Quiz 07/11/2022 (2)

An 80-year-old female presents with abnormal eye movements and cognition. She has also been experiencing problems with gait and memory. An MRI of the brain revealed midbrain atrophy, with relative sparing of the other brainstem structures. Which of the following is the most likely diagnosis?

Which of the following dermatomes are correctly matched with their associated anatomical location?

A 42-week gestational age female was born vaginally to a mother with a history of type 2 diabetes. Delivery was complicated by shoulder dystocia and required assistance with forceps. Apgar scores at 1 and 5 minutes were seven and nine, respectively. Her birth weight was 9 pounds and 6 ounces. Examination after birth showed there was no appreciable left arm bicep or deltoid function. What is the most likely etiology for this finding?

In normal neonatal development, which of the following structures myelinates the latest?

Which of the following pathologic findings is associated with posterior cortical atrophy?

Patients with Charcot-Marie Tooth disease that have the CMT1A phenotype will have which of the following genetic mutations?

Which of the following genes is responsible for familial ALS?

Which of the following is the relay center for hearing?

What seizure type is most commonly seen with the pathology shown in the image below?

A 42-year-old woman with multiple sclerosis presents to the ER after having worsening confusion over a few days with worsening weakness and vision loss. She was on natalizumab for years and her most recent JCV antibody titer was negative. She has missed several months of infusions due to her helping a sick relative. Her MRI shows multiple new T2 hyperintensities with enhancement in the periventricular and subcortical white matter. What is the most likely diagnosis?

Estrogen-containing oral contraceptive pills are known to reduce serum levels of which of the following medications?

A 74-year-old male with a past medical history of hypertension and hyperlipidemia is brought to the physician by his daughter who states that he has been exhibiting abnormal shaking of his hands and difficulty walking for the past year. On exam, the patient exhibits a shuffling gait, a tremor of his left hand at rest, and difficulty initiating movements. Which of the following is the most appropriate first-line therapy?

Which of the following is the mechanism of action of tetrabenazine?

Which of the following findings would most likely be found on histopathological analysis of the lesion shown in the image below?

A 32-year-old man was recently admitted to the stroke service for an intracranial hemorrhage secondary to an arteriovenous malformation. In addition to his acute focal neurological deficits, the following skin lesions are appreciated (see image below). A genetic syndrome is suspected. Based on the most likely clinical diagnosis, what is the risk of his son having the same condition?

A 19-year-old woman presents for evaluation of paroxysmal episodes of vertigo, spasticity, diplopia, and ataxia. She reports that her father and one of her two sisters have similar ailments. At the time of the appointment, there was no diplopia or ataxia appreciated. Myokymia was appreciated on electromyography. Which of the following is the most likely diagnosis?

What type of tumor is showcased in the pathologic image below?

A 68-year-old male with a 10-year history of Parkinson’s disease comes to the clinic for routine follow-up. He currently is taking levodopa/carbidopa, entacapone, and ropinirole. The patient is complaining of significant dyskinesias and unpredictable motor fluctuations between doses of levodopa/carbidopa. What is the most appropriate next best step in management?

Stiff person syndrome is associated with which of the following auto-antibodies?

A patient with a history of low back pain had an MRI of their lumbar spine completed as part of their workup. The T2 FLAIR image of the lumbar spine MRI is shown below. What is the most likely diagnosis?

Which of the following does this EEG tracing reveal?

An 8-year-old girl presents to the emergency room with a one-week history of progressive ataxia and headache. An MRI was performed and shown below. A biopsy was performed and Homer-Wright rosettes were found. What is the most appropriate treatment for this lesion?

Which of the following CSF findings would be expected in a patient with AIDP?

A 38-year-old male with a history of sarcoidosis and a one-week history of subacute onset of progressive weakness was admitted to the hospital with difficulty ambulating. On examination, you appreciate bilateral lower extremity weakness and hyporeflexia. Based on the clinical history and EMG findings shown below what is the most likely diagnosis?

389 Patients who had acute unilateral optic neuritis were enrolled between 1988-1991 in a study and followed for 15 years to assess their risk for developing multiple sclerosis (MS). They found that the probability of developing MS within 15 years was 50%. What type of study was this?

An 11-year-old girl has a neurosurgical resection of a brain mass. You are consulted post-op day 2 due to speech decline progressing to complete mutism. She does not have any weakness on exam. What type of tumor was most likely resected?

A 67-year-old woman with a past medical history of osteoporosis, hypertension, bipolar disorder, and a remote right MCA stroke is referred to the epilepsy clinic after experiencing her first epileptic seizure. An EEG has shown right temporal epileptiform discharges. Which of the following medications are most appropriate to start as initial therapy?

A 49-year-old female has had a 6-month history of difficulty swallowing and weight loss. On examination, she has generalized muscle loss. On strength examination, she has 3/5 proximally and 4/5 distally in the bilateral upper extremities. There are normal tongue movements and a normal jaw jerk reflex. Deep tendon reflexes in the bilateral upper extremities are normal and sensation is intact. An EMG is performed and outlined below. What is the most likely diagnosis?

Identify the lesion below.

A 34-year-old man with medically-refractory epilepsy has an MRI brain completed. The lesion shown (T2 sequence) does not enhance with contrast. If a surgical resection was performed, which of the following would be the most likely seen on pathologic analysis?

A 24-year-old patient had a severe unhelmeted motorcycle accident and despite best efforts by medication providers, he passed away two days later. An autopsy was performed. Identify the structure marked by the white arrow.

A 12-year-old male is brought to the clinic accompanied by his father who states that for the past 4 months he has been randomly flailing his arm during class and calling out in the middle of the teacher’s lectures. He has been sent to the principal’s office twice in the past month for his disruptions. His father states that this is very out of character for him. In the clinic, the patient has intermittent movements of his right arm, where he raises his arm above his head and then slaps it down on his leg. Additionally, during the exam, he blurts out words that do not make contextual sense in the conversation. Two months later, the patient follows up with his father who states that all of the abnormal movements and vocalizations have resolved. What is the most likely diagnosis?

A 4-month-old male with no significant past medical history was admitted to the epilepsy monitoring unit with episodes concerning for possible seizures. The family has noticed that events tend to occur when the child is angry. One episode is captured during his stay. The first symptoms appreciated were excessive crying followed by loss of tone and a few myoclonic jerks for 20 seconds. The patient then rapidly returned to baseline thereafter. There were no epileptiform discharges seen during this episode. Based on the most likely diagnosis, what is the most appropriate next step in management?

Which of the following arteries supplies the lumbar spinal cord?

A 30-year-old male presents with progressive dysphagia over the last month. Head imaging is completed and shown below. Which of the following is the most likely diagnosis?

A 62-year-old 50 kg female with a history of myasthenia gravis presents to the hospital with shortness of breath and generalized weakness felt to be secondary to a myasthenic crisis. Her medication history in the chart states she is currently taking pyridostigmine 60 mg QID, prednisone 20 mg daily, and just started cyclophosphamide 4 weeks ago. She was safely intubated in the ED with a NIF of -18 cm H2O and a VC of 600 ml. Select the appropriate next steps regarding pharmacotherapy and management for a myasthenic crisis.

I. Discontinue pyridostigmine
II. Increase the dose of prednisone
III. Increase the dose of azathioprine
IV. Consult general surgery for thymectomy
V. Consider PLEX or IVIg therapy
VI. Start rituximab

Which of the following medications do not worsen myasthenia gravis symptoms?

A 78-year-old male with a past medical history of depression and chronic alcohol use presents to the clinic with his wife for evaluation of a tremor. He states that the tremor is most severe at rest and disappears with purposeful movement. His wife states that he has been having slower movements over the past year. On physical exam, a “pill-rolling” resting tremor is noted in his left hand and the patient has difficulty with gait initiation and takes short steps when walking forward. Which dopaminergic pathway is most likely affected in this patient?

A 40-year-old woman with a history of neurofibromatosis type 2 is presenting to the clinic with chronic anosmia and progressive left eye vision loss. Visual acuity testing reveals 20/200 OS and 20/20 OD. There is a left eye afferent pupillary defect. Funduscopic examination shows pallor of the left optic disc and blurring of the disc margins of the right optic disc. An MRI brain is ordered to localize the lesion. Based on the clinical examination, where will the pathologic lesion most likely be found?

A 32-year-old Chinese male is referred to the epilepsy clinic after experiencing two episodes concerning for possible seizures. The first episode was unwitnessed, but the patient states that there was an abrupt loss of consciousness and that he woke up with the side of his tongue bitten and generalized myalgias. The second event was witnessed by his wife who noticed one day that the patient was unresponsive and lip-smacking for 2 minutes before returning back to baseline. MRI brain was performed and showed left-sided mesial temporal sclerosis. Which of the following medications should be avoided for this patient?