ROSH REVIEW-Neurology

One Step Further Question: If vesicles are noted in the external ear canal of a patient with vertigo, what is the appropriate therapy?

Answer: Acyclovir for herpes zoster oticus (Ramsay Hunt Syndrome).

One Step Further Question: Which electrolyte abnormality has been reported in patients taking high-dose phenytoin?

Answer: Hypocalcemia.

One Step Further Question: What is the most common cause of bilateral Bell's palsy?

Answer: Lyme disease.

One Step Further Question: Do patients with relapsing-remitting MS ever convert to other types of MS?

Answer: Most patients presenting with relapsing-remitting MS convert to secondary-progressive MS after 20 to 40 years

One Step Further Question: What are the classic neuropathologic changes found in patients with cerebral palsy?

Answer: Periventricular leukomalacia and matrix hemorrhages.

One Step Further Question: What is a myasthenic crisis?

Answer: Respiratory failure leading to mechanical ventilation.

One Step Further Question: What is a fatal complication of myasthenia gravis?

Answer: Respiratory failure.

One Step Further Question: Which common extrapyramidal symptom is irreversible?

Answer: Tardive dyskinesia develops over months to years of antipsychotic exposure and is usually irreversible

One Step Further Question: Which lobe of the brain controls hearing?

Answer: Temporal lobe.

One Step Further Question: What is the most common type of headache?

Answer: Tension headaches.

One Step Further Question: Which muscle group will show weakness and atrophy as carpal tunnel syndrome progresses?

Answer: The thenar muscle (abductor pollicis brevis) becomes weak and atrophied in carpal tunnel syndrome.

One Step Further Question: Which segment of the spine is most commonly involved in transverse myelitis?

Answer: Thoracic. The cervical spine is rarely affected.

One Step Further Question: In diagnosing peripheral artery disease, what is considered an abnormal ankle-brachial index?

Answer: < 0.9.

One Step Further Question: What receptor does sumatripan act on?

Answer: Sumatriptan is a selective 5-HT (1B/D) [serotonin] agonist.

One Step Further Question: Which class of drugs is first-line treatment for both Lewy-body dementia and Alzheimer's dementia?

Answer: Cholinesterase inhibitors.

One Step Further Question: At what systolic blood pressure range should a patient with spontaneous subarachnoid hemorrhage be maintained to prevent complications of the disease?

Answer: 140-160 mm Hg.

One Step Further Question: In a patient over 50 years old with a complaint of a new onset headache, what imaging is warranted?

Answer: CT scan of the brain.

Which of the following is recommended for secondary stroke prevention for non-cardioembolic transient ischemic attack and ischemic stroke? Aspirin Aspirin plus clopidogrel Cilostazol Indomethacin

Correct Answer ( A ) Explanation: Aspirin is recommended for secondary stroke prevention for non-cardioembolic transient ischemic attack (TIA) and ischemic stroke. Guidelines from the American Heart Association/American Stroke Association (AHA/ASA) recommend that patients with a non-cardioembolic atherothrombotic, lacunar, or cryptogenic stroke or TIA (and no contraindications) receive an antiplatelet agent to reduce the risk of recurrent stroke. These guidelines note that aspirin, clopidogrel, and the combination of aspirin-extended-release dipyridamole are all acceptable options for preventing recurrent noncardioembolic ischemic stroke or transient ischemic attack.

Which of the following physical exam techniques can be used to confirm the diagnosis of benign paroxysmal positional vertigo? Dix-Hallpike maneuver Epley maneuver Jendrassik's maneuver Phalen's maneuver

Correct Answer ( A ) Explanation: Benign paroxysmal positional vertigo is the most common form of positional vertigo with a lifetime prevalence of 2.4%. It is caused by calcium debris in the posterior semicircular canals. While 35% of cases are idiopathic, other etiologies include head trauma and other vestibular pathologies. Symptoms are of short duration and occur with certain movements of the head, such as looking upward or rolling over in bed. Nystagmus observed during the Dix-Hallpike maneuver helps confirm the diagnosis of benign paroxysmal positional vertigo. This maneuver involves specific positioning of the head and neck, a rapid movement from sitting to supine with the head hanging off the bed and observation for nystagmus. Benign paroxysmal positional vertigo generally resolves spontaneously and commonly recurs.

A 24-year-old athlete undergoes anterior cruciate ligament repair surgery. Two weeks later, he presents with excruciating pain distal to the knee. Fracture, soft tissue injury and intraarticular infection are ruled-out. Examination reveals a swollen, warm, red foot and ankle that is painfully sensitive to light touch. The other leg appears normal. The patient guards this area and active range-of-motion is restricted. Complete pinprick sensory testing is deferred as initial testing is too painful to continue. Vibration testing results in continued pain even after removal of the tuning fork. Which of the following medications will you most likely prescribe? Gabapentin Intranasal desmopressin Intravenous immunoglobulin Pyridostigmine

Correct Answer ( A ) Explanation: Complex regional pain syndrome (CRPS) is not commonly encountered in primary care. However, CRPS presents with profound signs and symptoms. CRPS-1 occurs after a noxious neurological event, such as soft tissue crush injury, immobilization, orthopedic surgery and podiatric surgery. Any insult to the integrity of peripheral nerves is a possible etiology. This condition is felt to be due to activation of peripheral nociceptors, causing an increase in neuroexcitatory agents in the spinal cord, leading to upregulation and sensitization of peripheral and central pain pathways. Lowered neuronal thresholds result in over activity and dysregulation of the sympathetic nervous system. The most common presenting symptoms include: neuropathic pain (spontaneous, burning, dysesthetic, diffuse), allodynia (pain felt from a nonpainful stimulus, such as clothes or bed sheets on the skin), hyperesthesia (exaggerated pain response to a painful stimulus), hypoesthesia (decreased sensation/numbness in a painful area), hyperpathia (continued sensation after a stimulus is removed, such as continuing to feel vibration after a tuning fork is removed), decreased range-of-motion and joint guarding, skin changes (edema, erythema, temperature alterations, changes in hair growth and nail composition) and motor impairment. A classic presentation is post-surgical patients with days-to-weeks onset of distal extremity diffuse neuropathic pain, edema and erythema, as in the above patient. Most patients are treated with a mix of medication options: corticosteroid burst, intranasal calcitonin, NSAIDs, short course of opioids, gabapentin, tricyclic antidepressants and transdermal clonidine or lidocaine.

A 42-year-old man displays personality changes and confusion for 2 days. He denies pain. Upon presentation, you discover a weak right leg, speech impairment and low-grade fever. Upper extremity and left leg strength, rectal sensation and muscle tone, reflex testing and four-extremity sensory examination are normal. Nuchal rigidity, involuntary tremors, clonus and spasticity are absent. A brain MRI shows left temporal lobe edema. Cerebrospinal fluid analysis has an elevated number of red blood cells and the Gram stain is negative for bacteria. Which of the following is the most likely diagnosis? Encephalitis Meningitis Myelitis Sydenham's chorea

Correct Answer ( A ) Explanation: Encephalitis and meningitis manifest with similar symptoms, especially early in the infection. The classic meningitis triad is fever, headache and altered mental status, while altered mental status plus focal neurologic deficit is more descriptive of encephalitis. Herpes simplex encephalitis causes 10% of all diagnosed cases. More than half of these cases are fatal if untreated. HSV-1, commonly contracted in childhood (cold sores, fevers, and blisters), reactivates in adulthood to cause the majority of cases. Symptoms include up to 5 days of fever and headache, followed by behavior and personality changes, seizures, speech and memory deficits, hallucinations and altered consciousness. Frontal lobe pathology, leading to behavior and personality changes, and temporal lobe pathology, leading to memory and speech problems, are most common. Herpes encephalitis is associated with elevated red blood cells on CSF analysis. In adults, T2-weighted MRI reveals hyperintensity corresponding to edematous changes in the temporal lobes, inferior frontal lobes, and insula, with a predilection for the medial temporal lobes. Foci of hemorrhage occasionally can be observed on MRI. Untreated HSV encephalitis is associated with high morbidity and mortality. Therefore, in suspected cases of encephalitis without an obvious source, empiric treatment with intravenous acyclovir is recommended. Young children and the immunocompromised are at greatest risk for HSV disease. Meningitis (B) presents in a similar fashion to encephalitis early on. However, encephalitis is typically associated with behavioral and personality changes and neurologic deficits. Myelitis (C) (infection or inflammation of the spinal cord), typically presents with spinal pain, various sensory deficits, extremity weakness, reflex changes and bowel or bladder changes. Sydenham's chorea (D) occurs mainly in children with acute rheumatic fever or other Group A beta-hemolytic Streptococcus infection. It is characterized by face, hand and feet rapid jerking movements. Adult presentation is extremely rare.

Which of the following patients in the setting of a known subarachnoid hemorrhage has the worst prognosis? A 39-year-old woman who is confused and has mild right lower extremity weakness A 55-year-old man with a severe headache and oculomotor nerve palsy A 65-year-old man with a mild headache, nausea, and an episode of vomiting A 70-year-old woman with headache, neck stiffness, and an episode of vomiting

Correct Answer ( A ) Explanation: The Hunt and Hess scale is one of several schemes used to classify the severity of a subarachnoid hemorrhage based on the patient's clinical condition at presentation. It is used as a predictor of outcome; higher grades correlate with lower survival rates. The patient who is confused with mild right lower extremity weakness is classified as Grade III and has a projected survival of 50%.

What level does the spinal cord terminate in adults? L1-L2 L2-L3 L3-L4 L4-L5

Correct Answer ( A ) Explanation: The level of the spinal cord varies by age as the spine grows. Knowing the level is essential knowledge prior to performing a lumbar puncture. In the fetus the spinal canal and spinal cord are equal in length. But, growth of the spinal cord does not keep pace with the growth of the spinal canal. In adults and older children the spinal cord (conus medullaris) terminates at the lower level of L1 spinal nerve or the body of L2. Therefore, the best approach for a lumbar puncture is between the L3-L4, L4-L5 or L5-S1 interspace. In infants, the cord terminates at L3 (B and C). Therefore, in infants a spinal needle should be placed in the L4-L5 (D) or L5-S1 interspace

A 59-year-old man presents to your office with a resting tremor, rigidity, and bradykinesia. You note that his handwriting is small and difficult to read when you look at the registration form he completed upon check-in. Which of the following is the most likely diagnosis? ACerebellar tremor BMultiple sclerosis CParkinson disease DPrimary writing tremor

Correct Answer ( C ) Explanation: Parkinson disease is a progressive neurodegenerative disorder. Individuals with Parkinson disease often exhibit a characteristic tremor, a shuffling gait, and a masked facial expression. However, the effects of Parkinson disease are much more widespread. Although the cardinal features of Parkinson disease are described as resting tremor, rigidity, bradykinesia, akinesia, postural instability, flexed posture, and "freezing" episodes, these do not all manifest at once. Early motor signs may be subtle and nonspecific. Often they are recognized only in retrospect. A decrease in arm swing or stride length on one side while walking can lead to pain in the shoulder, upper back, low back, or hip. Decreased fine motor coordination can cause difficulty with buttons and clasps. Thus, getting dressed in the morning may become a slower process. Additional movements may slow and decrease in amplitude. For example, handwriting often becomes smaller and more difficult to read. When tremors first appear, they often are intermittent and most obvious during stressful situations. As the disease progresses, physical signs become more obvious. Tremor often is more constant. However, it may be absent altogether in some people, especially older individuals. Parkinsonian tremor usually is present only at rest. Some people learn to control the tremor by keeping their hands active. As walking becomes more difficult, people with Parkinson disease tend to become more sedentary. Difficulty with initiating movement, in combination with worsening balance, can make rising from soft chairs and car seats an arduous process. As the disease advances, akinesia (lack of movement) and bradykinesia (slowness of movement) continue to become more prominent. Posture may become more stooped. People with Parkinson disease may attribute these signs to weakness or stiffness of their limbs and body. Cerebellar tremor (A) is a slow tremor that is absent during rest but appears and progressively increases in amplitude with movement, particularly with fine adjustments required for a precise movement. It is associated with cerebellar disease. Multiple sclerosis (B) patients often exhibit incoordination, dysarthria, and intention tremor along with weakness in one or more limbs and visual blurring due to optic neuritis. Primary writing tremor (D) is a rare condition characterized by a 4- to 7-Hz tremor in the hand while assuming a writing posture or during the writing task itself. Most patients are men. About one-third have a positive family history of writing tremor, and a similar number give a history of improvement after ethanol ingestion.

A 40-year-old woman presents to her primary care provider's office with complaints of droopy eyelids and difficulty chewing. She has difficulty keeping her eyes open as the day goes on. Throughout a meal, she has progressive difficulty chewing. She holds her jaw up at the end of the meal. Which of the following physical exam findings would this woman most likely exhibit? Decreased sensation to light touch in extremities Diminished or absent deep tendon reflexes Hyperreflexive deep tendon reflexes Normal deep tendon reflexes

Correct Answer ( D ) Explanation: A patient with myasthenia gravis will most likely exhibit normal deep tendon reflexes. Myasthenia gravis is an autoimmune disorder characterized by skeletal muscle weakness and fatigability. Hyperreflexive deep tendon reflexes (C) are seen in multiple sclerosis, spinal cord injuries, traumatic brain injuries, hyperthyroidism, and electrolyte abnormalities. Deep tendon reflexes are graded on a scale from zero to five. A zero represents an absent reflex and five represents sustained clonus.

One Step Further Question: What medication can be given as an alternative therapy to benztropine for patients with dystonic reactions?

Answer: Diphenhydramine

One Step Further Question: What is the mechanism of action of dabigatran, another medication used to treat patients requiring full anticoagulation?

Answer: Direct thrombin inhibitor.

One Step Further Question: What is the name of the maneuver used to diagnose benign paroxysmal peripheral vertigo?

Answer: Dix-Hallpike Maneuver.

One Step Further Question: How is meningococcal meningitis transmitted?

Answer: Droplets.

One Step Further Question: Which mosquito-transmitted viruses commonly cause encephalitis in the US?

Answer: Eastern and Western Equine, West Nile, St.Louis and LaCrosse viruses.

One Step Further Question: What is the initial work-up for acute foot drop and suspected common peroneal neuropathy?

Answer: Electrodiagnostics (electromyography and nerve conduction studies).

One Step Further Question: What cerebrospinal fluid finding is typical to diagnose Guillain-Barré syndrome?

Answer: Elevated cerebrospinal fluid protein with normal cerebrospinal fluid white blood cell count.

One Step Further Question: In addition to elevated ESR, what other lab abnormality is seen in 50% of patients with temporal arteritis?

Answer: Elevated liver function tests.

One Step Further Question: An accumulation of blood in the potential space between the dura and skull best describes which type of head injury?

Answer: Epidural hematoma.

One Step Further Question: What is the classic triad of bacterial meningitis?

Answer: Fever, altered mental status, nuchal rigidity.

One Step Further Question: What are the cornerstone therapies in multiple sclerosis?

Answer: Glucocorticoids and plasma exchange.

One Step Further Question: What is the timeline needed to diagnose chronic tension-type headache?

Answer: Headaches occurring more than 15 days per month for more than three months.

One Step Further Question: What systems need to be monitored for side effects from carbamazepine?

Answer: Hematologic (CBC) and hepatic (liver function tests).

One Step Further Question: What cerebrospinal fluid (CSF) findings are seen in bacterial meningitis?

Answer: High protein, low glucose, presence of polymorphonuclear cells.

One Step Further Question: What is the first line treatment of multiple sclerosis exacerbations?

Answer: High-dose methylprednisolone followed by a prednisone taper

One Step Further Question: What is a complication of cluster headaches?

Answer: Horner syndrome. The severity of pain is also associated with increased risk for suicide.

One Step Further Question: What upper extremity fracture often compromises the radial nerve?

Answer: Humeral shaft fracture.

One Step Further Question: What is the recommended antibiotic regimen for a brain abscess from an oral or sinus source?

Answer: Intravenous metronidazole and ceftriaxone (or cefotaxime) +/- vancomycin

One Step Further Question: Myasthenia gravis is often misdiagnosed in the elderly population as what condition?

Answer: Ischemic stroke, especially when there is new-onset facial weakness.

One Step Further Question: What is a Marcus Gunn pupil?

Answer: It is a relative afferent pupillary defect that results in bilateral pupil constriction when shining a light in the unaffected eye but an absent pupil response (or a paradoxial dilation) when a light source is directed toward the affected eye.

One Step Further Question: Traumatic femoral neuropathy may result in which motor symptoms?

Answer: Knee extension (quadriceps) weakness.

One Step Further Question: How does Lambert-Eaton syndrome clinically differ from myasthenia gravis?

Answer: Lambert-Eaton syndrome is associated with small cell lung cancer in about 50% of cases, and symptoms improve with repetitive use.

One Step Further Question: What intraneuronal inclusions are associated with Parkinson's disease?

Answer: Lewy bodies.

One Step Further Question: Of the sites commonly affected by primary hypertensive intracranial hemorrhage, which one has the strongest association with anticoagulation?

Answer: Lobar hemorrhages, which occur in the white matter of the different cerebral lobes.

One Step Further Question: Bilateral facial nerve paralysis should alert the provider to which potential diagnoses?

Answer: Lyme disease, infectious mononucleosis, HIV infection, botulism.

One Step Further Question: What imaging modality should be used to evaluate a patient for vertebrobasilar insufficiency?

Answer: MRI of the brain with MRA.

Question: What are 2 contraindications to pyridostigmine use?

Answer: Mechanical gastrointestinal obstruction and urinary obstruction.

One Step Further Question: Chronic migraine is strongly associated with which disorder?

Answer: Medication overuse (rebound phenomenon or rebound headaches).

One Step Further Question: What disease presents with symptoms of vertigo, sensorineural hearing loss and tinnitus?

Answer: Meniere disease.

One Step Further Question: Name the condition of lateral femoral cutaneous nerve entrapment under the inguinal ligament.

Answer: Meralgia paresthetica.

One Step Further Question: What antibiotics should be used in a patient with meningitis and a confirmed cephalosporin allergy?

Answer: Meropenem or chloramphenicol can replace the third generation cephalosporin.

One Step Further Question: What are the most common CSF findings in transverse myelitis?

Answer: Mildly elevated protein and pleocytosis are seen in 60% of cases.

One Step Further Question: What is Miller Fisher syndrome?

Answer: Miller Fisher syndrome is a rare form of GBS characterized by the triad of ophthalmoplegia, ataxia, and areflexia.

One Step Further Question: What is akathesia?

Answer: Motor restlessness characterized by the need to constantly be moving.

One Step Further Question: What is the most common acquired neurologic disability in young adults?

Answer: Multiple sclerosis.

One Step Further Question: What is the hallmark of myasthenia gravis?

Answer: Muscle weakness that worsens with activity and improves with rest.

One Step Further Question: The motor fibers of the trigeminal nerve inervate which muscles?

Answer: Muscles of mastication.

One Step Further Question: What medication should be administered to help reduce the vasospasm associated with subarachnoid hemorrhage?

Answer: Nimodipine.

One Step Further Question: What role do neuromuscular blocking drugs have in refractory status epilepticus?

Answer: Nondepolarizing neuromuscular blocking drugs will stop the tonic-clonic movements, but will not affect abnormal neuronal firing. Patient will need an EEG.

One Step Further Question: What are the three branches of the trigeminal nerve?

Answer: Ophthalmic, maxillary, and mandibular.

One Step Further Question: Which form of sumatriptan was found not to be an effective treatment for cluster headache?

Answer: Oral sumatriptan.

One Step Further Question: What is the difference between plegia and paresis?

Answer: Plegia refers to paralysis (flaccid, no movement) while paresis refers to weakness (movement, but abnormal strength).

One Step Further Question: What autosomal dominant familial kidney disease is the greatest risk factor for cerebral aneurysm?

Answer: Polycystic kidney disease.

One Step Further Question: What systemic disease is frequently associated with temporal arteritis?

Answer: Polymyalgia rheumatica (in 30-40% of patients).

One Step Further Question: What imaging modality can show abnormalities in patients with concussion in the acute setting?

Answer: Positron emission tomography (PET) scan.

One Step Further Question: If there is no response to gabapentin, what other pain medications could be tried?

Answer: Pregabalin, duloxetine and carbamazepine.

One Step Further Question: What medication should be given to a patient with status epilepticus and a possible isoniazid overdose?

Answer: Pyridoxine

One Step Further Question: What are the four types of multiple sclerosis?

Answer: Relapsing remitting, secondary progressive, primary progressive, or progressive relapsing.

One Step Further Question: What therapy should household contacts of patients with confirmed meningitis receive?

Answer: Rifampin (oral). Ciprofloxacin (oral) and ceftriaxone are alternatives.

One Step Further Question: What are some other, less common, diseases associated with optic neuritis?

Answer: Sarcoidosis and systemic lupus erythematous are associated optic neuritis and generally confer a poorer prognosis of visual recovery.

One Step Further Question: What is the treatment of idiopathic intracranial hypertension?

Answer: Serial lumbar punctures, acetazolamide and diuretics.

One Step Further Question: Which bacteria is responsible for the majority of food-borne toxicity in the United States?

Answer: Staphylococcus.

Question: What is the most common cause of bacterial meningitis in adults?

Answer: Streptococcus pneumoniae.

One Step Further Question: Which medication used for migraine treatment is contraindicated in patients with coronary or peripheral vascular disease?

Answer: Sumatriptan, because it causes vasoconstriction.

One Step Further Question: What is a complex concussion?

Answer: Symptoms persist >10 days and involve symptom recurrence with exertion and prolonged cognitive impairment.

One Step Further Question: Why should phenytoin be administered as an infusion over 45-60 minutes as opposed to a bolus?

Answer: The diluent in phenytoin causes hypotension if given rapidly.

One Step Further Question: What lab test is most likely to be abnormal in patients with spinal epidural abscess?

Answer: The erythrocyte sedimentation rate (ESR) is often elevated despite a frequently normal WBC count.

One Step Further Question: How long until the onset of action of phenobarbital?

Answer: The onset of action is 15-30 minutes.

One Step Further Question: The ulnar nerve is responsible for the sensation of what part of the hand?

Answer: The palmar and dorsal surface of the 5th digit and the medial aspect of the 4th digit.

One Step Further Question: What cranial nerve disorder is commonly characterized by bouts of sharp stabbing pain on one side of the face?

Answer: Trigeminal neuralgia.

One Step Further Question: What is the first-line abortive therapy for moderate to severe migraine headaches?

Answer: Triptans.

One Step Further Question: What electrocardiogram changes will you see in a patient presenting with weakness and severe hypokalemia?

Answer: U waves.

One Step Further Question: What is Tullio's sign?

Answer: Vertigo resulting from loud sounds, seen in superior canal dehiscence.

One Step Further Question: What is the most common long term complication of idiopathic intracranial hypertension?

Answer: Vision loss.

One Step Further Question: What finding on cerebrospinal fluid analysis is concerning for hemorrhage?

Answer: Xanthochromia

One Step Further Question: Can steroids be started for temporal arteritis prior to obtaining a biopsy?

Answer: Yes. The biopsy should be performed within 48 hours of starting steroids.

A 64-year-old man presents to the emergency department with acute onset of left arm and left leg weakness approximately one hour ago. He has slurred speech and is difficult to understand while obtaining the history. Vital signs are HR 80, BP 192/100, RR 12, T 37.0°C. Examination shows facial asymmetry with left upper and lower extremity weakness. What is the most important bedside diagnostic test to perform at this time? Chest radiograph Echocardiogram Electrocardiogram Fingerstick glucose

Correct Answer ( D ) Explanation: The patient in this question is presenting with acute onset of focal weakness of which the differential diagnosis includes cerebrovascular accident (CVA), transient ischemic attack (TIA), hypoglycemia, seizure with Todd's paralysis, complicated migraine, tumor and conversion disorder. Seizure disorder is best diagnosed either by witnessing seizure activity or obtaining historical information consistent with seizure. Seizure activity was not reported in this case so is a less likely diagnosis at this time. Complicated migraine and conversion disorder would be diagnoses of exclusion after completing the workup for other disease states and there is no report of headache on presentation. Acute onset of weakness from a brain tumor would likely occur in the setting of concomitant intracranial hemorrhage which would be diagnosed by completion of head CT or MRI and not with a bedside test. There are few laboratory tests that aid in the diagnosis of CVA or TIA except for a fingerstick glucose to exclude hypoglycemia, a common stroke mimic.

A 16-year-old girl presents to the ED complaining that she cannot walk up the steps. She has been well recently except for an episode of gastroenteritis two weeks ago. On exam, she has decreased strength in her bilateral lower extremities with absent patellar and ankle jerk reflexes. What is the most important next test to perform? Electromyelography Lumbar puncture MRI lumbar spine Vital capacity

Correct Answer ( D ) Explanation: This patient has Guillain-Barré syndrome (GBS), an immune-mediated peripheral neuropathy caused by myelin sheath destruction. It is often preceded by a viral illness, infection with Campylobacter jejuni, or vaccination. In the classic presentation of GBS, a viral illness is followed by an ascending symmetric weakness or paralysis with decreased or absent deep tendon reflexes. The most serious complication of GBS is respiratory failure from diaphragmatic weakness. Measuring the vital capacity or negative inspiratory force (NIF) is critical to assessing respiratory effort. These easily repeated tests predict developing diaphragmatic weakness and the need for prophylactic intubation. GBS is treated with supportive care, IV immune globulin, and possibly plasmapheresis. All patients with suspected GBS should be admitted to an intensive care unit.

One Step Further Question: What is the most commonly isolated pathogen in patients with Guillain-Barré syndrome?

Answer: Campylobacter jejuni.

One Step Further Question: What is the medical treatment for patients with trigeminal neuralgia?

Answer: Carbamazepine (primary) or gabapentin (secondary)

One Step Further Question: Vertical nystagmus is always associated with which type of vertigo?

Answer: Central vertigo; peripheral vertigo is associated with horizontal or horizontal rotary nystagmus.

One Step Further Question: What is the most common cause of unilateral hearing loss?

Answer: Cerumen impaction.

One Step Further Question: In which chromosome is the excess CAG repeats seen in patients with Huntington's disease?

Answer: Chromosome 4.

One Step Further Question: Which chromosome is the huntingtin gene located on?

Answer: Chromosome 4.

One Step Further Question: What is the name of the terminal end of the spinal cord?

Answer: Conus medullaris

One Step Further Question: What is the role of corticosteroids in CNS abscess?

Answer: Corticosteroids should only be given to patients with cerebral edema related to the abscess

One Step Further Question: What other toxin is commonly a co-inhalant in a fire with carbon monoxide?

Answer: Cyanide.

One Step Further Question: What test in patients with GBS correlates with need for intubation?

Answer: Decreased forced vital capacity.

One Step Further Question: Upon which chromosome is the gene responsible for Huntington disease located?

Answer: An expanded and unstable CAG trinucleotide repeat in the huntingtin gene on chromosome 4 is responsible for this disease.

One Step Further Question: What is the treatment of choice for a carotid artery dissection?

Answer: Anticoagulation (heparin followed by warfarin).

One Step Further Question: What changes in the brain are seen with Huntington disease?

Answer: Atrophy of the caudate nucleus and putamen.

One Step Further Question: What antidote should be present during edrophonium administration?

Answer: Atropine can be used to counteract the cholinergic effects of edrophonium.

One Step Further Question: What are the common nerve conduction study findings in axonal and demyelinating neuropathy?

Answer: Axonal: decreased action potential amplitude, relatively normal conduction velocity; Demyelinating: slowed conduction velocity, prolonged distal latencies.

One Step Further Question: What is the most common cause of vertigo?

Answer: Benign (paroxysmal) positional vertigo.

One Step Further Question: What are some treatments for essential tremor?

Answer: Beta-blockers or anti-convulsants may reduce benign essential tremor.

One Step Further Question: Is the Miller-Fisher variant of GBS associated with ascending or descending paralysis?

Answer: Descending.

One Step Further Question: Blockade of which receptor helps to reduce the incidence of extrapyramidal effects?

Answer: Blockade of muscarinic acetylcholine receptors suppresses the emergence of extrapyramidal effects.

One Step Further Question: In patients with neuroleptic malignant syndrome, what medications can be given instead of dantrolene?

Answer: Bromocriptine and amantadine.

One Step Further Question: What opportunistic infections are seen in HIV patients when the CD4 count is < 50 cells/mm3?

Answer: CMV retinitis and Mycobacterium avium complex.

One Step Further Question: Which medication class is considered effective for preventing vasospasms following an intracerebral hemorrhage?

Answer: Calcium channel blockers, particularly nimodipine, are effective for preventing vasospasms.

One Step Further Question: What antecedent bacterial infection is associated with GBS?

Answer: Campylobacter jejuni infection.

One Step Further Question: If a patient is given tissue-type plasminogen activator, how long should you wait before giving aspirin or clopidogrel?

Answer: 24 hours.

One Step Further Question: What is the score range of the Glasgow Coma Scale?

Answer: 3 (worst) to 15 (best).

One Step Further Question: What is the normal opening pressure in adults?

Answer: 5-20 cm H20

One Step Further Question: What is the upper age limit for tPA administration up to 4.5 hours?

Answer: 80-years-old.

One Step Further Question: Status migrainosus is defined by a headache that lasts at least how long?

Answer: A diagnosis of status migrainosus requires an ongoing migraine of at least 72 hours in duration.

One Step Further Question: What is the postictal period (state)?

Answer: A period lasting minutes to hours following the active stage of a seizure, in which the patient experiences headache, fatigue, amnesia, localized weakness, difficulty speaking and abnormal behavior, such as psychosis.

One Step Further Question: A positive Myerson sign, in which repetitive tapping over the bridge of the patient's nose produces a sustained, involuntary blink response, may be seen in which neurologic condition?

Answer: A positive Myerson sign may be seen in patients with Parkinsonism.

One Step Further Question: What is status migranosus?

Answer: A severe migraine headache that persists >72 hours. Associated symptoms are usually debilitating and hospitalization may be required.

One Step Further Question: Describe the pathophysiology of piriformis syndrome?

Answer: A tight, spastic or hypertrophied piriformis muscle compresses the nearby sciatic nerve.

One Step Further Question: What is Miller-Fischer syndrome?

Answer: A variant of GBS characterized by opthalmoplegia and ataxia.

One Step Further Question: What scoring system may help risk stratify patients with TIA?

Answer: ABCD2.

One Step Further Question: What is the most effective intervention to treat an acute ibuprofen overdose?

Answer: Activated charcoal.

One Step Further Question: In what setting is dual antiplatelet therapy with aspirin and clopidogrel indicated?

Answer: After percutaneous coronary intervention and stent placement.

One Step Further Question: What is the combination of elevated cerebrospinal fluid protein and normal white blood cell count called?

Answer: Albuminocytologic dissociation.

One Step Further Question: What are the most common types of dementia?

Answer: Alzheimer's and vascular dementia.

A 35-year-old woman presents with lower extremity weakness and ptosis of her right eye that she states has progressively worsened throughout the day. You suspect the diagnosis of myasthenia gravis. In addition to obtaining electromyogram testing, which of the following is the most appropriate antibody test to send at this time? Acetylcholine receptor antibody Antiglial nuclear antibody Antiparietal cell antibody Antiphospholipid antibody

Correct Answer ( A ) Explanation: Acetylcholine receptor (AChR) antibodies are present in approximately 85% of patients with myasthenia gravis who have generalized symptoms, but only 50% of patients with purely ocular involvement. In the absence of AChR antibodies, especially in subjects with generalized symptoms, testing for muscle-specific kinase antibodies is recommended. Both AChR and muscle-specific kinase antibodies have a high specificity for myasthenia gravis and confirms the diagnosis. The Lambert-Eaton myasthenic syndrome (LEMS) is one of the 1st recognized and most common neurologic paraneoplastic syndromes, and it is one of the only syndromes with direct evidence to support the role of autoantibodies in producing clinical disease. Antiglial nuclear antibody (AGNA) (B) binds to the SOX1 antigen, found in nuclei of specialized glia cells in the cerebellum. Antiparietal cell antibodies (C) react with gastric parietal cells and are present in more than 90% of patients with pernicious anemia. Antiphospholipid antibodies (D) are found in numerous acquired conditions and have been associated with increased risk of venous thrombosis.

A patient presents 90 minutes after acute severe headache, nausea, and vomiting. He states it began during sexual intercourse. He has no fever or focal neurologic signs. You would most likely expect to find which of the following abnormalities on a non-contrast CT of the brain? Bright, high-attenuation density within the subarachnoid space Bright, low-attenuation density within the brainstem Dark, high-attenuation density within the epidural space Dark, low-attenuation density within the ventricles

Correct Answer ( A ) Explanation: Aneurysmal rupture is the fourth most common cause of cerebrovascular disease, and if large enough, can be fatal. They arise from a congenital defect of the vessel wall's internal elastic lamina and media. The majority of all aneurysms occur on the internal carotid artery or its branches. Other locations include the basilar or vertebral arteries or their branches. The most common site is the anterior half of the circle of Willis at a bifurcation of a branching distal artery off of the circle. Most brain aneurysms are recognized only when they rupture, commonly between the ages of 35-65 years, resulting in subarachnoid hemorrhage, and presenting as acute onset severe headache, nausea, vomiting, and meningeal signs without fever. Rupture is not necessarily related to chronic hypertension; sometimes rupture follows a Valsalva maneuver, intense physical effort, or sexual intercourse. Diagnosis is confirmed with CT scanning, or the presence of blood on a lumbar puncture in the setting of a normal CT scan. On a non-contrast brain CT, fresh subarachnoid hemorrhage appears as a bright, white, high-attenuating, amorphous substance within the normally dark CSF-filled subarachnoid spaces. (non-contrast CT bright = bone, clotted blood; dark = air, fluid, fat; gray = brain). Complications include mass effect, vasospasm, and cerebral infarction, but also hydrocephalus and SIADH. Definitive treatment is built on early diagnosis, defining the vasculature with angiography and neurosurgical clipping. Aneurysmal rupture much less commonly occurs in the brainstem (B) vessels (basilar artery and its branches). A talking patient typically does not have brainstem pathology. Bright does not go with low-attenuation. Dark does not go with high-attenuation (C). Aneurysms do not bleed into the epidural space. Fresh subarachnoid hemorrhage clots almost immediately. It becomes denser than intravascular or intracellular fluid, and as such, it appears bright (high-attenuation density on non-contrast brain CT) within the normally dark cerebrospinal fluid spaces (in essence, subarachnoid space) and passageways (ventricular system). Although dark goes with low attenuation, and ruptured aneurysms typically produce blood in the ventricles, clotted hemorrhage appears bright, not dark (D).

Which of the following patients is most likely suffering from dementia? A confused 65-year-old man brought from a house fire who exhibits good long-term recall, no recall of immediate events, normal vital signs, carboxyhemoglobin level of seven A confused 65-year-old man brought from a house fire who exhibits poor long-term recall, no recall of immediate events, normal vital signs, carboxyhemoglobin level of 25 A confused 80-year-old man brought from home with fever, back pain, and urinary retention A confused 80-year-old man brought from home with fever, cough, and hypoxia A confused 80-year-old man brought from home with increased thirst, increased urination, and blood sugar monitor that reads "high"

Correct Answer ( A ) Explanation: Dementia results from a gradual loss of mental capacity with preservation of motor and speech. Dementia generally occurs in the elderly. Remote memories are often preserved. The most common types of dementia are Alzheimer's disease and vascular dementia, both insidious in onset. Symptoms may worsen acutely because of a concurrent medical condition. Physical exam and laboratory workup are generally unrevealing.

A previously healthy 62-year-old woman presents to your office with a complaint of dizziness. For the past two weeks, she has been experiencing an occasional spinning sensation that lasts approximately 20-30 seconds. She first noticed it while tilting her head back in the shower and also had symptoms when rolling over in bed. Which of the following is the most likely diagnosis? Benign paroxysmal positional vertigo Labyrinthitis Ménière disease Orthostatic hypotension

Correct Answer ( A ) Explanation: Benign paroxysmal positional vertigo (BPPV) is the most common type of vertigo in the United States. It is caused by loose calcium debris in the posterior semicircular canal of the ear. BPPV typically has a sudden onset and individuals notice it in connection with positional changes of the head, such as rolling over in bed, while showering, or sitting up quickly. The incidence is higher in women and BPPV generally occurs in individuals older than 50 years of age. Most cases of BPPV are either idiopathic or due to head trauma. Symptoms include recurrent episodes of vertigo that last less than one minute and resolve with positional changes of the head. Some individuals also experience nausea and vomiting. Diagnosis is based on history and physical exam, including nystagmus seen during provoking maneuvers. First-line treatment is with canalith repositioning maneuvers including the Epley maneuver.

Which of the following pathogens is most commonly associated with Guillain-Barré syndrome? Campylobacter jejuni Human immunodeficiency virus Influenza virus Varicella zoster virus

Correct Answer ( A ) Explanation: Campylobacter jejuni is the most common pathogen to cause Guillain-Barré syndrome. Guillain-Barre syndrome is an acute immune-mediated paralyzing illness typically caused by an infectious process. The most common mechanism involves an infection which causes an abnormal immune response that in turn reacts with the peripheral nerves leading to polyneuropathy. Campylobacter jejuni, a gram-negative bacterium, has been found to be the most common infectious precipitant of the disease. Characteristic findings of the disease include progressive and symmetric muscle weakness that starts in the legs and ascends proximally. Deep tendon reflexes are found to be decreased or absent. Diagnosis is made from the history and physical examination and is confirmed with analysis of the cerebrospinal fluid and nerve conduction studies. Complications include neuromuscular respiratory failure as the muscular weakness ascends proximally. Treatment includes supportive care with mechanical ventilation and plasmapheresis and intravenous immune globulin to modify the disease course.

A 30-year-old-man presents to office with headaches that have been occurring every day, lasting 3-4 hours associated with severe throbbing unilateral retroorbital pain and ipsilateral lacrimation and nasal congestion. This has been occurring for months and the patient complains of agitation and restlessness when he is having a headache. Which of the following is the most likely diagnosis? Cluster headache Migraine headache Temporal arteritis Tension headache

Correct Answer ( A ) Explanation: Cluster headaches occur most often in middle-aged men. They are described as recurrent, unilateral, and last from 15 minutes to 3 hours. The headache is nonpulsatile and constant, frequently occurring at night. Clinical findings include ipsilateral conjunctival injection, lacrimation, and nasal discharge. Cluster headaches classically take place in groups over days to weeks, occurring at the same time of day and in the same location. The neurologic exam should be normal. Treatment of an acute attack includes 100% oxygen, triptans, ergotamines, intranasal lidocaine, and butorphanol. Prophylaxis includes avoiding triggers such as alcohol, stress, and medications causing vasodilation. To prevent acute recurrence, a prednisone burst and taper may be helpful.

A 43-year-old man presents to the emergency department with a severe left-sided headache that started one hour ago, accompanied by stabbing unilateral periorbital pain. Physical exam reveals rhinorrhea, left-sided lacrimation, and redness of the eye. Administration of 100% oxygen provides modest relief. The headache could be best classified as which of the following types? Cluster headache Medication overuse headache Migraine headache Tension-type headache

Correct Answer ( A ) Explanation: Cluster headaches typically occur in middle-aged men, causing unilateral headaches with sharp periorbital pain. Lacrimation, rhinorrhea, redness of the affected eye, and restlessness commonly accompany the headache. A transient Horner syndrome with ptosis, miosis, and unilateral anhidrosis may be noted on physical exam. Diagnosis is clinical. Cluster headaches may be aborted with 100% oxygen via non-rebreather for 15 minutes. Acceptable medications for headache abortion include subcutaneous or intranasal sumatriptan, intramuscular or intravenous dihydroergotamine, or intranasal lidocaine. First-line preventative medications include oral lithium carbonate, verapamil, topiramate, or valproate.

A 38-year-old man presents with the worst headache of his life. A magnetic resonance angiogram is obtained which demonstrates a large unruptured cerebral aneurysm of the posterior communicating artery. Which of the following are you most likely to find on physical examination? Cranial nerve III palsy Cranial nerve V palsy Cranial nerve VI palsy Cranial nerve VII palsy

Correct Answer ( A ) Explanation: Cranial nerve III palsy is a likely physical examination finding in a patient presenting with a subarachnoid hemorrhage due to a cerebral aneurysm. A subarachnoid hemorrhage is bleeding within the subarachnoid space of the brain and is most commonly caused by a cerebral aneurysm or an arteriovenous malformation. A cerebral aneurysm is a ballooning of the blood vessels of the brain that when under continued pressure can rupture. This is a medical emergency which requires immediate intervention. An unruptured cerebral aneurysm is often incidentally found on brain imaging or when screening for a cerebral aneurysm if a patient has a strong family history. Patients are typically asymptomatic with an unruptured aneurysm but may become symptomatic if increased pressure on the aneurysm continues. Characteristic findings of both unruptured and ruptured cerebral aneurysms include headache, often the worst headache of life, cranial nerve III deficit, and visual loss. Cranial nerve III is the oculomotor nerve which can become compressed when a cerebral aneurysm is present at the junction of the internal carotid artery and posterior communicating artery, causing a unilateral strict downward gaze, ptosis, anisocoria, and blurred vision. When a cerebral aneurysm causes a cranial nerve III deficit with no other neurological symptoms, the aneurysm is likely to be unruptured, but is large enough to compress the nerve. Cranial nerve V palsy (B) is a palsy of the trigeminal nerve which controls sensation of the face and muscles of mastication and are rarely affected by a cerebral aneurysm. Typical findings of a cranial nerve V palsy include decreased sensation to the face and jaw deviation upon closure. Cranial nerve VI palsy (C) is a palsy of the abducens nerve which helps to control lateral movement of the eye. When this nerve is affected, the lateral rectus muscle becomes weak and the eye deviates inward towards the nose. The most common causes of a cranial nerve VI palsy include increased intracranial pressure, brain tumor, and stroke. Cranial nerve VII palsy (D) is a palsy of the facial nerve which innervates all facial musculature. When this nerve is affected there is drooping of the facial muscles and decreased taste on the anterior two-thirds of the tongue. An idiopathic cranial nerve VII palsy is most commonly known as Bell's palsy which is caused by a virus.

A 55-year-old woman with a history of hypertension and diabetes mellitus presents to the emergency department after becoming incoherent during a work meeting 45 minutes prior to arrival. Examination reveals a right facial droop and right upper extremity weakness. It is decided to proceed with thrombolysis with recombinant tissue-type plasminogen activator. Which of the following is an exclusion criterion for using reperfusion therapy? Current warfarin use with INR > 1.7 Lack of intracranial hemorrhage on computed tomographic imaging Myocardial infarction 6 months prior Significant head trauma one year prior

Correct Answer ( A ) Explanation: Current warfarin use with INR > 1.7 is an exclusion criterion for using reperfusion therapy. Inclusion criteria for the treatment of acute ischemic stroke with recombinant tissue plasminogen activator include age ≥ 18 years old, a clinical diagnosis of ischemic stroke causing measurable neurologic deficit, and onset of symptoms < 4.5 hours before beginning treatment. If the exact time of stroke onset is not known, it is defined as the last time the patient was known to be normal. There is a long list of exclusion criteria and relative exclusion criteria including, but not limited to, significant stroke or head trauma in the previous three months, previous intracranial hemorrhage, intracranial neoplasm, arteriovenous malformation or aneurysm, recent intracranial or intraspinal surgery, arterial puncture at a noncompressible site in the previous seven days, symptoms suggestive of subarachnoid hemorrhage, persistent blood pressure elevation (systolic ≥ 185 mmHg or diastolic ≥ 110 mmHg), serum glucose < 50 mg/dL, active internal bleeding, acute bleeding diathesis, platelet count < 100,000/mm3, current anticoagulant use with an INR > 1.7 or PT > 15 seconds, heparin use within 48 hours and an abnormally elevated aPTT, current use of a direct thrombin inhibitor or direct factor Xa inhibitor with evidence of anticoagulant effect by laboratory tests, evidence of hemorrhage or extensive regions of obvious hypodensity consistent with irreversible injury on head CT scan.

Nonsteroidal anti-inflammatory medications are the mainstay of treatment for a tension-type headache. Which of the following is a black box warning for ibuprofen? Gastrointestinal bleeding Hepatoxicity Rebound headache Respiratory depression

Correct Answer ( A ) Explanation: Gastrointestinal bleeding is a black box warning for ibuprofen and other nonsteroidal anti-inflammatory medications. Nonsteroidal anti-inflammatory medications are a class of medications that block the cyclooxygenase enzyme, thereby reducing the number of prostaglandins, or chemicals that support pain and inflammation, throughout the body. When the amount of prostaglandins are reduced, the severity of pain and inflammation also reduce. Prostaglandins are also produced by the cyclooxygenase enzyme to support the formation of platelets and protect the lining of the stomach from acid buildup. When a nonsteroidal anti-inflammatory medication is ingested and the number of prostaglandins is reduced, the stomach is no longer adequately protected from the buildup of acid. This results in the formation of gastric ulcers which ultimately results in gastrointestinal bleeding.

A 55-year-old man presents with amaurosis fugax, headaches, scalp tenderness, jaw claudication, occasional ear pain, malaise, and intermittent fevers. On exam, you note tenderness of his left temple. Laboratory studies reveal an erythrocyte sedimentation rate of 85. Which of the following is the most likely diagnosis? Giant cell arteritis Multiple sclerosis Myasthenia gravis Polymyositis

Correct Answer ( A ) Explanation: Giant cell arteritis (temporal arteritis) is a systemic autoimmune disorder. Pathologically, there is a granulomatous inflammation of large and medium-sized arteries. It is most common in women >50 years of age. Involvement may occur in any organ system, but the condition is characterized by subacute inflammation of the external carotid arterial system and vertebral arteries. Patients often describe a new temporal or diffuse headache that may be associated with transient visual loss, scalp tenderness, and jaw claudication. Patients may report a history of polymyalgia rheumatica (fever, myalgias, anorexia, weight loss, and arthralgias). If the diagnosis is suspected, treatment should be immediately initiated by administering steroids (prednisone 60 mg daily). Confirmation of the diagnosis is by temporal artery biopsy. Lab findings supportive of the diagnosis is an elevated ESR (50-100). Using the Westergren method, the value for a normal ESR is 30 mm/hr for a 60-year-old man; for women, top of normal range is age plus 10 divided by 2, so 35 mm/hr is the upper range of normal for a 60-year-old woman.

A 24-year-old woman presents to Urgent Care after tripping over her backpack and hitting her head at home. A friend noted brief period of unconsciousness lasting less than 3 seconds. Prior to arrival to urgent care, she vomits once. On your physical exam, she is noted to have a normal physical exam with normal vital signs. According to the Canadian CT Head Rule, which of the following is the most appropriate next step in management? Observation Transfer to emergency department for a CT scan of the head with contrast Transfer to emergency department for a CT scan of the head without contrast Transfer to emergency department for an MRI scan of the head

Correct Answer ( A ) Explanation: Head injuries can be life threatening therefore it is critical to identify those patients at risk for significant pathology. Serious head trauma can result in an epidural hematoma, subdural hematoma, subarachnoid hemorrhage, intracerebral hemorrhage, and skull fracture. For minor head trauma, guidelines help clinicians decide on whether a head CT scan is needed to diagnose or rule out an intracranial injury. One set of rules called the Canadian CT Head Rule devised a set of criteria seen below on which patients with minor head trauma require a head CT scan. The patient in the clinical scenario does not meet any of these criteria and therefore can undergo a period of observation. The patient should be watched closely for signs of increased intracranial pressure (due to expanding hematoma) such as vomiting, confusion, motor or sensory deficits, visual changes, altered consciousness. It should be noted that the Canadian CT Head Rule is a guideline and any time a clinician feels a patient may have an intracranial injury despite not meeting the Canadian CT Head Rule, the clinician should go ahead and get the head CT scan.

What is the genetic basis of Huntington disease? Expansion of the cytosine-adenine-guanine trinucleotide repeats in the HTT gene Hemizygous deletion of up to 28 genes on chromosome 7q11.23 Mutations in the fibrillin gene Mutations in the gene encoding phenylalanine hydroxylase

Correct Answer ( A ) Explanation: Huntington disease (HD) is an autosomal dominant inherited, neurologic disorder characterized by choreiform movements, dementia and psychiatric disturbances. The pathophysiology of the disease is still not fully understood. Diagnosis is with genetic testing. Asymptomatic patients with a known family history of HD may also request genetic testing prior to the onset of symptoms. Genetic testing in individuals with HD will show expansion of the cytosine-adenine-guanine trinucleotide repeats in the HTT gene. There is no cure and no disease-modifying treatment available at this time. Treatment focuses on supportive care for the patient and family along with management of symptoms. HD is progressive with the end stages of the disease being severe disability and then death. Hemizygous deletion of up to 28 genes on chromosome 7q11.23 (B) is found in Williams syndrome. Clinical manifestations of Williams syndrome include cardiac anomalies, impaired cognition, friendly personality, "elfin" facies and genitourinary abnormalities. Mutations in the fibrillin gene (C) are seen in Marfan syndrome, an autosomal dominant connective tissue disorder. Mutations in the gene encoding phenylalanine hydroxylase (D) cause phenylketonuria, a disorder that if untreated causes intellectual disability.

A 56-year-old woman with a history of diabetes mellitus type 2 presents to your office with a complaint of bilateral foot pain. She describes the pain as a burning sensation that occurs with rest and improves with activity. Her most recent hemoglobin A1C was 11.3%. Which of the following is the most appropriate initial therapy? Amitriptyline Lamotrigine Oxcarbazepine Oxycodone

Correct Answer ( A ) Explanation: Peripheral neuropathy is a major cause of morbidity in patients with diabetes mellitus. Risk factors for the development of diabetic neuropathy include duration and severity of hyperglycemia, hyperlipidemia, smoking and hypertension. Diabetic neuropathy is a symmetrical sensory polyneuropathy that generally affects the distal lower extremities first. Clinical manifestations of diabetic neuropathy include pain in the feet described as burning or tingling that occurs at rest and improves with activity. Symptoms are sometimes preceded by a recent change in glycemic control. There are three aspects to the treatment of diabetic neuropathy: glycemic control, foot care and pain management. The American Diabetes Association recommends a step-wise management strategy in patients with diabetic neuropathy that begins with excluding other neurologic etiologies and stabilizing glycemic control. Pharmacologic treatment should begin with tricyclic antidepressants such as amitriptyline and then may include anticonvulsants such as gabapentin.

You refer a 45 year old man with a movement disorder to a neurologist. She obtains a brain MRI which shows significant atrophy of the basal ganglia. She also comments on finding significant choreiform movements and ballism, as well as dementia. She initiates drug therapy, but asks you to follow-up for medication monitoring. Which of the following class of medications will you most likely be titrating? Benzodiazepine Dopamine-agonist Ergotamine-derivative Tricyclic antidepressant

Correct Answer ( A ) Explanation: Huntington's disease is an autosomal dominant genetic disorder with gradual onset and progression of chorea and dementia, usually starting at 35-50 years of age. The major pathology exists as neuronal loss, astrogliosis and eventual atrophy of the caudate nucleus and putamen of the basal ganglia, and the cerebral cortex. Three main symptoms exist: movement disorder (characterized by chorea, ballism, dystonia and parkinsonian features), cognitive disorder (mostly dementia) and mood or behavior disorder (depression, bipolar disorder, psychosis, personality disorders, sexual and sleep disturbance). Chorea is defined as excessive, spontaneous and abrupt movements or irregular frequency and random distribution. Examples include restlessness, fidgeting, gesture and facial expression alterations and dancelike gait. A milder form, choreathetosis, involves slow distal writhing movements, whereas more severe forms, ballism, is marked by gross, proximal, flinging movements of the extremities. Athetosis refers to contorted, twisting movements. Patients sometimes try to mask these choreiform movements as purposeful movements (parakinesia). If the abnormal movements interfere with daily function, consider treating Huntington's with benzodiazepines, dopamine-depleting or dopamine-antagonist medications.

A 23-year-old man presents in status epilepticus by EMS. They have given multiple doses of benzodiazepines without response. Which of the following tests is most important at this time? Fingerstick glucose Lumbar puncture Noncontrast head CT Serum sodium level

Correct Answer ( A ) Explanation: Hypoglycemia is a common and easily treated cause of seizures that is frequently overlooked. Seizures are defined as episodes of abnormal neurologic functioning caused by an excess activation of neurons. They are common with over 10% of the general population experiencing at least one seizure during their lifetime. Seizures have a number of causes and are typically separated into primary (epilepsy) and secondary. Secondary seizures may be provoked by trauma, intoxication, toxin exposure, organ failure, metabolic disturbances, tumors and secondary to drug effects. Hypoglcyemia is a common metabolic disturbance that can cause a host of neurologic symptoms including confusion, coma, seizures and focal neurologic deficits. Because hypoglycemia can be rapidly identified, rapidly treated and potentially fatal, a fingerstick glucose should be part of the immediate work up of any patient who presents with altered mental status.

Which of the following treatments is recommended as first-line management for patients with idiopathic intracranial hypertension? Acetazolamide Corticosteroids Serial lumbar punctures Ventricular Shunt

Correct Answer ( A ) Explanation: Idiopathic intracranial hypertension is caused by increased brain water content and decreased cerebrospinal fluid (CSF) outflow. Patients typically present with generalized headache that is gradual in onset. The headache may be worsened by eye movements. Additionally, any valsalva maneuver decreases cerebral venous return and will worsen the headache. Patients will often have visual complaints including transient vision loss secondary to ischemia of the visual pathways. Other typical symptoms include nausea, vomiting and lightheadedness. Fundoscopic examination reveals papilledema. Diagnosis is made based on an increased opening pressure found on lumbar puncture. Treatment focuses on decreasing CSF production initially with acetazolamide. Acetazolamide is a carbonic anhydrase inhibitor that can be used alone or in combination with furosemide.

In the initial diagnostic evaluation of a patient with undifferentiated polyneuropathy, which of the following approaches is most appropriate? Electrodiagnostics, then laboratory testing Electrodiagnostics, then nerve biopsy Laboratory testing, then electrodiagnostics Laboratory testing, then nerve biopsy

Correct Answer ( A ) Explanation: In the workup of suspected polyneuropathy, first assess for possible common underlying causes, such as diabetes mellitus, alcohol abuse, medication side effects, chronic renal impairment (uremia) and HIV by taking a complete detailed history. In those patients with common known underlying conditions and mild symptomatology, extensive initial diagnostic work up may be unnecessary. In those patients with unknown underlying conditions, or those with moderate, severe or progressive symptoms, initial diagnostic work-up begins with electrodiagnostic testing, such as electromyography and/or nerve conduction studies. Laboratory studies can be ordered based on electrodiagnostic testing results and history and examination findings.

Which of the following is correct regarding multiple sclerosis? ABecomes more prevalent the farther you live from the equator BMore common in men CRetinal tear is a common presenting symptom DWorsens during the course of pregnancy

Correct Answer ( A ) Explanation: Multiple sclerosis (MS) is most common in people of Northern European descent. Caucasians develop MS at nearly twice the rate of African Americans in the United States. In many areas of the world, MS is more prevalent in temperate latitudes (approaching 1 in 500 in some locations) and becomes less prevalent toward the equator (1 in 20,000 or rare case reports only in some locations). Multiple sclerosis is a disease characterized by multifocal areas of demyelination in the brain and spinal cord, with associated inflammatory cell infiltrates, reactive gliosis, and axonal degeneration. It typically presents in young adults with episodic neurologic dysfunction. Pathologically, most cases are characterized by multifocal areas of demyelination and gross gliotic scarring in the brain and spinal cord. Classic locations of these lesions, called plaques, are the optic nerves, periventricular white matter, deep white matter, juxtacortical white matter, corpus callosum, cerebellar peduncles, and dorsolateral spinal cord. The presentation of MS is quite variable. The classic presentation involves acute to subacute onset of impaired vision or sensation. Patients may often describe fatigue, depression, bladder urgency, weakness, impaired balance, and impaired coordination. Multiple sclerosis occurs 2- to 2.5-fold more frequently in women than in men (B), a sex predilection that is common in autoimmune diseases. Optic neuritis, not retinal tear (C) is a classic presenting syndrome, typically with visual symptoms in one eye. Many women with MS successfully have multiple children, and the symptoms of MS diminish during pregnancy (D), especially by the third trimester, when the frequency of exacerbations is reduced by approximately two thirds.

A 55-year-old woman with a past medical history of hypertension and high cholesterol is undergoing evaluation for transient slurred speech lasting approximately 10 minutes earlier in the day. Her work up has been unrevealing thus far and, in conjunction with the neurology service, a diagnosis of transient ischemic attack is made. Which of the following medication regimens is recommended for this patient upon her discharge? Aspirin 325 mg daily Aspirin 325 mg plus clopidogrel 75 mg daily Enoxaparin 40 mg daily Warfarin 5 mg daily

Correct Answer ( A ) Explanation: Patients who are diagnosed with transient ischemic attack (TIA) are at increased risk for having a cerebrovascular accident (CVA) days to weeks after the initial event. Of the patients who experience a CVA, up to 30% of them had a preceding TIA. The risk of stroke after a TIA is highest 24 hours after the initial event and many of those who go on to have a stroke do so within seven days. Antiplatelet therapy is considered appropriate anticoagulation therapy in patients with cerebrovascular disease and is indicated in most patients with TIA to help prevent an acute CVA. Aspirin, clopidogrel, combination aspirin plus dipyridamole, and ticlopidine are currently the four FDA-approved antiplatelet therapies for secondary stroke prevention. Aspirin is the most well-studied and widely prescribed of the approved antiplatelet medications. Lower doses appear to be as effective as higher doses and have a lower incidence of associated gastrointestinal bleeding. The recommended aspirin dose is 81 mg to 325 mg daily.

Which of the following disease entities can be treated with the Epley maneuver? Benign paroxysmal positional vertigo Meniere's disease Vertebrobasilar insufficiency Vestibular neuritis

Correct Answer ( A ) Explanation: The Epley maneuver may be curative in patients with benign paroxysmal positional vertigo (BPPV). Vertigo is defined as the sensation or illusion of movement, often spinning, when the patient is standing still. There are numerous causes of vertigo. For matters of diagnosis and management, they are often divided into peripheral causes and central causes. Peripheral causes involve pathophysiologic changes outside of the central nervous system and are typically benign (with the exception of acoustic neuroma). Central vertigo, on the other hand, is generally caused by a dangerous etiology affecting the central nervous system (vertebrobasilar insufficiency, cerebellar hemorrhage and occlusion of the posterior inferior cerebellar artery). BPPV typically involves rapid onset of vertigo with nausea and vomiting that is elicited with moving the head to a certain position and relieved by moving the head away from that position. It is typically caused by the presence of an otolith in one of the semicircular canals in the inner ear. A Dix-Hallpike test can be performed to aid in diagnosis and an Epley maneuver can be used to dislodge the otolith from the semicircular canal leading to a resolution of symptoms.

A 56-year-old man underwent a prolonged pelvic surgery. Two weeks later, he reports frequent tripping over his right foot. Strength examination reveals: 5/5 plantar flexion, 5/5 ankle inversion, 3/5 ankle eversion and 2/5 dorsiflexion. Light touch sensation is diminished over the lateral but not medial shin. Ankle and knee reflexes are intact. Injury to which of the following nerves is most likely causative? Common peroneal Femoral Sciatic Tibial

Correct Answer ( A ) Explanation: The common peroneal nerve is a division of the sciatic nerve. As it descends it wraps around the fibular head and then divides into its two distal branches of the superficial and deep peroneal nerves. Compression injury frequently occurs at the fibular head, mainly due to prolonged external pressure, as in prolonged bed rest, high casts or lengthy surgeries. Common peroneal neuropathy typically presents as a foot drop, in which the patient complains of a limp, tripping over the foot as they ambulate. Patients display a steppage gait to compensate, flexing the hip and knee more to help "clear" the dropped foot. Ankle weakness, namely dorsiflexion, is the culprit, as the main dorsiflexor (tibialis anterior) is a deep peroneal innervated muscle. Ankle eversion, powered by peroneus longus and brevis (two superficial peroneal innervated muscles) may also be weak, but plantar flexion and ankle inversion remain normal strength, as these muscles are innervated by the posterior tibial nerve. Paresthesias and numbness often accompany the dorsiflexion weakness, appearing mainly in the distribution of the superficial peroneal nerve (lateral lower leg and dorsum of foot).

A 32-year-old man who works as a carpenter presents with leg numbness. On physical exam, he has decreased light touch and pinprick sensation only in an area around his front pants pocket. Distal leg sensation, strength and reflexes are intact. Which of the following nerves is most likely involved? Lateral femoral cutaneous Pudendal Saphenous Sural

Correct Answer ( A ) Explanation: The lateral femoral cutaneous nerve is a direct branch of the lumbosacral plexus, whose main contribution is from the L2 and L3 roots. It travels inferiorly, running lateral to the femoral nerve and underneath the inguinal ligament, ultimately sending cutaneous branches to the proximal, mainly lateral, thigh. This is a common entrapment site for the lateral femoral cutaneous nerve, especially in patients with large abdomens, as in pregnancy or obesity. It also can also be irritated by repetitive trunk flexion in a patient who wears large belts, as in a carpenter whose tool belt compresses the inguinal region during repetitive forward bending. The pudendal nerves (B) innervate the pelvic floor musculature, and transmit sensation from the perineal region. The saphenous nerve (C), a distal branch of the femoral nerve, is a pure sensory nerve that transmits sensation from the medial lower leg, medial ankle and arch of the foot. The sural nerve (D), a distal branch of the tibial and common peroneal nerves, is also a pure sensory nerve, and transmits sensation from the posterolateral lower leg, lateral ankle and lateral foot.

What is the most common cause of vertigo? Benign paroxysmal positional vertigo Labyrinthitis Meniere's disease Vertebrobasilar insufficiency

Correct Answer ( A ) Explanation: The majority of vertigo is caused by peripheral diseases and are not life-threatening. Benign paroxysmal positional vertigo (BPPV) is the most common of the peripheral disorders. Vertigo is defined as an illusion of motion. It is classically described as sensation of the room spinning. There are a number of causes of vertigo. The critical action in determining the cause is to separate peripheral vertigo from central vertigo. Peripheral vertigo can be caused by vestibular neuritis, labyrinthtits, Meniere's disease, acute otitis media, perilymphatic fistula, trauma to the labyrinth, acoustic neuroma and BPPV. Each of these etiologies have classic findings and symptoms that can aid in differentiating the causes. BPPV is typically caused by the presence of an otolith in the labyrinth system. The Dix-Hallpike maneuver can be used to support the diagnosis of BPPV. After performing a Dix-Hallpike, an Epley maneuver can be performed to expel the otolith from the labyrinth.

A 77-year-old man presents with left sided weakness. The patient woke this morning with difficulty moving his left side. On examination, his leg is weaker than his arm. Which vascular structure is likely responsible for this stroke? Anterior cerebral artery Basilar artery Middle cerebral artery Posterior cerebral artery

Correct Answer ( A ) Explanation: The patient has a stroke syndrome characteristic of the anterior cerebral artery. Patients with ischemic insults to the anterior cerebral artery will often affect frontal lobe function causing patients to lack insight and have impaired judgment. Motor function and sensation is decreased on the opposite site of the body with the lower extremities affected more than the upper extremities. A basilar artery (B) stroke syndrome causes a wide variety of symptoms as this artery feeds the entire vertebrobasilar system or posterior circulation. This structure feeds the reticular activating system leading to impaired levels of consciousness or even loss of consciousness. The basilar artery gives rise to the posterior cerebral artery (D), which supplies the occipital lobe and cerebellum. Patients may experience visual changes related to the occipital lobe involvement. Cerebellar function is impaired demonstrated by nystagmus, difficulty with coordination and ataxia. Patients may also have nausea and vomiting with this vascular involvement. Strokes involving the middle cerebral artery (C) have significant motor and sensory loss on the opposite side of the lesion. The upper extremities are affected more than the lower extremities. If the patient's dominant hemisphere with the language center is involved (most commonly left side of the brain), aphasia is present.

A 32-year-old man presents with acute onset right-sided facial weakness that he first noticed upon waking this morning. You ask him to smile as seen in the image above. Which of the following clinical features, if present, suggests a poorer prognosis? A vesicle found on the right tympanic membrane Dysgeusia Hyperacusis Unilateral symptoms

Correct Answer ( A ) Explanation: The patient has idiopathic facial nerve paralysis (Bell's palsy). Bell's palsy is characterized by rapid onset of unilateral facial paralysis. More than half of patients have a prodromal viral illness (rhinorrhea, myalgias, etc). Bell's palsy is associated with ear pain, decreased tearing, an overflow of tears onto the cheek (epiphora), hyperacute hearing (hyperacusis), and dysgeusia (an impairment of taste). Idiopathic facial nerve paralysis involves the lower motor neuron. Features that suggest an upper motor neuron cause include a slow progression of symptoms, recurrent symptoms, and intact forehead movement. A herpetiform vesicular eruption on the tympanic membrane, pinna, external auditory canal, soft palate, oral cavity, face, or neck suggests a diagnosis of Ramsay Hunt syndrome (herpes zoster oticus). In Ramsay Hunt syndrome, the pain is more severe than in Bell's palsy, and the prognosis is worse, with a lower incidence of complete facial recovery and the possibility of associated sensorineural hearing loss. Bilateral facial nerve paralysis suggests a diagnosis of Lyme disease, which is treated differently than Bell's palsy or Ramsay Hunt syndrome. Bell's palsy and Ramsay Hunt syndrome are treated with steroids and antivirals for 7-10 days.

A 34-year-old man presents with complaints of progressive lower extremity weakness over the past 2 days associated with 2 weeks of diarrhea that has since resolved. Vital signs are HR 89, BP 160/95, and RR 12. On exam, you note symmetric lower extremity weakness with intact sensation and absent ankle reflexes. Which of the following would help to support the most likely diagnosis? Albuminocytologic dissociation Improvement with steroids Increased forced vital capacity Selective enhancement of the dorsal root ganglion on MRI

Correct Answer ( A ) Explanation: The patient is most likely suffering from Guillain-Barré syndrome, a progressive, ascending demyelinating polyneuropathy that typically begins with lower extremity weakness. Classically, GBS presents with symmetric lower extremity weakness, decreased or absent deep tendon reflexes, and little or no sensory involvement (although variable). It follows a respiratory or gastrointestinal infection by weeks to days. Common organisms implicated include Campylobacter jejuni, cytomegalovirus, Epstein-Barr virus, and Mycoplasma pneumoniae. Elevated protein with only a mild pleocytosis on cerebrospinal fluid analysis (termed albuminocytologic dissociation) is highly specific in the clinical setting of suspected GBS. Patients with probable GBS should receive a neurology consult and be admitted for either intravenous immunoglobulin (IVIG) therapy or plasma exchange.

A 67-year-old woman presents with a gradual onset headache that has been present for several days. Examination is notable for tenderness over the left temple. Vital signs are normal. Which of the following is the most appropriate test to order? Erythrocyte sedimentation rate Lumbar puncture with opening pressure Non-contrast head computed tomography Outpatient magnetic resonance imaging

Correct Answer ( A ) Explanation: The patient's symptoms suggest temporal arteritis. Temporal arteritis is a form of giant cell arteritis affecting large and medium-sized vessels. Temporal arteritis occurs most commonly in patients over 50. The pain may occur anywhere on the head, but is usually localized to the temporal region. Up to half of patients may report a history of jaw claudication with chewing due to vascular insufficiency of the masseter and temporalis muscles. Patients may also develop polymyalgia rheumatica involving pain in large, proximal joints throughout the body. The erythrocyte sedimentation rate is markedly elevated in the majority of patients. Definitive diagnosis is made with a temporal artery biopsy. Treatment is with long-term high dose steroids and should not be delayed pending biopsy. Untreated temporal arteritis leads to visual loss in 50% of patients.

During an exam, passive flexion of a patient's neck causes a reflexive flexion of his hips and knees. What is this finding called? Brudzinski sign Griesinger's sign Kernig's sign Levine's sign

Correct Answer ( A ) Explanation: This finding describes Brudzinski sign, which is used as an indicator of meningeal irritation such as in patients with acute meningitis. The test is performed by applying flexion to the patient's neck. A positive test is the observance of a reflexive flexion of the hips and knees. Care should be taken regarding a positive Brudzinski sign, as this does not always indicate acute meningitis. In addition to meningitis, it can also be observed in patients with subarachnoid hemorrhage or encephalitis. All conditions that cause meningeal irritation. Griesinger's sign (B) is swelling of the posterior auricular area and may be seen with certain types of sinus thrombosis. Kernig's sign (C) is usually assessed along with Brudzinski. It is performed by flexing the hip and knee to 90° and then attempting to extend the knee. This will cause significant pain in a patient with meningeal irritation.

A 40-year-old man presents to the ED with the chief complaint of headache for two days. The headache is right frontal, constant, and severe. He has tried ibuprofen and acetaminophen without relief of the pain. He denies history of headaches, recent trauma, nausea, vomiting, and syncope. He has a past medical history of cervical disk protrusion for which he takes ibuprofen and gets manipulation by a chiropractor. His vital signs are T 36.6°C, BP 142/90, HR 82, RR 16, and oxygen saturation 99%. On examination, his right pupil is 3 mm and reactive; his left pupil is 6 mm and reactive. Extraocular movements are intact. He is unable to keep his right eyelid open against resistance. Visual acuity is 20/30 in both eyes with glasses. Fundoscopic exam is normal. His neck is supple. Strength is 5/5 in all extremities, gait is normal, and Romberg is negative. Which of the following is the most likely diagnosis? Carotid artery dissection Cluster headache Retinal artery occlusion Temporal arteritis Vertebral artery dissection

Correct Answer ( A ) Explanation: This patient has a carotid artery dissection. This is the most frequent cause of stroke in patients < 45 years old. Risk factors include minor neck trauma (cervical manipulation in this patient), family history of arterial disease, and connective tissue disorders. The most common presenting symptoms are unilateral neck pain or headache around the eye or frontal area. The pain is classically abrupt in onset. Carotid artery dissection is associated with a partial ipsilateral Horner's syndrome of miosis and ptosis without facial anhidrosis. Horner's syndrome results from a disruption of sympathetic innervation anywhere along the sympathetic chain. A carotid bruit may be heard on exam. The diagnostic study of choice is a MRI/MRA. If left untreated, it can lead to cerebral ischemia or retinal artery infarction and vision loss. However, dissection of the vertebral artery (E) is typically associated with marked occipital or posterior neck pain and may be accompanied by focal neurological deficits, including ipsilateral facial numbness and contralateral pain and temperature sensory loss

A 91-year-old man was diagnosed with a middle cerebral artery thrombotic stroke last week. He currently has 3/5 motor strength in his left upper extremity and his left leg. According to the World Health Organization, this patient's hemiplegia would be considered a(an)? Disability Handicap Impairment Limitation

Correct Answer ( C ) Explanation: "Disability" is an umbrella term, covering impairments, activity limitations, and participation restrictions. An impairment is a problem in body function or structure. The United States government funds 3 major disability programs: Social Security Disability Insurance (SSDI), Supplemental Security Income (SSI), and worker's compensation. An activity limitation, or disability (A), is a difficulty encountered by an individual in executing a task or action. A participation restriction, or handicap (B), is a problem experienced by an individual in involvement in life situations. There is no specific definition of a limitation (D).

A 76-year-old woman with a history of hypertension presents with left sided headache and jaw pain. She states that over the last four weeks, she has had progressively worsening headaches and the pain in her jaw makes it difficult to eat. She also complains of decreased vision in her left eye for the last 6 hours. Exam reveals tenderness over the left temple and jaw. Which of the following is the most appropriate next step in management? ESR, CRP and initiate corticosteroid therapy ESR, CRP and referral to a rheumatologist for further management Non-contrast head CT scan Treat her headache with NSAIDs and refer to an ophthalmologist

Correct Answer ( A ) Explanation: This patient has a presentation consistent with temporal arteritis or giant cell arteritis. Temporal arteritis is a large vessel disease characterized by granulomatous inflammation. It is most common in women aged 50-70 years. Onset of symptoms is usually gradual and can have systemic manifestations including fever, weight loss and anorexia. The classic symptoms are visual loss in one eye, temporal artery tenderness and jaw claudication. Headache is the most common presenting symptom. The diagnosis can be made based on clinical manifestations alone if classic symptoms are present. If the patient does not have classic symptoms, an elevated ESR (> 50 mm/hr) and CRP can aid in diagnosis. Ultimately, a temporal artery biopsy is needed to confirm the diagnosis. It is critical to initiate empiric treatment with corticosteroids. Any delay can result in permanent loss of vision.

A 30-year-old woman presents to the ED complaining of pain in the right side of her face for the last two days. The pain is sharp, severe, and lasts several seconds. The pain is associated with an involuntary movement of the side of her mouth. In between episodes, there is no pain. She has no past medical history. Her vitals include a temperature of 36.6°C, BP 122/78, HR 80, RR 20. Her exam is normal. Which of the following medications has been shown to relieve this condition and is the first line treatment? Carbamazepine 100 mg twice daily Gabapentin 100 mg three times daily Phenytoin 100 mg three times daily Prednisone 60 mg once daily

Correct Answer ( A ) Explanation: This patient has trigeminal neuralgia (tic douloureux), a disorder of the trigeminal nerve (cranial nerve V). The treatment is carbamazepine 100 mg twice daily. Carbamazepine is thought to work by reducing postsynaptic tetanic contractions. It is used to treat trigeminal neuralgia and is used as an antiepileptic. It is the first line treatment and the main antiepileptic used for this condition. Trigeminal neuralgia is characterized by paroxysms of severe unilateral pain in the trigeminal nerve distribution, sometimes described as recurrent bursts of an electric shock. The pain lasts only a few seconds and most commonly occurs on the right side of the face. On exam, you may be able to elicit pain by tapping trigger zones along the distribution of the trigeminal nerve. Otherwise, there should be no demonstrable physical findings.

A 17-year-old boy experiences involuntary contractions of the facial and neck muscles after administration of haloperidol. The patient has arching of the back, deviation of the head to the left and protrusion of the tongue. What management is indicated? Benztropine Cyproheptadine Dantrolene Diazepam

Correct Answer ( A ) Explanation: This patient presents with a dystonic reaction after administration of a neuroleptic agent and should be given benztropine. Acute dystonia is the most common adverse effect seen after the administration of neuroleptic agents and occurs in up to 5% of patients. This reaction is caused by disruption in the balance between dopaminergic and cholinergic pathways in the basal ganglia. They tend to occur within 48 hours of administration of a neuroleptic agent. Patients often experience tongue protrusion, acute torticollis, sustained eye deviation (usually upwards) and arching of the back. Laryngospasm is rare but potentially life threatening. Because dystonic reactions occur as a result of cholinergic dominance, they should be treated with anticholinergic agents including benztropine and diphenhydramine. These agents typically reverse symptoms immediately.

A 44-year-old woman presents with involuntary movements of the neck, face and tongue. She reports that she was seen earlier in the Emergency Department for vomiting and received metoclopramide. What management is indicated? Benztropine intramuscular Lorazepam intramuscular MRI of the brain Noncontrast CT scan of the head

Correct Answer ( A ) Explanation: This patient presents with a dystonic reaction after the administration of metoclopramide and should be given benztropine. Acute dystonia is the most common adverse effect seen after the administration of neuroleptic agents and can occur in up to 5% of patients. It can also occur with medications that antagonize central and peripheral dopamine receptors such as metoclopramide. This reaction is caused by disruption in the balance between dopaminergic and cholinergic pathways in the basal ganglia. They tend to occur within 48 hours of administration. Patients often experience tongue protrusion, acute torticollis, sustained eye deviation (usually upwards) and arching of the back. Laryngospasm is rare but potentially life threatening. Because dystonic reactions occur as a result of cholinergic dominance, they should be treated with anticholinergic agents including benztropine and diphenhydramine. These agents typically reverse symptoms immediately.

A 32-year-old woman presents with intermittent double vision for one week. The patient states that she woke up this morning with no symptoms but now has double vision and this pattern has been going on for the last week. The patient states symptoms get better with rest. Physical examination reveals drooping of the upper eyelids and the left eye is unable to abduct. What is the next best step in management of this patient? Administer edrophonium Intravenous immunoglobulin MRI of the brain Noncontrast CT of the head

Correct Answer ( A ) Explanation: This patient presents with signs and symptoms concerning for myasthenia gravis (MG) and can have edrophonium administered to confirm the diagnosis. MG is an uncommon disease that affects women more commonly than men. The disease results from the presence of autoantibodies directed against nicotinic acetylcholine receptors (AChR) at the neuromuscular junction. This leads to destruction of AChRs resulting in muscle weakness. Muscle weakness that gets worse with use is the hallmark of MG. Patients will complain of weakness in muscle groups with repeated use. Ocular symptoms often manifest early and include ptosis, diplopia and blurred vision. These symptoms are typically worse at the end of the day. Respiratory failure presents later in the disease and may lead patients with a diagnosis of MG to present to the ED. The quality of respiration and ventilation can be quantified using a negative inspiratory flow (NIF) measurement. Patients with symptoms concerning for MG can be diagnosed using the edrophonium test. Edrophonium is a short acting acetylcholinesterase (ACh)-blocking agent which leads to increased ACh in the presynaptic cleft. Administration in patients with MG should lead to decreased symptoms. Use of edrophonium can result in potentially significant complications, such as symptomatic bradycardia and bronchospasm. It should be used with caution, if at all, in older patients, those with asthma/COPD and those with underlying cardiac disease.

A 33-year-old woman presents with intermittent, intense shooting pain to the left side of the face. She states that the pain begins near her ear and radiates to her chin. The pain is often brought on by chewing and brushing her teeth. Which of the following managements is most likely indicated? Carbamazepine Dental X-rays Mandible CT scan Prednisone

Correct Answer ( A ) Explanation: This patient presents with symptoms consistent with trigeminal neuralgia and should be treated with carbamazepine. Trigeminal neuralgia is a syndrome characterized by sudden paroxysms of lancinating pain in one or more of the trigeminal nerve distributions. The syndrome manifests with unilateral facial pain triggered by chewing, brushing the teeth or touching the affected areas of the face. Hot and cold temperature exposure can provoke symptoms as well. The maxillary (V2) and mandibular (V3) branches of the trigeminal nerve are most commonly affected. The diagnosis is made based on clinical presentation. First line therapy for trigeminal neuralgia is with carbamazepine. Refractory cases may require surgical management.

A patient is diagnosed with multiple sclerosis. Imaging shows that the majority of plaques are located in the cerebellum. Examination is positive for dysmetria and ataxia. Which of the following types of tremor would most commonly occur in this patient? Intention Pill-rolling Postural Rubral

Correct Answer ( A ) Explanation: Tremor is classified into three main types: postural, intention, and resting. An intention tremor is a tremor that occurs during movement that increases as a target is approached. The tremor is associated with cerebellar signs. The tremor is not seen at rest. Causes of intention tremor include cerebellar or brain stem disease, Wilson's disease, and drug toxicity. Pill-rolling tremor (B) is a type of resting tremor that may affect the fingers, hands, forearms, or feet and has a frequency of 4-6 Hz. Causes of resting tremor include basal ganglia lesions, parkinsonism, Wilson disease, heavy metal poisoning, hyperthyroidism, anxiety, drug withdrawal or toxicity, and benign hereditary tremor. Rubral tremor (D) is rare, occurring mainly with unusual infarcts of the midbrain's red nucleus. It occurs during rest, with movement against gravity (posture) and with intention. Postural tremor (C) occurs with sustained posture of an extremity. Symptoms increase with emotional stress or sleep deprivation and are not present during sleep. They also increase with use of TCAs, valproic acid, lithium, and bronchodilators.

A 42-year-old woman presents with severe facial pain. The patient reports she has had episodes of severe, shooting pain on the lower half of the left side of her face. Her neurologic examination is normal. Her medical history is positive for herpes zoster. Which of the following is the appropriate next step? Carbamazepine prescription CT scan of the head Prednisone prescription Referral for EEG

Correct Answer ( A ) Explanation: Trigeminal neuralgia is manifested by intermittent episodes of severe pain in the distribution of the fifth cranial nerve. The maxillary and mandibular branches of the trigeminal nerve are most commonly affected. Trigeminal neuralgia is thought to be idiopathic although there may be some component of vascular compression as this has been found on surgical exploration of the trigeminal nerve root. Patients will often describe physical triggers that lead to an episode of severe pain. These include: chewing, shaving, brushing the teeth or touching the involved area of the face. In the absence of any neurologic deficit, no imaging is necessary. The antiepileptic carbamazepine is the medical treatment of choice for this condition. Other medications that have been tried but not shown to have more effectiveness include gabapentin, phenytoin, valproic acid, baclofen, lamotrigine and levetiracetam. If medical therapy fails, surgical procedures aimed at the destruction of the trigeminal ganglion may be attempted. A CT scan of the head (B) is not indicated unless a neurologic deficit is identified. Structural lesions including tumors, vascular malformations and aneurysms could cause the neuralgia.

A previously healthy 63-year-old woman presents with vertigo that she first noted when she woke up this morning. Throughout the day, she has experienced intense spinning that calms down with movement. She has been nauseated and having nonbloody, nonbilious emesis. On exam, she has a horizontal nystagmus beating towards the left that is exacerbated by changing head position which resolves after a few moments. She is otherwise neurologically intact and has an unremarkable head and neck exam. Which of the following findings most strongly indicates this is a peripheral nervous system lesion? Lack of other neurologic deficits Nausea Nystagmus Sudden symptom onset

Correct Answer ( A ) Explanation: Vertigo is a challenging chief complaint that can stem from the central nervous system or the peripheral nervous system. Vertigo is a perception of movement when there is none. Central vertigo is most often due to a cerebrovascular accident involving the posterior fossa. Peripheral vertigo is most often due to cranial nerve VIII or vestibular lesions. Differentiating between central and peripheral lesions is critical as the treatment pathways are quite different. A lack of other neurologic deficits (A) points is most indicative of a peripheral nervous system lesion in this patient.

An 82-year-old woman presents to clinic with her daughter. Both are concerned with an acute change in mood, stating that beginning two days ago the patient felt depressed and irritable, described by the daughter as "having a short fuse." The patient reports not having felt this way in the past, even after her husband died 5 years ago. She can state her name, address and date properly. She is well groomed and has been eating and voiding normally, although she does complain of dysuria for the past four days. Which of the following is the most likely diagnosis? Delirium Dementia Disorientation Major depression

Correct Answer ( A ) Explanation: When caring for elderly patients, it is important to monitor cognitive status and functional status. This age group is commonly affected with three cognitive disorders: depression, delirium and dementia. Depression affects up to 20% of elderly patients, especially post stroke or concurrent with Parkinson's disease, cancer or loss of family members. It can be evaluated with a PDQ-9 test, and if found, should be treated accordingly. Delirium, or acute confusional state, is typically a reversible condition commonly due to an underlying medical condition. It most commonly occurs in hospitalized elderly patients. As compared to depression and dementia, delirium is characterized by acute and fluctuating changes in mood or behavior of which the patient is usually aware. Common sources of delirium include acute infection, thyroid dysfunction, stroke, medications (especially anticholinergics) or impairment of the cardiopulmonary and renal systems. It is evaluated with the Confusion Assessment Method (CAM) questionnaire. Treatment is structured on diagnosing and treating the underlying condition, providing frequent reorientation and using antipsychotic medication (off-label) for agitation or restoring the sleep-wake cycles.

A patient presents with nausea, vomiting, right-sided hemiplegia and non-occipital headache. His gaze is deviated to the left, but he denies loss of sensation. Thirty minutes later, he becomes stuporous and progresses into coma. The pupils are now fixed and dilated. Abnormal posturing is absent. A brain CT scan is ordered. In which of the following sites would you expect to find intracerebral hemorrhage? Left pons Left putamen Right cerebellum Right thalamus

Correct Answer ( B ) Explanation: A large putamen hemorrhage results in a rapid progression of hemiplegia, nausea, vomiting and headache over 30 minutes, which is quickly followed by ipsilateral deviation of the eyes, stupor, coma and mydriatic pupils ("blown-pupil", associated with brainstem compression and oculomotor nerve palsy). Acute management includes controlling intracranial pressure and hypertension. Surgical removal of clots is seldom successful, however, some neurologic function may be salvaged in those without coma or those with lobar clots. Pontine hemorrhages (A) are characterized by total paralysis, rapid coma, decerebrate rigidity (abnormal posturing marked by shoulder adduction, elbow extension, wrist pronation and flexion, digit flexion, leg extension and plantarflexion) and small (miotic), not mydriatic, reactive pupils. Cerebellar insults (C) are evidenced by vomiting, occipital headache, inability to stand, vertigo, and eye deviation to the opposite side of the lesion. In this scenario, the eye deviation makes sense, but not the presenting symptoms.Thalamic lesions (D) are marked by complete hemisensory loss. Also common is hemiparesis (bleeding extension into the internal capsule) and aphasia.

A 59-year-old woman with a history of poorly controlled hypertension presents to the emergency room for a sudden, very severe headache that began 50 minutes ago. She is disoriented and vomiting. A CT of her head showed no acute abnormalities. Which of the following is the next best in her management? Electroencephalogram Lumbar puncture MRI head Supportive care

Correct Answer ( B ) Explanation: A lumbar puncture is the next best step to rule out a spontaneous subarachnoid hemorrhage (SAH). This patient has exhibited the classic symptoms of a SAH: a sudden headache, often of severity never experienced previously, nausea and vomiting, and impairment of consciousness that may progress to coma. A physical exam can show nuchal rigidity. Focal neurologic signs usually only present in the setting of a focal intracerebral hematoma. The patient's history might reveal one or more risk factors for intracerebral hemorrhages, including recent head trauma, hypertension, tobacco use, or heavy alcohol use. Females of advancing age are at the greatest risk. At the first suspicion of a SAH, a CT scan, with CT angiography if possible, should be performed. If the CT scan does not confirm a hemorrhage, a lumbar puncture must be performed to fully rule out the diagnosis. An intracerebral hemorrhage is suspected when the cerebrospinal fluid is bloodstained in a quantity greater than that of a traumatic tap. Xanthochromia is also indicative of cerebral bleeding. Once diagnosed, patients need admission, and cerebral angiography should be done to determine the source of bleeding. Surgical clipping or endovascular treatment by interventional radiologists is necessary to definitively treat the hemorrhage. Following this, measures to prevent rehemorrhaging during the critical days following an intervention are necessary. These include bed rest with avoidance of physical exertion, and use of antiemetics, stool softeners, and antitussives as applicable. Seizure prophylaxis, usually with phenytoin, may also be used.

Which of the following characteristics helps to differentiate delirium from dementia? Attention is spared in delirium Delirium has an acute onset Delirium has no change in level of consciousness PET scanning is abnormal in delirium

Correct Answer ( B ) Explanation: An important distinction between delirium and dementia is the time of onset. Delirium is characterized by its rapid onset and fluctuating course. It leads to acute changes in mental status, marked by inattention, poor concentration, and fluctuating levels of consciousness. It commonly occurs in hospitalized elderly patients. Many disorders can have similar clinical features of delirium and patients with delirium are often mistakenly diagnosed with dementia or depression. Delirium, dementia, and depression can coexist within the same patient. When an acute change in mental status occurs, however, the diagnosis of delirium should be presumed until proven otherwise. Treatment involves identifying and treating the underlying causes. Clinicians should minimize the use of physical restraints as much as possible and encourage consistent presence of family or caregivers familiar to the patient.

A 45-year-old woman presents to her primary care provider with paroxysmal stabbing unilateral facial pain. She denies recent facial rash, trauma to the area, or other neurological complaints. Magnetic resonance imaging of the brain is normal. Which of the following is the most appropriate treatment? Baclofen Carbamazepine Lamotrigine Viscous lidocaine

Correct Answer ( B ) Explanation: Carbamazepine is the most appropriate first-line therapy for patients with trigeminal neuralgia. Trigeminal neuralgia is an uncommon facial pain syndrome characterized by unilateral electric shock-like pain in the distribution of the fifth cranial nerve (trigeminal nerve). The most common cause of trigeminal neuralgia is compression of the nerve root as it exits the pons. The most common source of compression is an aberrant artery or nerve. Other causes of compression include vestibular schwannoma, meningioma, epidermoid cysts, and saccular aneurysms. Trigeminal neuralgia is more common in women than men. Most cases begin after the age of 50 years. Trigeminal neuralgia classically presents with paroxysmal attacks of sharp pain in the distribution of one or more branches of the trigeminal nerve. The pain is typically maximal at onset and described as shock-like, stabbing, or electric. Magnetic resonance imaging with and without contrast is recommended in all patients with suspected trigeminal neuralgia to rule out structural causes. Carbamazepine is first-line therapy for patients with idiopathic trigeminal neuralgia or trigeminal neuralgia caused by neurovascular compression. Carbamazepine is typically started at 100 to 200 mg and gradually titrated by 200 mg each day until adequate pain relief is obtained. Slow titration is useful for minimizing the most common side effects of carbamazepine (e.g. drowsiness, dizziness, nausea, and vomiting). Surgery is reserved for patients with trigeminal neuralgia refractory to pharmacotherapy. Trigeminal neuralgia prognosis is variable.

30-year-old woman is pregnant with her third child. Her medical history is significant for cocaine abuse. She delivered her first and second low-birth-weight children preterm. Both of these children have congenital abnormalities. Which of the following diseases is this third fetus most at risk of developing? Cerebellar ataxia Cerebral palsy Charcot-Marie-Tooth disease Cystic fibrosis

Correct Answer ( B ) Explanation: Cerebral palsy (CP) is a neurodevelopmental disease caused by a non-progressive CNS insult that occurred during fetal or infant development. It is characterized by movement and posture abnormalities which are associated with a seizure disorder or disturbances of sensation, cognition, perception, behavior and communication, or both. Risk factors include birth weights <2,000 grams, sibling history of congenital malformations, maternal mental retardation or substance abuse (cocaine causes intrauterine ischemia) and birth injury. Three main classes of CP exist: (1) Dyskinetic (extrapyramidal) CP, in which intrapartum asphyxia leads to a need for postnatal resuscitation and eventual tetraplegia, pseudobulbar palsy, choreathetosis and dystonia; (2) Spastic (pyramidal) CP, which occurs gradually between the ages of 4-6 months, and manifests as extremity paralysis (spastic diplegia, triplegia or tetraplegia), gait abnormalities and decreased intelligence; (3) Mixed CP, in which both types of symptoms are present. The major complications which carry over into adulthood are mental retardation, learning disabilities, seizure, musculoskeletal pain, osteoarthritis, hip and back problems, bowel and bladder problems and gastroesophageal reflux. Treatment is based on aggressive and lifelong physical, occupation and speech rehabilitation. Cerebellar ataxia (A) is a childhood, not fetal, disease caused by previous infection with varicella-zoster virus, Epstein-Barr virus and echovirus. Charcot-Marie-Tooth disease (C), the most common inherited neurologic disorder, is characterized by myelin and axonal abnormalities that cause distal > proximal weakness and atrophy. Maternal age and substance abuse history are not specific risk factors for this condition. The biggest risk factor for cystic fibrosis (D) is family history, not maternal substance abuse.

A 22-year-old man presents to the ED with unilateral throbbing headache. He denies family history of headache, however, he has had this type of headache before. Beginning around age 20 years, he started having four to six "flares" a day for six weeks at a time. Each flare would last 30 minutes and they mainly occurred at night. Eventually they resolved, but reappeared after his 21st birthday and lasted for about two months. He had been asymptomatic until two days ago when the headaches returned. Physical examination shows conjunctival injection and rhinorrhea. Which of the following treatments should most likely be initiated first in this patient? Acetaminophen, aspirin, and caffeine Oxygen Topiramate Verapamil

Correct Answer ( B ) Explanation: Cluster headaches occur most often in middle-aged men. It is described as a recurrent, unilateral, excruciating periorbital headache that lasts from 15 minutes to three hours. Headaches are nonpulsatile and constant, frequently occurring at night. Cluster headaches are associated with ipsilateral conjunctival injection, lacrimation, and nasal congestion. Cluster headaches classically take place in groups over days to weeks, occurring at the same time of day and in the same location. Acute attacks can be treated with 100% oxygen. Other treatments include triptans, ergotamines, intranasal lidocaine, and butorphanol. Topiramate (C) and verapamil (D) are used in the prevention, not abortion, of cluster headache. Acetaminophen-aspirin-caffeine (A) is used more commonly in treating migraine, not cluster, headache.

A 23-year old man presents to the emergency department with a right-sided headache lasting 30 minutes. He reports having these headaches 3-5 times a day with associated rhinorrhea and lacrimation. Which of the following is the initial abortive treatment of choice? Naproxen Oxygen Prednisone Verapamil

Correct Answer ( B ) Explanation: Cluster headaches occur most often in middle-aged men. They are described as recurrent, unilateral, excruciating periorbital headache that lasts from 15 minutes to 3 hours. Headaches are nonpulsatile and constant and frequently occur at night. They are associated with ipsilateral autonomic symptoms such as eyelid edema, nasal congestion, lacrimation, and forehead sweating. Horner's syndrome may also be present. Cluster headaches classically take place in groups over days to weeks, occurring at the same time of day and in the same location. The mainstay of treatment is 100% oxygen with tripans used as an adjunct for abortive therapy. Other abortive therapies include intranasal lidocaine and butorphanol. Cluster headaches are associated with an increased suicide rate due to the associated severity of pain

A 33-year-old woman is being evaluated in clinic for pain in her left lower leg. She underwent an arthroscopic meniscus repair of the left knee three months ago. She denies any recent trauma. Patient reports sudden onset of pain and burning sensation in her leg. It does not worsen with physical activity or elevation. She denies fever or chills. On physical exam her left leg is erythematous, warm, swollen and tender to the lightest touch. Lower extremity pulses are 2+ bilaterally. Initial lab work reveals a normal complete blood count, erythrocyte sedimentation rate, and C-reactive protein. What is the most likely diagnosis? Cellulitis Complex regional pain syndrome Erysipelas Peripheral vascular disease

Correct Answer ( B ) Explanation: Complex regional pain syndrome is a form of chronic pain in a body region, but it most commonly affects the extremities. It is characterized by pain, swelling, skin changes, vasomotor instability, limited range of motion, and patchy bone demineralization. It frequently occurs after a soft tissue injury, surgery, or vascular event such as a myocardial infarction or stroke. The pathophysiology of complex regional pain syndrome is incompletely understood and most of the time there is no definable nerve lesion. Symptoms of complex regional pain syndrome include pain that is described as burning, tingling, throbbing or aching. The pain can be quite severe and out of proportion to physical exam. Many patients experience allodynia, which is pain from a stimulus that does not usually provoke pain. Other symptoms include sensitivity to cold or touch, swelling, skin changes such as thickened, rough skin, or muscle atrophy. A diagnosis of complex regional pain syndrome requires the presence of regional pain and sensory changes following a noxious event. It is usually made clinically as there is no laboratory or radiologic testing that alone is diagnostic.

40-year-old man with HIV presents with two weeks of progressive headache, malaise, and fever. On examination, he has mild nuchal rigidity, confusion, and a temperature of 38.2oC. Cerebrospinal fluid analysis shows a white blood cell count 360 cells/mL with a monocyte predominance, glucose 28 mg/dL, and protein 220 mg/dL. What is the treatment of choice? Acyclovir Amphotericin B Ceftriaxone Vancomycin

Correct Answer ( B ) Explanation: Cryptococcal meningoencephalitis is an opportunistic infection that occurs primarily in patients with advanced AIDS, although it can be seen in immunocompromised transplant patients as well. The majority of HIV-related cases occur when the CD4 count is < 100 cells/mm3. Patients present with progressive headache, nausea, malaise, and fever over the course of 1 - 2 weeks. Examination findings are typical for meningitis and include altered mental status, photophobia, and fever. Meningismus is less commonly seen. Presentations can be subtle and a high index of suspicion is needed for diagnosis. A CT scan of the brain is indicated if there are signs of increased intracranial pressure or focal neurologic deficits. Lumbar puncture should be performed with careful measurement of the opening pressure. Cerebrospinal fluid will show a mildly elevated white blood cell count with a monocyte predominance, decreased glucose, and mildly elevated protein. However, in some cases the cerebrospinal fluid will only show minor, if any, abnormalities. Cryptococcal antigen testing of the CSF is nearly 100% sensitive and specific. India ink staining will show budding organisms. Treatment of choice is intravenous amphotericin B in addition to oral flucytosine for 14 days followed by an 8 week course of oral fluconazole.

For the past two years, a 77-year-old man is more forgetful. He completely disagrees with this observation, but his daughter calls you concerned with her father's progressive memory loss. She states that her "stubborn" dad has become "more edgy" around the family, which only seems to occur at the end of the day. She adds that her dad has difficulties with remembering the names of the grandchildren. You schedule the patient and his daughter for an appointment. Which of the following tests will you most likely administer during this appointment? Confusion Assessment Method Mini-Mental State Examination PHQ-9 Stanford-Binet test

Correct Answer ( B ) Explanation: Elderly patients are at high risk of depression and cognitive changes, namely delirium (acute onset) and dementia (chronic onset). Dementia, an age-related progressive cognitive decline, affects 5% of those aged 71-80 years, and near 40% of those aged over 90 years. Many patients are unaware or ignore these cognitive changes, chalking them up to "getting old." Dementia presents with gradual, progressive memory loss. It is associated with word-finding and concentration problems, emotional lability, personality changes, social withdrawal, and difficulties with dressing, cooking, balancing the checkbook, and maintaining hygiene. There are multiple types of dementia: Alzheimer disease, vascular dementia, and other less common dementias. Alzheimer dementia is most common. Dementia can be evaluated with brief quantitative screening tests of cognitive function, such as the Folstein Mini-Mental State Examination (MMSE) or the Memory Impairment Screen. The MMSE may be useful to provide a baseline for future comparison. Controversy exists over the use of memantine and anticholinergic medications in the treatment of dementia. The Stanford-Binet test (D) is a cognitive ability and intelligence test used to diagnose developmental or cognitive deficiencies in children. It is not used in the evaluation of elder cognitive changes. T

34-year-old man presents to the psychiatric clinic because his wife thinks he has become more irritable, reckless, and paranoid over the past year. During the patient interview, he appears unable to prevent occasional, jerking movements of his arms and torso. He says his father developed the same problem in his 40's and died alone in a psychiatric hospital about 15 years later. Which of the following tests will most definitively diagnose this patient's likely condition? Brain MRI Genetic testing Lumbar puncture Positive emission tomography (PET) scan

Correct Answer ( B ) Explanation: Genetic testing is the most definitive method of diagnosing Huntington disease, the most likely disease in this patient. Huntington disease is a genetically-passed neurologic disorder with an autosomal dominant inheritance pattern. Patients usually present with symptoms between 30 and 50 years of age and typically first notice mild abnormal movements or intellectual changes. Irritability, personality changes, and antisocial behaviors are common. In the initial stages, the abnormal movements may merely be slight fidgeting or restlessness. However, over the next 15-20 years, the disorder will progress to cause obvious dementia and the stereotypic dystonic posturing and choreiform ("dance-like") movements that are hallmarks of the disease. Huntington disease may be suggested by cerebral atrophy of the caudate nucleus on CT or MRI scanning of the brain. A positive emission tomography (PET) scan might show a reduced striatal metabolic rate. However, genetic testing under the guidance of a licensed genetic counselor is diagnostic. There is no cure for Huntington disease and no treatments exist to slow its progress. Tetrabenazine is usually used to manage the dyskinesia. Phenothiazines or haloperidol may be recommended to provide additional control of movements and to palliate any behavioral disturbances. Clozapine is occasionally needed to manage severe psychiatric effects. All patients with Huntington disease should be given the option to have their offspring referred for genetic counseling to aid in informed reproductive planning.

Which of the following is true regarding hyperbaric oxygen therapy for carbon monoxide poisoning? It reduces mortality It reduces risk of neurologic sequelae It reduces the risk of dysrhythmia It reduces the risk of myocardial infarction

Correct Answer ( B ) Explanation: Hyperbaric oxygen therapy has been shown to decrease the risk for developing delayed neurologic sequelae in patients with carbon monoxide (CO) poisoning. CO is the most common cause of acute poisoning death in the developed world. CO is created through incomplete combustion of carbon containing materials. CO combines with deoxyhemoglobin to form carboxyhemoglobin (COHb). COHb is unable to carry oxygen. The bond between hemoglobin and CO is tight and is only slowly reversible. CO also inhibits cytochromes involved in cellular oxygen use thus poisoning mitochondrial oxidative phosphorylation. Patients can present with a host of neurologic symptoms including dizziness, confusion, coma and seizures. Additionally, significant metabolic acidosis and impaired oxygen utilization can lead to hypotension and cardiac arrest. Initial treatment focuses on supplemental oxygen therapy, which decreases the half-life of COHb. Hyperbaric oxygen (HBO) therapy can further decrease the half-life and can achieve a high PO2 level. HBO has only been shown, however, to prevent the development of delayed neurologic sequelae.

27-year-old man presents to your office with a complaint of numbness and weakness in his feet and fingers that began 24 hours ago. He tells you that two weeks ago he had an upper respiratory infection, but is otherwise healthy. Physical exam 4/5 motor of the lower extremities and absent deep tendon reflexes. Which of the following is the most likely diagnosis? Botulism Guillain-Barré syndrome Lyme disease Multiple sclerosis

Correct Answer ( B ) Explanation: Guillain-Barré syndrome (GBS) is a post-infection, immune-mediated polyneuropathy. The cardinal feature of GBS is progressive, ascending, symmetric muscle weakness that begins in the lower extremities. Two-thirds of patients with GBS report a viral or bacterial infection prior to the onset of symptoms, most commonly upper respiratory or gastrointestinal. Muscle weakness progresses over hours to days and may eventually affect the muscles of respiration. Diagnosis is initially made based on clinical presentation and may be confirmed with cerebrospinal fluid analysis or neurophysiology studies including nerve conduction studies or needle electromyography. Patients diagnosed with GBS need to be hospitalized for close monitoring until it is determined where the patient is in the course of the disease. Many patients develop respiratory failure and require admission to the intensive care unit. Supportive care is the most important treatment modality for patients with GBS. Patients should receive deep vein thrombosis prophylaxis, bladder and bowel care, physical therapy and emotional support as part of their management plan. Prognosis ranges from full recovery to death, based on severity of symptoms and progression of the disease.

A 27-year-old man presents to your office with a complaint of numbness and weakness in his feet and fingers that began 24 hours ago. He tells you that two weeks ago he had an upper respiratory infection, but is otherwise healthy. Physical exam reveals right-sided facial droop and absent deep tendon reflexes. Which of the following is the most likely diagnosis? Botulism Guillain-Barré syndrome Lyme Disease Multiple sclerosis

Correct Answer ( B ) Explanation: Guillain-Barré syndrome (GBS) is a post-infectious, immune-mediated polyneuropathy. The cardinal feature of GBS is progressive, ascending, symmetric muscle weakness that begins in the lower extremities. Two-thirds of patients with GBS report a viral or bacterial infection prior to the onset of symptoms, most commonly upper respiratory or gastrointestinal. Muscle weakness progresses over hours to days and may eventually affect the muscles of respiration. Diagnosis is initially made based on clinical presentation and may be confirmed with cerebrospinal fluid analysis or neurophysiology studies including nerve conduction studies or needle electromyography. Patients diagnosed with GBS need to be hospitalized for close monitoring until it is determined where the patient is in the course of the disease. Many patients develop respiratory failure and require admission to the intensive care unit. Supportive care is the most important treatment modality for patients with GBS. Patients should receive deep vein thrombosis prophylaxis, bladder and bowel care, physical therapy and emotional support as part of their management plan. Prognosis ranges from full recovery to death, based on severity of symptoms and progression of the disease.

A 48-year-old woman presents with a two-day history of weakness and loss of sensation in both feet. The weakness today is spreading to her bilateral lower legs and hands. What is the most likely diagnosis? Amyotrophic lateral sclerosis Guillain-Barré syndrome Myasthenia gravis Parkinson's disease

Correct Answer ( B ) Explanation: Guillain-Barré syndrome generally presents with progressive weakness that starts peripherally and progresses centrally. It is associated with ascending paralysis and loss of deep tendon reflexes. The course is usually acute or subacute and sometimes follows infections, surgeries, or inoculations. The disorder is thought to have an immunologic basis, but the exact mechanism is unclear. Neuropathic or radicular pain is common. In severe cases, life-threatening weakness of the muscles of respiration may occur. Most patients recover, although approximately 20 percent have persistent disability. Approximately three percent of patients have clinical relapses, often many years after the initial occurrence. Amyotrophic lateral sclerosis (ALS) (A) is a mixed upper and lower motor neuron disorder. Weakness is usually progressive over a longer term and sensory deficit usually does not occur. ALS can be associated with cognitive decline and can be familial.

A previously healthy 32-year-old man presents to your office with a complaint of tingling in both feet. He says that the tingling sensation started 3 days ago and now his lower legs feel weak and he is having difficulty walking. In addition, he is having trouble chewing his food. Physical exam shows absent patellar and Achilles reflexes. Which of the following is the most likely diagnosis? Diabetic neuropathy Guillain-Barré syndrome Multiple sclerosis Myasthenia Gravis

Correct Answer ( B ) Explanation: Guillain-Barré syndrome is an autoimmune disorder that affects the peripheral nervous system. The classic presentation involves symmetric, progressive muscle weakness along with weak or absent deep tendon reflexes. Facial weakness occurs in more than 50% of individuals. Diagnosis is generally based on clinical signs and symptoms, but may be confirmed by cerebrospinal fluid analysis which will reveal elevated protein with a normal white blood cell count. Treatment includes supportive care, admission to the ICU for monitoring of respiratory and cardiac function, plasma exchange and IVIG.

A 40-year-old man presents to the office with his wife who states that she has noticed some personality changes and aggressive behavior that began a few months ago. He is now having brief irregular involuntary movement of his arms, is unable to focus, and is having trouble problem solving. His wife also describes excessive blinking and head thrusts. Which of the following conditions are you suspicious for? Alzheimer disease Huntington disease Parkinson disease Wilson disease

Correct Answer ( B ) Explanation: Huntington disease is a fully penetrant autosomal dominant neurodegenerative disorder caused by an expanded trinucleotide (CAG) repeat in the gene for the protein huntingtin. The prevalence is approximately 10 per 100,000. Symptoms include a combination of a movement disorder, psychiatric disturbances, and cognitive dysfunction. Early on, the movement disorder is predominantly chorea, but parkinsonism and dystonia develop later. Some patients, especially those with juvenile onset, have a more rapidly progressive akinetic-rigid and dystonic form (the Westphal variant). Psychiatric manifestations, which are universal but widely variable, include personality changes, impulsiveness, aggressive behavior, depression, and paranoid psychosis. These psychiatric symptoms may precede the motor manifestations, and psychotropic drug therapy may be incorrectly blamed for the subsequent development of the movement disorder. Cognitive changes result in progressive subcortical dementia with disturbed attention, concentration, judgment, and problem-solving that differs from the typical cortical dementia of Alzheimer's disease. Oculomotor dysfunction most often manifestes in difficulties with refixating the gaze and a resulting tendency to use blinks and head thrusts is common. The diagnosis is confirmed by genetic testing. Wilson's disease (D) is characterized by Kayser-Fleischer rings, tremor, lack of coordination, drooling, and signs of liver failure.

A 67-year-old woman presents with severe unilateral pain in her face and head. She characterizes the symptoms as lasting only a few seconds and denies any associated nausea, vomiting, dizziness, or blurry vision. Her Head, Eye, Ear, Nose and Throat Exam (HEENT) and neurologic exam are normal. Which of the following statements is correct regarding this patient's condition? AFacial droop is associated with this condition BPain is most commonly located in the V2 and V3 cranial nerve distribution CPain is usually bilateral in the elderly population DPoor dentition is associated with this condition EThe typical pain attack lasts 30-60 minutes then resolves spontaneously

Correct Answer ( B ) Explanation: Trigeminal neuralgia manifests with unilateral facial pain characterized as lancinating paroxysms of pain in the lips, teeth, gums, and chin. The pain is often associated with triggers such as chewing, brushing the teeth, shaving, washing or touching the affected area of the face, swallowing, or exposure to heat or cold in the affected area. Pain most often occurs in the maxillary (V2) and mandibular (V3) distribution of the trigeminal (V) nerve. The ophthalmic division (V1) is rarely involved.

A 35-year-old woman with a known history of seizure disorder is actively seizing in the ED. Which of the following is the first-line medication and route to treat her seizure? Intramuscular fosphenytoin Intravenous midazolam Oral lorazepam Rectal diazepam

Correct Answer ( B ) Explanation: In an actively seizing patient, attention is always directed to the airway first. A patient having a generalized tonic-clonic seizure has a suppressed gag reflex and is prone to aspiration of gastric contents. Therefore, patients should be placed in the left lateral decubitus position. First-line pharmacologic management for an actively seizing patient is a parenteral benzodiazepine. Benzodiazepines directly enhance GABA-mediated neuronal inhibition, affect clinical and electrical manifestations of seizures, and are highly effective at terminating seizure activity. Benzodiazepines have been shown to be more effective than phenytoin at terminating status epilepticus. They are as effective as parenteral phenobarbital, but phenobarbital is associated with a higher risk of hypoventilation and hypotension. The intravenous route is the preferred route to administer an antiepileptic because this has the quickest onset of action. Therefore, intravenous midazolam is the preferred agent from the choices listed above. Midazolam's onset of action is within 1 minute. In addition to the intravenous preparation, it is available in both intranasal and buccal formulations. It also has the least cardiovascular effects among benzodiazepines. Lorazepam (C) is considered a first-line agent for an actively seizing patient and is the most popular agent used. However, oral medications are contraindicated in an actively seizing patient due to the prolonged onset of action and risk of aspiration. Diazepam (D) is also a first-line agent for an actively seizing patient and is most commonly used as a rectal preparation in the pediatric population where intravenous access is not readily available.

A 18-year-old man presents with a depressed mental status. Which of the following could be empirically given? Activated charcoal Dextrose and thiamine Flumazenil Syrup of ipecac

Correct Answer ( B ) Explanation: In the undifferentiated patient with depressed mental status, empiric delivery of dextrose and thiamine should be considered. Hypoglycemia can manifest with varied presentations. Patients may exhibit lethargy, somnolence, agitation, confusion, altered mental status, seizures, focal neurologic deficits or coma. In all patients with neurologic changes, a rapid finger stick should be obtained. However, in the absence of a finger stick, it is reasonable to empirically treat with dextrose as hypoglycemia is life threatening. 1-2 grams of dextrose/kg should be given intravenously. In patients in whom intravenous access cannot be established, glucagon can be given intramuscularly. This will increase circulating glucose levels by mobilizing glycogen stores. However, it will not work in those patients with limited glycogen stores (chronically ill, malnourished etc.). Thiamine should be given with dextrose to prevent the development of Wernicke's encephalopathy although the traditional "thiamine before dextrose" has little basis in the literature.

A 55-year-old woman with a history of hypertension and diabetes mellitus presents to the emergency department after becoming incoherent during a work meeting 45 minutes prior to arrival. On examination, a right facial droop and right upper extremity weakness is appreciated. A non-contrast head computed tomography scan is normal. Electrocardiogram reveals normal sinus rhythm. Which of the following the next appropriate step in management? Intravenous heparin bolus and infusion Intravenous recombinant tissue-type plasminogen activator bolus and infusion Magnetic resonance imaging of the brain Watchful waiting

Correct Answer ( B ) Explanation: Intravenous recombinant tissue-type plasminogen activator bolus and infusion is the next appropriate step in management. Recombinant tissue-type plasminogen activator bolus and infusion, or rtPA, is a fibrinolytic agent that converts plasminogen to plasmin in the presence of fibrin to initiate fibrinolysis at the site of thrombus formation, which improves blood flow to areas of the brain that are ischemic, but not yet infarcted, called the ischemic penumbra. A small number of patients with acute ischemic stroke will be eligible for reperfusion therapy, and an even smaller number will actually receive it. Recombinant tissue-type plasminogen activator has been shown to improve functional outcomes from ischemic stroke and that the benefits outweigh the risks for patients who receive treatment within 4.5 hours of symptom onset. The benefit of intravenous thrombolysis decreases continuously over time from symptom onset. Therefore, treatment must be given as soon as possible, rather than near the end of the time window. The most important factor in successful thrombolytic treatment of acute ischemic stroke is early treatment. Nonetheless, selection of appropriate candidates for thrombolysis demands a neurologic evaluation, a neuroimaging study, and determination of eligibility based upon inclusion and exclusion criteria.

A 63-year-old man presents to your office accompanied by his wife. She reports a cognitive decline over the last several months. Physical exam shows a shuffling gait and a reduced arm swing. He has no delusions, but is reacting to visual hallucinations. His affect is flat. He has deficits in short-term and long-term memory and confabulates when answering questions. A previous medical provider gave the patient an antipsychotic medication which caused catatonia. Which of the following is the most likely diagnosis? Frontotemporal dementia Lewy body dementia Normal pressure hydrocephalus Parkinson's disease

Correct Answer ( B ) Explanation: Lewy body dementia (LBD) is a dementia similar to Alzheimer's and is characterized by hallucinations, parkinsonian features, and extrapyramidal signs, as well as the cognitive dysfunction common in all dementias. Patients with LBD lose cholinergic neurons which cause the loss in cognitive functioning and a loss in dopaminergic neurons causes the loss in motor control. Lewy bodies occur in the brainstem, midbrain, olfactory bulb, and neocortex. Patients with LBD are very sensitive to neuroleptic and antiemetic medications that affect dopaminergic and cholinergic systems. They respond with catatonia, loss of cognitive function, or develop life-threatening muscle rigidity. Parkinsonism, and motor neuron disease. Normal pressure hydrocephalus (C) presents with abnormal gait, dementia, and urinary incontinence. Parkinson's disease (D) presents with bradykinesia, resting tremor, postural instability, autonomic dysfunction, and behavioral changes.

Which of the following statements is true regarding the local anesthetic lidocaine? It is a member of the class of ester anesthetics Symptoms of overdose include seizures and cardiac dysrhythmia The maximum dose is 3-5 mg/kg when prepared with epinephrine The mechanism of action is via calcium channel blockade

Correct Answer ( B ) Explanation: Lidocaine overdose results in CNS and cardiovascular problems and are secondary to sodium channel blockade. CNS symptoms include dizziness, headache, tingling, tinnitus, sedation, tremors and seizures. Cardiovascular effects include bradycardia, heart block and dysrhythmias. Lidocaine is an amide (A) anesthetic, not an ester. Other local anesthetics in the amide group include bupivacaine, prilocaine, mepicacaine, and etidocaine. Ester anesthetics include procaine, benzocaine, tetracaine, and cocaine. The mechanism of action of lidocaine is via sodium channel blockade, not calcium channel blockade (D). As a result, it provides local anesthesia by blocking nerve conduction. The maximum dose of Lidocaine is 3-5 mg/kg without epinephrine and 5-7 mg/kg with epinephrine (C).

A 65-year-old man presents to the ED complaining of persistent dizziness. He complains of nausea and feels like the room is spinning. He has a history of hypertension. Vital signs are within normal limits. Which of the following physical exam findings supports a central cause of his dizziness? Hearing loss Limb ataxia Patient remains steady when eyes are open and closed when performing the Romberg test Spontaneous nystagmus that is suppressed by visual fixation Sudden onset of intense, intermittent episodes of disequilibrium

Correct Answer ( B ) Explanation: Vertigo is the perception or sensation of movement. This may be described by the patient as a feeling of swaying, spinning, whirling, leaning, or tilting. The patient may also report feeling intoxicated. Nausea and vomiting may also be seen. Vertigo results from dysfunction in the vestibular system from either its peripheral or central components. Physical examination will reveal nystagmus in nearly all patients, often lateralizing to the affected side. Although less common than peripheral etiologies, central causes of vertigo are more concerning and include conditions such as vertebrobasilar insufficiency, brainstem and cerebellar infarct or hemorrhage, basilar artery migraine, and degenerative diseases such as multiple sclerosis. In general, when compared to peripheral vertigo, the symptoms of central vertigo are less acute and more persistent and may be associated with neurologic deficits. However, exceptions exist, particularly in those patients with cardiovascular risk factors. Limb ataxia is not seen with peripheral causes and is usually attributable to cerebellar lesions. A noncontrast head CT is a reasonable screening test with suspected central vertigo (and is most appropriate to evaluate for potential intracranial hemorrhage). Secondary to the fact that it does not permit adequate visualization of the cerebellum, it is not considered the definitive test. Magnetic resonance imaging with angiography is the study of choice in most cases of suspected central causes of vertigo.

A 32-year-old pregnant woman complains of burning pain on her right thigh. Neurological examination reveals hyperalgesic light touch sensation in the skin of the right, proximal lateral thigh. Further examination reveals normal right leg strength, normal distal leg sensation and a 2+ ankle reflex. Which of the following neuropathies is the most likely diagnosis? Femoral neuropathy Meralgia paresthetica S1 radiculopathy Tarsal tunnel syndrome

Correct Answer ( B ) Explanation: Meralgia paresthetica is the clinical syndrome of pain or dysesthesias, or both in the anterolateral, proximal thigh, due to a compressive neuropathy of the lateral femoral cutaneous nerve. Entrapment of this nerve under the inguinal ligament is common. Mean age of onset is 50-years-old, occurring equally in men and women. It is more common in patients with diabetes mellitus, obesity (higher Body Mass Index) and pregnancy, or those who have inguinal scar tissue or wear tight belts around the waist. Diagnosis is based on the above pain description, sensory abnormalities in the nerve's distribution and absence of abnormal lower leg neurologic findings. Electrodiagnostic findings are variable, as it is difficult to perform nerve conduction studies in obese individuals. Relief of pain with local injection of anesthetic confirms the diagnosis. Spontaneous remission is common, and 90% of patients respond to conservative treatment, which includes: education on weight loss and avoiding tight belts/garments and local steroid injection. Surgery is rarely necessary.

A 22-year-old woman is complaining of recurring headaches that have been worsening over the past several months. They are usually on the right side of her head causing a throbbing pain and she has noticed that they tend to occur more frequently around the time of her menses. She is very concerned because recently she has been seeing "spots" and feels a tingling in her left arm around the time of the headaches. She takes 400 mg of ibuprofen and goes to sleep in order to obtain relief. What is the most likely type of headache? Cluster Migraine Tension Thunderclap

Correct Answer ( B ) Explanation: Migraine headache is a common disorder affecting over 10% of the population. It is more common in women than in men and has several characteristics that can aid in its diagnosis. Migraine is most often unilateral, recurring, throbbing, and associated with certain recognizable triggers such as hormonal fluctuations during menses (there are many other possible environmental triggers). Non-steroidal anti-inflammatory medication and sleep will often relieve the symptoms. Although the presence of a visual, auditory, or sensory aura will help in the diagnosis, most patients do not experience an aura related to their migraine.

44-year-old woman presents with a bilateral throbbing headache and nausea that was preceded by vision changes of flashing lights and blind spots. Her past medical history is significant for poorly controlled hypertension. Currently, her blood pressure is 182/100 and neurological examination is normal. CT scanning reveals no intracranial pathology. Which of the following is the most appropriate headache treatment at this time? High flow oxygen Ibuprofen Sumatriptan alone Sumatriptan plus ergotamine

Correct Answer ( B ) Explanation: Migraine headaches are characterized by 4-72 hours of unilateral or bilateral head pain which is associated with neurologic, autonomic and psychological symptoms. Three subtypes exist. Migraine with aura only accounts for 20% of migraines. This type of headache is preceded by 30 minutes to 1 hour of prodrome symptoms: weakness, paresthesias and several visual changes including scotoma (blind spots), hemianopia, fortification (zizag patterns) and scintilla (flashing lights). Migraine without aura is headache without preceding prodrome symptoms. Finally, chronic migraine (or episodic migraine without aura) is a progressive form characterized by increasing symptom frequency and comorbid psychiatric conditions such as depression, anxiety, bipolar disorder, panic disorder and obsessive-compulsive disorder. The pathophysiological basis of migraine is brain "hypersensitivity" caused by trigeminal nerve and upper cervical nerve excitation of perivascular inflammation. Up to 90% of migraine sufferers have a positive family history. The majority of patients are adult females. Associated nonprodromal symptoms include nausea and vomiting, sensitivity to light (photophobia; prefer to be left alone in a dark room), sensitivity to sound (phonophobia), blurred vision and polyuria. Abortive treatment consists of acetaminophen-aspirin-caffeine, NSAIDs (ibuprofen), antiemetics, ergotamine and triptan medications. Preventive treatment of migraine consists of tricyclic antidepressants, beta-blockers, anticonvulsants, ergot-derivatives, tizanidine, occipital nerve blocks, botulinum toxin injection and calcium channel blockers. Triptan medications (C and D) have adrenergic-agonistic properties. Therefore, they should not be used in patients with cardiovascular or cerebrovascular disease, angina, severe hypertension and peripheral vascular disease. Clinicians must never combine ergot-derivatives with triptan medications.

A 16-year-old boy presents to the office with 10 headaches over the last 3 months. These headaches last 8-10 hours, are unilateral, pulsatile, get worse with physical activity, and often accompanied by nausea with emesis. The headaches have caused him to miss school. Ibuprofen has minimal relief. Which of the following is the the most likely diagnosis? Cluster headache Migraine headache Rebound headache Tension headache

Correct Answer ( B ) Explanation: Migraine headaches are divided into two categories: those without aura (formerly known as common migraine), and those with aura (also known as classic migraine). Migraines typically present among patients 10-30 years of age. Migraine without aura is described as a recurrent headache lasting 4-72 hours. Diagnosis of migraine without aura requires two of the following characteristics: unilateral distribution, pulsatile quality, severity limited daily activities, and exacerbation by physical activity. One of the following characteristics must also be present: nausea or vomiting, photosensitivity, and sensitivity to noise or smell. Migraine with aura presents with symptoms similar to those of migraine without aura, but the headache is preceded by an aura. An aura is a reversible symptom indicative of focal cerebral dysfunction. Examples of auras include: gradual onset and spread of scotomas, scintillations, hemianoptic field defects, unilateral paresthesias or numbness, unilateral weakness, and speech disturbances. The patient in the clinical scenario meets criteria for migraine without aura.

A 23-year-old woman complains of a headache for 24 hours. She has no history of headaches, but they run in her family. Which of the following headaches is most likely to be a migraine? A fluctuating headache with seizure activity A pulsatile unilateral headache with nausea A sudden onset headache that is worse at onset An orbital headache with lacrimation

Correct Answer ( B ) Explanation: Migraine headaches can be remembered by the mnemonic POUND: Pulsatile, 4-72 hOurs, Unilateral, Nausea or vomiting, Disabling. Presence of at least four of these criterion has a likelihood ratio of 24 for a migrainous etiology of headache. Migraines are thought to be vascular in origin. They are treated with a variety of medications, including dihydroergotamine (DHE), triptans, metoclopramide, chlorpromazine, and ketorolac. Placement of the patient in a dark, quiet room and administration of IV fluids can also be helpful. Migraines can present with neurologic symptoms such as visual disturbances (scotoma), paresthesias, and auras, which can make the diagnosis difficult. In those patients without a history of headaches, more serious and life-threatening causes must be ruled out before a diagnosis of migraine is firmly established. A fluctuating headache with seizure activity (A) should prompt the clinician to consider central venous thrombosis (CVT), a venous occlusion from hypercoagulable states. CVT presents with headache, vomiting, seizures, and fluctuating neurologic symptoms. It is diagnosed by MRV. Cluster headaches (D) present with unilateral, severe, orbital, supraorbital, or temporal pain lasting 15-180 minutes. Patients often have ipsilateral red conjunctiva, lacrimation, nasal congestion, rhinorrhea, miosis, and/or ptosis. The patient is usually pacing and restless. High-flow oxygen is effective in up to 70% of patients. A sudden onset headache that is worst at onset (C) is concerning for subarachnoid hemorrhage (SAH), which is typically maximal at onset, sudden, and different from prior headaches. SAH is an emergent condition because of the many complications, including rebleed, infarction, and intracranial hypertension

A 35-year-old woman makes an appointment to establish primary care. Three months ago she was diagnosed with myasthenia gravis which is now only mildly symptomatic and is properly treated by a neurologist. Which of the following would you most expect to find during her intake physical examination? Hyporeflexia Ptosis Sensory abnormalities Weak hypothenar muscles

Correct Answer ( B ) Explanation: Myasthenia gravis (MG) is a disease of neuromuscular transmission characterized by fluctuating weakness and fatigability of certain muscles, namely those of the eyes, face and other cranial nerves. Pathological findings include a widened synaptic cleft, decreased surface area of the postsynaptic button, decreased number of postsynaptic acetylcholine receptors and the presence of anti-acetylcholine receptor antibodies. Symptoms typical of MG are drooping eyelids (ptosis), diplopia, strabismus, dysphonia, dysarthria and dysphagia. Electrodiagnostics are primarily used in confirming a diagnosis. Treatment includes anticholinesterase drugs (-stigmine), thymectomy, corticosteroids, plasmapheresis and intravenous immunoglobulin. Hyporeflexia (A), or areflexia, is not characteristic of MG. MG is a disorder of the motor, not sensory, nerves. Sensory exam (C) is intact in those with MG. Severe, advanced cases of MG may show muscle weakness, however, it is usually proximal, not distal. The hypothenar muscles (D) would not likely be affected in MG. Very few cases display distal weakness.

What is the most common cause of nontraumatic intracerebral hemorrhage in adults? Cerebral amyloid angiopathy Hypertensive vasculopathy Malignant hypertension Vascular malformations

Correct Answer ( B ) Explanation: Nontraumatic intracerebral hemorrhages (ICH), while significantly less common than ischemic strokes, have an in-hospital mortality rate of up to 60%. Hypertensive vasculopathy, the result of long-standing hypertension, is the leading cause of intracerebral hemorrhage. The result of degenerative changes and eventual rupture of small penetrating arteries that branch off major intracerebral arteries, hypertensive intracranial hemorrhage occurs most frequently in deep cerebral locations such as in the putamen. Hemorrhage in this area can result in hemiplegia, hemisensory loss, homonymous hemianopsia, gaze palsy and coma. Cerebral amyloid angiopathy (A) is the leading cause of lobar hemorrhage in elderly patients. It is characterized by deposition of amyloid beta peptide deposits in small and medium sized cerebral blood vessels. Malignant hypertension (C) can also result in intracerebral hemorrhage, however it a less common cause than vasculopathy secondary to long-standing hypertension. Other causes of ICH in adults include vascular malformations, drug intoxication, especially sympathomimetics such as cocaine, blood dyscrasias and hemorrhagic transformation of ischemic strokes. Vascular malformations (D), such as arteriovenous malformations and aneurysms, are the most common cause of intracerebral hemorrhage in children.

A 82-year-old woman with a history of diabetes mellitus, coronary artery disease, and congestive heart failure is hospitalized for sepsis secondary to a urinary tract infection. She complains of disequilibrium and tinnitus which has developed over the past few days. In addition, she cannot hear high frequency sounds well. Which of the following of her medications is the most likely culprit? Aspirin Gentamicin Hydrochlorothiazide Metformin

Correct Answer ( B ) Explanation: Numerous medications can cause injury to the inner ear, known as ototoxicity. By affecting the cochlea, semicircular canals, and otoliths, ototoxic medications result in clinical symptoms of high frequency hearing loss, tinnitus, disequilibrium, and vertigo. Over 100 classes of medications have been implicated as ototoxic. Aminoglycoside antibiotics are the most commonly implicated class of medications. Kanamycin, amikacin, and neomycin preferentially injure the cochlea and affect hearing, while streptomycin and tobramycin affect the vestibular system, causing disequilibrium and vertigo. Gentamicin can affect both the cochlea and vestibular systems. The exact mechanism of aminoglycoside-induced ototoxicity is unknown, and may be multifactorial. Proposed mechanisms include free radical-induced damage to the inner ear, direct toxic effects on cellular membrane potentials, and loss of sensory hair cells in the cochlea and vestibule. Risk factors for aminoglycoside-induced ototoxicity include higher doses, elevated blood levels, and longer durations of therapy. Ototoxicity is more likely in elderly patients and those with underlying renal insufficiency. Other medications associated with ototoxicity include macrolide antibiotics, vancomycin, loop diuretics, antineoplastic agents, salicylates, and quinine. If ototoxicity is suspected, the offending agent should be stopped. Prognosis for recovery of function depends on the ototoxic agent.

Olanzapine is a commonly used medication in patients with schizophrenia. Which of the following statements is true regarding the rapid dispersing formulation (wafer) of olanzapine? At overdose levels it will cause bradycardia It is considered to be bioequivalent to olanzapine tablet Rarely associated with orthostatic hypotension The risk of extrapyramidal side effects is higher than with haloperidol

Correct Answer ( B ) Explanation: Olanzapine is an atypical antipsychotic used in the treatment of schizophrenia, bipolar disorder, and acute agitation. It affects multiple receptors (serotonin, dopamine, muscarinic, adrenergic, histamine), but has the greatest affinity as a blocker of the serotonin (5HT2A) receptor and dopamine (D2) receptor. It is available in oral, rapid dispersible (wafer), and intramuscular preparations. In the ED, the rapid dispersible preparation is particularly valuable to manage acutely agitated patients due to its rapid absorption through the oral mucosa and resulting in rapid somnolence. Both oral and rapid dispersible (wafer) preparations are considered bioequivalent.

An elderly man presents with his wife to establish medical care. She is concerned about his multiple falling episodes. He slowly walks into the office in a stooped posture, taking short steps and holding onto the wall. You discover resting tremor and upper extremity rigidity on examination. He doesn't smile. There are no involuntary facial, mouth, wrist or shoulder movements present. Which of the following is the most likely diagnosis? Huntington's disease Parkinson's disease Paroxysmal kinesigenic choreathetosis Tardive dyskinesia

Correct Answer ( B ) Explanation: Parkinson's disease is a syndrome characterized by resting tremor, bradykinesia, muscular rigidity, and loss of postural reflexes. It is most commonly seen in individuals older than 60 years of age. It results from the depletion of dopamine in the substantia nigra, leading to unopposed cholinergic activity. Patients may present with resting tremor or "pill rolling" that is less severe during voluntary movement. Bradykinesia commonly manifests as decreased arm swing while walking. Rigidity is evident during passive movement of limbs (cogwheeling). A shuffling gait and impaired postural reflexes are seen. Masklike facial expressions are seen with decreased frequency of eye blinking. Seborrhea of the scalp and face is common. The first line treatment is levodopa and carbidopa, the latter of which inhibits peripheral conversion of dopamine. Selegiline (MAO inhibitor) may be used as an adjunct to levodopa by inhibiting the degradation of levodopa and is sometimes used to improve declining response to levodopa. Paroxysmal kinesigenic choreathetosis (C) is a rare hyperkinetic movement disorder characterized by involuntary movements which are triggered by sudden voluntary movements. Ballism, chorea and dystonia are common. It occurs before age 20 years, more commonly in females.

Which of the following medications is considered first line therapy for absence seizure? Carbamazepine Diazepam Ethosuximide Phenytoin

Correct Answer ( C ) Explanation: Absence seizures are sometimes referred to as petit mal seizures. The hallmark of absence seizures is an abrupt and sudden-onset impairment of consciousness, interruption of ongoing activities, a blank stare, possibly a brief upward rotation of the eyes. If the patient is speaking, speech is slowed or interrupted; if walking, he or she stands transfixed; if eating, the food will stop on its way to the mouth. Usually, the patient will be unresponsive when addressed. In some cases, attacks are aborted when the patient is called. The attack lasts from a few seconds to half a minute, and evaporates as rapidly as it commenced. Absence seizures generally are not followed by a post-ictal state. This is in contrast to the majority of seizure disorders. Electroencephalography (EEG) shows generalized spike-and-slow wave discharges. The traditional treatment of absence seizures is with ethosuximide. An alternative agent is valproic acid.

A 14-year-old girl presents for evaluation. Two weeks ago, she collided with an opponent while playing soccer. She was assessed on the field and complained of a headache and dizziness. She was instructed to sit out for the remainder of the game. She continues to complain of headache, dizziness, and difficulty concentrating. Which of the following is the most likely diagnosis? Concussion Post-concussion syndrome Second impact syndrome Tension headache

Correct Answer ( B ) Explanation: Post-concussion syndrome is the sequelae of a mild traumatic brain injury or concussion that is defined by the continuation of symptoms of headache, dizziness, and cognitive impairment. A mild traumatic brain injury is a mild injury to the brain caused by a blunt force or shaking of the brain inside of the skull. It is defined as a Glasgow Coma Scale of 13-15 and generally patients do not lose consciousness. A concussion is a subset of a traumatic brain injury and can also be defined as mild, moderate, or severe. Symptoms of a concussion include headache, dizziness, nausea, vomiting, blurred vision, amnesia, disorientation, and loss of consciousness. Post-concussion syndrome is the continuation of these mild traumatic brain injury symptoms. While the specific timeline that separates a concussion from post-concussion syndrome is still not well established, symptoms that continue seven to 10 days after the initial injury are considered to be caused by post-concussion syndrome. Patients typically begin to feel relief within one month and treatment is aimed toward symptom relief and education.

A 21-year-old man presents to the emergency department with headache, fever, and vomiting. He has been febrile and acting lethargic for the past three days. On exam, a petechial rash is noted on his lower extremities and sacral area. Which of the following signs is most likely to be positive in this patient? Kehr sign Kernig sign Murphy sign Obturator sign

Correct Answer ( B ) Explanation: The Kernig sign is most likely to be positive in patients with meningitis. Meningitis is an inflammatory disorder of the meninges. Meningitis can be caused by bacteria, viruses, parasites, or drugs. The most common causes of community acquired bacterial meningitis are Streptococcus pneumoniae, Neisseria meningitidis, and Listeria monocytogenes. Healthcare-associated bacterial meningitis is usually occurs after neurosurgery and is usually caused by Staphylococci and gram-negative bacilli. Some of the most common risk factors for meningitis include age extremes (< 5 years or > 60 years), diabetes mellitus, renal insufficiency, immunosuppression, human immunodeficiency virus (HIV) infection, crowded housing (e.g. military recruits, college dorms), intravenous drug use, and alcoholism. Patients with bacterial meningitis typically present with fever, headache, and altered mental status. Nuchal rigidity is present in the majority of patients. Nuchal rigidity can be assessed by actively or passively flexing the neck, or with Brudzinski sign or Kernig sign. A positive Brudzinski sign occurs when the patient flexes his or her hips and knees in response to neck flexion. Kernig sign is positive when the knee cannot be fully extended when the patient is lying supine with the hip flexed. Many patients with meningitis caused by N. meningitidis will have characteristic skin manifestations like petechiae and purpura. Laboratory studies should include complete blood count, blood cultures, and lumbar puncture. A computed tomography (CT) scan may be required prior to lumbar puncture to assess for increased intracranial pressure. The typical cerebrospinal fluid findings, in patients with bacterial meningitis, are increased white blood cell count and decreased glucose. Antibiotics should be initiated as quickly as possible after the lumbar puncture is performed. Intravenous antibiotics should be directed against the most likely bacteria based on the patient's age, underlying risk factors, and comorbid diseases. Dexamethasone is a common adjunctive therapy used to diminish rates of permanent neurologic sequelae such as hearing loss, intellectual impairment, and focal deficits. The Kehr sign (A) is the shoulder pain due to irritation of the diaphragm. Kehr sign is referred pain due to common nerve origin of the supraclavicular nerve and the phrenic nerve. Conditions that can cause Kehr sign include ruptured spleen, splenic abscess, and ruptured ectopic pregnancy.

You are seeing a 79-year-old woman who suffers from severe diabetic polyneuropathy. She is taking antihyperglycemic medications to maximize her glycemic control. In an effort to improve her care, you recommend a pain medication. Which of the following medications would be most appropriate to trial? Aspirin Gabapentin Glipizide Oxycodone

Correct Answer ( B ) Explanation: The goal of treating polyneuropathy is two-fold: reducing, removing or managing any underlying condition, and using neuropathic pain medications. Gabapentin substantially reduces the pain associated with polyneuropathy. It is generally well tolerated. A typical starting dose in the elderly population is 100 mg at night, with a gradual increase to twice a day and then three times a day over 1-2 weeks. Slow, further titration may be necessary to optimize pain control, but must be balanced with the possible side effects of sedation and dizziness. Tricyclic antidepressants, such as amitriptyline are also generally considered effective.

A 26-year-old man was involved in an automobile collision and sustained a pelvic fracture. He complains of medial ankle paresthesias which radiate into the arch of his foot. Which of the following nerves is most likely involved? Obturator Saphenous Superficial peroneal Sural

Correct Answer ( B ) Explanation: The largest nerve to emerge from the lumbosacral plexus is the femoral nerve, whose distal branches innervate the anterior thigh muscles and skin. Its most distal branch, the saphenous nerve, is a pure sensory nerve that transmits sensation from the medial lower leg, medial ankle and arch of the foot. The main contribution to the saphenous nerve is from the L4 root. The lumbosacral plexus lies anterior to the sacroiliac joints within the pelvis, and can be damaged post pelvic trauma, especially from an anterior-to-posterior force which results in sacroiliac joint disruption. A proximal femoral nerve injury may result in distal saphenous symptoms. The obturator nerve (A) is another branch of the lumbosacral plexus. It innervates the thigh adductors and only has a small sensory contribution to the medial thigh, superior to the knee. The superficial peroneal nerve (C) sends sensory branches to the skin of the anterolateral lower leg and dorsum of the foot. The sural nerve (D) is also a distal lower extremity sensory nerve, but it supplies sensory innervation to the posterolateral lower leg, lateral ankle and lateral foot.

A 14-year-old treated for sinusitis two weeks ago presents to your ED with worsening headache and fevers over the last week. His mother states that he has been sleeping most of the day and brought him in because he was having trouble walking. He has had no vomiting, vision changes, photophobia, neck pain, or trauma. His vital signs are within normal limits for his age. Your examination shows a pale appearing, somnolent male who wakes and answers questions appropriately. He has a normal cranial nerve exam, negative Kernig's and Brudzinski's signs, but is unable to heel-to-toe walk and has a foot drop on the left. Of the following, what is the next best step to establish the diagnosis? Blood culture Brain CT scan with contrast Electroencephalogram Lumbar puncture

Correct Answer ( B ) Explanation: This patient has a subdural empyema from direct extension of his sinusitis. Diagnosis of a brain abscess is challenging as the presentation is inconsistent. However, it should be considered in all patients with headache, fever, and focal neurological signs. Pain may be sudden or gradual in onset. Only half of these patients have fevers and some present without a headache. Frontal abscesses commonly cause generalized seizures and may be the only presenting sign. Meningeal signs are usually absent (unless an abscess is occipital), and signs of increased intracranial pressure usually take days to develop. The best test for diagnosing a brain abscess is magnetic resonance imaging with gadolinium contrast, however this is not always available in the emergency department. Brain CT with contrast is the next best test to confirm a brain abscess.

A 19-year-old woman presents to the ED with pain and dyschromatopsia in her right eye. She also describes varying degrees of intermittent paresthesias over the previous month and occasional transient gait disturbance. An MRI shows white matter pathology. Which of the following lumbar puncture findings is associated with her condition? IgM and IgG antibodies to B. burgdorferi Pleocytosis and oligoclonal bands of immunoglobulin G Positive VDRL Xanthochromia

Correct Answer ( B ) Explanation: This patient has multiple sclerosis, which is a neurologic disorder that causes variable motor, sensory, visual, and cerebellar dysfunction as a result of multiple focal areas of CNS demyelination. The patient's orbital pain is likely due to optic neuritis. Dyschromatopsia is the change in color perception and may be more prominent than visual disturbance. Although MRI is the gold standard, lumbar puncture can aid in the diagnosis. The lumbar puncture in approximately 50% of the cases will show pleocytosis, which is an increased number of lymphocytes. In 85%-95% of cases, there will be oligoclonal bands of immunoglobulin G.

A 77-year-old man comes to your clinic having difficulty with daily activities. He finds it hard to rise from chairs, and has slowness to start movements. His wife is present and adds that his voice even seems softer. On exam, you note a pill-rolling tremor in his right hand. Which of the following additional findings is consistent with this patient's suspected disease? Chorea Masked face Ptosis Short-term memory impairment

Correct Answer ( B ) Explanation: This patient is exhibiting signs and symptoms of Parkinsonism, a movement disorder characterized by tremor, rigidity, bradykinesia, postural instability, and "masked" or emotionless facial features. Other findings may include a mild decline in intellectual function, a decreased blink rate, a softened voice, impairment of fine motor skills, anosmia, and a shuffling gait. Parkinsonism is relatively common, affecting all ethnic groups and genders equally. The most common etiology of Parkinsonism is idiopathic Parkinson's disease, which usually presents between ages 45-65 with a steady progression of symptoms. This disease is due to a degeneration of the dopaminergic nigrostriatal system responsible for maintaining a balance of dopamine and acetylcholine, neurotransmitters necessary for normal movement. Diagnosis relies on ruling out other similar neurodegenerative conditions. There is no current cure for Parkinson disease or treatment to slow progression, though therapies to improve functionality are available as necessary. Levodopa is commonly used, as this medication is converted to dopamine in the body and can temporarily relieve all symptoms when used in combination with carbidopa to allow for appropriate conversion of the medication. Dopamine agonists such as bromocriptine have similar efficacy and can be given with levodopa or before this medication is initiated. Selective monoamine oxidase inhibitors may also be used with levodopa for patients becoming unresponsive to levodopa alone. Other options include amantadine for relief of mild disease symptoms, anticholinergic medications to control patients' tremor and rigidity, and atypical antipsychotics to manage the confusion and psychosis that may occur with dopaminergic therapies. Physical and speech therapy are usually appropriate adjuncts. Finally, deep brain stimulation remains a surgical option to improve functionality in appropriate candidates.

A 28-year-old woman with myasthenia gravis presents with progressive shortness of breath that started several hours ago. She takes pyridostigmine every 6 hours and has not missed any doses. Yesterday she was prescribed an unknown antibiotic for sinusitis at an urgent care clinic. On exam, her vital signs are within normal limits, and she does not appear in respiratory distress. What is the next immediate step in management? Begin intravenous immunoglobulin (IVIG) Measure negative inspiratory force Obtain a chest X-ray Start corticosteroids

Correct Answer ( B ) Explanation: This patient is experiencing a myasthenic crisis, which is impending respiratory failure with an associated need for mechanical ventilation. Myasthenic crisis may be precipitated by a variety of factors, most often a concurrent infection. It can also follow a surgical intervention, pregnancy, childbirth, or tapering of immunosuppressive medications. In addition, myasthenic crisis can occur spontaneously as part of the natural history of myasthenia gravis (MG) itself. A number of drugs can increase the weakness in myasthenia and should be considered as possible precipitants in this setting. This is of more concern with certain antibiotics (aminoglycosides, erythromycin, and azithromycin); cardiac drugs (beta-blockers, procainamide, and quinidine); and magnesium. An objective measure of respiratory status (i.e., negative inspiratory force [NIF] or forced vital capacity [FVC]) should be trended to evaluate the need for mechanical ventilation.

6-year-old boy presents to your office because his teachers noticed that he frequently tends to stare off during some of his schoolwork and will not respond to their questions. His mother states that she has seen him stare off during dinner but assumed that he was just ignoring her. What is your next step in the evaluation of this child? Order a MRI Order an EEG Reassure the mother and tell her it is likely nothing Send him to the ER for urgent treatment

Correct Answer ( B ) Explanation: This patient is having absence seizures (petit mal) and requires an EEG. Absence seizures are characterized by sudden-onset staring spells, usually lasting approximately 10 seconds, with immediate recovery. Clinically, patients may experience eye fluttering or altered postural tone that may occur many times per day. The diagnosis is confirmed by EEG, which shows characteristic generalized 3 Hz spike-and-wave discharges. Childhood absence epilepsy is an idiopathic generalized epilepsy that has an onset between 4 and 14 years of age with a peak at 5 to 7 years. The prognosis is generally favorable with approximately 80% of children outgrowing their seizures by adolescence.

A 65-year-old man is brought to the ED after a fall. He says he has had trouble walking "for a while." His examination is significant for normal cranial nerve function, normal strength, a resting tremor, and difficulty stopping when he is walking. What is the cellular pathology associated with this condition? Demyelination Lewy bodies Loss of anterior horn cells Neurofibrillary tangles

Correct Answer ( B ) Explanation: This patient is showing signs of Parkinson's disease, a chronic neurologic condition. It is characterized by intracellular cytoplasmic inclusions called Lewy bodies, dopaminergic neuron loss in the substantia nigra, and depigmentation along with gliosis of pigmented areas in the midbrain. The symptoms can be remembered with the mnemonic TRAP: resting Tremor, cogwheel Rigidity, Akinesia, and impairment of Posture or equilibrium. It is treated with drugs to increase central dopamine levels, dopamine receptor agonists (carbidopa and levodopa), and anticholinergic agents (which offset the movement effects induced by diminished dopamine). There is no cure for Parkinson's disease. The most common cause of death is respiratory failure. Demyelination (A) is associated with multiple sclerosis and Guillain-Barré syndrome. Loss of anterior horn cells (C) is associated with amyotrophic lateral sclerosis (ALS), a progressive degenerative of upper and lower motor neuron disease. Neurofibrillary tangles (D) and amyloid plaques are thought to be responsible for Alzheimer's disease, which is a progressive cognitive disorder

A 35-year-old woman presents to clinic three hours after the onset of a recurrent right frontal pulsatile headache that starts roughly one hour after experiencing visual loss. You suspect migraine headache with aura. What are you likely to find on physical examination? Bitemporal hemianopsia Normal visual fields Right homonymous hemianopsia Right temporal field loss

Correct Answer ( B ) Explanation: You are likely to find normal visual fields on physical examination of a patient suspected of migraine headache with aura. A migraine headache with aura is a disorder characterized by findings of an aura with symptoms of reversible changes in vision, hearing, speech, or motor followed by a unilateral pulsatile headache that could last from 4-72 hours. A migraine headache is often triggered by stress, hormones, malnutrition, lack of sleep, and weather changes. The symptoms of aura are typically gradual in onset and last 5-60 minutes. At the end of the aura, symptoms return to normal, but then a severe headache begins. Visual changes that typically occur during an aura begin with a small area of visual loss lateral (or temporal) to the area of visual fixation. This area can expand to affect a quadrant or even a hemifield of the visual field. When the aura resolves, the visual deficit returns to normal, first resolving the central visual field where the initial deficit occurred. Bitemporal hemianopsia (A) is a visual field deficit in which the outer halves of both fields are missing. This is typically caused by an injury or lesion of the optic chiasm where the optic nerves cross and is not a common finding in migraine headache with aura. Right homonymous hemianopsia (C) is the loss of the right half of the visual field in both eyes. This is typically caused by a stroke, brain mass, or trauma to the brain and is less likely to be seen in migraine headache with aura. Right temporal field loss (D) is the loss of vision in the right eye's temporal hemifield. This is a finding of migraine headache with aura, but when the symptoms of aura resolve, the visual field deficit also resolves. If a patient presents hours after the completion of an aura, findings of visual field loss is less likely to be found on physical examination.

A 20-year-old woman presents with numbness in the left arm, left eye pain and blurry vision, and generalized weakness. She states she has had similar episodes in the past. Physical examination reveals a visual acuity of 20/20 in the right eye and 20/100 in the left eye. She also has decreased sensation in the left arm. Which of the following is true about the patient's disease? It is more common in men than women Lumbar puncture will reveal CSF pleocytosis and elevated IgG Lumbar puncture will reveal increased opening pressure Non-contrast head CT is diagnostic

Correct Answer ( B ) Explanation: This patient presents with multiple neurologic symptoms and findings that cannot be accounted for by a single lesion, which is highly suggestive of multiple sclerosis (MS). MS is an inflammatory disease of the central nervous system (CNS). The inflammatory disease involves demyelination of regions within the CNS with sparing of axons. The clinical picture is highly variable and typically involves symptoms occurring in different parts of the body, which are intermittent, recurrent and "separated by time and space." Common symptoms include unilateral vision changes or loss, optic neuritis, sensory loss, weakness, paresthesias, ataxia, bladder dysfunction, diplopia, and depression. Intranuclear ophthalmoplegia accounts for diplopia. Lhermitte sign, an electrical shock type sensation radiating down the back of the neck with flexion, is common as well. A common complaint is worsening of current symptoms with a change in temperature (i.e. fever, exercise or a warm shower). This is referred to as Uhthoff's phenomenon. Analysis of CSF is abnormal in 90% of cases with pleocytosis (50%) and elevated gamma globulin (70%) being the most common findings. MRI of the brain and spinal cord is a sensitive test that will show plaques (demyelination) in 95% of patients.

A 21-year-old woman presents with progressive headaches for one month. She also complains of intermittent blurred vision bilaterally and increased headache with coughing. Fundoscopic examination reveals the above. What abnormality is typically found on lumbar puncture in patients with this presentation? Decreased glucose Elevated opening pressure Elevated red blood cell count Elevated white blood cell count

Correct Answer ( B ) Explanation: This patient presents with signs and symptoms consistent with a diagnosis of idiopathic intracranial hypertension (IIH), which can be diagnosed by an increased opening pressure on lumbar puncture. IIH, previously called pseudotumor cerebri and benign intracranial hypertension is a relatively common disease. It is typically seen in obese women. The exact pathophysiology of the disease is not fully understood but it seems to result from a decreased outflow of cerebrospinal fluid (CSF) and increased brain water content. The headache is typically gradual in onset and generalized. It may be worsened by eye movement or valsalva maneuver (or other maneuvers that increase intracranial pressure). Visual complaints are common and can range from transient blurring of vision to vision loss. The headache may also be accompanied by nausea, vomiting and dizziness. Physical examination reveals papilledema and patients may also have visual field defects. Diagnosis is made by an increased opening pressure on lumbar puncture. Opening pressure will be elevated (above 250 mm H2O).

A 14-year-old boy presents with headache, fever and altered mental status. He was recently seen and evaluated for sinusitis one week ago, but was not given antibiotic treatment at that time. A CT scan of the head is performed. What management is indicated? Ceftriaxone, metronidazole and admit Ceftriaxone, metronidazole and neurosurgery consultation Ceftriaxone, vancomycin and neurosurgery consultation Pyramethamine, sulfadiazine and admit

Correct Answer ( B ) Explanation: This patient presents with signs, symptoms and imaging consistent with an intracranial abscess. Immediate management involves administration of antibiotics covering the most likely pathogens and neurosurgical consultation. CNS abscess is unusual in immunocompetent hosts but can result from direct spread from other infections. The most common causes from direct spread are dental infections, otitis media and sinus infections. Patients with multiple abscesses should raise suspicion for endocarditis. Streptococcus species and anaerobic bacteria are the predominant causative agents. Patients with a history of trauma or intracranial surgery are also at risk for MRSA. CT scan is the diagnostic modality of choice and typically is followed by a lumbar puncture (unless there are signs of increased intracranial pressure), which aids in determining the etiologic agent. Location, size and number of abscesses dictate management. Patients with a single abscess are more readily treated with neurosurgical intervention while those with multiple abscesses are usually treated with antibiotics alone. Initial antibiotics in patients without a history of neurosurgery should be with a third generation cephalosporin and an agent covering anaerobic bacteria like metronidazole.

An 18-year-old man presents with lower extremity weakness for 4 days. He states that he had a bout of diarrhea 2 weeks ago that resolved but then began feeling weak in his legs. Over the last 24 hours, he has begun to feel weak in his arms and has difficulty breathing. Physical examination reveals 3/5 strength in both lower extremities and 4/5 strength in both upper extremities along with decreased deep tendon reflexes. What management is indicated for the suspected diagnosis? Ceftriaxone and vancomycin Intravenous immunoglobulin MRI of the brain Oral steroids

Correct Answer ( B ) Explanation: This patient presents with symptoms and signs concerning for Guillain-Barre syndrome (GBS) and should be treated with intravenous immunoglobulin (IVIG). GBS is a peripheral nerve disorder with an unpredictable and heterogeneous clinical course. The most common form is the acute inflammatory demyelinating polyneuropathy (AIDP) accounting for 90% of cases. Most patients present with symptoms days to weeks after the resolution of a gastrointestinal or upper respiratory illness. Symptoms are worse in the lower extremities than in the upper extremities and typically, weakness in ascending. The descending version is called the Miller Fisher variant. In addition to the progressive, symmetric weakness, patients will have variable sensory findings and decrease in deep tendon reflexes. Notably, anal sphincter tone is spared. Respiratory compromise is common and approximately 33% of patients will require intubation and mechanical ventilation. The mortality and recurrence rate are both 3%. All patients presenting with symptoms concerning for GBS should have vital capacity and negative inspiratory pressure (NIF) measured frequently as a decrease predicts the need for intubation. Lumbar puncture typically shows elevated protein with a mild pleocytosis. Treatment should be started with either IVIG or plasma exchange.

Tonya is a 35-year-old nurse who presented to the emergency department last week for a neurologic event. She had fatigue, generalized dull aching, decreased color vision in her left eye, and right arm and leg weakness that lasted for 48 hours. A magnetic resonance imaging study of the brain showed no evidence of a stroke but revealed scattered T2 lesions. She has never had these symptoms before. What is the treatment for this acute episode? Glatiramer High-dose corticosteroids Interferon beta Low-dose corticosteroids

Correct Answer ( B ) Explanation: This woman presents with the classic signs and symptoms of multiple sclerosis. In most cases, the disease follows a relapsing-remitting pattern, with short-term episodes of neurologic deficits that resolve completely or almost completely. A minority of patients experience steadily progressive neurologic deterioration. Typical symptoms include sensory disturbances, motor weakness, optic neuritis, impaired coordination, and fatigue. Multiple sclerosis is a clinical diagnosis. Two neurologic deficits lasting for at least 24 hours, separated in space and time are considered diagnostic. Magnetic resonance imaging is highly sensitive for revealing sharply delineated regions of demyelination throughout the central nervous system white matter, especially in periventricular areas, and can be used to diagnose multiple sclerosis in cases not meeting the threshold for clinical diagnosis. Cerebrospinal fluid is also obtained for diagnostic purposes. The analysis typically demonstrates oligoclonal bands and an increased immunoglobulin G concentration. Exacerbations affect 85% of patients with multiple sclerosis; infections and stress may play a role. For those with significant, acute symptoms, corticosteroids are the treatment of choice. The initial drug therapy for acute episodes, whether CIS or a subsequent acute episode, is intravenous corticosteroids. Corticosteroids decrease T-cell response to antigens, production of cytokines, secretion of immunoglobulins, and levels of myelin basic protein in cerebrospinal fluid. Treatment with high-dose intravenous methylprednisolone (more than 500 mg/day for at least 3 days) is a typical recommended treatment. Disease-modifying agents should be initiated early in the treatment of multiple sclerosis to forestall disease and preserve function. Symptom management constitutes a large part of care; neurogenic bladder and bowel, sexual dysfunction, pain, spasticity, and fatigue are best treated with a multidisciplinary approach to improve quality of life. Interferon beta (C) or glatiramer (A) should be used as the first disease-modifying therapy, and treatment should be initiated as soon as possible after diagnosis in patients. Patients who start on interferon beta and either cannot tolerate treatment or for whom the treatment is not effective are typically changed to glatiramer and vice versa. Low-dose corticosteroids (D) are not an indicated treatment for patients with multiple sclerosis.

A 44-year-old woman presents complaining of dizziness. She states that the room spins to the right every time she turns her head quickly. These episodes last three to five seconds and resolve on their own. Which of the following additional findings is most likely to be present in this patient? Low frequency hearing loss Reproduction of vertigo with tragal pressure Resolution of symptoms with the Epley maneuver Vertical nystagmus with the Dix-Hallpike maneuver

Correct Answer ( C ) Explanation: Benign paroxysmal positional vertigo (BPPV) occurs when otoliths that reside in the utricle of the inner ear are displaced into the posterior semicircular canal. When the head is turned rapidly, these otoliths move within the canal, and the patient feels the illusion of rotational motion. The otoliths typically settle within three to five seconds, leading to resolution of symptoms. To diagnose BPPV, the Dix-Hallpike maneuver is performed. The patient is placed in a sitting position on a bed or examination table. The patient's head is turned 45º and is laid supine quickly with his or her head hanging off the table. A positive test occurs with the development of horizontal or rotational nystagmus and reproduction of vertigo. This is repeated for both sides, with each repetition of the maneuver having decreased intensity and duration. A positive Dix-Hallpike maneuver indicates pathology in the posterior canal ipsilateral to the direction of the patient's head. To treat BPPV, the Epley maneuver is performed. For example, if the patient has a positive Dix-Hallpike maneuver on the right, his or her head is rotated 45º to the right, and is laid supine quickly with his or her head hanging off the table. This is identical to the Dix-Hallpike test, except this position is held for one to two minutes. The patient's head is then rotated 90º to the left, and is again held for one to two minutes. The patient is then instructed to lie on his or her left side, and the head is rotated to the left 90º. The patient will be looking at the floor at a 45º angle. After being held for one to two more minutes, the patient is moved back to the seated position. When performed correctly, the Epley maneuver has a greater than 90% success rate.

A 36-year-old woman presents to the Emergency Department with progressive lower back pain. She denies history of fever or trauma, but the pain worsened acutely when she bent over to pick up a box earlier today. Which of the following features is most concerning for cauda equina syndrome? Decreased patellar reflex on the right Decreased sensation of the lateral foot Pain radiating down both legs Positive straight leg raise

Correct Answer ( C ) Explanation: Cauda equina syndrome is a neurosurgical emergency that results from sudden compression of multiple lumbar and sacral nerve roots. Often the result of massive central disk herniation, it can also be caused by trauma, malignancy, and epidural abscess or hematoma. Patients present with acute onset of lower back pain with pain, weakness, and numbness affecting multiple levels and both legs. Urinary retention is the most consistent examination finding and has a negative predictive value of 99%. Patients may complain of overflow urinary incontinence. Other findings include saddle anesthesia, decreased rectal tone, and fecal incontinence. Emergent neurosurgical consultation is indicated for operative decompression.

Which of the following medications helps prevent cerebral vasospasm in patients with spontaneous subarachnoid hemorrhage? Lisinopril Metoprolol Nimodipine Verapamil

Correct Answer ( C ) Explanation: Cerebral vasospasm is a delayed complication of spontaneous subarachnoid hemorrhage that may develop between two days and two weeks after the event. Vasospasm may lead to further neurological deterioration which causes significant morbidity related to subarachnoid hemorrhage. Nimodipine, a calcium-channel blocker, has been shown to reduce the occurrence of secondary ischemia with a favorable trend towards reducing case fatality. The mechanism of this improvement is unclear and this benefit has not been demonstrated in other calcium-channel blockers. It is appropriate to administer nimodipine 60 mg po every 4 hours. If the patient is unable to swallow, the nimodipine should be crushed and given via nasogastric tube as there is no evidence for the efficacy of intravenous nimodipine.

Chronic use of which of the following medications would most likely cause gingival hyperplasia? Clonazepam Clozapine Phenytoin Promethazine

Correct Answer ( C ) Explanation: Chronic administration of phenytoin can lead to gingival hyperplasia. Phenytoin is an anticonvulsant medication that is commonly used in the management of epilepsy and status epilepticus. Phenytoin acts by stabilizing neuronal membranes by increasing efflux or decreasing influx of sodium ions across neuronal membranes in the motor cortex, which prolongs the effective refractory period. Phenytoin can be administered orally or intravenously. Intramuscular or subcutaneous administration should be avoided due to the severe risk of local tissue destruction and necrosis. Phenytoin has a black box warning against intravenous infusion faster than 50 mg/min due to increased risk of severe hypotension and cardiac dysrhythmias. Phenytoin is contraindicated in patients with sinus bradycardia, sinoatrial block, second and third degree heart block. Phenytoin should be used with caution in patients with diabetes mellitus, hepatic impairment, porphyria, and hypothyroidism. Common adverse reactions include nausea, vomiting, rash, nystagmus, ataxia, somnolence, gingival hyperplasia, hirsutism, folic acid deficiency, and Peyronie disease. Phenytoin is pregnancy safety class D. Trough concentrations are recommended for routine monitoring, and dosage adjusted based on the patient's serum albumin.

You are evaluating a patient with polyneuropathy. His nerve conduction study reveals a predominantly demyelinating injury process. Which of the following laboratory serum tests would best delineate this patient's diagnosis? Creatinine Hemoglobin A1C Hepatitis B panel Thyroid function studies

Correct Answer ( C ) Explanation: Electrodiagnostic testing is initially used in the evaluation of polyneuropathy, with the goal of defining a predominant injury process, either axonal nerve damage or demyelinating nerve damage. Specific lab tests should be reserved until after this determination, as a certain disease tends to cause a certain nerve injury pattern:

In which of the following ways does an essential tremor differ from the tremor of Parkinson's disease? Essential tremor can be treated with dopamine agonists Essential tremor is a pill rolling tremor Essential tremor is exacerbated by action Essential tremor is unilateral

Correct Answer ( C ) Explanation: Essential tremor is usually symmetric and exacerbated by action, whereas the tremor of Parkinson's disease is usually asymmetric and at rest. Essential tremor is usually symmetric and exacerbated by action, whereas the tremor of Parkinson's disease is usually asymmetric and at rest.

A patient presents to clinic with a complaint of three weeks of bilateral dull frontal head pain. He denies symptoms of photophobia or phonophobia. He has experienced these symptoms infrequently in the past. After being assigned a large company project, his symptoms have increased. What is the most likely diagnosis? Cluster headache Medication overuse headache Migraine headache without aura Tension-type headache

Correct Answer ( D ) Explanation: Tension-type headache is the most common type of headache and is the second-most common disorder in the world. It is characterized by mild to moderate, bilateral, non-pulsating head pain that is often described as "dull", "band-like", or "pressure". There are no associated symptoms with these headaches. The most common precipitating factor is stress, but neck movements, hunger, and odors can also trigger a tension-type headache. Diagnosis is made clinically and treatment includes oral over-the-counter analgesic medications including aspirin, ibuprofen, and acetaminophen

A patient is diagnosed with Campylobacter jejuni enteritis, receives treatment and recovers. Two weeks later, he presents with weakness in his legs followed by weakness in his arms. He denies dyspnea, but reports diplopia and dysphagia. Examination reveals a mild fever, extraoccular muscle palsies, hyporeflexia, distal paresthesias and intact proprioception. Laboratory testing is significant for a normal CBC and a PaO2 of 88 mmHg. Which of the following is the most appropriate intervention at this time? Continuous positive airway pressure (CPAP) Corticosteroids Plasmapheresis Vitamin B12

Correct Answer ( C ) Explanation: Guillain-Barre syndrome (GBS) is a symmetric, progressive ascending muscle weakness that usually starts in the legs and may be acute or subacute. The condition is life-threatening if respiratory or swallowing muscles are involved. GBS can follow minor respiratory or GI illness, inoculation, or surgical procedures. It carries a poorer prognosis when it follows Campylobacter jejuni infection. The clinical hallmark of GBS is lack of deep tendon reflexes. Patients experience weakness of >2 limbs that typically begins with the proximal lower extremities. As weakness progresses, patients may experience shortness of breath, constipation, facial weakness, dysphagia, ophthalmoplegia, dysarthria. And sensory disturbances. Lumbar puncture results will demonstrate increased CSF protein but a normal cell count. Treatment is supportive. Plasmapheresis or IVIG may improve recovery time and decrease residual neurologic effects.

54-year-old woman presents with bilateral lower extremity weakness. She has no achilles or patellar reflexes. Her sensation appears intact. Her strength is 3 out of 5. An MRI of the thoracolumbar spine is normal. Which of the following is the most appropriate next step in making the diagnosis? CT myelogram Edrophonium test Lumbar puncture Pulmonary function tests

Correct Answer ( C ) Explanation: Guillain-Barré syndrome (GBS) is a polyneuropathy characterized by symmetric weakness typically beginning distally and spreading proximally. Sensory abnormalities may also occur although motor weakness predominates. Some patients present days to weeks after an antecedent upper respiratory or gastrointestinal illness. GBS is diagnosed clinically with the assistance of some additional tests including a lumbar puncture. The results of the lumbar puncture demonstrate a markedly elevated protein and mild pleocytosis. New onset myasthenia gravis is diagnosed by both the clinical symptoms of muscle weakness and fatigability in combination with a positive edrophonium test (B). An antibody against the nicotinic acetylcholine receptors at the neuromuscular junction causes myasthenia gravis. Edrophonium is an acetylcholinesterase inhibitor leading to higher levels of acetylcholine at the neuromuscular junction. When administered, weakness (particularly ptosis) improves.

27-year-old woman presents with a complaint of transient vision loss in her right eye. She states that she has had multiple similar episodes in the past six months. She also complains of incomplete bladder emptying, intermittent tremors and intermittent weakness in her left arm. The patient has a family history of multiple sclerosis. Which of the following is the best diagnostic test for the suspected diagnosis? CSF testing for myelin basic protein CT scan of the spine MRI Serum oligoclonal bands

Correct Answer ( C ) Explanation: MRI is the diagnostic modality of choice for multiple sclerosis (MS). Multiple sclerosis is characterized by demyelination of axons within the central nervous system. Patients present with symptoms that are "scattered in time and space" meaning that they have intermittent symptoms in anatomically disparate locations. Patients may have patchy motor and sensory deficits and bladder dysfunction. Spinal cord lesions typically involve the posterior columns, lateral spinothalamic tracts and the corticospinal tract. Spinal MRI is the diagnostic modality of choice because it can be used to rule out causes of cord compression while also show lesions diagnostic for MS.

A previously healthy 18-year-old woman presents to the emergency department with complaints of fever, headache, and neck stiffness. She is accompanied by her sister, who expresses concern because the patient seems suddenly confused and cannot remember what she did yesterday. After you administer empiric intravenous antibiotics, which of the following is the next best step? Chest X-ray Complete blood count with differential Lumbar puncture Urinalysis

Correct Answer ( C ) Explanation: Meningitis is an inflammation of the tissues surrounding the brain and spinal cord (meninges) and may be of infectious (bacterial, viral, or fungal) and various other etiologies. The classic clinical manifestations include nuchal rigidity, fever, and altered mental status. Patients often present with headache as well. All patients with suspected meningitis should have lumbar puncture (LP) to evaluate the cerebrospinal fluid (CSF) unless this procedure is contraindicated. There are no absolute contraindications to LP. Relative contraindications include patients with evidence of increased intracranial pressure, thrombocytopenia, bleeding diathesis, or spinal epidural abscess. Acute bacterial meningitis is a medical emergency and left untreated or treated late is almost universally fatal. Treatment involves addressing systemic complications and initiating empiric antibiotic therapy as soon as possible.

What is the most common cause of an intracranial neoplasm? Astrocytoma Meningioma Metastases Pituitary adenoma

Correct Answer ( C ) Explanation: Metastatic disease (primarily from lung cancer) is the most common cause of an intracranial neoplasm. Other cancers that commonly metastasize to the brain include breast and colon carcinoma. The malignant gliomas (A) (anaplastic astrocytomas and glioblastoma multiforme) are the most common glial tumors. These are located in the cerebral hemispheres. Meningiomas (B) are generally benign, slow-growing tumors that originate in the meninges. Pituitary adenomas (D) are tumors originating from the pituitary gland and are often first noted when the patient exhibits visual impairment from compression of the optic chiasm.

A patient presents to the emergency department with a complaint of recurrent symptoms of vomiting, sensitivity to loud noises, and a severe pulsating headache over the right temporal region that has lasted over the past ten hours. What is the most likely diagnosis? Cluster headache Migraine headache with aura Migraine headache without aura Tension-type headache

Correct Answer ( C ) Explanation: Migraine headache without aura is defined as a unilateral, pulsatile severe headache that lasts between 4-72 hours and is accompanied by nausea, vomiting, photophobia, or phonophobia. The onset of symptoms is typically triggered by emotional stress, menstruation, malnutrition, or sleep deprivation. Migraine headaches is a genetic disorder and is more common in women than men. It can be divided into multiple subtypes of migraine headaches including migraine headaches without aura, migraine headaches with aura, menstrual migraines, and vestibular migraines. An aura is a focal neurological symptom that occurs before the onset of headache. This phase includes the gradual development of positive or negative symptoms such as bright visual lights, tinnitus, rhythmic movements, visual loss, loss of hearing, or the inability to move a body part. This stage typically lasts no longer than one hour before the onset of a headache. If a patient does not experience symptoms of an aura, but does exhibit signs and symptoms of a migraine headache, a diagnosis of migraine headache without aura can be made. Treatment includes administration of non-steroidal anti-inflammatory medications for mild symptoms and oral triptans plus antiemetic medication for moderate to severe attacks.

Which of the following is the most common type of migraine headache? Basilar-type migraine Migraine with aura Migraine without aura Ophthalmoplegic migraine

Correct Answer ( C ) Explanation: Migraine headaches tend to be familial and affect women twice as often as men. The underlying pathophysiology is thought to be vasogenic inflammation. The first headache usually occurs in an individual in the teens or twenties. The headache is often unilateral, pulsating in quality, moderate to severe in intensity, and exacerbated by routine activities. The side of the headache can vary with individual attacks and may be bilateral up to 40% of the time. The onset is usually gradual and the attacks last from 4-72 hours. The migraine without aura is most common (80%) and is usually with associated nausea, vomiting, photophobia, or phonophobia. Pharmacological treatment is divided into abortive therapies (limit the intensity and duration of an episode) and prophylactic therapies (decrease the frequency and intensity of attacks).

A 34-year-old woman presents with pain behind her left eye that is worse with eye movements. She adds that the vision in that eye, which was formerly "perfect," has been getting worse over the past few days. An exam is notable for a visual acuity of 20/80 in her left eye, impaired color vision, and an afferent pupillary defect. Which of the following underlying conditions will this patient most likely have? HIV infection Hyperlipidemia Multiple sclerosis Polymyalgia rheumatic

Correct Answer ( C ) Explanation: Multiple sclerosis is strongly associated with optic neuritis, the most likely diagnosis in this patient. Optic neuritis, a demyelinating disease of the optic nerve, usually presents in female patients and causes unilateral loss vision loss to develop over several days. The degree of impairment can range from mild to no perception of light. Central visual field loss and impaired color vision may also occur. Patients often have pain behind the affected eye that worsens with ocular movements. A relative afferent pupillary defect may be seen in the affected eye and a fundoscopic exam may show swelling of the optic disk and flame-shaped peripapillary hemorrhages. The vision loss in acute optic neuritis usually resolves more rapidly when treated with intravenous methylprednisolone for 3 days, followed by an oral steroid taper. If vision loss and pain persist beyond 2 weeks, a CT or MRI scan of the head and orbits is necessary to rule out a lesion compressing the optic nerve. However, most cases of optic neuritis are related to multiple sclerosis, which is diagnosed in 50% of patients within 15 years of their first episode. Female gender and the presence of white matter lesions on a brain MRI make developing multiple sclerosis more likely. Starting interferon therapy in a patient with optic neuritis who has multiple white matter lesions can prevent development of multiple sclerosis in a percentage of patients.

Which of the following is characteristic of myasthenia gravis? Descending paralysis Improvement of muscle strength with repeated stimulation Positive ice bag test Ptosis worse in the morning

Correct Answer ( C ) Explanation: Myasthenia gravis is caused by the autoimmune destruction of the nicotinic acetylcholine receptors on the postsynaptic membrane. As a result of this autoantibody destruction, over time less total receptors are available. At the remaining receptors, the autoantibodies are competing with acetylcholine for the binding sites. With repeated stimulation, fewer receptor sites are both physically available because of destruction and competition, but also because many remain in the refractory period. As a result, muscles weaken and fatigue, which is the hallmark of the disease. Most commonly the first manifestation of the disease is ocular symptoms including ptosis, diplopia and blurred vision. Over the course of the disease, involvement of the respiratory and bulbar muscles may develop. In order to make the diagnosis, 2 options exist. The first is a bedside edrophonium test although the production of this compound is discontinued. Edrophonium is an acetylcholinesterase blocker and therefore causes an accumulation of acetylcholine in the synapse improving symptoms. Symptoms of myasthenia gravis improve with cooling and so the ice bag test is another alternative diagnostic bed-side maneuver. In both scenarios, the distance between the upper and lower eyelids with ptosis is measured. The ice bag test is based on the physiologic principle of improving neuromuscular transmission at lower muscle temperatures, the eyelid muscles are the most easily cooled by the application of ice. After the application of either the ice bag or administration of edrophonium, the distance between the lids is re-measured to assess for a difference.

An 18-year-old college student presents to the emergency department with headache, fever, and stiff neck for two days. He thought he had a cold and has been taking acetaminophen without relief of his headache. He has no medical history. His parents did not have him vaccinated because of worries about autism. Vital signs are T 38.8.°C, BP 110/50 mm Hg, HR 137/min, and RR 25/min. He is sleepy but arousable. When you flex the patient's head, you note flexion of the patient's lower extremities (hips and knees). Skin exam reveals diffuse petechiae. What is the most likely diagnosis and recommended therapy? Cryptococcus; amphotericin B and flucytosine Herpes simplex virus; acyclovir Neisseria meningitidis; ceftriaxone plus vancomycin Streptococcus pneumoniae; ceftriaxone plus vancomycin West Nile virus; supportive care

Correct Answer ( C ) Explanation: Neisseria meningitidis is a common cause of meningitis in crowded living conditions such as college dorms and military barracks. The classic signs of meningitis are fever, headache, neck stiffness, and mental status change. Petechiae and purpura may also be seen with more advanced cases of meningococcemia. N. meningitidis is an encapsulated bacterium that gains entry through the upper airway and spreads hematogenously to the subarachnoid space. Elements of the capsule trigger an inflammatory cascade, causing the brain and meninges to become edematous, leading to decreased CSF drainage. Disruption of the blood-brain barrier also allows entry of protein and water into the brain, resulting in vasogenic edema. The Brudzinski sign is positive if flexion of the neck results in passive flexion of the hips and knees. It is more sensitive than the Kernig sign. Diagnosis of meningitis is made with lumbar puncture (LP). The LP typically reveals an opening pressure > 30, WBC > 1000 with > 80% PMNs, glucose < 40 mg/dL, protein > 200 mg/dL, and cultures test positive for gram negative bacteria. Early treatment of meningitis is essential. Antibiotics should be administered as quickly as possible, even before lumbar puncture.

An 80-year-old homeless man is found in his riverside tent with confusion and fever in July. On physical examination, the patient is disoriented with T 38.2°C, HR 122 bpm, BP 106/84 mm Hg, and oxygen saturation 99% on room air. You note splenomegaly, flaccid paralysis, and multiple mosquito bites. Which lab abnormality would lead you to suspect West Nile encephalitis in this patient? Decreased CSF glucose level Leukocytosis Lymphopenia Markedly increased serum ALT/AST

Correct Answer ( C ) Explanation: Patients with West Nile encephalitis (WNE) will have leukopenia with a pronounced and prolonged lymphopenia, which can aid in distinguishing it from other causes of encephalitis. WNE is an arthropodborne virus (arbovirus) endemic to the Middle East and now found throughout the United States. It is transmitted by the Aedes mosquito, with birds serving as the intermediate host. Most people with WNE will remain asymptomatic or have a mild viral syndrome. Patients with encephalitis will present with new psychiatric symptoms, cognitive deficits, seizures, flaccid paralysis, and tremors. Most will have a headache and low-grade fever. In patients with meningoencephalitis, a lumbar puncture will show a pleocytosis with mostly lymphocytes, normal to elevated glucose, and increased protein. Decreased CSF glucose (A) is found in patients with bacterial or fungal meningitis. It is generally normal in viral meningitides. A mildly increased ALT/AST (D) may be seen in WNE, Epstein-Barr virus, Rocky Mountain spotted fever, and ehrlichiosis; however, markedly elevated transaminases are not generally part of arboviral infection. Leukocytosis (B) in a patient with WNE suggests an overlying bacterial infection.

You suspect postconcussive syndrome in a previously healthy young woman who was involved in a motor vehicle collision last week. Initial neuroimaging and neurologic examination were normal. Emergency department management was mainly supportive. Currently, she complains of memory and concentration difficulties. Neurologic examination is normal. Which of the following would you recommend at this time? Lumbar puncture Noncontrast MRI Referral to a cognitive therapist Repeat CT scan

Correct Answer ( C ) Explanation: Postconcussive syndrome is a constellation of symptoms which occur after head trauma. The underlying pathology is based on axonal injury of the cerebrum. Psychological abnormalities also play a role in the development of this syndrome. There are several different definitions, however, most agree on the following: minor head injury (GCS 13-15), no CT scan abnormalities, and at least 3 of the following symptoms which begin within several weeks post injury: headache, dizziness, irritability, fatigue, insomnia, memory deficits, concentration deficits, photophobia or phonophobia. Other symptoms include attention deficits, delayed reaction time, and decreased appetite and libido and personality changes. Neurological exam is normal. No treatment has been proven beneficial, but analgesics, antiemetics, psychotherapy, cognitive therapy, vocation counseling and stress management are frequently recommended. Some of the best evidence points toward patient education, reassurance and regular follow-up as being effective.

A 29-year-old woman has a 10-year history of migraine headaches. She had been using ergotamine to abort her headaches, but is now having one or two headaches per week that are interfering with work. Which of the following is the most appropriate preventive therapy? Ketorolac Promethazine Propranolol Sumatriptan

Correct Answer ( C ) Explanation: Preventive treatment may be necessary if migraine headaches occur more frequently than two or three times a month or significant disability is associated with attacks. Propranolol is one beta-blocker that is frequently used as a first-line prophylaxis for migraines. Once a prophylactic drug has been found to improve occurrence of migraines, it should be continued for several months. If a patient remains headache-free, the dose may gradually be tapered down and eventually withdrawn. Other medications shown to be successful in the prophylactic treatment of migraines include antiepileptics (topiramate, valproic acid), calcium channel blockers (verapamil), and antidepressants (amitriptyline). Recently, botulinum toxin type A injections have been approved by the US Food and Drug Administration (FDA) for migraine prevention.

You are caring for a 60-kg patient who has been seizing for 30 minutes. You have already administered 4 mg of IV lorazepam and 1200 mg of phenytoin without termination of seizure activity. Which of the following should most likely be your next step? Administer another bolus of IV phenytoin Administer IV fosphenytoin Administer IV pentobarbital Administer IV sodium bicarbonate

Correct Answer ( C ) Explanation: Seizing patients become apneic and may vomit, compromising their airway and putting them at significant risk for hypoxia and aspiration. Failure to respond to optimal benzodiazepine and phenytoin loading functionally defines refractory status epilepticus. At this point, a third line agent such as pentobarbital (15 mg/kg IV) should be administered. Use caution when adding barbiturates to benzodiazepines because their co-administration may potentiate respiratory depression. Propofol is an alternative to pentobarbital and has the advantages of a short half-life and rapid clearance. These agents are given by IV drip and titrated to a burst-suppression pattern in the EEG tracing. Consider also other causes of benzodiazepine-resistant seizures such as isoniazid toxicity (treated with pyridoxine) and hyponatremia.

A 20-year-old man is seen in the emergency department for sudden onset left-sided severe headache with associated syncope, nausea, and vomiting. He has a history of migraines but states this feels different from his usual headaches. A non-contrast CT scan of his head today is negative for hemorrhage. What is the most appropriate next step in management? Emergent MRI Neurosurgery consult for aneurysm clipping Perform a lumbar puncture Repeat a CT scan with contrast

Correct Answer ( C ) Explanation: Subarachnoid hemorrhage (SAH) classically presents with a sudden severe "thunderclap" headache which patients may describe as "the worst headache of my life." The headache can be lateralized and may be associated with a syncopal episode, nausea, and vomiting. Vitreous (pre-retinal) hemorrhage, called Terson's syndrome, or seizure may also be present and are poor prognostic indicators. Almost half of patients report a sudden and severe headache one to three weeks before the SAH event. These "sentinel headaches" are thought to represent a minor bleed. Change from a patient's typical headache or a thunderclap headache should prompt immediate and emergent evaluation by non-contrast head CT. Non-contrast CT has high sensitivity for SAH (almost 100 percent); however, CT may miss small bleeds or those with atypical symptoms, such as isolated neck pain. Considering the disastrous and likely fatal consequences of missing this diagnosis, lumbar puncture is recommended in patients with a negative initial CT in whom SAH is a concern. Studies have shown lumbar puncture to be 100 percent sensitive in the detection of SAH missed by CT scan.

Which of the following medications should be used in the treatment of cluster headache if high-flow oxygen is ineffective? Aspirin Caffeine Sumatriptan Verapamil

Correct Answer ( C ) Explanation: Sumatriptan is a selective serotonin receptor agonist used as first-line pharmacotherapy to treat migraine headaches with or without aura and cluster headaches in addition to oxygen therapy or when oxygen treatment is ineffective. Cluster headache is characterized by severe orbital, supraorbital, or temporal head pain accompanied by unilateral sweating, conjunctival injection, lacrimation, miosis, ptosis, rhinorrhea, agitation, or restlessness. It most commonly affects men and symptoms may be short-lived, but can attack up to several times a day. The mainstay of treatment is the deliverance of oxygen via a nonrebreathing facial mask. In addition to this, using pharmacotherapy such as a triptan, particularly sumatriptan, has been shown to increase the rate of recovery. Sumatriptan can be used orally, intranasally, or through an injection. In the case of a cluster headache, a subcutaneous injection has been found to be the most effective. Verapamil (D) is a calcium channel blocker that can be used as preventative treatment for a cluster headache. This medication has been shown to reduce attack frequency if taken daily, but has not been proven to be used as first-line treatment during an acute attack.

Which of the following can be used to distinguish a seizure from a syncopal episode? Loss of consciousness Loss of urinary continence Post-ictal period Tongue biting

Correct Answer ( C ) Explanation: Syncope and seizure are often confused and difficult to differentiate. However, patients with syncope should not experience a post-ictal period or prolonged state of confusion. Syncope is defined as a sudden, transient loss of consciousness along with a loss of postural tone. The majority of cases are benign but determining which cases are potentially life-threatening or are harbingers of bad outcomes is difficult. All syncopal episodes result from the same pathophysiology; dysfunction of both cerebral hemispheres or dysfunction of the reticular activating system in the brainstem. After a syncopal episode, patients may have brief (seconds) episodes of confusion but will not experience a true post-ictal period.

32-year-old woman complains of a band-like pressure around her forehead that radiates down to the back of her neck. These headaches occur twice a week on average and last for approximately 1 hour in duration. Her neurological exam is within normal limits and she has no other associated symptoms. What is the best initial abortive treatment? Amitriptyline Caffeine Ibuprofen Promethazine

Correct Answer ( C ) Explanation: Tension-type headaches cause pain that is mild or moderately intense and is described as tightness, pressure, or a dull ache. The pain is usually experienced as a band extending bilaterally back from the forehead across the sides of the head to the occiput. Patients often report that this tension radiates from the occiput to the posterior neck muscles. In its most extensive form, the pain distribution is "cape like," radiating along the medial and lateral trapezius muscles covering the shoulders. Tension-type headaches can last from 30 minutes to several days and can be continuous in severe cases. In addition to its characteristic distribution and intermittent nature, the history obtained from patients with tension-type headache discloses an absence of signs of any serious underlying condition. Patients with tension-type headache do not typically report any visual disturbance, fever, stiff neck or recent trauma. Treatment goals for patients with tension-type headache should include recommending effective over the counter analgesic agents and discovering and ameliorating any circumstances that may be triggering the headaches. Research confirms that NSAIDs, such as Ibuprofen, and acetaminophen are effective in reducing headache symptoms. Patients with chronic tension-type headache should limit their use of analgesics to two times weekly to prevent the development of chronic daily headache. Repeated use of analgesics, especially ones containing caffeine or butalbital, can lead to "rebound" headaches as each dose wears off and patients then take another round of medication. Common features of chronic daily headache associated with frequent analgesic use are early morning awakening with headache, poor appetite, nausea, restlessness, irritability, memory or concentration problems, and depression. If the patient requires analgesic medication more frequently, adjunctive headache medications can be initiated. Smoking cessation is an important issue to address in patients with chronic tension-type headache. The number of cigarettes smoked has been "significantly related" to the headache index score and to the number of days with headache each week.

An 88-year-old woman with osteoporosis sustained a hip fracture after falling. The fracture was surgically treated, and she was discharged to the inpatient rehabilitation unit. Her main complaint now is leg pain and numbness. On examination, you find hamstring and ankle weakness, an absent ankle reflex and numbness on the posterolateral leg. Hip flexion, hip abduction and knee extension strength is intact. Injury to which of the following nerves is most likely causative? Femoral Obturator Sciatic Superior gluteal

Correct Answer ( C ) Explanation: The hip is a very common site for osteoporotic fractures, especially in an elderly woman. Hip dislocation or fracture is a very common cause of compressive sciatic neuropathy. Other causes include prolonged bed rest, piriformis syndrome and pelvic masses. Proximal sciatic neuropathy results in a wide array of symptoms, including: sensory changes in the peroneal, tibial and sural distributions (medial lower leg and arch of foot are likely spared due to saphenous innervation); weakness in most of the leg except hip flexion and extension (femoral and gluteal nerve innervation), hip abduction and adduction (superior gluteal (D) and obturator (B) nerve innervation) and knee extension (femoral nerve (A) innervation); absent ankle, decreased hamstring, and preserved knee reflexes; and local pain which radiates thru the posterior thigh and leg. Electrodiagnostics (electromyography [EMG] and nerve conduction studies [NCS]) are helpful in distinguishing sciatic neuropathy from L5 or S1 radiculopathy.

Which of the following tests for function of the median nerve? Abduction of the index finger against resistance Extension of the wrist against resistance Pincer function of the thumb and index finger Sensation of the dorsum of the first webspace

Correct Answer ( C ) Explanation: The median nerve, which is derived from the medial and lateral cords of the brachial plexus, contains nerve roots from C5-T1. In the forearm, it gives rise to the anterior interosseous nerve, which supplies the deep muscles of the anterior forearm, and the palmar cutaneous nerve which innervates the skin of the lateral aspect of the palm. It then crosses through the carpal tunnel terminating as the recurrent branch, responsible for innervating the thenar muscles, and the palmar digital branch. The palmar digital branch innervates the palmar surface and fingertips of the thumb, index, and middle digits, as well as the lateral two lumbrical muscles. Motor function of the median nerve can be tested by having the patient make an "OK" sign (pincer function of the thumb and index finger) or oppose the thumb and fifth finger. Sensation at the volar tip of the index finger confirms sensory function of the median nerve. The ulnar nerve is responsible for abduction of the index finger against resistance (A). Extension of the wrist against resistance (B) and sensation of the dorsum of the first webspace (D) is a function of the radial nerve.

A 17-year-old girl presents to your office with a complaint of worsening headaches. The headaches occur most days of the week, worsen with activity, and are sometimes associated with vomiting. She denies a family history of headaches. She is taking ibuprofen for her headaches and doxycycline for acne. Exam is significant for papilledema. A brain MRI is normal, and on lumbar tap the opening pressure is elevated. What is the best next step in treatment? Start acetazolamide Start rizatriptan Stop doxycycline Stop ibuprofen

Correct Answer ( C ) Explanation: The patient has idiopathic intracranial hypertension (formally known as pseudotumor cerebri) as evidenced by papilledema, normal imaging and elevated opening CSF pressure. Idiopathic intracranial hypertension should be suspected in any older child or adolescent with headaches and papilledema. Patients typically describe diffuse headaches that may worsen on straining. It is often associated with visual loss or diplopia, transient visual obscurations, pulsatile tinnitus, limited abduction of one or both eyes, and rarely a decreased level of consciousness. The disorder typically affects obese, hirsute-appearing young women. Idiopathic intracranial hypertension has many drug-induced etiologies as well, including tetracyclines, oral contraceptives, sulfonamides, hypervitaminosis A, phenytoin, corticosteroids and nitrofurantoin. It is appropriate to stop doxycycline to determine if that is the underlying cause of her headaches.

A 35-year-old man is administered IV metoclopramide for migraine abortive therapy. One hour later the patient is noted to have the following activity seen in the video above. Which of the following medications should be administered to the patient? Bromocriptine Dantrolene Diphenhydramine Muscarine

Correct Answer ( C ) Explanation: The patient is having an acute dystonic reaction secondary to metoclopramide. Acute dystonic reactions manifest as involuntary spasms that often involve the face, neck, back, or limb muscles. Although dystonic reactions are occasionally dose related, these reactions are more often idiosyncratic and unpredictable. Dystonic reactions are commonly associated with typical antipsychotics (haloperidol, droperidol, chlorpromazine) but can also occur, albeit less frequently, with atypical antipsychotics (clozapine, olanzapine) and other antidopaminergic drugs like metoclopramide. They reportedly arise from a drug-induced alteration of dopaminergic-cholinergic balance in the nigrostriatum (i.e., basal ganglia). Most drugs produce dystonic reactions by nigrostriatal dopamine D2 receptor blockade, which leads to an excess of striatal cholinergic output. High-potency D2 receptor antagonists are most likely to produce an acute dystonic reaction. Acute treatment consists of administration of anticholinergic agents such as diphenhydramine or benztropine. Patients should continue therapy for at least 48 hours to prevent recurrence and be referred back to their prescribing physician for medication adjustment.

Which of the following medications should be used as prophylactic therapy for patients with recurrent migraine headaches? Dihydroergotamine (DHE) Ketorolac Propranolol Sumatriptan

Correct Answer ( C ) Explanation: The pharmacologic treatment of migraine headaches is divided into abortive and prophylactic therapies. Prophylactic therapies are intended to decrease the frequency and intensity of attacks and is indicated for patients who have frequent attacks (>2 episodes per month), prolonged attacks lasting more than 48 hours, or attacks that are severe and debilitating. Beta-adrenergic blocking agents such as propranolol reduce both the frequency and severity of migraine headache and are most widely used for prophylaxis. Other medications used for migraine headache prophylaxis include calcium channel blockers, tricyclic antidepressants, anticonvulsants, and monoamine oxidase inhibitors

A 22-year-old man presents to the ED with sustained spasm of his neck to the right. Which of the following drugs is he most likely to be taking? Benztropine Cocaine Haloperidol Ziprasidone

Correct Answer ( C ) Explanation: This man is having a dystonic reaction, which is a common side effect of haloperidol and other typical antipsychotic drugs. Haloperidol is a high-potency antipsychotic that blocks dopamine-2 receptors at the basal ganglia, which can lead to acute dystonia shortly after drug initiation (50% occur within 48 hours, 90% within five days) and sustained movement disorders (parkinsonism and tardive dyskinesia) with prolonged use. Symptoms of acute dystonia include intermittent, involuntary, and uncoordinated hyperkinetic movements most often affecting the tongue, face, neck, trunk, or extremities. Treatment is with IM or IV benztropine or diphenhydramine, and recovery is rapid after medication administration.

A 32-year-old woman complains of back pain and difficulty urinating for five days. The pain is located in her lumbar spine and is constant and dull. She says she feels the urge to urinate every five minutes, but only a small amount of urine is expressed. On physical exam, her vital signs are T 38.4°C, BP 110/72 mm Hg, HR 115 bpm. Cardiac exam reveals a 2/6 systolic murmur. Lung exam is normal. Abdominal exam reveals a large, tender suprapubic mass. Musculoskeletal exam reveals tenderness over her lumbar spine and track marks consistent with IV drug use in her left antecubital fossa. Neurological exam reveals 4/5 strength in her bilateral lower extremities. What is the most likely cause of her symptoms? Bladder cancer Necrotic uterine leiomyoma Spinal epidural abscess Tubo-ovarian abscess

Correct Answer ( C ) Explanation: This patient has a spinal epidural abscess causing cauda equina syndrome. Urinary retention with or without overflow incontinence is the most common finding of cauda equina syndrome. Spinal epidural abscesses classically present with back pain, fever, and neurologic deficits. In this case, it is urinary retention. Some risk factors for epidural abscess include injection drug abuse, immunocompromised state, recent spinal surgery, and cancer. This patient has a cardiac murmur and track marks on her arm confirming her history of IV drug abuse.

A 45-year-old businessman presents for "shakiness" of his hands for several months. He notices it most when giving a presentation at work, and adds that his voice "quivers" at those times too. No other symptoms are present. He says his father had a similar problem for most of his adult life. Which of the following medications is the best initial selection for this condition? Botulinum toxin A Donepezil Propranolol Rasagiline

Correct Answer ( C ) Explanation: This patient is showing symptoms of benign essential (familial) tremor, for which the best initial treatment option in lifestyle-limiting disease is the beta-blocker propranolol. Essential tremor usually presents with a postural tremor of the hands or head that is often worsened by psychic stress. When laryngeal muscles are involved, the patient's voice may shake as well. Of note, the legs are usually spared in this condition. Though the cause is uncertain, there appears to be an autosomal-dominant inheritance pattern in some cases. Patients may be initially affected at any age. Essential tremor usually becomes more prominent with age, though significant disability is rare. Symptomatic treatment is limited to patients with lifestyle-limiting tremors. In addition to propranolol, other therapies include primidone, alprazolam, topiramate, or gabapentin.

A 73-year-old woman with a history of hypertension presents with a unilateral headache for three weeks. She states that she has a throbbing pain at her right temple and has pain in her jaw with opening and closing. The vision in her right eye has worsened over the previous day. Her blood pressure is 173/100 mm Hg. What treatment is indicated? Carbamazepine Labetalol Methylprednisolone Non-contrast head CT scan

Correct Answer ( C ) Explanation: This patient presents with a unilateral, subacute headache with associated jaw claudication and vision change; symptoms consistent with temporal arteritis. Temporal arteritis or giant cell arteritis is a systemic inflammatory process of small and medium-size arteries. The most commonly involved vessels are the ophthalmic vessels and the extracranial branches of the aortic arch. The disease typically affects patients over 70 years of age and is more common in women than in men. Patients present with a subacute headache that is throbbing in nature and may be present for weeks to months. Often, patients will have symptoms for more than 2 months. Patients may also report jaw claudication secondary to vascular insufficiency of the masseter and temporalis muscles. Physical examination may reveal tenderness over the temporal artery. Systemic symptoms may also be present including fever, joint pains, and weight loss. Diagnostic testing in the Emergency Department generally begins with an erythrocyte sedimentation rate (ESR) with a cutoff of 50 mm/hour although the level may be >100 mm/hour. However, the ESR will be normal in 10-25% of patients. The gold standard diagnostic test is a temporal artery biopsy. In patients with a high-clinical likelihood of temporal arteritis, treatment should be initiated regardless of initial diagnostic testing as delay can lead to permanent visual loss. Prednisone should be started at 60 - 120 mg/day. If vision loss is present, methylprednisolone is the recommended medication.

A 20-year-old woman presents with weakness in her left wrist. She states that she fell asleep in a chair after a night of heavy drinking. On physical examination, she is unable to extend her wrist. What nerve is compromised? Axillary nerve Median nerve Radial nerve Ulnar nerve

Correct Answer ( C ) Explanation: This patient presents with a wrist drop caused by radial neuropathy also called "Saturday Night Palsy." The radial nerve arises from the C5-T1 roots. It controls extension of the fingers, thumb, wrist and elbow. Symptoms of radial neuropathy depend on the location of compression. Compression in the axilla (typically from improper use of crutches) causes weakness of extension at the elbow, wrist and fingers. More typically, the nerve is compressed between the humeral shaft and another hard surface and results in weakness with extension at the wrist and fingers. This typically occurs from deep sleep (often secondary to inebriation). About 90% of radial nerve mononeuropathies that occur during sleep, coma or anesthesia recover within 6-8 weeks. Patients should be placed in a wrist splint with 60 degrees of dorsiflexion to prevent atrophy and contractures. The axillary nerve (A) causes weakness of the shoulder (deltoid) and decreased sensation over the shoulder. Median nerve neuropathy (B) causes numbness in the 1st, 2nd and 3rd digits. Ulnar nerve (D) neuropathy symptoms depend on location but do not cause weakness with extension at the wrist.

An 82-year-old man presents with slurred speech and unilateral arm weakness that has resolved. His non-contrast head CT is negative and he is given aspirin. Which of the following is the most appropriate management plan? Discharge home with PMD follow-up Low molecular weight heparin MRI and MRA of the head and neck Transcranial dopplers

Correct Answer ( C ) Explanation: This patient's symptoms are concerning for a transient ischemic attack (TIA), neurologic deficits that occur and then resolve within 24 hours. Most patients with a TIA will resolve within one hour (the previous definition). Patients who have a TIA are at high risk for stroke in the period immediately following a TIA and therefore further testing is warranted in a timely fashion. In most cases, patients should be admitted to an observation unit or hospital in order to rule out any reversible causes of the TIA. Testing includes vascular imaging to identify any critical stenosis. The MRI will also identify the subset of patients with TIA symptoms who ultimately have an infarct on MRI. If MRA is not available, carotid dopplers are an acceptable alternative. Once the vasculature has been evaluated, patients may be discharged with outpatient follow-up. Routine testing that often occurs during the observation period includes cardiac telemetry for dysrhythmia (particularly atrial fibrillation) and echocardiography to evaluate for valvular lesions or intracardiac thrombus.

32-year-old woman presents to her primary care provider's office because of uncontrollable, jerky movements of her arms and mood changes that have progressed over the past 8 months. Her father died at the age of 55 from dementia and her grandmother died in a "mental institution". During the interview, she displays irregular arm movements and has difficulty concentrating. No focal neurological deficits are noted. Which of the following is the most likely diagnosis? Amyotrophic lateral sclerosis Creutzfeldt-Jakob disease Huntington's disease Tardive dyskinesia

Correct Answer ( C ) Explanation: This woman most likely has Huntington's disease. Huntington's disease (HD) is an autosomal dominant disorder that results in progressive neurodegeneration. HD is caused by an unstable and expanded CAG trinucleotide repeat in the huntingtin gene. Patients with longer repeats tend to present with symptoms at an earlier age and have a faster disease progression. The age of onset is typically 30-50 years of age. Chorea is the key feature and defining symptom of HD. Chorea is a rapid, uncontrollable, irregular, jerky movement of the face, trunk, or extremities. Chorea typically progress from mild movements that may appear as fidgeting, to widespread, disruptive flailing. Many patients with HD also experience depressed mood, irritability, apathy, paranoia, delusions, or hallucinations. The majority of patients have progressive cognitive decline, which typically manifests as indecisiveness, inability to multi-task, and decreased word fluency. Diagnosis of HD is based upon family history and genetic testing. Genetic testing is 98.8% sensitive and 100% specific for HD. Brain magnetic resonance imaging (MRI) is often used to rule out other conditions. HD is incurable, and treatment is limited to symptomatic management and improving quality of life. Tetrabenazine is often used to manage chorea. Other medications for controlling chorea include risperidone, haloperidol, and amantadine. Psychotic features are often managed with atypical neuroleptics. Depression is usually managed with selective serotonin reuptake inhibitors or tricyclic antidepressants. Amyotrophic lateral sclerosis (A) is a neurodegenerative disorder characterized by progressive muscle weakness, muscle atrophy, and cognitive decline. Patients with amyotrophic lateral sclerosis (ALS) may present with reduced dexterity, foot or wrist drop, extremity weakness, slurred speech, or difficulty swallowing. Most cases of ALS are sporadic. ALS is usually fatal within 3-5 years. Creutzfeldt-Jakob disease (B) is spongiform encephalopathy caused by prions. Creutzfeldt-Jakob disease (CJD) can be inherited or, more commonly, acquired. CJD can be acquired from eating infected meat. A family history is usually absent in CJD. CJD manifests with rapidly progressive dementia and mental status changes. Tardive dyskinesia (D) is the involuntary movement of the face, tongue, lips, trunk, and extremities. Tardive dyskinesia is caused by long-term dopaminergic antagonist drugs. Tardive dyskinesia is typically seen in patients with schizophrenia and bipolar disorder. Tardive dyskinesia most commonly manifests as persistent chewing movements and recurrent tongue protrusion.

Your patient presents for an evaluation of foot pain and numbness. After taking a history and performing a physical exam, you suspect polyneuropathy. Which of the following is the most appropriate initial diagnostic test? Lumbar puncture Nerve biopsy Nerve conduction study Skin biopsy

Correct Answer ( C ) Explanation: When considering polyneuropathy in a differential diagnosis, first stratify the symptoms as mild, moderate or severe, then assess for common underlying causes, such as diabetes mellitus, alcohol abuse, medication side effects, chronic renal impairment (uremia) and HIV. In those patients with common known underlying conditions and mild symptomatology, extensive initial diagnostic work up may be unnecessary. In those patients with unknown underlying conditions, or those with moderate, severe or progressive symptoms, initial diagnostic work-up begins with electrodiagnostic testing, which includes nerve conduction studies and electromyography. The main goal of electrodiagnostic testing is to determine if a condition is due to a nerve (neuropathy) or muscle (myopathy) disorder. Once neuropathy is considered, electrodiagnostics are used to assess the predominant underlying pathology, namely axonal injury or demyelinating injury. This information is a main differentiator of polyneuropathy.

Which of the following is most characteristic of a patient with idiopathic intracranial hypertension? Abnormal CSF chemistry analysis Opening pressure of 15 cm H2O on lumbar puncture Transient visual loss Unilateral papilledema

Correct Answer ( C ) Explanation:The headache is usually generalized and made worse by maneuvers that impair cerebral venous return (e.g. Valsalva and bending forward). Visual complaints occur commonly and patients may have transient visual disturbances several times a day. After these episodes, periods of visual loss can occur. These episodes likely occur secondary to ischemia of the visual pathways. On physical examination, the patient may have an enlarged blind spot and loss of peripheral vision. Rarely, a sixth nerve palsy occurs. It is most commonly seen in young, obese women of childbearing age. Additional risk factors include the use of oral contraceptives, anabolic steroids, tetracyclines and vitamin A. Headache is the predominant condition of the disease.

A 56-year-old woman presents with burning pain and tingling on the palmar surfaces of digits 1-3 of her right hand. The pain has been present for several months and awakens her at night. There is no atrophy or weakness of her hand. However, volar wrist percussion causes a shock-like pain extending to the palmar surfaces of digits 1-3. Which of the following tests proved positive in this examination? Finkelstein Phalen Spurling Tinel

Correct Answer ( D ) Explanation: A Tinel test is considered positive when percussion of the volar wrist in the region of the median nerve produces tingling or a shock-like pain in the palmar surfaces of digits 1-3. This test is generally sensitive and specific for clinically diagnosing carpal tunnel syndrome, an entrapment neuropathy due to some form of compression or narrowing of the carpal tunnel that impinges the median nerve. Other physical exam findings may include a positive Phalen sign in which flexion of both wrists to 90 degrees for one minute causes pain or tingling in the median nerve distribution. The carpal compression test, in which the clinician applies steady, direct pressure over the carpal tunnel to elicit tingling, may be even more sensitive for the condition. Carpal tunnel syndrome generally presents with pain and tingling in the median nerve distribution and may initially bother the patient only during sleep. A history of performing repetitive activities may be noted. Diabetes mellitus and fluid retention during pregnancy can also contribute to carpal tunnel compression. A work-up for carpal tunnel syndrome may include an ultrasound to observe flattening of the median nerve, or electromyography and nerve conduction studies to differentiate it from a more proximal neuropathy. A trial of wrist extension splinting, as well as NSAIDs and corticosteroids, may help. Surgical carpal tunnel release can be considered for symptoms beyond 12 months of conservative treatment. A Finkelstein test (A) requires sharp, ulnar deviation of the thumb while the fingers are flexed around the thumb into a fist. Pain at the wrist is suggestive of deQuervain tenosynovitis. A Phalen test (B) is positive when flexion of both wrists to 90 degrees for one minute causes pain or tingling in the median nerve distribution. Like A Tinel test, a positive Phalen test suggests carpal tunnel syndrome. A Spurling test (C) requires the patient to rotate and extend the neck to one side while the clinician gently applies an axial load to the neck. Nerve root compression is likely when this test produces cervical radiculopathy.

21-year-old man presents with a headache. What feature should raise the concern for a subarachnoid hemorrhage? Age <40 Fever History of intravenous drug use Sudden onset of headache

Correct Answer ( D ) Explanation: A sudden onset of headache should raise concern for subarachnoid hemorrhage (SAH). SAH is a life-threatening disorder that involves extravasation of blood into the subarachnoid space. It accounts for 10% of all strokes. SAH can either be traumatic or spontaneous. Spontaneous SAH is associated with intracranial aneurysms about 80% of the time. Patients typically present with a sudden onset of severe headache that is classically described as "the worst headache of their life." The headache of SAH is often associated with nausea and vomiting (75%) and may have concomitant neck stiffness (25%) and seizures (17%). Meningismsus is present in half of patients. The workup of a patient with a suspicion of a subarachnoid hemorrhage typically involves a noncontrast head CT followed by a lumbar puncture if imaging is unremarkable. Although the ability of CT to detect SAH in patients presenting early in the course of disease has improved greatly over the last decade, lumbar puncture is still recommended to rule out the disorder.

A 57-year-old woman is brought by ambulance to the emergency room. Three hours ago she began having a headache with nausea and noticed a loss of sensation on most of the right side of her body. A non-contrast computed tomography scan of her head shows a 2 cm area of enhancement in the brain parenchyma. Which of the following is the most likely location of the patient's lesion? Cerebellum Frontal lobe Temporal lobe Thalamus

Correct Answer ( D ) Explanation: A thalamic hemorrhage classically presents with contralateral hemisensory loss. Other symptoms such as hemiparesis and ocular symptoms can also occur when the internal capsule and other nuclei are involved. Symptoms are acute and progress rapidly. Most patients present with headache and often nausea as well. Non-contrast CT scan is the diagnostic test of choice for brain hemorrhage as it can be done quickly and new blood will enhance on the scan (older blood will appear dark). Prognosis is in large part related to the amount of bleeding. Cerebellar (A) hemorrhages most often present with symptoms of increased intracranial pressure due to compression of the fourth ventricle (lethargy, nausea, vomiting). Compression of the brainstem can also occur leading to comatose before seeing hemiparesis. Other lesions of the cerebellum can cause various forms of ataxia. Frontal lobe (B) lesions can cause a contralateral palsy due to the primary and pre-motor cortex in the frontal lobe but the primary sensory cortex is in the parietal lobe. Broca's area is also in the frontal lobe and damage would lead to an expressive aphasia (good understanding but difficulty producing comprehendible speech). A lesion in the dominant temporal lobe (C) could lead to a Wernicke's aphasia (receptive aphasia) where the patient speaks clearly but has trouble understanding.

A previously healthy 48-year-old man presents with fever, headache and signs of meningeal irritation. He has no significant past medical history. He has no rash and has not had any recent medical procedures. Which of the following is the most common bacteria responsible for the suspected diagnosis? Haemophilus influenzae Neisseria meningitidis Staphylococcus aureus Streptococcus pneumoniae

Correct Answer ( D ) Explanation: Adult bacterial meningitis has several microbiologic etiologies. The most common cause is Streptococcus pneumoniae (30-60% of cases) for all age categories. The second most common bacteria is Neisseria meningitidis (10-35%), which more commonly affects older children and young adults, and more commonly occurs is associated with a petechial rash. Haemophilus influenzae is also causative (<5%), and occurs most commonly in those with recent neurosurgical procedures or possible CSF leaks. Listeria monocytogenes (5-10%) and Gram-negative rods (1-10%) are also causative and occur more frequently in elderly patients or those with alcoholism, immunosuppression, cancer and recent medical or neurosurgical procedures. Empiric treatment in healthy adults is ceftriaxone plus vancomycin. Consider ampicillin if the patient is older than 50 years or has an alcohol abuse history in order to cover Listeria monocytogenes.

A 15-year-old boy presents to the ED with an acute onset of altered level of consciousness. There is no history of trauma. He is completely unresponsive to external stimuli, including pain. Which of the following is the most likely cause of coma in this child? Brain malformation Encephalitis Inborn error of metabolism Toxic ingestion

Correct Answer ( D ) Explanation: Consciousness is a state of awareness of one's self and the environment. Coma is a state of no response to any stimuli, including pain. Alterations between these two endpoints deteriorate in a pattern of reduced awareness of self, then environment, then an absence of arousal. Confusion is a loss of clear thinking with impaired decision-making and cognition. Disorientation accompanies or follows confusion, and is characterized as being unaware of time, then place, then memory then self. Delirium follows and is marked by mental and motor excitement, irritability and agitation. Lethargy follows, where the patient is in a state of limited responsiveness, even to moderate stimulation. Stupor is marked by deep sleep or unresponsiveness, however, patients will respond to repeated moderate stimuli. Coma follows. The most common cause of acute onset of altered consciousness in the pediatric population is toxic ingestion.

A 42-year-old man presents to your office with complaints of uncontrollable fidgeting, irritability and difficulty with short-term memory. He tells you that his father had similar symptoms and died at a young age. Which of the following is the most appropriate next step in management? Discussion of end-of-life issues Magnetic resonance imaging Positron emission tomography scan Referral for genetic testing

Correct Answer ( D ) Explanation: Huntington disease (HD) is an autosomal dominant inherited, neurologic disorder characterized by choreiform movements, dementia and psychiatric disturbances. Patients with early symptoms of HD present with mild chorea which may be mistaken for fidgeting. Cognitive decline occurs and the behavioral aspect of the disorder often presents with symptoms of depression, mania, psychosis or changes in personality. Diagnosis is with genetic testing and patients who are suspected to have HD should be referred as soon as possible. Asymptomatic patients with a known family history of HD may also request genetic testing prior to the onset of symptoms. Currently there is no cure and no treatment that will modify the disease process. Treatment focuses on supportive care for the patient and family along with management of symptoms. Prognosis is poor, as HD is a progressive disorder that leads to disability and death.

A young woman reports 3 months of worsening vision, noting blurred and diplopic abnormalities. She also complains of left leg and right arm weakness. Examination reveals extraoccular palsies, poor visual acuity, nystagmus, left leg and right arm strength deficits and a positive Romberg sign. You suspect a central inflammatory demyelinating process. Which of the following imaging and results would help to confirm a diagnosis? ANoncontrast head CT scan with cervical spine central stenosis BNoncontrast head CT scan with ventricular enlargement CNoncontrast T1-weighted brain MRI with corpus callosal hyperintensities DNoncontrast T2-weighted brain MRI with periventricular hyperintensities

Correct Answer ( D ) Explanation: Multiple sclerosis (MS) is a chronic multifocal demyelinating neurologic disorder that involves different parts of the CNS at various points in time. There is a female to male ratio of 2:1. Prevalence increases with further distance from the equator. Clinically patients present with focal limb weakness, numbness, paresthesias, a band like sensation around the trunk or a limb, and ataxia. Diplopia, dysarthria, intention tremor, and bladder dysfunction are also seen. Optic neuritis (sudden loss or blurring of vision in one eye) is the presenting symptom in 25% of cases. Symptoms are transient, lasting days to weeks. Lhermitte sign (an electric shock-like sensation down the spine with flexing the neck) may be positive, with paresthesias in the trunk and limbs with neck flexion. An afferent pupillary defect may be seen. Diagnosis is usually made by MRI, which is the most sensitive study. The classic brain noncontrast MRI findings are multiple T2 hyperintensities found most commonly in the periventricular white matter, brainstem, cerebellum and spinal cord. CSF analysis on lumbar puncture may show oligoclonal bands. Treatment is with interferon-beta1b and 1a, glatiramer acetate and symptomatic therapy with corticosteroids.

Which of the following is true about myasthenia gravis? Limb weakness and dysarthria are the most common presenting symptoms Muscle weakness tends to worsen after long periods of rest Optic neuritis is commonly associated with the condition Symptoms are decreased by cooling

Correct Answer ( D ) Explanation: Myasthenia gravis (MG) is an autoimmune disease characterized by muscle weakness and fatigue seen particularly with repetitive use of voluntary muscles. It is caused by antibodies that attack and reduce the number of nicotinic acetylcholine receptors leading to a decrease in response to acetylcholine. Cooling improves the symptoms of myasthenia gravis. This is the basis of the "ice test." When ptosis is present, an ice pack is placed over the affected eye for two minutes, and the ptosis resolves. It is thought that local cooling results in a slowing of the kinetics of the acetylcholine receptor, allowing for a prolonged effect of acetylcholine and improvement of symptoms. The most frequent initial symptoms of myasthenia gravis are bulbar, typically with ptosis and diplopia (A) due to ocular muscular weakness, or weakness of the levator palpebrae superioris. Muscle weakness tends to improve (B) after long periods of rest. Patients become weaker with repetitive activity. Optic neuritis (C) is associated with multiple sclerosis, not myasthenia gravis.

A previously healthy 28-year-old woman presents to your office with complaints of worsening overall muscle weakness and drooping eyelids for the past month. Physical exam reveals a mask-like face with ptosis. Sensory exam and deep tendon reflexes are normal. Which of the following is the most likely diagnosis? Bell's palsy Guillain-Barré syndrome Multiple sclerosis Myasthenia gravis

Correct Answer ( D ) Explanation: Myasthenia gravis is an autoimmune disorder of neuromuscular transmission that has two forms, ocular and generalized. Ocular myasthenia gravis presents with weakness of the eyelids and extraocular muscles. Generalized myasthenia gravis presents with a combination of ocular, bulbar, limb, and respiratory muscular symptoms. Patients generally present with ocular symptoms initially, which progress to the facial and bulbar muscles, and then to muscles in the trunk and limbs. Most cases of myasthenia gravis are idiopathic, although certain medications such as antibiotics, beta-blockers, lithium and prednisone can exacerbate symptoms. Certain findings have also been found to be associated with the disorder including predisposition to other autoimmune diseases such as rheumatoid arthritis and systemic lupus erythematosus. The approach to diagnosing myasthenia gravis is a clinical one based on history, symptoms and physical exam findings. Treatment of myasthenia gravis includes a number of modalities including use of anticholinesterase agents to treat symptoms, rapid and chronic immunomodulating treatments, and surgical treatment with thymectomy.

A 79-year-old woman with Parkinson's disease is being treated with three-times-a-day carbidopa/levodopa for the past 5 years. She recently developed cervical dystonia and involuntary tongue movements, both of which have caused feeding impairments. She does not report problems with tremor or rigidity. Her medical history is significant for constipation and hypotensive episodes. Which of the following pharmacologic interventions is recommended at this time? Add benztropine, and switch to five-times-a-day dosing of carbidopa/levodopa Add nothing, but switch any immediate-release carbidopa/levodopa to a controlled-release formulation Add ropinirole, and switch to once-a-day dosing of carbidopa/levodopa Continue the current carbidopa/levodopa dosing, but add pramipexole

Correct Answer ( D ) Explanation: Parkinson's disease is mainly characterized by its impairment on voluntary movement, leading to gait, balance and fine motor abnormalities which significantly affects functionality. Carbidopa/levodopa (immediate-release) is the most effective medication for this disease, and is the mainstay first-line medication used, especially early in the disease course (<5 years). Unfortunately, long-term use of carbidopa/levodopa can cause tardive dyskinesia and further motor control complications, which may cause further functional limitations, in up to 40% of patients treated with this drug for >5 years. As disease progresses, increasing the dose of carbidopa/levodopa is common practice. Dopamine agonists, such as bromocriptine, pramipexole and ropinirole, are also considered first-line treatment of motor abnormalities, albeit they have less benefit than carbidopa/levodopa, but fortunately have less motor side effects than levodopa. Overall, when carbidopa/levodopa has reached its end usefulness, there are several options to consider. Some clinicians will split up the total daily carbidopa/levodopa dose into 5 smaller doses a day. Others will decrease the carbidopa/levodopa dose, and some will convert any controlled-release preparations into immediate-release preparations. Another viable option is to add, or switch to adjuvant medications like dopamine-agonists (pramipexole). Other adjuvants include monoamine oxidase-B inhibitors like selegiline, catechol-O-methyltransferase inhibitors like tolcapone and entacapone, and anticholinergics like benztropine. The most sensible option above is to add an adjuvant medication.

A young woman presents with bilateral headache. She describes it as a band-like pressure from her forehead to neck, feeling as if her "head is in a vice." She denies prodromal symptoms, nausea and photophobia. Cervical spine range-of-motion and skull palpation do not reproduce the headache. Which of the following is the most likely diagnosis? Cluster headache Migraine without aura Occipital headache Tension-type headache

Correct Answer ( D ) Explanation: Tension-type headache (TTHA) is the most common type of recurring headache other than migraine and the most common type overall. Tension headaches present as a bilateral headache with pain in the frontal and occipital regions in a bandlike distribution. Headaches are exacerbated by stress, fatigue, glare, or noise. It also often involves contraction of the scalp and posterior neck muscles, with a normal neurologic exam. Treatment is with aspirin, NSAIDs, and ergotamines.

A 10-year-old girl presents to the Emergency Department with weakness in her legs. It began yesterday and since has progressed up her legs. Reflexes are diminished in her bilateral lower extremities and are normal in her upper extremities. Sensation is intact throughout. She denies recent illness or sick contacts. Her family returned from a camping trip at a national park last week, but otherwise she has not traveled. During the camping trip, the child ate canned and boxed foods that the family had packed, as well as fish that the family caught and cooked. They also went hiking and swam in a freshwater stream. What intervention is most likely to improve the child's symptoms? Botulism immune globulin Intravenous Immunoglobulin Pyridostigmine Removal of a tick from the child

Correct Answer ( D ) Explanation: The child's ascending weakness and lower extremity hyporeflexia are concerning for Guillain-Barré syndrome or tick paralysis. In light of the child's recent camping trip, as well as no recent illnesses, tick paralysis is most likely. Symptoms of tick paralysis do not begin until a female tick has been attached to a human for four to seven days. The child should be examined for a tick, including careful inspection of the scalp, axillae, interdigital webs, genitourinary areas, and buttocks, where the child or the family may not have noticed them. A fine-toothed comb may aid in identification of scalp ticks. The primary offending ticks in the United States include the Rocky Mountain wood tick, American dog tick, Lone Star tick, black-legged tick, and western black-legged tick. Fortunately, if a tick is identified, removal of the tick from the child typically results in improvement of symptoms within a few hours. No other clinical intervention is recommended.

A 19-year-old woman at 34 weeks gestation presents with a one-day history of progressive left-sided facial weakness. She notes a recent history of an upper respiratory infection. On exam, she is unable to raise her left eyebrow, close her left eye, or smile on her left side. She also reports decreased taste sensation. Her neurologic exam is otherwise unremarkable. Which of the following complications is most commonly associated with the patient's diagnosis? Cavernous sinus thrombosis Descending paralysis Hearing loss Keratitis

Correct Answer ( D ) Explanation: The patient has unilateral facial nerve palsy with no other focal neurologic or systemic findings. Although the differential diagnosis for facial nerve paralysis is extensive and includes infectious, traumatic, and neoplastic etiologies, the most common diagnosis is idiopathic Bell's palsy. Bell's palsy is characterized by the sudden onset of facial nerve paralysis with symptoms usually peaking by 48 hours. Some 60% of patients have a viral prodrome. In making the diagnosis, careful consideration must be given to the motor function of the forehead; sparing of this region would be characteristic of a central, and not peripheral, etiology. Because the orbicularis oculi muscles are involved, this results in incomplete closure of the eyelids on the affected side and leads to corneal exposure keratitis. It is important to prescribe lubricating eye drops, to keep the corneal epithelium from breaking down, and to patch the affected eye at nighttime. Cavernous sinus thrombosis (A) is a cerebral venous thrombosis that presents with orbital pain, proptosis, and oculomotor palsies. The cavernous sinus contains CN III, IV, V1, V2, and VI. Resultant facial sensory nerve loss is common. Bell's palsy is associated with abnormally acute hearing (hyperacusis) (C), not the loss of hearing.

A 13-year-old boy with no past medical history presents with a headache 3 days after a closed head injury. The patient states that he stood up from kneeling and hit the top of his head on a wood cabinet. There was no loss of consciousness or seizure activity. In addition to the headache, he complains of difficulty concentrating at school and dizziness. His physical examination is unremarkable. What management is indicated? CT scan of the head with contrast CT scan of the head without contrast MRI of the brain Referal to primary care physician

Correct Answer ( D ) Explanation: The patient presents with minor head trauma and complaints consistent with a concussion and should have follow-up arranged with their primary care provider or concussion specialist. A concussion is a minor traumatic brain injury (TBI) that is often seen in MVCs and collision sports (football, hockey). It is typically caused by a rotational injury or an acceleration-deceleration injury. Patients will present with a number of non-specific symptoms including headaches, dizziness, confusion, amnesia, difficulty concentrating, and blurry vision but do not have focal neurologic findings. Despite the absence of severe intracranial injury, patients can have chronic and debilitating symptoms from concussions. Neurology referral is recommended, as patients should have functional testing and tracking of their symptoms for resolution. It is vital to council patients to avoid contact sports or activities that increased the risk of recurrent injury as these patients are at risk for more severe injury with second impact.

A 45-year-old woman presents complaining of one day of vision loss in her left eye associated with a left retro-orbital headache. She denies trauma or preceding illness. On exam, her vital signs are T 36.8ºC, BP 112/80 mm Hg, HR 74 bpm. Visual acuity is 20/20 in the right eye, 20/400 in the left eye, and 20/40 using both eyes. She has a left-sided afferent pupillary defect. Visual field testing reveals left-sided central vision loss. Which of the following is most accurate regarding her condition? Strict blood glucose control reduces the likelihood for future recurrences The diagnosis is confirmed by measurement of an intraocular pressure > 20 The diagnosis is made by magnetic resonance venography (MRV) The syndrome is associated with multiple sclerosis Therapy should be aimed at removing the acute obstruction of the ophthalmic artery

Correct Answer ( D ) Explanation: The patient's exam is consistent with optic neuritis, an acute monocular loss of vision caused by focal demyelination of the optic nerve. Most cases are retrobulbar and do not involve any visible changes on fundoscopy (especially during an acute episode). But, on occasion and with more protracted cases, optic disk pallor may be seen. An afferent pupillary defect, however, is always present. Approximately 30% of patients presenting with acute optic neuritis develop multiple sclerosis within 5 years. The classic clinical syndrome of MS consists of recurring episodes of neurologic symptoms that rapidly evolve over days and slowly resolve.

A 10-year-old boy presents to the emergency department complaining of nausea, vomiting, perioral numbness, blurry vision, and feeling his tactile sensation of hot temperature seems off. He admits to eating out and having grouper at a local restaurant. No other family members report illness. Which of the following is the most appropriate treatment? Activated charcoal Antihistamine Hemodialysis Mannitol

Correct Answer ( D ) Explanation: The poisoning is most consistent with Ciguatera toxicity. Ciguatera poisoning is endemic to fish in the south Pacific Ocean and less so in the lower Atlantic states. Since these fish are exported around the world, the toxicity can occur anywhere. Ciguatoxin concentrates in larger fish (barracuda, grouper, red snapper, and parrot fish) that eat smaller fish. The small fish feed in coral reef dinoflagellates that form the toxin (Gambierdiscus toxicus). The toxin is odorless, colorless, and tasteless and is not deactivated by heat. Symptoms begin two to 30 hours after ingestion and are associated with gastrointestinal findings (abdominal pain, vomiting, and diarrhea), unique neurological findings (hot-cold reversal, sensation of tooth looseness, ataxia, and altered mental status), and cardiovascular findings (bradycardia, hypotension, pulmonary edema). Management is mainly supportive with antiemetics, IV fluids for hypotension and atropine for bradydysrhythmia. Mannitol is administered to prevent or alleviate neurologic morbidity.

52-year-old man presents with decreased sensation in the upper extremities and chronic neck pain. He states that the loss of sensation has been progressive for months. On physical examination, he has decreased sensation to pain over the upper back, shoulders and arms with intact proprioception and light touch. What disorder does this patient exhibit? Anterior cord syndrome Brown-Séquard syndrome Central cord syndrome Syringomyelia

Correct Answer ( D ) Explanation: This patient exhibits signs and symptoms of syringomyelia. Syringomyelia is defined as the presence of a cavitary lesion in the spinal cord. The lesion is chronic and progressive and symptoms vary based on the location of the syrinx. The most common complaints are headache and neck pain but sensory disturbance, cranial nerve dysfunction (lower) and gait abnormalities are frequently noted as well. When the syrinx occurs in the upper portions of the cord (specifically the neck) patients present with a cape-like distribution of loss of pain and temperature sensation in the upper extremities with preservation of light touch and proprioception. Physical examination shows sensory loss along with muscle wasting and weakness in the hands and arms. Symptoms may be worsened by a Valsalva maneuver. It is best imaged by MRI. Anterior cord syndrome (A) is characterized by loss of motor function below the lesion and preservation of position, touch and vibration sensation. Brown-Séquard syndrome (B) presents with ipsilateral motor function loss and contralateral pain and temperature sensation loss. Patients with central cord syndrome (C) present with bilateral motor paresis and sensory impairment.

12-year-old boy presents with facial weakness for two hours. He states he woke up this morning and noticed that the right side of his face "doesn't move the right way." Examination reveals a right facial droop of the upper and lower face. He is unable to fully close his right eye. Examination is otherwise unremarkable. What management should be pursued? CT scan of the head Intravenous thrombolytics Lumbar puncture Prednisone

Correct Answer ( D ) Explanation: This patient presents with a peripheral facial nerve (CN VII) palsy or Bell's palsy, which should be managed with oral steroids (prednisone). Bell's palsy is an idiopathic, peripheral facial nerve palsy. The facial nerve innervates the muscles of the scalp, external ear and the muscles of facial expression. Additionally, the sensory portion supplies the anterior two thirds of the tongue. Patients will typically present with complaints of weakness of the facial muscles including the mouth, eyelid and forehead. One of the critical issues is to ensure that the paralysis is, in fact, peripheral and not central. In central facial nerve palsy, the muscles of the upper part of the face (forehead in particular) are spared. This is due to the fact that the upper third of the face has dual innervation; it receives fibers from the contralateral facial nerve. Thus, in a patient who has sparing of the upper part of the face, further diagnostic workup should be performed to determine the cause of central facial nerve paralysis. Peripheral facial nerve palsy is treated with prednisone 1 mg/kg/day for 7-10 days. The role of antivirals (i.e. acyclovir) is controversial as it is unclear if herpes simplex virus 1 is a causative organism. Additionally, artificial tears and taping the affected eye closed at night are recommended to prevent drying out of the cornea and subsequent abrasions or ulcerations.

A 45-year-old man with a history of alcohol abuse presents with numbness and weakness of the left hand. He states he slept on a bench last night and awoke this morning with the symptoms. Physical examination reveals decreased sensation over the first, second, and third digits and a wrist drop. What management is indicated? CT of the cervical spine MRI of the brain Noncontrast CT scan of the head Wrist splint and follow up with neurology

Correct Answer ( D ) Explanation: This patient presents with a radial nerve palsy likely caused by direct compression and should have a wrist splint placed and given follow up with a neurologist. The radial nerve arises from the C5-T1 roots, exits the brachial plexus and passes into the spiral groove behind the proximal humerus. It bifurcates at the antecubital fossa. This nerve controls extension of the fingers, thumb and wrist. Compression of the radial nerve in the axilla occurs secondary to the improper use of crutches or from an extended period of unconsciousness with prolonged compression. This pathology is typically called a "Saturday night palsy." Patients with radial nerve palsy present with weakness in extension of the fingers and wrist. Spontaneous recovery often occurs but may take 2-4 months. During this time, the wrist should be splinted with 60 degrees of dorsiflexion in order to prevent atrophy and contractures.

A 63-year-old man with chronic alcoholism presents with headache, fever and neck pain. Physical examination reveals neck stiffness. What antibiotics should be administered? Ampicillin alone Ceftriaxone alone Ceftriaxone and vancomycin Ceftriaxone, vancomycin and ampicillin

Correct Answer ( D ) Explanation: This patient presents with signs and symptoms concerning for meningitis and should receive antibiotics covering the most likely organisms including Listeria monocytogenes. Meningitis describes an inflammation of the meninges. Patients typically present with headache, fever, neck pain and possibly, altered mental status. Diagnosis is made by analysis of cerebrospinal fluid (CSF). The most useful diagnostic tests are a CSF cell count and gram stain. Gram stain may reveal the causative organism. However, antibiotics should not be withheld while either waiting to perform the lumbar puncture or waiting for the CSF results. Patients with a high suspicion for meningitis should have appropriate antibiotics started as soon as possible. The most common organisms in healthy adults are Streptococcus pneumoniae and Nisseria meningitides. Both of these organisms are covered by ceftriaxone however there is a small percentage of resistant S. pneumoniae requiring treatment with vancomycin. Other organisms are more common based on age group and comorbid conditions. Listeria monocytogenes is a more common cause of meningitis in very young infants, elderly patients and those with chronic alcoholism or immunosuppression. L. monocytogenes is typically treated with ampicillin.

A 63-year-old man with a history of alcoholism presents with fever, headache and altered mental status. What antibiotics should be administered? Ceftriaxone, ampicillin Vancomycin, cefazolin, ampicillin Vancomycin, ceftriaxone Vancomycin, ceftriaxone, ampicillin

Correct Answer ( D ) Explanation: This patient presents with symptoms concerning for meningitis and should immediately have antibiotics started covering the most likely pathogens. Bacterial meningitis typically presents with a rapidly progressive course. There are a number of causative organisms but the most common are Streptococcus pneumoniae, Neisseria meningitidis and Listeria monocytogenes. Streptococcus pneumoniae ​is the most common organism in all adults, while N. meningitidis is more likely to be encountered in younger adults. L. monocytogenes is more common at the extremes of age (neonates and the elderly) and in patients with immunosuppression and alcoholism. Patients may experience fever, headache, photophobia, neck pain, vomiting, altered mental status, lethargy and seizures. Kernig's and Brudzinski's signs (physical findings consistent with nuchal rigidity) are classic findings but are seen in less than 50% of adults. A non-contrast head CT should be performed in patients with suspected meningitis if they have signs of increased intracranial pressure or mass effect (focal neurologic findings, altered mental status or papilledema) and in those with significant immunocompromise (i.e. HIV/AIDS). The diagnosis of meningitis is made by analysis of the CSF after lumbar puncture. Antibiotics should not be withheld from patients while waiting for CT scan or lumbar puncture. While awaiting the results of CSF and blood cultures, broad-spectrum antibiotics covering the most likely pathogens should be started. Vancomycin is administered to cover resistant S. pneumoniae and a third generation cephalosporin (ceftriaxone) for N. meningitidis. Ampicillin should be given for patients who are at risk for L. monocytogenes.

33-year-old man with schizophrenia treated with haloperidol presents with involuntary, writhing movements of the tongue and face. Additionally, the patient has choreoathetoid movements of the trunk and arms. What management is indicated? Benztropine Diphenhydramine Lorazepam Stop haloperidol

Correct Answer ( D ) Explanation: This patient presents with tardive dyskinesia (TD) and should have his haloperidol therapy stopped. TD is characterized by involuntary movements, particularly of the face and tongue that occur after years of treatment with neuroleptic agents. The movements are described as grimacing, writhing and choreathetoid in nature. Duration of treatment, cumulative dose, patient age and the presence of preexisting brain damage are directly related to the incidence of TD. In patients with mild symptoms, cessation of the neuroleptic drug or decreasing the dose may result in resolution of symptoms. Patients with moderate to severe symptoms are harder to treat. Reserpine and tetrabenazine have shown some benefit in combination with stopping the offending agent. Benztropine (A) and diphenhydramine (B) are effective in treating acute dystonic reactions and akathesia but are not effective in treating TD. Lorazepam (C) will lead to sedation of the patient but will not treat TD.

A 55-year-old obese woman with a history of rheumatoid arthritis managed with NSAIDs presents after a gradual onset of numbness in her hands over several weeks. She reports the pain is worse at night after she comes home from work as a court reporter. She denies any fever, recent illness, or trauma to the area. Her vital signs are within normal limits. On exam, she has numbness, paresthesia, and pain in the thumb, index, and long fingers. She is worried because her brother had numbness in his right hand last month and was told he had a stroke. Which of the following will confirm her diagnosis? Anteroposterior and lateral films of the bilateral forearms Finkelstein's test Froment's sign Phalen's maneuver

Correct Answer ( D ) Explanation: This patient with bilateral numbness, paresthesia, and pain in the median nerve distribution that gradually worsens most likely suffers from carpal tunnel syndrome. Her risk factors for this disease include obesity, rheumatoid arthritis, and repetitive strain of the wrists as a court reporter. Though she is understandably worried about a stroke, a quick bedside test can both reassure you and her of carpal tunnel syndrome. To perform Phalen's maneuver, ask the patient to raise his or her arms in front of them to shoulder level, bring the dorsum of his or her hands together in the midline, and hold his or her wrist in complete, forced flexion for 30-60 seconds. This increases the pressure in the carpal tunnel, pinching the median nerve between the transverse carpal ligament and the distal end of the radius. Recreation of symptoms (tingling and numbness over the median nerve distribution) is considered a positive test. Froment's sign (C) tests for ulnar nerve palsy. The patient is asked to hold a piece of paper between the thumb and index finger in a pinching grasp. The examiner then tries to pull the paper out from the patient's grasp. The test is positive if the patient must compensate by flexing the flexor pollicis longus of the thumb to keep the object in place.

A woman presents with 30 minutes of double vision, vertigo, difficulty swallowing, and difficulty speaking. During her initial evaluation, these symptoms resolve and her neurologic exam returns to normal. Which of the following is the most appropriate diagnosis and the most likely affected artery? Embolic ischemia - anterior cerebral artery Thrombotic stroke - left anterior descending artery Transient ischemic attack - middle cerebral artery Transient ischemic attack - vertebrobasilar arteries

Correct Answer ( D ) Explanation: Transient ischemic attacks (TIAs) are characterized by an abrupt onset of focal neurologic symptoms lasting less than 24 hours and often lasting only five to 20 minutes. TIAs suggest impending thrombotic-ischemic stroke. Carotid pathology leads to TIAs demonstrated by hemiparesis, hemisensory, aphasia, confusion and transient monocular blindness. Vertebrobasilar insufficiency leads to TIAs marked by hemiplegia or quadriplegia, varying sensory changes, blindness, hemianopsia, diplopia, vertigo, dysarthria, dysphagia and facial, motor, and sensory change. Investigation includes Doppler ultrasonography of the carotids and vertebrobasilar system. Head CT scan will not show any acute changes. MRA angiography may also be indicated. Selected cases may require endarterectomy, angioplasty or lifelong anticoagulation. Middle cerebral artery (C) lesions do not produce vertigo. Embolic ischemia typically produces symptoms that last longer than three hours and anterior cerebral artery (A) lesions do not produce vertigo and diplopia.

A 28-year-old woman complains of sudden onset of lower back pain that began two days ago. The pain radiates down both legs. On exam, she has 3/5 strength bilaterally, decreased sensation in her lower extremities, decreased patellar reflexes, and a distended urinary bladder. An MRI of her spine reveals no masses. Which of the following is the most likely diagnosis? Guillain-Barré Syndrome Spinal epidural abscess Syringomyelia Transverse myelitis

Correct Answer ( D ) Explanation: Transverse myelitis is an acute spinal cord disorder characterized by paraplegia, a transverse level of sensory impairment, and sphincter disturbance. The specific etiology is unknown, although it is noted to follow a viral infection in 30% of patients and is commonly referred to as postinfectious myelitis. Patients often complain of back pain and may have a low-grade fever, raising the concern for an epidural abscess. An MRI is usually performed to rule out compressive lesions of the spine. Despite the absence of evidence, most sources still recommend high dose steroids as first line treatment. Although spinal epidural abscess (B) has many clinical similarities to transverse myelitis, the population at risk for this diagnosis is generally limited to individuals with a history of intravenous drug abuse or prior spinal instrumentation

A 63-year-old woman with a history of a recent viral upper respiratory infection presents with vertigo that started this morning after she woke up. Throughout the day, she experienced intense spinning associated with head movements. She reports nausea and nonbloody, nonbilious emesis. She denies recent trauma and pain. On exam, she has horizontal nystagmus beating towards the left that is exacerbated by changing head positions and resolves after a few moments. She notes decreased hearing on the right. She is otherwise neurologically intact and has an unremarkable head and neck exam. What is the most likely diagnosis? Benign paroxysmal positional vertigo Cerebrovascular accident of the posterior circulation Vertebral artery dissection Vestibular labyrinthitis

Correct Answer ( D ) Explanation: Vertigo is a challenging chief complaint that can stem from the central nervous system or the peripheral nervous system. Vertigo is a perception of movement when there is none. Central vertigo is most often due to a cerebrovascular accident of the posterior cerebral circulation. Peripheral vertigo is most often due to cranial nerve VIII or vestibular lesions. When hearing is affected in conjunction with vertigo, it is most likely a peripheral lesion. Vestibular labyrinthitis is inflammation along the vestibulocochlear nerve (CN VIII). It most commonly occurs after a viral illness (e.g. viral URI) or otitis media, lasts for several days, and then often resolves. It is the second most common cause of peripheral vertigo behind benign paroxysmal positional vertigo (BPPV). BPPV does not result in hearing loss. Treatment is symptomatic until the condition resolves.

A 59-year-old man has been experiencing left sided hearing loss over the past several months and he is no longer able to listen to a telephone on his left side. He also has left side tinnitus and feels off-balanced when walking. An audiogram shows high tone hearing loss in the left ear and a 30% word discrimination score. Which of the following is the most likely diagnosis? Labyrinthitis Meniere's disease Presbycusis Vestibular schwannoma

Correct Answer ( D ) Explanation: Vestibular Schwannoma usually presents with a triad of progressive ipsilateral hearing loss, ataxia, and tinnitus. It is a tumor of the vestibular portion of the acoustic (VIII) cranial nerve and has historically been referred to as an acoustic neuroma (although this term is a misnomer). A typical finding is ipsilateral sensorineural hearing loss in the high tones and a poor word discrimination score (hence the frequent complaint of not being able to use a telephone in that ear). Diagnosis is made with neuroimaging (contrast enhanced MRI is most common) and treatment options include: 1) continued observation with periodic imaging 2) radiosurgery 3) surgical resection. Meniere's disease (B) is a disorder of endolymphatic hydrops. It presents with a similar triad of hearing loss, tinnitus, and vertigo. Some key distinctions include: 1) recurrent bouts of severe and often disabling vertigo (as opposed to ataxia) and 2) hearing loss will come and go and is typically in the low tones (as opposed to the high tones). Labyrinthitis (A) is inflammation of the inner ear usually caused by a viral infection and often follows an upper respiratory infection. The vertigo and hearing loss have an acute onset and are self-limited to a few weeks. Treatment is symptomatic in most cases. Presbycusis (C) is age-related hearing loss that typically begins past the age of 65 and is almost always bilateral affecting both ears equally.

A 65-year-old woman presents to the ED for right-sided facial weakness that began acutely 1 hour prior to arrival. She woke up from sleep this morning with a dry right eye and is now unable to move the right side of her mouth. You ask the patient to smile and raise her eyebrows and you note a right-sided facial droop and no forehead wrinkling on the right side. Which of the following is the most appropriate next step in management? Administer a small dose of edrophonium, followed by a full dose, and observe the patient for improvement of her symptoms Administer intravenous prochlorperazine Obtain a blood glucose level, basic laboratory work, and CT scan of the head Obtain a National Institutes of Health Stroke Scale, bring the patient to the CT scanner, and consult a neurologist Provide prescriptions for corticosteroids, antivirals, artificial tears, and an eye patch, and have the patient follow up with her physician

Correct Answer ( E ) Explanation: This patient has Bell's palsy, a partial or complete paralysis of the facial nerve (cranial nerve VII). Typical symptoms include sudden onset of unilateral facial paralysis, including the forehead, decreased tearing, hyperacusis, and loss of taste sensation on the anterior two-thirds of the tongue that can progress over 1 to 7 days. Many patients describe a viral prodrome. The physical exam is key in the diagnosis to establish the presence of a peripheral rather than a central 7th nerve palsy. Upper and lower facial weakness is present in the peripheral nerve palsy. In central 7th nerve palsy, common in stroke syndromes, only the lower facial weakness is present. The forehead is spared because of bilateral hemispheric innervations to the forehead muscles. Treatment includes corticosteroids, antivirals, artificial tears, and eye protection.

An 85-year-old man presents to the emergency department with altered mental status. Which finding would suggest a diagnosis of delirium rather than dementia? Acute onset Depressed mood Memory impairment Pupillary dilation

orrect Answer ( A ) Explanation: Delirium is a common mental disorder seen in patients with a medical illness, especially older adults. Delirium is characterized by an acute onset (usually hours to days) disturbance in attention and cognition that is caused by a medical condition, substance withdrawal or intoxication, or a side effect from medication. The changes in mental status cannot be explained by a preexisting condition and are not related to a reduction in level of arousal. Patients with delirium may also exhibit psychomotor behavioral and emotional disturbances. The differential diagnosis for delirium is extensive, and diagnostic evaluation involves both identifying that delirium is present as well as determining the underlying medical cause. Obtaining a history and performing a physical exam on a patient with delirium is challenging. History should be obtained from family members or friends and physical exam should include a focused assessment with review of vital signs, skin condition, level of hydration and clues to an infectious etiology. Treatment of delirium involves managing the behavior disturbance along with finding and treating the underlying medical cause.

A 50-year old woman presents to her primary care provider's office with complaints of difficulty walking and balancing. The weakness started in both of her feet, but has now progressed to her knees and hips. She has an unremarkable past medical history except for a recent gastrointestinal infection with Campylobacter jejuni. Which of the following exam findings would this woman most exhibit? Choreiform movements and dyskinesia Diminished or absent deep tendon reflexes Hyperreflexia of the patellar reflex Resting tremor and immobile faces

orrect Answer ( B ) Explanation: Patients with Guillain-Barré syndrome would most likely have diminished or absent deep tendon reflexes. Guillain-Barré syndrome (GBS) encompasses a heterogeneous group of acute immune-related polyneuropathies. GBS most commonly presents following a preceding respiratory or gastrointestinal infection, which is thought to trigger an autoimmune response against axons. Campylobacter jejuni is the most commonly recognized precipitant of GBS. The hallmark symptoms of GBS are bilateral, progressive, symmetrical muscle weakness and paralysis, accompanied by diminished to absent deep tendon reflexes. In 90% of cases, the weakness begins in the legs and ascends. Tachycardia, hypotension, and other manifestations of autonomic dysfunction occur in approximately 70% of patients. Sensory involvement is usually less obvious than motor symptoms. The clinical course of GBS typically progresses over a period of two weeks. The cerebrospinal fluid of patients with GBS typically has an elevated protein concentration and a normal white blood cell count. Antibody testing may be of benefit in some GBS variants. All patients with suspected GBS should be admitted due to the risk of respiratory compromise. Intensive care unit admission is warranted for any patients with labile dysautonomia. Plasmapheresis and intravenous immunoglobulin are the main treatment methods. Corticosteroids are not typically indicated. Physical, occupational, and speech therapy should be considered for most patients. Most patients are able to ambulate independently within six months and about half of patients regain full strength within one year.

A 49-year-old woman with a history of osteoarthritis presents stating that she feels like the room around her is spinning. She reports a similar episode three weeks ago, and since then, has had an intermittent ringing sound in her ears. Her husband adds that over the same time frame she also has developed some difficulty hearing from the left ear. During your exam, the patient has an episode of non-bilious vomiting and states that the room is spinning again. Vital signs are normal. Which of the following is the most likely diagnosis? Benign paroxysmal positional vertigo (BPPV) Ménière's disease Salicylate toxicity Vestibular neuronitis

orrect Answer ( B ) Explanation: This patient has Ménière's disease. This disorder is associated with increased endolymph within the cochlea and labyrinth. The common triad is tinnitus, vertigo, and unilateral hearing loss (sensorineural). A key finding in Ménière's disease is fluctuating hearing loss. Episodes are abrupt in onset and associated with nausea and vomiting. There are often long, symptom-free intervals between attacks. Salicylate (aspirin) toxicity (C) is associated with tinnitus and reversible hearing loss. The patient has a history of osteoarthritis and may be using aspirin for her pain; however, salicylate toxicity is usually associated with bilateral hearing loss. Symptoms of vertigo are also uncommon in such patients.

A 3-year-old boy with no past medical history presents with a seizure. His mom states that he had a cough and a fever for the last day and then she witnessed him having 45 seconds of tonic-clonic movements. In the emergency department, the patient has normal vital signs except for a temperature of 102°F. Physical examination reveals a well-appearing boy who is playful and interactive. He has no neurologic findings. What management is indicated? Admit for electroencephalography Lumbar puncture Noncontrast CT scan of the head Observation, reassurance, and follow up with pediatrician

orrect Answer ( D ) Explanation: This patient presents with a simple febrile seizure and the parents should be reassured and instructed to follow up with the child's pediatrician. Febrile seizures are defined as a seizure occurring in the presence of a fever without central nervous system (CNS) infection or other cause. A simple febrile seizure describes a brief (<15 minutes) generalized seizure in a developmentally normal child between the ages of 6 months and 5 years. A complex febrile seizure occurs when there are multiple seizures during the same illness, the seizures are prolonged or focal. Febrile seizures are common and occur in 2-5% of children. Children who experience a febrile seizure are likely to have a recurrence (30%) and at a 2-3% risk of developing epilepsy. Seizure activity usually ceases on its own without specific management but if the child has a seizure in the ED, they can be treated with benzodiazepines. Children with simple febrile seizures do not require any specific testing beyond a good history and physical examination. Administration of antipyretics has not been found to reduce the likelihood of recurrence. Reassurance and follow up with their pediatrician are the most important aspects of management.