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100

A 73-year-old woman is evaluated for progressive left arm weakness. One year earlier, she noted difficulty with fine movements of her left hand. Within a few months, her entire left upper extremity became stiff and slow, such that she now cannot use this limb. Her left leg has also become rigid. She notes brief jerky movements of her left arm. She takes no medication.

On physical examination, vital signs are normal. Language and memory are intact, but she speaks slowly and has difficulty with multistep commands. Results of cranial nerve testing, including extraocular movements, are normal. Muscle strength is preserved throughout. Her left upper and lower extremities are rigid and slow. Her left hand is forced into a bent position and cannot be opened by the examiner. She needs support during ambulation because of stiffness of her left leg. Her sensory perception is preserved, but she cannot identify an object placed into her left hand when her eyes are closed. Cerebellar examination findings, plantar response, and deep tendon reflexes are unremarkable.

What is the most likely diagnosis?

Corticobasal degeneration

Multiple system atrophy
Parkinson disease
Progressive supranuclear palsy
Amyotrophic lateral sclerosis

100

A 66-year-old woman is evaluated for a 6-month history of slowed gait and difficulty lifting her feet off of the ground when she first starts to walk. She becomes incontinent without feeling the urge to urinate. Her family reports that she seems to be thinking more slowly.

On physical examination, vital signs are normal. Gait is magnetic, with hesitancy and shuffling.

MRI of the brain shows ventriculomegaly in the lateral ventricles and third ventricle. There is no obstruction in the cerebral aqueduct.

What is the most appropriate next step in management?

High-volume cerebrospinal fluid removal

Lumboperitoneal shunt procedure
Placement of a temporary lumbar drain
Ventriculoperitoneal shunt procedure 

100

A 60-year-old woman comes to the office to discuss the best strategy to prevent cognitive impairment. She has no medical problems and takes no medications. She does not use tobacco, has 1 to 2 alcoholic drinks per week, and is sedentary.

On physical examination, vital signs and other findings are unremarkable.

Results of cognitive screening testing are normal.

What is the most effective preventive measure?

Physical exercise training 

Cognitive training
Donepezil
Vitamin E supplementation

100

A 64-year-old man is hospitalized for right-sided weakness and slurred speech of 24 hours' duration. He has hypertension and dyslipidemia.

On physical examination, blood pressure is 190/88 mm Hg and oxygen saturation  is 98% with the patient breathing ambient air. The remaining vital signs are normal. Abnormal physical examination findings are confined to right facial weakness and right arm and leg pronator drift. National Institutes of Health Stroke Scale score is 3.

ECG is normal. Serum creatinine  level is 0.9 mg/dL (79.6 µmol/L), and cardiac biomarkers are negative.

CT scan of the head shows a left frontal infarction. CT angiogram shows 80% stenosis of middle cerebral artery.

Medical therapy with aspirin, clopidogrel, and high-intensity atorvastatin is initiated.

What is the most appropriate immediate treatment?

No additional intervention or treatment

Chlorthalidone
Furosemide
Intracranial stenting
Lisinopril 

200

A 61-year-old man is evaluated for pain and weakness in his hands and feet. He has a history of bilateral carpal tunnel syndrome that improved after surgery 1 year ago, but symptoms returned 2 months ago. He has noted progressive pain and numbness below his knees and diminished sweating. He also has episodic light-headedness on standing, dry eyes, heart failure with preserved ejection fraction, left ventricular hypertrophy by echocardiography, and erectile dysfunction. Current medications are amlodipine, metoclopramide, artificial tears, and sildenafil.

On physical examination, vital signs are normal. The presence of orthostatic hypotension is confirmed. Pupils react sluggishly to light. Extraocular movements are preserved. Weakness in right thumb abductor and bilateral foot dorsiflexors is observed. Sensory loss to temperature and pain below the knees and elbows are noted. Vibration is preserved at the ankles. Deep tendon reflexes are preserved in upper extremities and diminished in lower extremities.

Fasting plasma glucose level, glucose tolerance test results, serum and urine protein electrophoresis and immunofixation results, rapid plasma reagin results, and anti-Ro and anti-La antibody titers are normal.

ECG is remarkable for low voltages.

What is the most appropriate diagnostic test to perform next?

Abdominal fat pad biopsy

Anti–muscle-specific kinase antibody measurement
Myotonic dystrophy gene test
Serum GQ1B antibody measurement 

200

A 21-year-old woman comes to the office requesting clearance to resume playing collegiate soccer. Two weeks ago, she sustained a mild traumatic brain injury in a head-to-head collision during a match and, for the next 3 days, had episodic dizziness and recurrent headaches lasting minutes to hours; symptoms resolved spontaneously with a combination of physical and cognitive rest. She resumed normal activities and has been free of symptoms for the past 10 days. She has taken no medication. Neuroimaging was not performed at the time of injury.

Physical examination findings, including vital signs and neurologic examination results, are normal.

What is the most appropriate next step in management?

Clearance to return to play

Head CT
Neuropsychological testing
Vestibular therapy 

200

A 39-year-old woman is evaluated for a 3-month history of low energy, lack of interest in activities, and poor sleep. She has relapsing-remitting multiple sclerosis and a history of vitamin D deficiency. Medications are glatiramer acetate and a vitamin D supplement.

On physical examination, vital signs are normal. All other examination findings, including those from a neurologic examination, are unremarkable.

Two weeks ago, a routine MRI of the brain showed a stable number of T2 lesions, and none of these lesions demonstrated contrast enhancement on T1-weighted images.

What is the most appropriate management?

Screen for depression

Initiate modafinil
Measure serum 25-hydroxyvitamin D level
Switch to natalizumab

200

A 44-year-old man is evaluated for muscle cramps and fatigue. He frequently experiences cramps after prolonged exertion. Within the past few months he has developed difficulty climbing stairs and feels that his walking has become unsteady. He has no family history of neuromuscular disease.

On physical examination, vital signs are normal. There is no muscle tenderness on palpation. Muscle strength is reduced in bilateral hip flexors and arm abductors and preserved distally. Deep tendon reflexes are preserved but exhibit delayed relaxation. Percussion over biceps muscles leads to formation of a raised muscular ridge. Gait has a waddling quality. Sensory examination and muscle tone are normal. Plantar responses are flexor. There are no involuntary movements.

What is the most likely cause of this patient's findings?

Hypothyroidism

Copper deficiency
Hypokalemia
Vitamin D deficiency
Vitamin E deficiency 

300

A 29-year-old woman is evaluated in the emergency department for loss of consciousness. This morning her mother found her unconscious in bed, drooling, and shaking all her limbs. The patient was unresponsive for 3 to 4 minutes, and was confused for about 5 minutes after regaining consciousness. By the time she reached the emergency department, she had recovered completely. She has had no recent illness, including infection, head trauma, or history of prior similar episodes.

She has no history of alcohol or illicit drug use. She has no other medical problems and her only medication is a combined oral contraceptive.

On physical examination, vital signs are normal. All other examination findings, including those from a neurologic examination, are unremarkable.

Results of a comprehensive metabolic profile and toxicology screen are normal. A CT of the head without contrast in the emergency department is normal. An electroencephalogram has been scheduled.

What is the most appropriate management?

Brain MRI without contrast

CT of the head with contrast
Levetiracetam
Lumbar puncture 

300

A 55-year-old man is evaluated in the hospital for paralysis present on awakening from anesthesia after surgical repair of an aortic dissection. He reports midthoracic back pain. He had been well without evidence of infection before surgery. Other medical problems include hypertension and dyslipidemia. Current medications are atorvastatin, hydrochlorothiazide, and losartan.

On physical examination, vital signs are normal. Muscle tone is flaccid in the legs and normal in the arms. Muscle strength is normal in both arms and no movement in both legs. Reflexes are 2+ in arms and 0 in legs. Sensation to pinprick is mildly impaired in the legs, and vibratory and position sensation are intact.

What is the most likely diagnosis?

Spinal cord infarction

Guillain-Barré syndrome
Idiopathic transverse myelitis
Lumbar disk herniation

300

A 78-year-old woman is evaluated for a 1-year history of progressively worsening memory impairment. She can no longer handle her finances, as she doesn't remember how to fill out checks properly. She lives with her spouse and is independent in all other basic activities of daily living.

On physical examination, vital signs are normal. Montreal Cognitive Assessment score is 21/30, with difficulty copying the cube, recalling the five words, and naming the day, date, and month noted.

Results of laboratory evaluation are normal.

What is the most appropriate imaging test to perform next?

MRI of the brain without contrast

Amyloid PET scan
Fluorodeoxyglucose PET scan
Head CT with contrast 

300

A 63-year-old man is evaluated for myalgia and proximal weakness in both upper and lower extremities. He has diabetes, hypertension, and coronary artery disease. Current medications include metformin, empagliflozin, lisinopril, and atorvastatin.

On physical examination, blood pressure is 132/82 mm Hg; other vital signs are normal. Proximal muscle tenderness is noted. All other findings, including those from the neurologic examination, are normal.

Laboratory studies show an erythrocyte sedimentation rate  of 15 mm/h, hemoglobin A1c  level of 6.8%, and serum creatine kinase  level of 250 U/L.

What is most likely the cause of this patient's symptoms?

Statin-induced myopathy

Immune-mediated necrotizing myopathy
Polymyalgia rheumatica
Proximal lumbosacral radiculoneuropathy 

400

A 20-year-old woman is evaluated for intermittent double vision that occurs when she looks from the corner of her eyes and disappears when she covers one eye. She also reports fluctuating weakness and frequent nausea and diarrhea. She has had a history of exercise intolerance and intermittent muscle cramps since middle school. She also has postural orthostatic tachycardia syndrome, irritable bowel syndrome, and fibromyalgia. Her sister, brother, mother, and maternal aunt have similar muscle, neurologic, and multiorgan symptoms, but her father and a second brother do not. Current medications are propranolol and duloxetine.

On physical examination, vital signs are normal. BMI is 20. There is bilateral impairment of ocular adduction along with bilateral ptosis. Lower facial muscles show weakness. Muscle tone is reduced, but deep tendon reflexes are preserved. Proximal weakness is noted in all extremities. There is no delayed relaxation with grip or percussion. Mental status, sensory examination, and coordination are intact.

Electromyogram shows myopathic changes.

What is the most likely diagnosis?

Mitochondrial myopathy

Acid maltase deficiency
Becker muscular dystrophy
Inclusion body myositis
McArdle disease 

400

A 74-year-old woman is evaluated in the hospital at 2:00 AM for agitation. She was admitted 3 days ago with acute diverticulitis. Over the last 24 hours, the patient has been alternately agitated or drowsy, sometimes confused when awake, and tonight has experienced visual hallucinations. Her only other medical problem is long-standing depression treated with paroxetine, which has been discontinued. Medical records indicate she does not drink alcohol. Her current medications include oxycodone, scheduled every 6 hours, and piperacillin-tazobactam.

On physical examination, temperature is 37.2 °C (99.0 °F), and the remaining vital signs are normal. Oxygen saturation is 97% with the patient breathing ambient air. The abdomen has diminished but present bowel sounds, and there is mild tenderness to palpation without guarding in the left lower quadrant. The remainder of the physical examination is normal. The patient is alert and vigilant but inattentive to questioning and demonstrates disorganized thinking.

An order is written to hold the oxycodone.

What is the most appropriate next step in management?

Paroxetine

Diphenhydramine
Lorazepam
Zolpidem
No additional treatment 

400

A 76-year-old woman is evaluated in the emergency department for a stroke. She is given intravenous alteplase within 3 hours of onset. She also has hypertension. Her only outpatient medication is amlodipine.

On physical examination, temperature is 37.6 °C (99.7 °F), blood pressure is 160/76 mm Hg, pulse rate is 68/min and irregular, and oxygen saturation  is 96% with the patient breathing ambient air. She has global aphasia with no speech production, right facial weakness, and lack of movement in right arm and leg. National Institutes of Health Stroke Scale score is 18.

ECG shows atrial fibrillation.

CT of the head shows no acute findings.

What is the most appropriate next step in management?

CT angiography of the brain

Intravenous heparin
Intravenous labetalol
No additional intervention or treatment 

400

A 38-year-old man is admitted to the hospital for surgical resection of a glioblastoma. Following resection, he is expected to remain in intensive care for 2 days and discharged home by the third day.

What is the most appropriate measure to prevent venous thromboembolism immediately following surgery?

Mechanical prophylaxis

Low-molecular-weight heparin
Unfractionated heparin
No prophylaxis is needed 

500

A 56-year-old woman is evaluated for changes in her voice. Over the past year her voice has become strained and strangled, and she has had problems with expressing certain consonants. She reports no pain or shortness of breath but has intermittent tightness in her throat. She notes no other symptoms or medical problems and takes no medications.

On physical examination, vital signs are normal. Her voice is hoarse, and she speaks with a strained and pressured voice disrupted by sudden pauses, but she can sing without any problems. The remainder of the neurologic examination is normal.

Video laryngoscopy reveals slow, sustained, and nonrhythmic pulling of vocal cords toward midline with intermittent relaxations.

What is the most likely diagnosis?

Dystonia

Chorea
Functional disorder
Myoclonus
Tic 

500

A 30-year-old man is evaluated for a 1-hour history of a severe headache. He has had similar headaches for the past 2 weeks that start in the evening, last 2 to 3 hours, and are characterized by intense left-sided pain that is periorbital and piercing. Associated features are photophobia, nausea, and ipsilateral tearing. Nasal sumatriptan, acetaminophen, and ibuprofen have not relieved the pain. He has a 7-pack-year smoking history.

On physical examination, vital signs are within normal limits. Left ptosis and miosis are noted.

Results of laboratory studies and MRI of the brain with contrast are normal.

What is the most appropriate next step in management?

Subcutaneous sumatriptan

Carbamazepine
Indomethacin
Magnetic resonance angiography of the head and neck 

500

A 26-year-old man is evaluated for a 5-month history of jerking episodes that occur three to four times monthly. The jerking lasts less than 1 second. He is aware during the episodes and retains a complete memory of the event. He reports that sometimes the jerking occurs in clusters and he will have 10 to 20 jerks over a period of 30 to 90 seconds, often on awakening from sleep. He denies confusion or loss of muscle tone with either the single or clustered events. He has no other medical problems and takes no medications.

On physical examination, vital signs are normal. The neurologic examination is normal.

What is the most likely diagnosis?

Myoclonic seizures

Focal motor aware seizures
Generalized tonic-clonic seizures
Tonic seizures

500

A 31-year-old man is evaluated for a 5-mm saccular aneurysm in the anterior communicating artery found incidentally on a magnetic resonance angiogram of the head obtained to investigate new-onset trigeminal neuralgia. The patient is otherwise asymptomatic and has no additional medical problems. His only medication is carbamazepine.

On physical examination, vital signs, general physical and neurologic examinations are normal.

What is the most appropriate management?

Serial magnetic resonance angiography

Aneurysm clipping
Aneurysm coiling
Catheter-based angiography
CT angiography 

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