Neuro Vignettes

A 51-year-old man presents to the emergency department due to headache, nausea, and pupillary abnormalities after a physical altercation. The patient was in his usual state of health until there was a fight that resulted in head trauma. Medical history is significant for hypertension and chronic alcohol abuse disorder of over 15 years, which is treated with hydrochlorothiazide and disulfiram. His blood alcohol level is 0.32%. On physical examination, the patient appears confused and a dilated pupil that is unresponsive to light. A non-contrast head CT is shown.

Acute subdural hematoma

A 78-year-old right-handed male is brought in by ambulance after being found down in his home. After being aroused, the patient has difficulty answering questions and appears to be frustrated by his inability to communicate. He is able to speak his name and a few other words but his speech is not fluent. Subsequent neurologic exam finds that the patient is able to comprehend both one and two step instructions; however, he is unable to repeat phrases despite being able to understand them. He also has difficulty writing despite retaining fine motor control. CT reveals an acute stroke to his left hemisphere. Damage to which of the following sets of structures would be most likely to result in this pattern of deficits?

Broca Aphasia

A 79-year-old man with a long-standing history of hypertension and diabetes presents to the emergency department with sudden-onset of loss of motor function on the left side of his body. Neurologic exam shows normal sensation throughout the body. MRI of the brain is obtained and shows a small sub-cortical infarct involving a perforating vessel. Which of the following is the most likely underlying pathogenesis?

This patient has a lacunar infarct, which is most often caused by lipohyalinosis of small perforating vessels predisposing to occlusion.

An 82-year-old right-handed woman is brought in by ambulance after being found down in her home. On presentation, she is found to be awake but does not follow directions or respond to questions. She is able to speak and produces a fluent string of nonsensical words and sounds. She does not appear to be bothered by her deficits. Subsequent neurologic exam finds that the patient is unable to comprehend any instructions and is also unable to repeat phrases. CT scan reveals an acute stroke to her left hemisphere.

Wernicke aphasia

A 78-year-old man presents to his primary care clinic and is accompanied by his wife. He feels well, but his wife is concerned that he has become more confused over the past few weeks. He enjoys socializing with family and friends, but sometimes has difficulty finding words and recently forgot his niece's name. He walks around his neighborhood for exercise, but has the tendency to wander and get lost. He has a history of hypertension and atrial fibrillation. Current medications are warfarin and dronedarone. Physical exam is unremarkable. Recent lab results were within normal limits.

Alzheimer Disease

A 61-year-old male presents with left-sided hand weakness and trouble with walking. He is not sure why these symptoms occur. On physical exam, tongue fasciculations are appreciated. He has slow speech. The left upper extremity shows forearm atrophy and depressed reflexes. The right lower extremity is hypertonic, with 3+ reflexes and positive Babinski sign.

Amyotrophic Lateral Sclerosis

A 61-year-old man is brought to the emergency room with slurred speech. According to the patient's wife, they were watching a movie together when he developed a minor headache. He soon developed difficulty speaking in complete sentences, at which point she decided to take him to the emergency room. His past medical history is notable for hypertension and hyperlipidemia. He takes aspirin, lisinopril, rosuvastatin. The patient is a retired lawyer. He has a 25-pack-year smoking history and drinks 4-5 beers per day. His father died of a myocardial infarction, and his mother died of breast cancer. His temperature is 98.6°F (37°C), blood pressure is 143/81 mmHg, pulse is 88/min, and respirations are 21/min. On exam, he can understand everything that is being said to him and is able to repeat statements without difficulty. However, when asked to speak freely, he hesitates with every word and takes 30 seconds to finish a short sentence.

Anterior cerebral artery and middle cerebral artery watershed area

A 17-year-old man presents to the emergency department after a physical altercation that resulted in a stab wound. He was stabbed at level T12 in the back. On physical examination, there is significant muscle weakness affecting the whole right lower extremity. There is also impaired vibration and proprioception sense of the right leg and loss of pain and temperature sense of the left leg.

Brown-Sequard Syndrome

A 21-year-old male presents to the ED with a stab wound to the right neck. The patient is alert and responsive, and vital signs are stable. Which of the following neurologic findings would most likely support the diagnosis of right-sided spinal cord hemisection?

Brown-Sequard syndrome, is characterized by ipsilateral loss of tactile, vibration, and proprioception loss, contralateral pain and temperature sensation loss, and ipsilateral paresis.

A 75-year-old woman with a history of stroke 1 year ago was found unconscious on the floor of her home by her son. The patient was brought to the emergency department by ambulance but expired prior to arrival. An autopsy was performed and showed the cause of death to be a massive ischemic stroke. The coroner also examined sections taken from the area of her prior stroke. Which histologic finding would be prominent in the area of her stroke from one year prior?

By approximately one month after an ischemic infarct, the involved area will have formed a glial scar, which histologically appears as a cyst formed by astrocyte processes.

Neuro Vignettes An infant boy is brought to the emergency department due to lethargy and enlarging head circumference. The parents deny any recent infections or trauma to the patient. They report he had 2 episodes of emesis and is inconsolable. On physican exam, head circumference is larger than expected. An MRI of the head is performed, which shows aqueductal funnelling and enlargment of the lateral and third ventricles.

Congenital aqueductal stenosis

A 32-year-old woman is brought to your office by her husband. The husband says that she had been acting strange lately. She has been forgetful, and she sometimes becomes angered for no reason, which is highly unusual for her. She has also been having random, uncontrollable movements, which are also new. On examination, she appears withdrawn and flat. On further questioning, she reveals that her father died at age 45 from a movement disorder. Which of the following is the pathological hallmark of the patient's condition?

Huntington's disease is a progressive neurodegenerative disorder characterized by loss of neurons in the caudate nucleus and putamen.

A 40-year-old male with Down syndrome is brought to your clinic by his mother. She reports that over the past few months he has started having difficulty managing his daily routine at his assisted-living facility and no longer seems like himself. She says that last week he wandered away from the facility and was brought back by police. Additionally, he has stopped taking his regular antiepileptic medication, and she is concerned that he might have a seizure. TSH is checked and is normal. Which of the following is most likely to be responsible for this man's current presentation?

Down syndrome occurs due to trisomy of chromosome 21, which also encodes the beta-amyloid precursor protein. Alzheimer disease occurs at an earlier age in patients with Down syndrome due to abnormal metabolism and accumulation of beta-amyloid protein in amyloid plaques.

A 42-year-old male presentswith confusion, headache, and fever. The patient is unable to answer questions. A head CT is negative for a space-occupying lesion or hemorrhage. An MRI is shown. A lumbar puncture is performed, with cerebral spinal fluid (CSF) analysis showing a lymphocytic pleocytosis and normal glucose. PCR of the CSF is positive for HSV-1.

Encephalitis

A 72-year-old female is brought in by ambulance after being found down in her home. Her daughter discovered her after returning from work and does not know how long she has been down. Physical exam reveals right sided paralysis with a positive babinski sign. She is also found to produce strained stuttering speech with no perceivable meaning. She is unable to follow any instructions and cannot repeat speech. An MRI is obtained showing a left MCA infarct.

Global Aphasia

A 32-year-old man presents to the emergency department with worsening lower extremity weakness. The patient was in his usual state of health until approximately one week prior to presentation, where he developed increased difficulty climbing the stairs. Now he is unable to stand. A few weeks ago he developed bloody diarrhea, which he attributes to drinking unpasteurized milk. On physcal examination, he has bilateral lower extremity 1/5 strength with absent patellar reflexes.

Guillain-Barre syndrome

A 25-year-old man presents to the ER after a street fight. His girlfriend reports that he was struck with a bat in the head. After the initial hit, she reported that he briefly lost consciousness but then was normal for several hours. Since then, he has been rapidly decompensating. He is grabbing his head in pain and his mental status is altered. Vital signs are stable. Neurological exam reveals that his right pupil is dilated and his right lid is drooping. If this patient's symptoms are allowed to progress without intervention, which of the following would be expected to occur?

Herniation of the uncus

An 81-year-old woman is brought to the emergency room by her son after witnessing the patient fall and hit her head. The son reports that the patient was in her usual state of health until she complained of chest palpitations. This startled her while she was climbing down the stairs and lead to a fall. Past medical history is significant for hypertension and atrial fibrillation. Medications are lisinopril, metoprolol, and warfarin. Temperature is 99°F (37.2°C), blood pressure is 152/96 mmHg, pulse is 60/min, respirations are 12/min, and pulse oximetry is 98% on room air. On physical examination, she is disoriented and at times difficult to arouse, the left pupil is 6 mm and non-reactive to light, and the right pupil is 2 mm and reactive to light. A right-sided visual field defect is appreciated on visual field testing. There is 1/5 strength on the right upper and lower extremity; as well as 5/5 strength in the left upper and lower extremity. A computerized tomography (CT) scan of the head is shown. Which of the following most likely explains this patient's symptoms?

Herniation of the uncus

A 20-year-old man presents with a two-year history of motor restlessness that has progressed to uncontrollable choreiform movements. He has moderate dementia with a severe gait disturbance as well as agitation and problems with his mood. MRI scan of the brain shows atrophy of the cerebral cortex and caudate nucleus

Huntington Disease

A 27-year-old gentleman is brought into the ED after being stabbed in the back by a knife. In addition to the pain from the wound, he complains of weakness in his left leg. Upon physical examination you find that he has no other visible injuries; however, he has 2/5 strength in the left lower extremity. Complete neurologic exam also finds a deficit in vibration sense and light touch on the left lower extremity as well as a loss of pain and temperature sensation in the right lower extremity. Which of the following lesions would result in the syndrome described?

Ipsilateral weakness and loss of light touch and proprioception associated with a contralateral loss of pain and temperature sensation is characteristic of Brown-Sequard syndrome. The weakness in Brown-Sequard is ipsilateral to the lesion, so the answer is left cord hemisection.

A 16-year-old woman presents to the emergency department with a recent convulsive episode. The mother reports that this episode occured a few hours after awakening, described her movements as myoclonic, and involving the upper extremity. As time progressed, her myoclonus evolved into a generalized tonic-clonic seizure that lasted 3-4 minutes. The patient reported to sleeping only a few hours per night due to upcoming examinations. Physical exam is unremarkable. EEG shows 5-Hz polyspike and slow-wave discharges.

Juvenile myoclonic epilepsy

A 40-year-old man presents with increasing muscle weakness. He reports difficulty rising from seated position and difficulty climbing stairs. He denies any blurry vision or any rashes on his body. He has a 100-pack-year history of smoking. Physical exam reveals proximal muscle weakness, decreased deep tendon reflexes, and dry mucous membranes. A bedside edrophonium test is conducted, which is negative. His chest radiography shows a suspicious finding - the round opacity shown in the square.

Lambert-Eaton Syndrome

A 67-year-old male presents with poor attention and getting lost while driving. He is accompanied by his wife. She reports that he would have episodes of staring into space and disorganized speech. His wife also reports that he would have full conversations with himself in the living room. She says that he was talking to his brother, who is deceased. Postural instability and bradykinesia is noted on physical exam.

Lewy Body Dementia

A 32-year-old women presents to her physician complaining of double vision. This has been very distressing for her. She has a past medical history significant for type 1 diabetes, treated with a continuous subcutaneous insulin pump. Upon further questioning, she mentions she experienced arm weakness and numbness that resolved spontaneously over the course of a couple weeks. Physical examination is notable for impaired adduction of the right eye, and nystagmus on abduction of the left eye on left lateral gaze.

Multiple Sclerosis

A 19-year-old male is brought to the emergency department by his college roommate due to confusion and difficulty with arousing from sleep. The patient reports severe generalized headache, neck stiffness, and muscle aches. Temperature is 102.2°F (39°C), blood pressure is 102/68 mmHg, pulse is 107/min, and respirations are 22/min. On physical exam, a petechial rash is distributed on the thorax and extremities. While supine, neck flexion lead to involuntary knee flexion.

Meningococcal meningitis

A 33-year-old woman presents to her primary care physician with headache, nausea, and visual disturbances. The patient was in her usual state of health until yesterday, when she experienced a pulsatile bilateral headache that caused her to have one episode of emesis. Her headache is accompanied by seeing a shimmering light that distorts her vision, photophobia, and phonophobia. Medical history is unremarkable, and the patient recently began menses. Ibuprofen and acetaminophen have not improved her symptoms. Neurologic examination is unremarkable.

Migraine headaches

A 42-year-old woman presents to her primary care physician for fatigue. She reports that her fatigue is worse towards the end of the day. She also notes that while bathing her nephew in the shower her head would "drop." At times when she is watching television or reading a book she sees double. Lastly, she would see her left or right eyelid droop after returning from work. On physical exam, there is right-sided ptosis after sustaining upward gaze for a few minutes. A tensilon test is performed, which is demonstrated in the clinical image below. Serologic studies return positive for anti-acetylcholine receptor antibodies. Preparations are made to have a computerized tomography scan of the chest.

Myasthenia Gravis

A 62-year-old man presents to his primary care physician due to trouble with walking and difficulty with concentrating. The patient describes his walking as if he is "stuck to the ground." On gait testing, the patient has magnetic gait and on montreal cognitive assessment (MoCA) testing, he has a deficit in executive functioning. Laboratory tests are ordered and return normal. Lumbar puncture shows a normal opening pressure and magnetic resonance imaging (MRI) of the head is shown.

Normal pressure hydrocephalus

A 63-year-old male is brought to the physician by his wife for the evaluation of a tremor. The tremor is worse at rest, and decreases in severity with purposeful movement. The patient reports to having difficulty with initiating voluntary movement, and his wife states that the patient's movements have been slow. On exam, there is seborrheic dermatosis on the nasolabial folds. There is a "pill-rolling" resting tremor accentuated when the patient is asked to perform mental calculations. Resistance to passive movement at the elbow joint is noted. On gait testing, there was difficulty with initiating gait, as well as the patient taking short steps when walking forward.

Parkinson Disease

A 65-year-old woman presents to the emergency department with meaningless speech. The patient was in her usual state of health until 3 hours prior to presentation, where her daughter noticed her mother having "strange speech." On physical examination, her speech is fluent, has paraphasic errors, and comprehension and repetition is impaired. On visual field testing there is a right upper quadrant field-cut.

Wernicke aphasia

A 47-year-old male with a medical history significant for hypertension, recurrent urinary tract infections, mitral valve prolapse, and diverticulosis experiences a sudden, severe headache while watching television on his couch. He calls 911 and reports to paramedics that he feels as if "someone shot me in the back of my head." He is rushed to the emergency room. On exam, he shows no focal neurological deficits but has significant nuchal rigidity and photophobia.

SAH

A 58-year-old male presents with difficulties in concentration and worsening insomnia. These symptoms have been progressively worsening over the past month. Startle myoclonus is appreciated on physical examination. Electroencephalography shows biphasic sharp wave patterns.

Spongiform Encephalopathy / Creutzfeldt-Jakob Disease

A 58-year-old woman presents to the emergency department with a severe headache of sudden onset. The headache occurred immediately while at rest, and she describes the headache as the most severe she has ever had. Her headache symptoms are also accompanied by photophobia. She has tried ibuprofen and sumatriptan, but it has not improved her symptoms. Medical history is significant for migraine headache, hypertension, cigarette smoking, and alcohol use disorder. Physical examination is notable for nuchal rigidity. A non-contrast head CT is shown.

Subarachnoid hemorrhage

A 31-year-old presents with self-described complaints of being "fidgety and irritable" that is unlike his "calm personality a few years ago". What is concerning to him is that his father was diagnosed with a similar condition at the age of 38. His father began a progressive decline - losing interest in his life and family, becoming messy, experiencing involuntary movements, and worsening dementia as he grew older. Genetic tests were performed on the patient which confirmed that he has a larger number of repeats than his father. He is concerned that the disease may begin earlier for him or may have already started. Which of the following trinucleotide repeats is found in this disease?

The above presentation of the father with progressive dementia, chorea, and losing interest in his life suggests that the father may have Huntington's disease (HD), which is caused by CAG trinucleotide repeats.

A 38-year-old man with mental retardation (IQ 50), facial features that include epicanthic folds and low-set small ears, and hands pictured in Figure A, is brought in by his elderly parents, who provide constant care. They are concerned that over the past 3 years, he has become increasingly forgetful and less interested in conversing and sharing his thoughts. If a post-mortem brain autopsy were conducted, the most likely histopathological feature in his brain underlying these changes would be:

The clinical presentation is consistent with Alzheimer's disease in a patient with Down syndrome (trisomy 21). On brain autopsy, the hallmark histopathological changes of Alzheimer's disease are extracellular beta-amyloid plaques and intracellular aggregates of phosphorylated tau (neurofibrillary tangles).

A 53-year-old man is brought by his daughter to the clinic. She lives a town away but visits often. She reports that on recent visits, his mood has been volatile, ranging from aggressive at some moments to depressed at others. She has noticed some new jerky movements which she has never seen before and has been quite forgetful. She is concerned that he might be abusing alcohol and drugs. What changes would you expect in the brain of this patient?

The patient presents with signs and symptoms of Huntington disease. This condition is marked by a decrease in GABA at the caudate nucleus.

A 28-year-old female presents to her primary care doctor complaining of new onset blurry vision. She first noticed her vision getting blurry toward the end of the day several days ago. Since then, she reports that her vision has been fine when she wakes up but gets worse throughout the day. She has also noticed that her eyelids have started to droop before she goes to bed. On exam, she has bilateral ptosis that is worse on the right. Administering edrophonium to this patient leads to an immediate improvement in her symptoms. Which of the following is most likely true about this patient's condition?

The most likely diagnosis for this patient is myasthenia gravis (MG). MG is associated with the development of a thymoma and thymectomy can therefore lead to an improvement in symptoms in some patients.

A 5-year-old patient is brought to the emergency department by his parents for concerning behavior. His parents relate that over the past 3 weeks, he has had multiple episodes of staring into space, lip smacking, and clasping his hands together. The patient has his eyes open during these episode but does not respond to his parents' voice or his name. These episodes last between 1-2 minutes after which the patient appears to return back to awareness. The patient is confused after these episodes and appears not to know where he is for about 15 minutes. These episodes occur once every few days and the most recent one happened about 10 minutes before the patient arrived to the emergency department. On arrival, the patient is mildly confused and does not know where he is or what recently happened. He is slow to respond to questions and appears tired. Which of the following is the most likely diagnosis in this patient?

The patient displays loss of consciousness/awareness with motor automatisms like lip-smacking and hand-clasping characteristic of a complex partial seizure.

A 48-year-old man presents to the ER with a sudden-onset, severe headache. He is vomiting and appears confused. His wife, who accompanied him, says that he has not had any trauma, and that the patient has no relevant family history. He undergoes a non-contrast head CT that shows blood between the arachnoid and pia mater. What is the most likely complication from this condition?

The patient has had a subarachnoid hemorrhage (SAH), and arterial vasospasm is the most common complication from this disease.

A 25-year-old male rugby player presents to the emergency room complaining of a severe headache. He is accompanied by his teammate who reports that he had a head-to-head collision with another player and briefly passed out before regaining consciousness. His past medical history is significant for a pilocytic astrocytoma as a child treated successfully with surgery. His family history is notable for stroke in his father. His temperature is 98.9°F (37.2°C), blood pressure is 160/90 mmHg, pulse is 60/min, and respirations are 20/min. On examination, he is lethargic but oriented to person, place, and time.

The patient in this vignette presents with a post-traumatic headache preceded by a momentary loss of consciousness, a scenario that is suggestive of epidural hematoma. Rupture of the middle meningeal artery, a branch of the maxillary artery, leads to the development of an epidural hematoma.

A 59-year-old male presents to his primary care physician complaining of muscle weakness. Approximately 6 months ago, he started to develop gradually worsening right arm weakness that progressed to difficulty walking about three months ago. His past medical history is notable for a transient ischemic attack, hypertension, hyperlipidemia, and diabetes mellitus. He takes aspirin, lisinopril, atorvastatin, metformin, and glyburide. He does not smoke and he drinks alcohol occasionally. Physical examination reveals 4/5 strength in right shoulder abduction and right arm flexion. A tremor is noted in the right hand. Strength is 5/5 throughout the left upper extremity. Patellar reflexes are 3+ bilaterally. Sensation to touch and vibration is intact in the bilateral upper and lower extremities. Tongue fasciculations are noted. Which of the following is the most appropriate treatment in this patient?

The patient in this vignette presents with asymmetric limb weakness suggestive of amyotrophic lateral sclerosis (ALS). The only medication known to slow the progression of ALS is riluzole.

A 62-year-old man is brought to his primary care physician by his wife who is concerned about the patient's frequent falls. Approximately 6 months ago, she started noticing that he was walking more slowly than usual. He has fallen more than 6 times in the past month, and she is worried that he will sustain a serious injury if he does not stop falling. The patient is a retired banking executive and was active as a triathlete until the age of 60. He does not smoke and drinks 2-3 alcoholic beverages per day. His family history is notable for normal pressure hydrocephalus in his mother and Alzheimer dementia in his father. His temperature is 97.8°F (36.6°C), blood pressure is 131/81 mmHg, pulse is 68/min, and respirations are 19/min. On exam, his movements appear slowed and forced. He shuffles his feet when he walks. Tone is increased in his upper and lower extremities bilaterally. This patient's condition is most strongly associated with which of the following histologic findings on brain autopsy?

The patient in this vignette presents with bradykinesia, frequent falls, a shuffling gait, and muscle rigidity suggestive of Parkinson disease (PD). PD is characterized by Lewy bodies (round eosinophilic inclusions of α-synuclein).

A 61-year-old woman presents to her primary care doctor with her son who reports that his mother is not acting like herself. She has gotten lost while driving several times in the past 2 months and appears to be talking to herself frequently. Of note, the patient's husband died from a stroke 4 months ago. The patient reports feeling sad and guilty for causing so much trouble for her son. Her appetite has decreased since her husband died. On examination, she is oriented to person, place, and time. She is inattentive, and her speech is disorganized. She shakes her hand throughout the exam without realizing it. Her gait is slow and appears unstable. This patient's condition would most likely benefit from which of the following medications?

The patient in this vignette presents with dementia and parkinsonism suggestive of Lewy body dementia (LBD). The first-line treatment for LBD involves anti-cholinesterase inhibitors (e.g., rivastigmine or donepezil).

A 73-year-old man presents to his primary care doctor with his son who reports that his father has been acting strangely. He has started staring into space throughout the day and has a limited attention span. He has been found talking to himself on several occasions and has gotten lost while driving twice. He has occasional urinary incontinence. His past medical history is notable for a stroke 5 years ago with residual right arm weakness, diabetes, hypertension, and hyperlipidemia. He takes aspirin, glyburide, metformin, lisinopril, hydrochlorothiazide, and atorvastatin. On examination, he is oriented to person and place but thinks the year is 1989. He is inattentive throughout the exam. He takes short steps while walking. His movements are grossly slowed. A brain biopsy in this patient would most likely reveal which of the following?

The patient in this vignette presents with impaired cognition, inattention, hallucinations, and Parkinsonian symptoms suggestive of Lewy body dementia (LBD). Eosinophilic intracytoplasmic inclusions represent clumps of alpha-synuclein protein that can be found in the brains of patients with LBD.

A 32-year-old man presents to his primary care provider with right leg weakness and numbness. He reports a 2-day history of "clumsiness" in his right lower extremity after playing in his company's annual weekend-long charity baseball tournament. He says a similar episode happened 1 year ago during the same tournament. He has a history of major depressive disorder and right distal radius fracture status post-closed reduction and casting after falling 2 years ago. He takes fluoxetine. He also reports that he several years ago he had an isolated episode of decreased vision and inability to move his right eye that resolved on its own. He has a 15-pack-year smoking history and drinks 3-4 beers per week. His temperature is 98.4°F (36.9°C), blood pressure is 115/65 mmHg, pulse is 85/min, and respirations are 18/min. On exam, hip flexion, knee extension, and ankle dorsiflexion are all 3/5 on his right and 5/5 on his left. Right Achilles and patellar reflexes are 1+ on the right and 2+ on the left. He has decreased sensation to light touch throughout the right leg especially below the knee. Additional questioning would most likely reveal which of the following additional features about this patient's current symptoms?

The patient in this vignette presents with unilateral lower extremity numbness and weakness with a history of similar prior episodes suggestive of a multiple sclerosis (MS) flare. MS symptoms can often be exacerbated in the heat.

A 65-year-old patient with a history of atrial fibrillation presents to the emergency room complaining of sudden-onset speech difficulty. Her husband notes that they were eating dinner earlier in the night, when he suddenly noticed the change. On admission, her blood pressure is 135/80 mmHg, heart rate is 85 beats per minute, temperature is 98.6 degrees Fahrenheit, and SpO2 is 99% on room air. Her MRI is shown below in Figure A. Which of the following speech deficit is the patient most likely to experience?

The patient presents with a left posterior frontal lobe infarct in the territory of Broca's area, which is associated with difficulty with speech production and repetition.

A 26-year-old man presents to his primary doctor with one week of increasing weakness. He reports that he first noticed difficulty walking while attending his sister's graduation last week, and yesterday he had difficulty taking his coffee cup out of the microwave. He remembers having nausea and vomiting a few weeks prior, but other than that has no significant medical history. On exam, he has decreased reflexes in his bilateral upper and lower extremities, with intact sensation. If a lumbar puncture is performed, which of the following results are most likely?

The patient's clinical presentation is most consistent with Guillain-Barre syndrome. Cerebrospinal fluid (CSF) analysis in this syndrome is notable for "albuminocytologic dissociation," meaning that there is increased protein with a normal cell count, glucose, and opening pressure.

A 71 year-old female is brought to the emergency room by her husband. The husband reports that they were taking a walk together one hour ago, when his wife experienced sudden, right arm and leg weakness. He noticed that she had slurred speech, and that she was not able to tell him where she was. The patient underwent an emergent CT scan, which was unremarkable, and was treated with tissue plasminogen activator (tPA). Which of the following EKG findings increases a patient's risk for this acute presentation?

The patient's presentation is most consistent with an acute ischemic stroke, which is associated with atrial fibrillation.

4768) A 4-year-old previously healthy male presents with 4 days of intermittent vomiting and 5-6 daily loose stools. His mother noted bloody stools and decreased oral intake of food and water over the last 24 hours. He is normally in daycare; however, he has been home for the past 3 days. On physical exam his temperature is 102.2°F (39°C), blood pressure is 140/90 mmHg, pulse is 120/min, respirations are 22/min and O2 saturation is 99% on room air. He has dry mucous membranes. On abdominal exam you note diffuse tenderness to palpation without rebound or guarding. There are no masses, hepatosplenomegaly, and bowel sounds are hyperactive. Ultrasound of the right lower quadrant is negative for appendicitis. Stool is guaiac positive. He receives 15mg/kg acetaminophen and fluids are started. The next day, he complains of lower extremity weakness and tingling. On repeat exam, lower extremity strength is 3/5 with diminished patellar deep tendon reflexes. Which of the following lab findings would most likely be seen in this patient?

This child with diarrhea and fever followed by lower extremity weakness and hyporeflexia has Guillain-Barre syndrome (GBS) after gastrointestinal illness. Cerebrospinal fluid (CSF) will show increased cerebrospinal fluid protein with normal cell count (albuminocytologic dissociation).

A 25-year-old man with no significant past medical history is brought in by ambulance after a witnessed seizure at home. On physical exam, temperature is 102.3 deg F (39.1 deg C), blood pressure is 90/62 mmHg, pulse is 118/min, and respirations are 25/min. He is unable to touch his chin to his chest and spontaneously flexes his hips with passive neck flexion. Appropriate empiric treatment is begun. CT head is unremarkable, and a lumbar puncture sample is obtained. Gram stain of the cerebrospinal fluid (CSF) reveals gram-positive diplococci. Which of the following would you expect to see on CSF studies?

This clinical presentation is consistent with bacterial meningitis, likely due to Streptococcus pneumoniae (given gram-positive diplococci). Cerebrospinal fluid (CSF) studies in bacterial meningitis typically show elevated opening pressure, elevated protein, and low glucose.

An 81-year-old woman presents to your office accompanied by her husband. She has been doing well except for occasional word finding difficulty. Her husband is concerned that her memory is worsening over the past year. Recently, she got lost twice on her way home from her daughter's house, was unable to remember her neighbor's name, and could not pay the bills like she usually did. She has a history of hypertension and arthritis. She has no significant family history. Her medications include a daily multivitamin, hydrochlorothiazide, and ibuprofen as needed. Physical exam is unremarkable. Which of the following is associated with an increased risk of this patient's disease?

This elderly woman presenting with memory loss, word-finding difficulty, and getting lost in familiar places most likely has Alzheimer's disease. ApoE4 is associated with an increased risk of sporadic Alzheimer's disease.

A 72-year-old man with longstanding history of diabetes mellitus and hypertension presents to the emergency department with sudden-onset numbness. On your neurological exam, you note that he has loss of sensation on the left side of his face, arm, and leg. His motor strength exam is normal, as are his cranial nerves. Which of the following is the most likely explanation for his presentation?

This patient had a stroke with total sensory loss, consistent with thalamic stroke.

A 59-year-old woman with a past medical history of atrial fibrillation currently on warfarin presents to the emergency department for acute onset dizziness. She was watching TV in the living room when she suddenly felt the room spin around her as she was getting up to go to the bathroom. She denies any fever, weight loss, chest pain, palpitations, shortness of breath, lightheadedness, or pain but reports difficulty walking and hiccups. A physical examination is significant for rotary nystagmus and decreased pin prick sensation throughout her left side. A magnetic resonance image (MRI) of the head is obtained and shows ischemic changes of the right lateral medulla. What other symptoms would you expect to find in this patient?

This patient has lateral medullary (Wallenberg) syndrome as demonstrated by her vertigo, rotary nystagmus, and decreased pain sensation in the setting of ischemic changes in the lateral medulla on MRI. Other symptoms characteristic of lateral medullary syndrome include decreased gag reflex.

A patient is transferred from an outside hospital by family request. The patient is a 76-year-old gentleman who developed acute onset left-sided weakness four days ago with the imaging findings seen in Figure A. Despite aggressive care, the patient dies shortly after transfer. The family requests an autopsy. What histological finding would you expect to find on evaluation of the patient's brain?

This patient is 4 days removed from a large ischemic stroke. At this point in time the predominant histological finding would be macrophage infiltration and phagocytosis.

A 26-year-old man is brought to the emergency department due to right-sided facial and upper extremity weakness and aphasia. The patient was in his usual state of health until two hours prior to presentation, when he was eating breakfast with a friend and acutely developed the aforementioned symptoms. Medical history is unremarkable except for mild palpitations that occur during times of stress or when drinking coffee. Physical examination is consistent with the clinical presentation. Laboratory testing is unremarkable and a 12-lead electrocardiogram is normal. A non-contrast head CT and diffusion-weighted MRI shows no intracranial hemorrhage and an isolated superficial cerebral infarction. Transthoracic echocardiography with agitated saline mixed with air shows microbubbles in the left heart. There is a possible minor effusion surrounding the heart and the ejection fraction is within normal limits. Which of the following is most likely the cause of this patient's clinical presentation?

This patient is presenting with symptoms concerning for an acute stroke and a positive "bubble study", which is most likely due to a patent foramen ovale (PFO) leading to cryptogenic stroke.

A 73-year-old woman is brought in by her daughter stating that her mom has become increasingly forgetful and has trouble remembering recent events. Her memory for remote events is remarkably intact. The patient is no longer able to cook for herself as she frequently leaves the stove on unattended. She has recently been getting lost in her neighborhood even though she has lived there for 30 years. Her mood is not depressed. Decreased activity in which of the following areas of the brain is known to be involved in the pathogenesis of Alzheimer's disease?

This patient likely has Alzheimer's dementia. The pathogenesis of Alzheimer's involves decreased production of acetylcholine caused by decreased activity of choline acetyltransferase in the nucleus basalis of Meynert and hippocampus.

A 73-year-old man presents to your office accompanied by his wife. He has been experiencing a tremor in his right hand for the last several months that seems to be worsening. He does not have any other complaints and says he's "fine." His wife thinks that he has also had more difficulty walking. His history is significant for hypertension and an ischemic stroke of the right middle cerebral artery 2 years ago. His medications include hydrochlorothiazide and daily aspirin. On physical exam you note that the patient speaks with a soft voice and has decreased facial expressions. He has a resting tremor that is worse on the right side. He has increased resistance to passive movement when you flex and extend his relaxed wrist. He has 5/5 strength bilaterally. Neuronal degeneration in which of the following locations is most likely responsible for the progression of this disease?

This patient presenting with a resting tremor, decreased expression, and rigidity most likely has Parkinson's disease. Parkinson's disease is a degenerative that is associated with neuronal degeneration in the substantia nigra pars compacta.

A 74-year-old African-American woman is brought to the emergency department by her home health aid. The patient was eating breakfast this morning when she suddenly was unable to lift her spoon with her right hand. She attempted to get up from the table, but her right leg felt weak. One hour later in the emergency department, her strength is 0/5 in the right upper and right lower extremities. Strength is normal in her left upper and lower extremities. Sensation is normal bilaterally. An emergency CT of the head does not show signs of hemorrhage. Subsequent brain MRI shows an infarct involving the internal capsule. Which of the following is true about her disease process?

This patient presents with a pure motor stroke, which is one of the stroke syndromes caused by lacunar infarcts. The most important predisposing risk factors for lacunar infarcts are diabetes and hypertension.

A 38-year-old woman presents to her primary care physician for evaluation of 3 months of increasing fatigue. She states that she feels normal in the morning, but that her fatigue gets worse throughout the day. Specifically, she says that her head drops when trying to perform overhead tasks. She also says that she experiences double vision when watching television or reading a book. On physical exam, there is right-sided ptosis after sustaining upward gaze for a 2 minutes. Which of the following treatments may be effective in treating this patient's diagnosis?

This patient who experiences muscle weakness that is worse after repetitive use and develops ptosis on sustained upward gaze most likely has myasthenia gravis, which can be treated with thymectomy if a thymoma is identified.

A 75-year-old man is brought to the emergency room after being found unconscious in his home. His medical history is unknown. On physical examination he does not demonstrate any spontaneous movement of his extremities and is unable to respond to voice or painful stimuli. You notice that he is able blink and move his eyes in the vertical plane. Based on these physical exam findings, you expect that magnetic resonance angiogram will most likely reveal an occlusion in which of the following vessels?

This patient with quadriplegia and preservation of vertical gaze most likely has locked-in syndrome due to an occlusion of the basilar artery.

An 15-year-old boy is brought to the emergency department after he passed out in the hallway. On presentation, he is alert but confused about why he is in the hospital. He says that he remembers seeing flashes of light to his right while walking out of class but cannot recall what happened next. His next memory is being woken up by emergency responders who wheeled him into an ambulance. A friend who was with him at the time says that he seemed to be swallowing repeatedly and staring out into space. He has never had an episode like this before, and his past medical history is unremarkable. Which of the following characteristics is most likely true of the cause of this patient's symptoms?

This patient who saw visual flashes and then lost consciousness while demonstrating automatisms most likely had a complex partial seizure that started in the left occipital lobe and secondarily generalized.

A 23-year-old man presents to the emergency room following a stab wound to the back. He was in a bar when he got into an argument with another man who proceeded to stab him slightly right of the midline of his back. He is otherwise healthy and does not take any medications. He has one previous admission to the hospital for a stab wound to the leg from another bar fight 2 years ago. His temperature is 99°F (37.2°C), blood pressure is 115/80 mmHg, pulse is 100/min, and pulse oximetry is 99% on room air. Cardiopulmonary and abdominal exams are unremarkable; however, he has an abnormal neurologic exam. If this wound entered his spinal cord but did not cross the midline, which of the following would most likely be seen in this patient?

This patient with a right midline stab wound to the back has a hemisection of the spinal cord, or Brown-Sequard syndrome, which would result in ipsilateral flaccid paralysis at the level of the lesion.

A 68-year-old man is brought to the emergency department by ambulance after he was found to be altered at home. Specifically, his wife says that he fell and was unable to get back up while walking to bed. When she approached him, she found that he was unable to move his left leg. His past medical history is significant for hypertension, atrial fibrillation, and diabetes. In addition, he has a 20-pack-year smoking history. On presentation, he is found to still have difficulty moving his left leg though motor function in his left arm is completely intact. The cause of this patient's symptoms most likely occurred in an artery supplying which of the following brain regions?

This patient with acute onset lower extremity weakness most likely experienced a stroke of the anterior cerebral artery, which is the artery that supplies the cingulate gyrus.

An 89-year-old woman is admitted to the neurology intensive care unit following a massive cerebral infarction. She has a history of hypertension, ovarian cancer, and lung cancer. Her medications include lisinopril and aspirin. She has smoked a few cigarettes each day for the last 60 years. She does not drink alcohol or use drugs. An arterial line and intraventricular pressure monitor are placed. You decide to acutely lower intracranial pressure by causing cerebral vasoconstriction. Which of the following methods could be used for this effect?

This patient with cerebral edema can be treated with therapeutic hyperventilation to decrease cerebral blood flow.

A 68-year-old man is brought to the physician by his wife because she is concerned about his speech being irregular. Specifically, she says that over the last 8 months, her husband has been saying increasingly nonsensical statements at home. In addition, he is no longer able to perform basic verbal tasks such as ordering from a menu or giving directions even though he was an English teacher prior to retirement. She also reports that he has recently started attempting to kiss strangers and urinate in public. Finally, she has also noticed that he has been frequently binge eating sweets even though he was previously very conscientious about his health. When asked about these activities, the patient does not have insight into his symptoms. Which of the following would most likely be seen in this patient?

This patient with disinhibition, early aphasia, hyperorality, and compulsiveness most likely has frontotemporal dementia, which would present with hyperphosphorylated tau inclusion bodies.

A 68-year-old man is brought to the physician by his wife because she is concerned about his speech being irregular. Specifically, she says that over the last 8 months, her husband has been saying increasingly nonsensical statements at home. In addition, he is no longer able to perform basic verbal tasks such as ordering from a menu or giving directions even though he was an English teacher prior to retirement. She also reports that he has recently started attempting to kiss strangers and urinate in public. Finally, she has also noticed that he has been frequently binge eating sweets even though he was previously very conscientious about his health. When asked about these activities, the patient does not have insight into his symptoms. Which of the following would most likely be seen in this patient? Review Topic

This patient with disinhibition, early aphasia, hyperorality, and compulsiveness most likely has frontotemporal dementia, which would present with hyperphosphorylated tau inclusion bodies.

A 57-year-old man presents to his primary care physician with a 2-month history of right upper and lower extremity weakness. He noticed the weakness when he started falling far more frequently while running errands. Since then, he has had increasing difficulty with walking and lifting objects. His past medical history is significant only for well-controlled hypertension, but he says that some members of his family have had musculoskeletal problems. His right upper extremity shows forearm atrophy and depressed reflexes while his right lower extremity is hypertonic with a positive Babinski sign. Which of the following is most likely associated with the cause of this patient's symptoms?

This patient with muscle weakness (on physical exam has mixed upper and lower motor neuron signs) most likely has amyotrophic lateral sclerosis, which is associated with mutations in the superoxide dismutase type 1 (SOD-1) gene.

A 71-year-old man presents to his primary care physician because he is increasingly troubled by a tremor in his hands. He says that the tremor is worse when he is resting and gets better when he reaches for objects. His wife reports that he has been slowing in his movements and also has difficulty starting to walk. His steps have been short and unsteady even when he is able to initiate movement. Physical exam reveals rigidity in his muscles when tested for active range of motion. Histology in this patient would most likely reveal which of the following findings?

This patient with resting tremor, bradycardia, rigidity, and instability most likely has Parkinson disease, which could be seen on histology as round eosinophilic inclusions of alpha-synuclein.

A 34-year-old woman comes to the clinic complaining of numbness and tingling of her right arm for 2 days. She reports that she was washing dishes when she felt a burning sensation along her right forearm. The patient has been relatively healthy except for an episode of right eye pain and vision loss 4 years ago. She does not recall specific details but claims that "it just went away on its own after a couple of days." The patient denies any recent travel, trauma, loss of consciousness, speech changes, weakness, or vision change but does endorse gastroenteritis about 1 week ago. She is sexually active with multiple partners and rarely uses contraception. Her last HIV testing 2 weeks ago was negative. A magnetic resonance image (MRI) of her brain is shown in Figure A. What is the most likely explanation for this patient's symptoms?

This patient's neurologic symptoms that are disseminated in space and time (right arm paresthesia and optic neuritis) are characteristic of multiple sclerosis, which results from autoimmune inflammation and demyelination of oligodendrocytes by T-cells.

A 44-year-old man presents to his primary care physician due to a tremor. His tremor has been progressively worsening over the course of several weeks and he feels embarrassed and anxious about going to social events. He says these movements are involuntary and denies having an urge to have these movements. Medical history is significant for depression which is being treated with escitalopram. His mother is currently alive and healthy but his father committed suicide and had a history of depression. Physical examination is remarkable for impaired saccade initiation and brief, abrupt, and non-stereotyped movements involved the right arm. He also has irregular finger tapping. Which of the following is the best treatment for this patient's symptoms?

This patient's non-stereotyped and sudden movements of his right arm, impaired saccade initiation, and psychiatric symptoms (e.g., depression), are highly suggestive of Huntington disease. Deutetrabenazine is a pharmacologic treatment for his motor symptoms (chorea).

A 70-year-old woman with no significant medical history begins to experience memory loss and personality changes. Over the next few months, her symptoms become more severe, as she experiences rapid mental deterioration. She also starts to have sudden, jerking movements in response to being startled and gait disturbances. Eventually, she lapses into a coma and dies eight months after the onset of symptoms. What process likely caused this woman's illness?

This woman most likely suffered from Creutzfeldt-Jakob Disease (CJD), which results from the conversion of an a-helix in a normal protein, termed prion protein (PrPc), to a ß-pleated form. The new ß-pleated protein (PrPSc) resists degradation and facilitates conversion of normal proteins to the abnormal form.

A 28-year-old woman with a history of migraines presents to your office due to sudden loss of vision in her left eye and difficulty speaking. Two weeks ago she experienced muscle aches, fever, and cough. Her muscle aches are improving but she continues to have a cough. She also feels as though she has been more tired than usual. She had a similar episode of vision loss 2 years ago and had an MRI at that time. She has a family history of migraines and takes propranolol daily. On swinging light test there is decreased constriction of the left pupil relative to the right pupil. You repeat the MRI and note enhancing lesions in the left optic nerve. Which of the following is used to prevent progression of this condition?

This young female presenting with sudden loss of vision, difficulty speaking, and a history of a similar episode 2 years ago is most concerning for multiple sclerosis (MS). Natalizumab prevents progression of symptoms and is used in long-term management.

A 70-year-old man presents to the emergency department with confusion after having a convulsive episode. Prior to having a seizure, the patient reported to having a progressively worsening headache that awoke him from sleep, and right-sided weakness over the course of 7 months. On physical exam, there is weakness 2/5 strength throughout the right-side, and a left pupil that is unresponsive to light. A computerized tomography (CT) scan of the head shows a ring-enhancing lesion with surrounding cerebral edema.

Uncal herniation

You see a patient with new-onset neurologic deficits in the ED. What neurological exam findings would you expect if the patient's MRI angiography is as shown in Figure A? (left-sided lesion labeled with arrow)

You see a patient with new-onset neurologic deficits in the ED. What neurological exam findings would you expect if the patient's MRI angiography is as shown in Figure A? (left-sided lesion labeled with arrow)

A 67-year-old male is seen by neurology after he was noticed to be speaking strangely by his family. After acute treatment with tissue plasminogen activator (tPA), the patient is able to recover most of his speech. Subsequent neurologic exam finds that the patient is fluent while speaking and is able to comprehend both one and two step instructions. Noticeably the patient remains unable to complete tasks involving verbal repetition.

conduction aphasia, which is caused by isolated damage to the arcuate fasciculus.

A 59-year-old presents with right-sided hemiparesis, right-sided sensory loss, leftward eye deviation, and slurred speech. A head CT is performed which is significant for a hyperdense lesion affecting the putamen. The patient has a history of hypertension treated with hydrochlorothiazide, but is non-adherent. Which of the following is most likely associated with the cause of this patient's neurological deficits?

intraparenchymal hemorrhage (IPH)