Neuro
Contusions
Anterior/inferior frontal lobes or anterior temporal lobe, where the bone hots rough bone surface. Appears as a white bleb.
Describe hypertensive encephalopathy.
Cerebral vasoconstriction occurs when there is systemic hypertention to maintain proper flow and volume (AUTOREGULATION). Cerebral autoregulation fails in patients with hypertensive encephalopathy leading to edema and hemorrhage. This should be considered in patients that have a diastolic greater than 120. It is frequently accompanied with retinal ischemia and renal disease.
What is peripheral vertigo is provoked by noise?
Meniere syndrome: This is due to diminished resorption of endolymph leading to rupture of the membranes, sudden mixing of the endolymph and perilymph, leading to a sudden change in pressure. This results in damage to hair cells, low pitched tinnitis and hearing loss. Perilymph fistula: This is due to a small leak between inner and middle ear due to trauma, leading to sudden change in pressure in the middle ear. This results in hearing loss.
Describe Meningeoma.
Meningeomas are almost always benign and SLOW growing. They may be very large when found due to the slow growth. They are typically found on scans for other reasons and may be asymptomatic. They develop from the dura, may calcify and are typically very hard. Meningeomas may irritate the surrounding cortex leading to seizures (presenting sign). They may develop inferior to the frontal lobe and push on the surrounding structures leading to personality change with loss of smell. These are often resctable and respond to radiation therapy. They also very rarely undergo malignant transformation. Meningeomas are made up of plump spindle or polygonal cells which form lobules in some areas. Fasicles of cells tend to stream and form small whorls. Psammoma bodies (round calcifications) are often present but mitoses and necorsis is uncommon. Psammoma bodies (whorls with layers of calcium), cellular whorls with pseudo inclusions.
What are the causes of peripheral vertigo?
A relatively common and isolated condition is vestibular neuronitis or acute labrynthitis. It results in a single, monophasic attack of vertigo. Symptoms usually come on quickly and resolve within a few days to weeks. Acoustic neuroma is another condition to consider. This typically produces mild vertigo because it is very SLOW growing. It is much more common to be identified for visits due to hearing loss. This is a benign tumor.
How is vegetative state diagnosed?
Absence of: awareness of self and environment, purposeful or voluntary behavioral response to stimuli, and language comprehension or expression. Presence of: sleep-wake cycles, autonomic functions, cranial nerve/spinal reflexes. Potential signs are: Spontaneous roving eye movements, utter sound, grimace to pain, yawn/chewing movement, withdrawal from noxous stimuli, move head towards sound.
Describe simple focal (partial) seizure
All begin in a particular part of the cortex. Note that all can generalize. Motor: clonic jerking of the contralateral side, can spread (jacksonian march), and is followed by Todds paralysis (fatigue of the muscle) Somatosensory: parasthesia on the contralateral side, can be followed by diminished sensation. Can also involve the visual cortex and the olfactory-gustatory cortex.
67 year old patient was unable to be awoken in the morning. He had complained of mild headache and chills the day prior. The patient has a past history of HTN and BPH. He takes HCTZ daily. He has not traveled recently. T 39.8, P 100, BP 210/100. RR 20. SaO2 96%. symmetric, reactive 3mm pupils. Withdrawal of extremities to stimulation. Hyperreactive DTRs. Extreme nuchal rigidity, and tendency for hips and knees to flex. Labs reveal increases CSF pressure. Large increase in WBCs, specifically neutrophiles. Increase protein. Markedly decreased glucose.
Bacterial meningitis or viral encephalitis is highest on the differential. Note that this is not consistent with viral meningitis. Bacterial meningitis almost always produces acute meningitis. The typical signs are fever, headache, and meningismus (pain in neck, particularly on flexion due to stretching of the meninges). Patients are often sensitive to light. The patients tend to appear very ill. There may also be signs of increased intracranial pressure. This may lead to stroke due to inflammation of the blood vessels or seizure due to irritation of the underlying gray matter. Viral encephalitis: This may begin with signs and symptoms of viral meningitis, is distinguished by altered consciousness. This may progress to seizure or focal neurologic symptoms that would never be seen in uncomplicated viral meningitis (HSV1) Important to perform an urgent non-contrast CT, general metabolic labs, blood culture, and an LP. Sequence of treatment should be head CT and antibiotics (ceftriaxone, vancomycin, and ampicillin). Then do an LP once abscess has been ruled out. Then send for gram stain and culture and perform necessary lab tests. Steroids will also be given to reduce inflammation.
Describe Cerebellar Tumors.
Cerebellar tumors in children represent a particularly important phenomenon. The most common tumor in children are Cerebellar astrocytomas. Note that these are often curable (which is different than adult astrocytomas). These are RARE in adults.
Describe febrile seizures.
Common in childhood and considered benign (susceptibility resulting from an age related lowered seizure threshold). Features that identify an increased in underlying epilepsy are: Duration of seizures (longer than 15 minutes), focal or partial seizure (vs generalized) and repetitive seizure episodes within a 24 hour period of illness or fever. Note that having an abnormal neurologic profile or underlying brain injury is the most important risk factor for the development of epilepsy. Children with febrile seizures have a slight increase in rate of epilepsy.
What is the standard sequence of the five deteriorating variables?
Consciousness will progress from stupor to coma. Respiration will progress from eupnea --> Cheyene-Stokes --> central neurogenic hyperventilation --> to ataxia and finally --> apnea Pupils will progress from normal reactive --> small reactive --> midposition fixed VOR will progress from normal nystagmus --> loss opf fast component --> dyconjugate response --> loss of tonic phase Motor will deteriorate from normal mobility --> localizing noxous stimuli --> withdrawal response --> decorticate posturing -->decerebrate responses --> loss of motor responses (other than spinal reflexes).
Describe imaging of CNS tumors.
MRI is more sensitive since intracranial lesions that are of the same radiodensity as brain can be missed with uninhanced CT. The injection of intravascular contrast often helps to clarify the lesion since many tumors have blood vessels that do not have a normal BBB. PET scan may be used to show the high metabolic activity of tumors (although this is VERY expensive).
Imaging Dura mater
Fused with the inner skull periosteum. There are reflections of dura from the venous sinuses. Epidural space is between the dura and the naked bone of the skull. Epidural hematomas dont generally cross structures
What are diagnostic possibilities for demyelinating disease?
Lupus, thyroid disease, B12 deficiency. Lyme disease, Sarcoidosis, neurosyphilis, AIDs. All of these are considered the great mimics of central nervous system disease.
77 year old patient is referred for daily headache, weight loss and intermittent fever of one months duration. Family has noticed the patient becoming increasingly confused. There is no neck stiffness and the vitals/physical are normal. There is a depressed mental status exam. CBC, metabolic panel are normal. MRI is with contrast reveals diffuse meningeal enhancement. CSF pressure is increased. Increased WBCs, all lymphocytes. Total protein is 89. Low glucose.
Differential for this patient is chronic meningitis: - Cryptococcal meningitis. - TB infection. - Carcinoma meningitis (typically seen in immunosupressed patients, cancer or AIDs) - Sarcoid, rheumatologic disease, CNS vasculitis.
What are the two processes that depress consciousness?
Diffuse suppression of the cerebral cortical activity (wither by damage or suppression (encephalopathy)). Damage to the reticular formation (of the rostral brainstem to the diencephalon) (supression (less common) or damage (more common due to the fact that it is in a small confined area = inchemia/infarct, hemorrhage, neoplasm, abscess, or direct trauma). Important to treat this patient with flumazenil, naloxone and thiamine.
What are the symptoms of MS?
Fatigue, depression, bladder dysfunction, spasticity, ataxia and weakness. Note that fatigue is a common symptom that appears to get worse with overheating. Optic neuritis is very common (very likely to report). Numbness or tingling.
Describe Gliomas.
Gliomas are tumors that arise from the support cells of the CNS (glial cells). These are the most common intraxial primary brain tumors, where astrocytoma is the most aggressive. There are different grades gliomas: - Glioblastoma multiform: this is the most common and the most malignant. There are pathologic features of necrosis (outgrowing the blood supply), pseudopalisading of cells and proliferation of endothelial cells. ooligodendrogliomas: these tend to be less aggressive, often present with seizures. Note that they can calcify. The tumor will have a fried egg cell morphology and chicken wire capillaries. Unfortunately there is rarely a cure for glial tumors. They tend to infiltrate into surrounding brain structures and are therefore not totally resectable. Radiation can reduce size but is not curative.
38 year old patient presents to the ED with left are weakness that evolved over the past few days. He has a mild headache with no nausea or vomiting. He denies recent fevers, chills or weight loss. There is noted mild left sided facial droop. Mild increase in tone on the left side. Hyperreflexia on the left. There are multiple ring enhancing lesions. Chest X-ray is normal. WBC is 2.8. Has a positive HIV test.
Lesion most likely at the right supratentoral area involves the motor pathway. Differential is: - infectious lesion (taxoplasmosis, bacterial brain abscess, TB), JC virus, neoplasm (cerebral lymphoma). The light colored ring is due to inflammation of the brain tissue. The surrounding darker areas is due to edema. The symptoms are headache and focal signs (depending on where the lesion is). CSF may not me abnormal, especially if the lesion is encapsulated. Cerebral taxoplasmosis often appears as ringed enhancing lesions. A low CD4 count strongly correlates with generating cerebral taxoplasmosis. These are very responsive to antibiotic therapy. Note that CNS lymphoma can look very similar and is also associated with AIDs.
75 year old male presents with 2 month history of right greater than left lid drooping (ptosis). Mainly noted when he is tired. He also has described problems with swallowing liquids. He feels generally weak. PMH is significant for hypothyroidism.
Localization of the story fits best with the NMJ. This is most likely generalized myasthenia gravis. The fatigability is one of the biggest indicators for thinking this is MG. This is an autoimmune disease with autoantibodies directed against the nicotinic Ach receptors in the NMJ. Incidence is highest in young women and adult men. Note that thymic tumors or hyperplasia may be found in MG patients. The earliest symptoms are seen in the eyes with ptosis and diplopia. The second most commonly affected muscles are the pharynx and the soft palate with problems swallowing and speaking. The antibodies cause a downregulation of the receptors leading to a reduction in the miniature end plate potential. Anticholinesterases (pyridostigmine) improves the symptoms of MG by increasing the concentration of Ach in the synapse. There is also Lambert Eaton Myasthenic Syndrome: It may be associated with carcinoma (LEMS may improve after removal of the tumor) (usually bronchogenic small-cell carcinoma). This condition is due to the reduction of the amount of Ach tht is released into the synapse due to defective Ca2+ channels. Note that this causes weakness that tends to IMPROVE with exercise! Botulism is another disorder of Ach release blockade (may be seen with infection of C. botulinum). The toxin binds to synaptobrevin which is needed for vessicle dockage and NTG release. This presents with descending paralysis (starting at the head and neck first).
What are the causes of polyneuropathy?
Metabolic (diabetic, hypothyroid, liver failure) Nutritional (Deficiency in the B vitamins) Toxic (alcohol, medications, heavy metals) Infectious (HIV, Lyme, Syphilus) Inflammatory (Guillain Barre, CIDP, vasculitis) Dysproteinemia (Amyloidosis, MGUS) Hereditary (Charcot-Marie-Tooth disease)
Describe bacterial meningitis.
Most bacterial meningitis originates in the nose. The bacteria may gain access to the meninges through the connecting venous system. They may gain access through the arterial system from bacteremia and breakdown of the BBB. An exudate or pus accumulates in the subarachnoid space once there is invasion of the meninges. This material is comprised mostly of neutrophiles. This can lead to swelling of the cranium and obstruction of CSF flow. Death can result from an increase in intracranial pressure. Stroke can occur due to inflammation of the arteries in the subarachnoid space (this can occur after the patient has started to recover). You should profilacticaly treat these patients immediately once bacterial meningitis is suspected. Antibiotics should be given before you start the lengthy lab evaluation. You should image the brain before performing an LP because patients with abscesses should not be tapped. CSF pressure is usually elevated. WBC count is elevated (neutrophiles). Protein is usually high. Glucose is normal at first and then decreases (a very low glucose is a bad prognostic sign). CSF will be coudy yellow/white.
Describe musclar distrophies
Non-inflammatory degenerative conditions of muscle that are genetically determined, not effectively curable, and progressive. The type is usually classified based on inheritance and distribution of weakness. Duchenne Dystrophy: this is an X-linked disorder due to the dystrophin gene (functions by attaching the contractile elements to the muscle membrane. Another protein performs this function early in life, thus, presents after the child is walking. The child will experience a progressive decline in muscle strength (wheelchair bound by 10 and death in early 20s). Beckers Dystrophy: this is a less severe mutation in the dystrophin gene that causes later onset of symptoms. Myotonic dystrophy is unique in that it tends to affect distal muscles and causes notable myotonia (delayed relaxation of muscles). There are two distinct genes that may have the abnormal repeats. Type-1 myotonic dystrophy is more common and affects distal sites (first affecting the muscles of the hand). There are numerous non-neurologic findings (frontal balding, testicular atrophy, diabetes, arrhythmias, and cataracts. It progresses slowly where many patients succumb to respiratory arrest and infection in the 5th decade. This is a autosomal dominant trinucleotide repeat disease, affecting the myotonic protein kinase gene. These repeats tend to get longer with subsequent generations. Limb-girdle muscula dystrophy: this is a heterogeneous group of disorders that tends to appear in adolescence or in early adulthood with proximal limb weakness. The weakness usually progresses slowly but may spontaneously revert.
Describe how to diagnose MS.
Note that there is no specific biological test for MS. The diagnosis requires a combination of clinical, imaging and laboratory investigation. MS requires multiple white matter lesions separated in space and time. Demonstrating time you must wait for a second clinically apparent event that is at least 1 month after the first. Lesions that enhance indicates that there is disruption of the BBB and that the lesion is less than 1 month old. The most common sites to find lesions are Periventricular, Juxtacortical, Infratentoral (cerebellum or braintsem) and Spinal cord. Lab studies for MS include: CSF is analyzed for specific antibodies. These appear as oligoclonal bands. There is also likely IgG production in and around the brain. Exacerbations are typically seen in patients with MS, where the symptoms must last for more than 24 hours and 1 month must pass for the distinction of two exacerbations.
Male collapsed while walking. He is unresponsive, has no pulse and apneic. Cardiogenic shock returned pulse. Patient is unresponsive to nose tickle with no movement of the extremities, pupils were 2mm, symmetrical and responsive to light. VOR was intact. Patient has a history of hyperlipidemia and takes ASA and a statin. Eyes were open during the day and horizontal roving movements were noted. Patient would grimace with nasal tickle after several days and demonstrated bilateral decorticate posturing. Also had a positive babinski sign.
Note that this patient is in a vegetative state (there is absent awareness of self and environment but wakefulness is retained). The vegetative state is caused by diffuse or multifocal brain lesions that disconnect the polymodal cerebral cortices from the thalami, but, spare the brainstem and hypothalamus. Two clinical disorders are most common: diffuse hypoxic/ichemic neuronal damge to the thalamus or cortex suffered during cardiac arrest, or diffuse axonal injury from a traumatic injury caused by torque force.
Describe tension type headaches.
Pain is often mild to moderate, generalized and constant. Where the location is often bilateral, cervical or bifrontal. There is no warning, no specific timing as well as few or no associated symptoms. Note that this is what a migraine is not (no vomiting, nausea, photophopia or phonophobia).
Describe cluster headaches.
Pain is usually VERY severe and begins in the orbit and is unilateral. It is usually predictable, and the timing is regular and predictable, occurring in clusters over weeks. They usually last less than 1 hour. This is diagnosed through the presentation of clustering of severe headaches, that are short in duration, over weeks to months and then resolve.
20 year old patient at party with lack of sleep starts complaining of a burning odor. Began sniffing the air and repetitive lip smacking and licking of his lips. Then he began chewing and swallowing. He did not seem aware of environment and would not respond to questions. He then ran outside, 3 minutes later he crouched down and asked what happened (memory loss during the episode).
Patient has an olfactory aura, followed by oral automatism and altered awareness. This is followed by postictal confusion. This represents a copmplex partial seizure (focal seizure with impaired consciousness).
65 year old patient with CAD, HTN and HLD as well as migraines, presents to the clinic with recurrent headaches. Reports a history of intermittent right-sided throbbing headaches, with associated nausea and vomiting and photophobia years ago. Has experienced right sided frontal-temporal headaches within the last months. The headache has a dull quality. They are 6/10 and are not associated with nausea or vomiting. She fell a couple months ago but states that she did not hot her head hard. Reflexes are asymmetrical with an increase in the left patellar. Note that the patient has an extensive smoking history.
Patient is presenting with right sided headache and left sided hyperreflexia (may suggest right sided lesion). - Tumor: metastatic tumor from the lung - Temporal arteritis: She is of the appropriate age for this. Constitutional symptoms include weight loss, fever, anemia and fatigue. Evaluation through ESR and artery biopsy. Treat with steroids. - Hemorrhage (subdural hematoma from the fall). Perform the following tests: - ESR: evaluate for temporal arteritis. - CT: to rule out hematoma. - MRI (with and without contrast): to evaluate for tumor.
Describe medication overuse headaches (analgesic rebound).
Physiologic dependence on daily analgesic medication will develop bifrontal headaches when attempt to stop the medication. Are often noted in the morning. Treatment includes education and withdrawal of the medication. Several weeks may pass before symptom relief
Describe the autoimmune myopathies.
Polymyositis: usually appears in people between 30-60, and presents as an insidious onset of symmetric, proximal muscle weakness, occasionally accompanied by muscle soreness. Occasionally this is associated with another autoimmune disease or cancer (lymphoma, lung, bladder). Dermatomyositis: includes insidious weakness of the proximal muscles along with a purple rash (eyelids, elbows, knuckles and toes). This tends to be seen in children from 5-15 and later in adults in their 50s and 60s. Lymphoma, lung cancer, breast and ovarian cancer should also be considered. Inclusion body myositis: this is a slowly symmetrical myopathy that affects proximal muscles as well as the flexors of the fingers and wrists. Note that the sedimentation rate is variably elevated (ESR). This condition is characterized by cytoplasmic inclusions on muscle biopsy. This does not respond to immunosupression. Note that CK levels are elevated which indicates muscle damage. ESR is also elevated indicating an immune response. Prednisone or IVIG are the treatments used in myopathy.
What are the causes of central vertigo?
Posterior circulation ischemic stroke. Brainstem demylenation. Cerebellar pontine angle mass. Malformation. Migraine. Medication toxicity (aspirin).
21 year old patient with diabetes and epilepsy (treated with carbamazepine) brought to the ED as having a tonic-clonic seizure. Complained of the flu earlier in the day, tried to nap, then patient was found having a tonic-clonic seizure. Seizure had stopped and then started again with no apparent consciousness between the episodes.
Status epilepticus is continuous generalized seizures. The most common cause of status epilepticus is anticonvulsant withdrawal, next is metabolic and then infection. Termination of seizure medications results in a temporarily heightened seizure susceptibility. Treat this patient with benzos, IV anticonvulsant (fosphenytoin, valproate), barbituates and generalized anesthetics. It is important to stop the seizure ASAP, the cahnce of dying increases the longer the seizure goes on. Many survivors are left with severe brain damage. - Hyperthermia and hypoxia lead to massive neuronal damage. - Massive autonomic activity leads to severe BP changes and arrhythmia
Describe subarachnoid hemorrhage.
Sudden onset of severe headache should be considered subarachnoid hemorrhage until proven otherwise. 90% arise from congenital derived arterial outpouching that lie in the bifurcations of major components of the circle of willis. There can be nucal rigidity and low grade fever due to chemical meningitis from the breakdown of blood products.
What are the red flags (SNOOPS) to consider for headache evaluation?
Systemic symptoms: weight loss, fever. Neurologic Symptoms or abnormal signs. Onset: sudden. Older: new onset or progressive. Previous headache hostory (first, different or changing). Secondary risk factors: cancer, HIV.
Describe the different types of MS.
The classic type is relapse remitting type: the first incidence appears as a clinically isolated syndrome (an example would be an episode of optic neuritis producing vision loss in one or both eyes). This clinical syndrome often improves over days to months but often doesnt return to baseline. Other events of MS will follow where the diagnosis will then be made. There is usually some increasing disability with cumulative defect with each episode. The idea of treatment is to reduce the number of episodes. Secondarily progressive type: this starts out like relapse remitting MS but then begins an upward slope of of increasing neurologic deficit in between the episodes. This form tends to be more aggressive and less responsive to treatment. Primary progressive type: This is difficult to diagnose because there is no distinctive episodes and there isnt a sudden onset. Progressive relapsing type: this has a combination of progressive disability from the beginning with some relapses and remissions.
What are the signs of an expanding lesion?
The first signs would be due to local effects on sensory and motor function. There are then characteristic changes in five variables once the mass compresses the diencephalon (upper brainstem): consciousness, respiration, pupils, VOR and motor response. This is usually due to uncal herniation.
50 year old man presents with 6 month history of weakness of his left hand. He says that his right hand occasionally goes numb. There is atrophy and weakness of the left abductor pollicus brevis. Tinel sign is positive at the right wrist and negative at the elbows. Phalen sign is positive on the right. Lower stregth test identifies 4+/5 left ankle dorsoflexion, inversion and eversion. Fasciculations are noted on the left forearm and hand muscles.
This is consistent with carpel tunnel syndrome as well as cervical myelopathy with left lower cervical radiculopathy (ALS: both upper and lower motor signs). An important finding in local nerve damage is that the nerve becomes very mechanically sensitive at the site of injury (Tinel sign). This is a mononeuropathy that is caused by entrapment of the median nerve at the carpel tunnel.
Patient with hypertention, had a severe headache an hour prior to the visit followed by falling to the ground. Patient was then unable to move left arm or leg. Patient then lost consciousness. Patient presents with no purposeful motor responses to command. Cheyne-Stokes respirations. Enlarged right pupil, minimally reactive to light, normal pupil on the left. VOR reveals a tonic phase with the absence of a fast jerk.
The lesion is most likely in the right cerebral hemisphere. This is probably a hemorrhage due to the suddenness. Large pupil on the right indicates that there is pressure in the CN III. The midbrain is showing signs of dysfunction. Much of the brainstem is intact, indicated by the tonic phase of the caloric test. Lack of the fast jerk indicates that the cortex is not functioning. Expanding masses, including tumors, abscesses or hemorrhage. There is rostral to caudal deterioration: cortical function lost first, then dysfunction of the diencephalon. Midbrain dysfunction follows mostly due to herniation of the uncas. There is brainstem involvement lastly. Very important to treat this patient with mannitol and hyperventilation (decrease pressure).
Describe Viral Encephalitis.
The most common and important cause of viral encephalitis is HSV1. The virus lives dormant in the trigeminal ganglion. This site is adjacent to the medial part of the temporal lobe. It activates and spreads for unknown reasons, but commonly causes mental status changes and seizures. This is associated with headache, fever, and possible meningismus. Note that these patients look much more ill than viral meningitis patients.
37 year old female patient presents to the clinic with onset of quick (dance like) movements that are rapid, jerky, and irregular or the face and limbs. The patient has been on medium dose Fluoxetine for the past three years due to depression. Her father had similar symptoms but has since passed away. He was described as an angry man who would have jerking irregular movements.
The patient is most likely suffering from Huntingtons disease. This is an autosomal dominant disease with complete penetrance, where there is a trinucleotide CAG repeat in the Huntington gene. - Chorea: quick movements that are rapid, jerky, and irregular, usually affecting the muscles of the face and limbs. - Behavioral changes: Many patients suffer from psychiatric syndromes (depression, BP disorder, Anxiety and elevated mood). The median survival is 13 years due to degeneration of the cuadate nucleus. There are few treatment options: - For Chorea and psychosis: start with benzodiazepines, then neuroleptic agents. - Note that Haloperidol should be used as a lost resort for treating chorea. Do not give patients Levodopa (will make them worse). Dopamine blockers can lead to tardive dyskinesia.
26 year old patient with past medical history of migraine with aura is presenting to the ED with "worst headache of my life". This headache came out of the blue after soccer practice. She reports a sharp stabbing pain all over and nausea. Vitals were within normal limits. Patient is currently taking oral contraceptives and pain meds for headaches (has not taken today).
The possible causes of this patients headaches are: - VST: patient is at risk due to birth control. Pain is most likely due to stretching of the pain sensitive veins that drain into the sinus. Hypercoagulability and increased osmolarity are the main predisposing factors. - Subarachnoid hemorrhage: This should be HIGHLY suspected (worst headache of my life. May be due to trauma from soccer. Accompanied by the sudden onset of severe pain and vomiting. Pain persists and the patient looks very ill. May also have nuchal rigidity. - Epidural hematoma: Can be due to trauma of the side of the head. Important to order a CT: to identify a subarachnoid hematoma. MRV: this will identify a VST. Xanthachromia: this will identify a subarachnoid hematoma (this will be identified on an LP).
Describe the pathophysiology of migraines.
The trigeminal nerve (innervates the meninges) it intricately involved in migraines. There is a connection between cortical spreading depression and activation of trigeminal nerve affarents. Triggering of cortical spreading depression leads to long lasting increase in blood flow within the middle meningeal artery which is dependent on activation from the trigeminal nerve through release of vasogenic substances (substance P, neurokinin A and CGRP). Aura: note that the aura is much more specific and a diagnosis of migraine may be made on the basis of an aura. Note that migraines without aura are much more common. The most common symptoms involve vision, somatosensory and language. Unilateral location, pulsating quality, moderate-severe pain. Nausea and/or vomiting, photophobia and phonophobia. Fully reversible aura symptoms (lasting 50-60 minutes). Exclude a TIA.
Describe metastasis of tumors in the CNS.
There are many tumors that metastasize to the brain and the spinal cord. Lung, breast, and melanoma are the most common (note that prostate cancer rarely mets to the brain). Lymphoma is a form of cancer that can either originate in the brain or metastasize to the brain. Patients with severe immunosuppressed (HIV), are at high risk for primary CNS lymphoma.
Describe generalized seizures.
There is abnormal electrical activity of the entire cerebral cortex at once. Presumed that the generation of the seizure arises outside the cortex (RF or thalamus). Patients will have loss of consciousness, tonic contraction, expulsion of air against a closed glotis, emptying of the bladder, large pupils and erratic pulse. The most common syndrome of epilepsy is JME: there are several genetic abnormalities that result in a common clinical picture. Note that the EEG is characteristically abnormal, even in between episodes. Shown as spike-wave. Note that alcohol and sleep deprivation are triggers for JME. JME is a lifetime illness. There may be many years seizure free with medication (Valproate, Leviteracetam, Topiramate, Lomotragine), but will reoccur with drug cessation. Note that carbamazepine can worsen these patients. Many metabolic induced seizures fit the grand mal category: ionic abnormalities, sedative withdrawal in addicts, hypoglycemia, hypoxia and hyperthermia.
64 year old patient complains of 6 week history of progressive confusion, right sided weakness, poor coordination and trouble reading. She has also had difficulty walking for 10 days. There is a right homonymous hemianopia. Inability to read anything including things she had written. Right lower facial weakness. Right upper extremity pronator drift. Hyperreflexia on the right with upgoing toes.
There is upper motor neuron weakness (Babinski) involving the left primary motor cortex. Alexia without agraphia (can recognize words but can write) and right homonymous hemianopia (vision loss of the right field) which indicates occipital lobe involvement and splenium of the corpus callossum. The splenium is the region that transfers visual information between the two hemispheres. She can see the left visual field but cant relay the information to the cortex. Tumor is most likely based on the course (primary or metastatic). Steroids (dexamethasone) can reduce vasogenic edema and improve symptoms, chemotherapies (temazolamide), radiation or surgical resection are treatment options.
Describe Fungal meningitis.
These are chronic and is found in patients that are immunosuppressed such as cancer or AIDs patients. The most common fungus is Cryptoccocus neoformans. Patients often have a headache and a low grade fever. These symptoms are usually more mild and less acute. It is more common for patients to present with fatigue and malaise early on. This often results in more cognitive defects that result over the period of several months, often presenting as confusion or dementia. CSF pressure may be elevated. WBCs will be increased, mainly consisting of lymphocytes. Protein is VERY high and glucose is low. India ink prep is used to visualize the pathogen. Think fungus if the patient presents sick, confused with yellowish CSF.
Describe Intracranial Tumors.
These are classified according to the cell of origin. CNS neoplasms are seen in all age groups, but the frequency of various types differ with age. - Posterior cranial fossa, cerebellar and spinal cord predominate in children. - Middle and anterior cranial fossa tumors predominate in adults. Metastatic CNS tumors are uncommon in children. Breast, lung and melanoma are common mets, seen in adults. Note that these are all stage 4 at diagnosis and contain sharp edges. Gliomas are the most common primary intracranial tumors. Astrocytomas (glioblastoma multiforme) is the most aggressive.
Describe epidural hematomas.
These are most often arterial and are usually die to transection of the middle meningeal artery. Trauma is associated with concussion and loss of consciousness. The patient may awaken from this (lucid interval) and then loose consciousness again from brainstem distortion. Surgical evacuation is critical.
Describe Primitive Neuroectodermal Tumors.
These are most often seen in choldren and young adults. They are common in the posterior fossa (esopecially in the area of the cerebellum and the fourth ventricle). These are often called "small blue tumors", because they have a heavy concentration of nuclei on pathologic specimens, giving a strong appearance of basophilic blue dots. They are sensitive to chemotherapy and radiation but are not completely curable in most cases. Some of the particular tumors in this category include pineal tumors and meduloblastomas. Rosettes typical.
Describe petit mal (absence) seizures.
These are seizures of childhood that are thought to originate in the upper brainstem. Patients show many short episodes (few seconds) of staring spells of which the patient has no memory. These can be brought on by hyperventilation. The eyelids may flutter and patient may exhibit chewing. There is a spike-wave pattern seen on EEG (this may indicate why the absence seizures are typically negative symptoms). Spike represents diffuse depolerization. Wave represents diffuse inhibition (this is adequate to prevent behavioral symptoms). Note that most children have spontaneous remission.
Describe infantile spasms and age dependent epileptic encephalopathies.
These carry a high rate of permanent cognitive impairment and there is a poor response to anti-epileptic medication. This is very serious and affects children between 4-18 months. Caused by trisomy abnormalities, brain malformation, brain injury (birth hypoxic-ischemic brain injury), neurocutaneous disorder or metabolic abnormalities. There may be tonic, atonic or mixed, ranging from head drop to massive axial flexion spasms. Seizures occur in series, separated by a few seconds with the infant crying in between. Sometimes described as jacknifed or lightning spasms. EEG is characteristic showing a pattern called hypsarrhythmia (chaotic high amplitude poorly synchronized activity, multifocal spikes). Treat patients with ACTH (other standard antiepileptic drugs are unsatisfactory). There is a poor prognosis.
Describe Ependymomas.
These derive from the lining tissue of the ventricles and the central canal of the spinal cord. They have a tendency to be seen in younger patients. Some of these tumors are completely resectable but most require adjuvant treatment. Cells form perivascular formations with nuclear free zones.
55 year old noted memory and word retrieval difficulties a couple months prior. Has has low energy, has been less motivated and has been sleeping for longer periods of time. Family has noted "personalty" change. Has been having increased headaches over the last month which are different than his typical migraines (milder and not associated with photophobia or phonophobia or nausea). They would awaken him from sleep. He had a sudden onset of right arm jerking followed by loss of consciousness which is the reason for visit. Had depressed mental status, bilateral pailledema, mild facial droop, hyperreflexia, and positive Babinsky.
This can be localized to the left frontal lobe. Tumor is most likely based on the timing. This can either be primary or secondary. Intraaxial (Glioma, metastisis). Extraaxial (meningioma). Seizures are the initial manifestation of 25% of adults with neoplasm and localize the tumor to the cerebral hemisphere. A focal seizure may localize the tumor.
A 15 year old high school student is having difficulties with balance for the past month. Was also noting to have mild headaches, especially in the morning. He developed a rather sudden onset of severe headache and vertigo and nausea. He was unable to walk. He had vertical nystagmus with the left eye intorted towards the nose. He was unable to look up. pupil was 3mm, equal and mildly reactive to light. There was dysmetria with finger to nose and heal to shin testing bilaterally. There was a positive babinky sign.
This can be localized to the posterior fossa: cerebellum and brainstem. Pressure on the dorsum of the midbrain can result in parinauds (paralysis of upgaze). Pressure on the brainstem is affecting the upper motors (Babinky). Headache is most likely due to ventricular obstruction and internal hydrosephaly, The type of nystagmus suggests a central lesion of the vestibular system (vertical). Important to image the brain, MRI with and without contrast.
7 month old infant is seen in the clinic for recurrent spells. Parents describe multiple episodes where his head falls forward and he has flexion at the waist and abduction of his arms. The events seem to cluster several times daily. The child has decreased babbling over the past couple weeks and is not interested in toys.
This case is consistent with infantile spasms. This is a generalized epileptic encephalopathy. There is also evidence of loss of developmental milestones (cortical dysfunction).
Patient was found unresponsive. Patient takes lisinopril and sertraline as well as ativan (benzo). Patient had shallow regular breathing that deteriorated into apnea. Pupils were 2mm, sluggishly but symmetrically reactive to light. There was no purposeful response to either command or nose tickle. VOR appeared intact.
This case is indicative of metabolic/toxic encephalopathy (the brainstem is more resistant). The deterioration of the breathing indicates that the patient is getting worse. The pupillary light reflex is usually the most resistant to toxic/metabolic/drug related depression and is typically the last reflex to be detected. Stupor and coma normally present in depression of function in both cerebral hemispheres in metabolic abnormality or toxic insult.
38 year old female woke up 2 weeks ago with horizontal double vision. It would resolve when she closed her eyes. She suffered from episodes of painful vision loss in her left eye five years prior. It was suggested that she may have had optic neuritis (initial isolated symptom). She developed burning and tingling sensation involving her left arm, trunk and leg about a year ago. Red images appear washed out on the left (color desaturation). The left eye does not cross the midline on lateral gaze. There is also right beating nystagmus of the right eye. There is hyperreflexia on the the left. MRI revealed several periventricular and juxtacortical hjyperintensities and one small pontine hyperintensity that do not enhance. Cervical spinal cord demonstrated intramedullary hyperintensity. CSF analyzed showed: 5 oligoclonal bands, an IgG index of 1.7%, and a myeline basic protein level of 2.8. There was normal cell count, protein and glucose.
This clinical history is suggestive of relapse remitting MS. There is activation of T-helper cells (some reason), which become hyperactive and aggressive. They are attracted to and adhere to the vessels of the BBB. This causes a breakdown of the BBB and perivascular invasion of the CNS. They then encounter antigens in the CNS and mount an immune attack against the myelin. Most cases present with numbness, tingling, parasthesias, blurring of vision or mild weakness which is due to slowing of conduction rather thanm complete block. Treatment: consider IV steroids for acute treatment of the exacerbation. You should also consider first line MS treatment for this patient: - Beta-interferon (modulates lymphocyte function, reverses BBB disruption and alters expression of a number of cytokines). - Galatamir acetate (functions as an altered peptide ligand, where it stimulates regulatory T-cells that will suppress the immune reaction in the CNS). - Natalizumab is also an effective treatment but can cause reactivation of the JC virus.
30 year old patient is presenting to the clinic for evaluation of headaches with associated changed in vision. She has had headaches for several years where the headaches have increased in frequency recently and are now daily. They are moderate-severe and often throbbing in quality. She has intermittent double vision and vision becoming grayed out. Her headaches are sometimes associated with nausea and photophobia. She currently takes occasional ibuprofen. Patient is overweight. Fundoscopic exam is indicative of increased intracranial pressure (optic disk swelling bilaterally).
This differential for this patient is: - Migraine: There are red flags, so other possibilities must be ruled out first. - Idiopathic intracranial hypertention: Overweight, female, childbearing age, vision problems. Hallmarks include increased intracranial pressure (evaluated on LP), CN 6 palsy and risk of vision loss. Note that this needs to be treated ASAP. - Tumor: This should be considered when there is an increase in intracranial pressure. She does not smoke so met is unlikely. - VST: This should always be considered when there is an increase in intracranial pressure. Note that this usually presents abruptly.
A 62 year-old patient presenting with difficulty using her right arm over the last 6 months. She states that she has noticed increasing difficulty buttoning clothes and that her handwriting is becoming more "cramped". Her right arm also feels "tight" and often aches later in the day. She says that her balance is "OK for my age" and that her left arm is fine. She denies any history of tremor. She has no significant past medical history and does not take any medications. Family history is negative for neurological disease. On examination, She holds her right arm slightly flexed and stiffly against her side when walking. She is unable to button her shirt with her right hand alone, although she can accomplish this when using either both hands or just her left hand. Attempts at rapid, repeating movement (tapping her fingers) are performed much more slowly with her right than with her left hand. Her strength and reflexes are normal in all limbs, and there were no abnormal movements noted. Her right arm resisted passive movements and she did not appear to be able to relax it during examination. Muscles around her right shoulder and upper arm were somewhat tender to palpation. She walks with a narrow base with a shortened stride length and decrease arm swing on the right. She has mild postural instability on retropulsion testing (i.e., when you stood behind her and pulled her back toward you). There were no sensory abnormalities and cerebellar exam was normal.
This is Parkinsons disease
Describe motor neuron disease.
This is a general term for conditions that result in degeneration of the anterior horn cells and upper motor neurons of the cerebral cortex that give rise to the descending tracts. Lower motor signs (atrophy, fasciculations and decreased reflexes). Upper motor signs (spasticity, increased reflexes and up going toes). Most patients have signs for both upper and lower motor disease and are diagnosed with amyotrophic lateral schlerosis (ALS). ALS affect older adults in the 50s-70s. Note that weakness is of two types and may present very differently in the arms and in the legs of a single patient. The arms may show more lower motor symptoms, where the legs may show more upper motor symptoms. The disease starts unilaterally but progresses to involve many muscles bilaterally over few months. Note that there is no good treatment for ALS, but, riluzole does slightly delay the progression of the disease. The average patient survives 3-5 years with death resulting from weakness of the respiratory muscles.
Describe Creutzfeldt Jakob Disease.
This is a prion disease that produces spongiform changes in the brain. This is seen as a large number of holes on histological examination. This can occur spontaneously or can be transmitted through contact with infected material. There is a progressive course with prion disease, where the outcome is rapidly progressive dementia with no treatment. The CSF is normal unless tested for specific proteins. MRI imaging often shows abnormalities on diffusion weighted scans, consisting of hyperintensities or white spots in the thalamus and posterior parts of the brain. Patients may also have myoclonic jerks in addition to the dementia. Ataxia is another common clinical feature.
Describe locked in syndrome.
This is a state of profound paralysis (not a disorder of consciousness). Produced when there is a large infarction or hemorrhage in the pontine tegmentum and base producing paralysis. Patients present with pinpoint pupils. ALS, Guillain-Barre or polyneuropathy patients can experience locked in. NOTE that these patients will be able to communicate with vertical eye movement.
Describe Giant cell (temporal) arteritis.
This is a systemic vasculitis which rarely occurs in patientsunder 50. The clinical picture may include: - Polymyalgia rheumatica (malaise, loss of energy, proximal joint pain). - Non specific headaches: sometime associated with tenderness over the temporal artery. - Evidence of arterial insufficiency. There may be unique symptoms of jaw claudication or infarction of the tongue or scalp. There may also be retinal ischemia and blindness. Lastly, there may also be infarction of the brainstem, cerebellum, and occipital lobe. The treatment is with steroids. The ESR is usually VERY high and temporal artery biopsy is diagnostic (need to collect one inch).
Describe Idiopathic intracranial hypertention.
This is also called pseudotumor cerebri. Should be considered in obese females of childbearing age. LP is used for diagnosis . This effects vision (papilledema). There is no mass seen on imaging. You should always consider cerebral venous sinus thrombosis first. This is believed to result from a decrease in CSF resorption, with pressure buildup. It is most often identified with findings of papilledema but imaging is negative for an associated mass. High estrogen is believed to bredispose this disease, thus you should also consider polycyctic ovarian disease. Exogenous estrogen, Vtiamin A and tetracyclines are also associated as predisposing factors.
Describe the minimally conscious state.
This is altered consciousness characterized by a profound lack of responsiveness but partial intermittent evidence of awareness of self and environment. These patients are likely to respond to environmental and sensory stimuli. Note that Levodopa or dopamine agonists (parkinsons medications) lead to an increase functioning in minimally conscious patients.
Imaging Arachnoid mater
This is apposed to the dura and is tightly bound to the venous sinuses due to the arachnoid granulations. Subdural space is the potential space between the arachnoid and the dura. The spece between the arachnoid and the pia is called the subarachnoid space and contains the CSF.
Subdural hematoma
This is between the arachnoid and the dura (is a potential space). Appears as a hyperdense crescent collection. May extend along the falx and the tentorum. Will lead to a midline shift. Typically caused by tearing of the bridging veins. Typically countercoup.
Epidural hematoma
This is between the dura and the bone. Appearance is biconvex (does not cross structures. Note that there is a good prognosis. This typically involves the middle meningeal artery and is associated with fracture. Injury is croup.
Subarachnoid hemmorhage
This is between the pia and the arachnoid (location of the CSF). This will appear as whiteness on outside of the brain, in sulci.
Cytotoxic edema
This is caused by cell death in the cortex. Note that the cortex becomes less dense (hypodensity) (this makes sense becuse you are loosing tissue). This is indicative of a stroke!
Describe Neurosyphilis,
This is caused by treponema pallidum. The primary infection is usually a painless ulcer, called a chancre. The spirochete can produce chronic damage after several years of latency. It can attack the brain producing chronic encephalitis which represents tertiary syphilis dementia. It can also lead to chronic meningitis. Lyme disease can also lead to this clinical picture late in the disease.
Describe increased intracranial pressure.
This is characteristic of many intracranial masses and it is related to tumor size, edema, rapidity of growth, ventricular system obstruction, rate of CSF absorption, venous obstruction. Headache is the most common symptom associated with an increase in intracranial pressure, which occurs in many people that have rapidly expanding masses and less often with slow growing tumors. The headache is often worse in the morning after the patient has been horizontal for an extended amount of time (increased edema). Nausea and vomiting are also common. The most easily observed and telling sign of increased intracranial pressure is papilledema, swelling of the optic nerve head, which is associated with loss of retinal venous pulsations and enlargement of the blind spot on visual field testing.
Describe brain death.
This is the irreversible cessation of brain functions.Most cases result from massive traumatic brain injury, intracranial hemorrhage, meningitis, or diffuse hypoxic-ischemic neuronal damage from cardiac arrest or asphyxia. Intracranial pressure becomes greater than arterial blood pressure and cerebral blood flow stops. Four cardinal criteria: Deeply comatose, unresponsive to stimuli, absent pupillary light reflex, ventilator dependent. Irreversable loss of brain function.
Describe peripheral vertigo.
This is due to damage to the inner ear or to the 8th cranial nerve. Abnormal vestibular functions normally produce abnormal eye movements. It is specifically associated with jerk nystagmus. The nystagmus is in one particular direction (named for its fast component (right nystagmus)). Nystagmus will always be wore when the person moves their head towards the fast phase. There may be hearing loss or tinnitus due to damage of the auditory mechanism. There should be no other signs of damage to the nervous system (inability to walk is due to severity of the vertigo). You can typically predict how severe the vertigo is by how severe the nystagmus is.
52 year old female with progressive upper and lower extremity weakness. She has difficulty walking up stairs and lifter her arms. She has been taking a low dose statin for 5 years. She has constipation and some redness over her chest and neck over the past few months. She has a mild erythematous rash. ESR is elevated at 80, CK is high at 4000.
This is indicative of Dermatomyocytes (myopathy). Myopathy: proximal weakness, normal sensation, no hyperreflexia. Evaluation of weakness is seen when asking patients to rise out of a chair or holding arms up against gravity. Note that there is a statin myopathy, but this is rare considering that the patient has been on treatment for 5 years.
48 year old presents with 4 month history of slowly progressive weakness and generalized fatigue. She is having difficulty turning in bed, walking upstairs, opening jars, and knitting. She also feels unsteady on her feet (especially at night). She describes tingling in her hands and feet. Sensory exam shows impaired vibratory and joint position sense in the toes and impaired vibratory sense in the fingers. Altered pin prick and temperature sensation. Reflexes are absent throughout. Rhomberg is positive. She has high arches. She also has type 2 diabetes.
This is indicative of peripheral polyneuropathy. This is a common condition where the longest peripheral nerve fibers are usually affected first. The affect can be seen in the axon, myelin or both. It is usually symmetrical. The nerves to the lower extremity are the most reliant on a good supply of metabolic substrates and also have the greatest exposure to toxins. Loss of sensation (numbness) is the most common complaint. Prickling, tingling or burning are also commonly described. Many patients that with large fiber damage in the feet complain about balance trouble (and have a poitive rhomberg sign). Such patients may have difficulty walking in the dark. Diabetes is the single most common cause. Alcohol intake can also cause polyneuropathy (this is most likely due to deficiency in the B vitamins). HAART medication and antiseizure medication can be neurotoxic and lead top polyneuropathy. Infections such as tertiary syphilis, HIV, and lymes disease are also causers of the disease. Note that there is also familial peripheral neuropathies: Charcot-Marie-Tooth Disease (CMT) Note that most familial neuropathies are progressive over many years. Distal weakness, sensory loss, high arches and hammer toes are typical signs.
Describe Tuberculosis meningitis.
This is more common in children and the immunosuppressed (AIDs) and typically represents reactivation of the bacteria. Patients usually have low grade fever, headaches and night sweats. The picture is confusion or disorientation that can be confused with progressive dementia. Hydrocephalus with increased intracranial pressure is common due to decreased CSF flow at the base of the brain.
25 year old male has 5 year history of slowly progressive weakness. He is a thin man with with decreased muscle bulk throughout. Neuro exam is positive for 4/5 hip flexion. Shoulder abduction is 4+/5. He has mild difficulty getting out of a chair. His mother was also thought to have muscle weakness.
This is most consistent with muscular dystrophy (there is a slow progression and a family history). DDx includes limb girdle MD, Beckers MD (X linked). Note that the age of onset is not consistent with Duchene MD. This patient will need an EKG and Echo (risk of arrhythmia and heart failure in myopathy).
A nine year-old boy is brought in by his mother after a teacher complained that the boy was continually moving and "making faces" as well as engaging in disruptive behaviors. These included inappropriate, repetitive touching. He had been diagnosed with ADHD in the past year and started on a stimulant medication (methylphenidate) upon going back to school one month ago. His academic performance had worsened somewhat over the prior two years and that was what prompted the evaluation that led to the diagnosis of ADHD. Examination: He was very "fidgety", with "twitching"-type movements that continued even when asked to hold his hands steady out in front of him. He also had frequent grimacing. Many of these movements repeated themselves at irregular intervals. He appeared to fixate on the otoscope and, when asked not to touch it, continued to reach out for it. He frequently cleared his throat and sniffed even though his mucous membranes were clear.
This is most likely Tourette syndrome.
55-year-old surgeon, reports a 3 year history of increasing tremor with his right hand while drinking coffee, eating peas with a fork, writing, and making surgical incisions over the past year. He also described shaking in his right arm when operating the TV remote. His wife calmly stated that, "When he's relaxed he never shakes, but I've seen both his hands do it when he plays cards." Better control after drinking alcohol. Fatigue or psychological stress seemed to make the tremor worse. He has no significant past medical history and takes no medications. There is a family history of similar tremors in his father and paternal grandmother. Tremor on asking the patient to hold both arms outstretched before him, worse on the right. Accentuated tremor on both sides when asked to touch his nose and then the examiner's fingertip repetitively in sequence several times. No dysmetria was noted. No tremor when sitting quietly or when walking with the hands by the side.
This is most likely an essential tremor.
40 year old patient recently back from a business trip to India is presenting with numbness and tingling in her legs. She feels generally weak but attributes this to a recent illness with diarrhea and poor appetite.
This is most likely polyneuropathy due to campylobacter infection leading to Guillain-Barre Syndrome. This is an immune demyelinating disease. This is unique due to its rapid course and severe weakness, produced by immune attack on myelin. It is critical to recognize this disease because it can quicly involve the respiratory muscles leading to death. REFLEXES are usually lost early. Protein is markedly elevated in the CSF, where there are few, if any WBCs in the CSF.
Describe subdural hematoma.
This is typically due to disruption of veins that are bridging from the brain to the venous sinus. This is more likely to occur in older patients. The hematoma tends to grow SLOWLY because the veins are a LOW pressure system. The signs and symptoms will develop over days to even months. Therapeutic anticoagulation predisposes to subdural hematomas. The presenting picture may be depression of consciousness, difficulty with cognitive function and chronic headache.
Describe cental vertigo.
This is typically milder then peripheral vertigo and much less common. Note that the amount of nystagmus is usually greater than the patients illusion of movement. The nystagmus may be in multiple directions, including in the vertical direction. Central vertigo is NOT associated with hearing loss and there are often abnormalities in the nervous system.
Patient brought to the ED. She had been complaining of episodic jerking and twitching of the right hand and arm over the past week. The jerking had progressed to the face and leg where she then blacked out. She then began to shake all over. She was weak in her right arm as she came out of it. She complained of a sore tongue and had lost control of her bladder. She has been experiencing dull left temporal headache that is worse in the morning.
This patient had clonic activity of her right arm and hand (simple partial seizure) followed by a secondary generalization with tonic-clonic activity (tongue biting and urination are indicative). Jacksonian march: this is progression of motor symptoms from the upper extremity to the face, trunk and lower limbs (spreading of seizure from the focus). Note that seizures can generalize from either the corpus callosum or the rostral brainstem. Postictal depression can occure after the focal seizure and involves the area of cortex that is most involved: this is known as Todds paralysis if the motor cortex is involved. Any type of mass lesion (tumor, vascular malformation, or abscess can lead to this), cerebrovascular disease or TBI can also result in a focal seizure. Treat patient with Carbamazepine, Levetiracetam, phenytoin.
43-year-old patient was referred for neurological consultation. She described herself as being "nervous and fidgety," and volunteered that she had had "uncontrollable movements" of her face and arms for at least three years. These abnormal involuntary movements have slowly increased in severity. Her family has not observed them when she is asleep. There were no other aggravating or ameliorating factors. She also complained that her thinking was not as clear as in the past. Past medical history is notable for anxiety and irritable bowel syndrome. She does not take any medications. She was taking metoclopramide a few years ago but only took it occasionally for nausea. Patient exhibited: Continuous, irregular, jerky, uncontrollable movements of her face, tongue, jaw, head, and upper extremities. Ability to recall only 1 of 3 items after 5 minutes. Her clock drawing was impaired. Intact power and sensation in all four limbs and the face bilaterally. Normal finger nose finger and heel to shin testing, Normal deep tendon reflexes and gait.
This patient has Huntingtons disease.
18 year old highschool student is brought to the clinic due to episodic morning episodes of throwing his juice glass. The muscle jerks were isolated but sometimes occurred in clusters. There was no warning or aura. He had full awareness during the episodes. Parents described brief staring spells when he was a child that the patient was not aware of. Patient had his first generalized convulsive seizure during photic stimulation.
This patient has a history of myoclonic jerks as well as one generalized tonic-clonic seizure. This most likely represents Juvenile Myoclonic Epilepsy (JME). The patient has a history of Absence seizures (staring spells). There is a high genetic occurrence, where there will often be a mutation in the GABA receptor.
60 year old patient presents to the clinic with asymmetric progressive rhythmic movements of the of right arm while at rest, which seems to get better when the patient moves, but there is decreased movement of the left arm with walking. Patient also presents with insomnia, decreased facial expressions, and shuffling. These symptoms started 2 months prior and have steadily gotten worse. Biopsy of substantia nigra neurons reveals that there are cytoplasmic inclusion bodies consisting of synuclean.
This patient is presenting with signs of Parkinsons disease. This is usually sporadic, where there are loss of dopaminergic neurons in the substantia nigra pars compacta (direct and indirect pathway). There will be a loss of pigmented dopaminergic neurons as well as cytoplasmic inclusion bodies containing synuclean. There need to be 2/3 symptoms in order to make a diagnosis: - Static tremor: Rhythmic movement of the limbs noted with the limb in a wresting position that seems to get better with movement. - Bradykinesia: A decreased ability to initiate movement, may be noticed as weakness or "inability for signal to get to the limb". - Rigidity: this is an increase in resting muscle tone. Patients may also note difficulty with facial expressions, shuffling, freezing of gait. Dementia occurs in 20-30% of patients. Cogwheeling = rigidity + tremor. May progress rapidly where the median survival is 15 years (lengthened with treatment and without dementia). Neuroleptic drugs (risperidone, haldol) can cause parkinsons symptoms. Death occurs due to pulmonary infection and aspiration. Treat with Levodopa (converted to dopamine in the body). - Amantadine (antiviral medication used against pulmonary infections). - Deep brain stimulation over the subthalamic nucleus (indirect pathway).
45 year old patient presents with shaking when holding the hand out in a still position. She notices that the shaking gets worse when moving and grabbing for things. She notices that the shaking is present in both hands and is of the same character and magnitude. These symptoms started 3 years ago, but have slowly gotten worse. Exam shows difficulty with finger to nose test. Family history reveals that her mom and grandmother also suffer from shaking that is similar to hers.
This patient is suffering from essential tremor. This is autosomal dominant with incomplete penetrance. It can also arise sporadically. Usual age of onset is between 25-55. The disease is defined with the symptoms of: - Postural: shake when held in sustained position. - Intentional: worsens with action. - Symmetric. Can involve the hands, head or voice. Also, most patients have a cerebellar component. Disease will worsen with age, and is slowly progressive. Treatment with beta blockers (propranolol). Barbituates, benzos, topiramate. Some may require surgery.
55 year old patient describes waking up with severe dizziness. States that the dizziness is worse when she moves her head. Not aggravated by coughing or sneezing. Dizziness is a spinning sensation, she describes severe nausea and difficulty walking. History of tinnitus in both ears. Patient has right sided beating horizontal nystagmus. Has an ataxic gait, where she veers to the left.
This patient is suffering from severe peripheral vertigo. A left peripheral vestibular/labrynthine lesion can lead to right horizontal nystagmus (fast phase to the right) and patients often veer towards the side of the lesion. The most likely diagnosis is vestibular neuritis.
23 year old patient presents to the clinic for evaluation of headaches. Been having headaches 1-2 times per week for the last 2 years. They have been slowly increasing in frequency over the past 3 months and has 4-6 headache free days per month. Headaches have a throbbing quality, usually located over the left temporal and occipital regions. 7/10 intensity where the headache lasts most of the day. She gets relief from sleep. She often feels nausea and photophobia during the attack. Describes shimmering lights in the peripheral visual field that progress. She takes ibuprofen that used to be effective but is no longer.
This patients differential is: - Migraine with aura (episodic vs. chronic). - Medication overuse headache. - Note that a secondary headache is unlikely in this case. The clinical picture is most consistent with a medication overuse headache and chronic migraine with aura. Note that this picture does not fit with cluster headaches (duration of the headache). Chronic: greater than 15 days per month. Episodic: less than 15 days per month. Manage this patient: start with topamax, limit NSAIDs, and sumatriptan for acute treatment.
Patient with history of hypertention is found unresponsive. The patient takes hydrochlorithiazide and amlodapine. Respirations were very irregular and shallow. pupils were imm with questionable reactivity. Only eyemovements were brisk downward eye movements with slower return to midposition. VOR is absent. Corneal reflex is absent. There is no gag reflex, no motor response to nose tickle, positive babinski sign and normal spinal reflexes.
This presentation is indicative of coma-injury to the reticular activating system (RAS) it the pons (the most caudal extent). Pontine pupils indicate pontine localization with intact midbrain. Occular bobbing also indicates pontine localization. Irregular breathing (atixic) is also indicative of pontine localization. A pontine lesion (ischemia or infarction) should be suspected. These patients may have an expanding lesion that leads to brain death (apnea, increase in pupil size as well as absence of intracranial blood flow).
24 year old patient awoke with pain in her right eye and blurring of her vision. She notes that everything looks gray off to the right side of her right eye, while everything looks normal with the left eye. her right eye also hurts when she moves it. Visual acuity is 20/40 on the right. There was an afferent pupillary defect (APD) on the right (Marcus Gunn Pupil). Note that the fundiscopic exam is normal.
This presentation is most likely optic neuritis. This is aa clinically isolated syndrome which increases the risk of subsequent demyelinating episodes (multiple sclerosis). 1 episode of demyelination and 1 objective finding is associated with having a clinically isolated syndrome (these patients are at risk for MS). All criteria are met clinically with 1 objective change on exam with 2 symptomatic episodes or 2 objective changes on exam with 1 symptomatic episode (this is probable MS). All MS criteria are met clinically, with 2 episodes and 2 lesions (clinically defined MS). MS can be diagnosed on a single MRI scan showing multiple lesions ad different developmental stages (some enhancing and others not). High dose IV steroids are useful for an acute attack. Note that we are not good at determining when clinically isolated syndromes will develop into MS.
Vasogenic edema
This s caused by leaky capillaries (damage to the BBB). Note that the whit matter gets darker. Gray matter is accentuated. This is indicative of a tumor, inflammatory disease, hypertention.
Describe benign positional vertigo.
This should be considered if movement precipitates attacks. This is relatively common. This may develop spontaneously in the elderly or may be provoked by head trauma. Looking up is the classic head position that brings on an attack. Also bending over and rolling over in bed. Vertigo begins within suddenly after movement, reaches a maximum, and then resolves after 15sec - 1min. Treatment with cannilith re positioning maneuver is effective because this is probably due to loose otoliths in the inner ear. These are made of calcium carbonate crystals and may dissolve on their own. This will terminate the attacks, however, they can reform.
What are the causes of stupor and coma?
Traumatic brain injury*, contusion, hemorrhage, raised ICP. Neoplasm. Infections (meningitis and encephalitis). Brain abscess or empyema: sepsis or infection in the elderly or demented patient*. Infarction (brainstem, cerebellum, cortex). Metabolic encephalopathies*. Toxic encephalopathies* (alcohol, benzos, barbituates, opiods, anticonvulsants, anticholinergics, antipsychotics).
What are common non-localizing manifestations on neurologic exam that are associated with mass lesion?
Unilateral or bilateral sixth nerve palsies caused by rostrocaudal displacement of the brainstem with stretching of the abducense nerve. Third nerve dysfunction. Ischemia-infarction of the occipital cortex due to cerebral artery compression. Hydrocephalus secondary to tumor compression and occlusion of the fourth ventricles or foramen of manro, which causes diffuse hemispheric dysfunction that may obscure the local compressive and destructive signs of the mass itself.
Describe the evaluation of vertigo.
Vertigo is the illusion of movement (imbalance of signals to the vestibular apparatus). Peripheral causes: these relate to damage of the inner ear receptors or the the 8th cranial nerve. Central causes: these include damage to the centers that process vestibular signals in the CNS (mostly located in the brainstem, including vestibular nuclei and vestibulocerebellum). Note that a positive lateral head thrust test is indicative of peripheral vertigo.
Describe complex focal (partial) seizures.
results from partial seizure beginning in the frontal, temporal (less often in the parietal association). These manifest with behavioral, visceral and affective phenomenon (linbic cortex has the lowest threshold). This is a common seizure disorder (structures are vulnerable to metabolic injury). There may be peculiar/bad smells, bizzare abdominal sensations, fear, anxiety. As well as lip smacking, chewing or sniffing. The hippocampus is not functioning properly: this type of behavior is associated with memory loss (note that its usually due to other causes than the actual seizure). Note that there is collateral inhibition that prevents generalization in some patients, where other patients will generalize. It is important to order an MRI and EEG. Treat with Levetiracetam, Lamotragine, Carbamazepine, Valproic acid.
Key points for imaging a stroke
start off with non-contrast CT. There are 4 signs: dense MCA, loss of ray/white matter differentiation, loss of insular ribbon, effacement of sulci. Stroke is a clinical diagnosis!
