Quiz 07/27/2022 (1)

An 8-year-old child with a past medical history of tuberous sclerosis presents urgently to the emergency room with lethargy. Funduscopic examination shows blurring of the optic discs. An MRI is performed and shown below. Which of the following is the most likely cause of the patient’s symptoms?

A 65-year-old male presents with an acute onset of back pain, bilateral lower extremity weakness, and bladder incontinence. A physical exam reveals decreased strength and loss of pain and temperature sensation in both legs. Vibratory and light touch sensation and proprioception remain intact. A lesion to which of the following arteries would most likely be responsible for this clinical presentation?

A 50-year-old female with a history of small cell lung cancer treated with chemotherapy and radiation presents to the neuromuscular lab with progressive right upper extremity weakness. The video below is a recording of her EMG of the biceps brachii at rest. What is the abnormal pattern shown?

A 25-year-old male with a history of sensorineural hearing loss and tinnitus has an MRI brain ordered as part of his workup (see below). A genetic syndrome is suspected. Which of the following is also likely to be found on imaging?

A 52-year-old woman is brought to the emergency room in respiratory distress. She is accompanied by her husband who stated that they were eating at a high-end sushi restaurant when his wife started to complain of perioral numbness and tingling. Two hours later she started to complain of generalized weakness and trouble breathing. Accidental poisoning is suspected. Based on the available information, what is the mechanism of action of the poison ingested?

Which of the following is classically seen on electromyography in a patient with an acute neuronal injury?

A 65-year-old male is brought in by his daughter due to progressive headache and memory difficulties. MRI of the brain was ordered and is shown below (Left: T2 FLAIR, Right: T1 with contrast). A biopsy of the lesion would most likely reveal which of the following?

A 16-year-old male with no significant past medical history presents to the emergency room with vision complaints and coordination issues. Four days ago, he began experiencing double vision and since then has had progressive difficulty picking up objects and buttoning his shirts. On physical exam, the patient has a decreased range of motion of the extraocular muscles bilaterally, as well as areflexia and ataxia when walking. What is the most likely diagnosis?

Which of the following is not one of the absolute exclusionary criteria for tissue plasminogen activator (tPA)?

A 32-year-old female with an acute ischemic stroke was sent for an urgent angiography. Below is the image captured from this study. What is the likely etiology of the patient’s acute ischemic stroke?

A 65-year-old male is referred to the EMG lab with a recent right leg injury and associated weakness. Based on the EMG shown below, where is the most likely site of neuronal injury?

You are caring for a patient with Lewy body dementia who is having difficulty with myoclonic jerks. Which of the following is considered the first-line treatment for cortical myoclonus?

A 6-month-old previously healthy male is seen in your clinic due to feeding difficulties and weakness for the past 8 days. The mother reports that he has also not had a bowel movement in 3 days. On physical exam, the patient demonstrates a weak cry, severe hypotonia, and hypo-responsiveness. No one else in the family is sick, however, they have been eating homemade molasses for the past 4 weeks. This patient’s condition is caused by a toxin affecting which structure?

A 24-year-old female in her third trimester of pregnancy, with no other past medical history, presents with complaints of 1-2 times monthly episodes of sudden onset severe room spinning, nausea, and vomiting for 3 months. They happen randomly and are not associated with head movement. During an episode, sounds and bright light seem to bother her more. She denies motor weakness or hearing change. Her spells resolve after about 1 hour. She endorses she has had bad headaches in the past, but not with these episodes. She endorses she gets car sick easily. On neurologic examination, she has no focal findings, and her vitals are within normal limits. She had an MRI brain with MRA and MR Venogram, all of which were unremarkable. Which is the most likely etiology?

A 78-year-old male with a past medical history of diabetes presents to the clinic with a 2-year history of progressive lower extremity numbness and pain. He describes the pain as “pins and needles”. Physical examination reveals loss of pinprick sensation to the ankles bilaterally, mute Achilles reflexes, and moderate difficulty with tandem gait. A diagnosis is made based on examination and clinical history. What is the mechanism of action of the most appropriate first-line therapeutic agent?

A healthy 24-year-old female was admitted to the epilepsy monitoring unit for spell classification. What state is the patient during this segment of recording?

A 69-year-old male presents to the clinic with his wife who states that for the past 10 months the patient has become increasingly slow and more clumsy with daily activities. He has also been complaining of dizziness, especially in the morning when he first gets out of bed and has fallen once in the past month. On physical exam, it is observed that the patient has a 21 mmHg drop in systolic blood pressure when going from a sitting to a standing position. A brain MRI is ordered and shows a cruciform hypointensity in the pons. What is the most likely diagnosis?

Identify the tract marked by the red arrow in the spinal cord diagram below.

A 6-year-old girl presents to the clinic with frequent episodes of awakening from sleep in extreme fear. Sometimes she has a brief twitching of the limbs with this. She has these events in clusters on some nights, lasting seconds to minutes. On further questioning, you discover the mother had a similar problem when she was around this age, which slowly got better over time. Both patient and mother are otherwise healthy with no intellectual deficits or symptomatic features. Which of the following gene mutations do they both likely share?

A 38-year-old man with a past medical history of HIV, depression, and asthma presents after experiencing a first-time seizure. He was last seen normal about 45 minutes ago by his husband, who witnessed him seizing for about 10 minutes. A CTH is performed and normal. A lumbar puncture is performed and reveals the findings below. Which of the following is the most likely cause of the patient’s symptoms?

Which of the following is a common side effect of dimethyl fumarate?

A 3-year-old female with a past medical history of recurrent sinus infections is brought to the clinic by her father because of unsteadiness on her feet for the past 4 months. Her father states that she began walking at 12 months. However, more recently, she has been losing her balance and has been falling. On physical exam, she has truncal ataxia and an ataxic gait. An ocular exam reveals dilation of the retinal capillaries. Labs are ordered that show an elevated alpha-fetoprotein. Which of the following is the most likely diagnosis?

Which of the following medication regimens would put a patient at the highest risk of medication overuse headaches?

A 15-day-old female is evaluated in the NICU after being born to a G2P2 mother. On exam, the infant is jaundiced. Abdominal ultrasound shows severe hepatosplenomegaly. CT scan of the head shows hydrocephalus and periventricular calcifications, and a fundoscopic exam shows bilateral chorioretinitis. Serological testing is most likely to show infection with which organism?

The ophthalmic branch of the trigeminal nerve exits the skull from which cranial foramina?

A 13-year-old boy presents with worsening severe headaches, nausea, vomiting, sleepiness, and confusion. The symptoms have been episodic, improved somewhat by lying recumbent. An MRI is done and shows the following. Which of the following is the best treatment option?

A 55-year-old male presents to the clinic with a complaint of right lower extremity pain and weakness. On examination of the right lower extremity, there is decreased sensation to pinprick on the medial calf and foot, 4/5 strength with knee extension, and a mute patellar reflex. These findings are most likely due to radiculopathy of which of the following nerve roots?

Which of the following are actions of the superior rectus muscle?

A 20-year-old male presents to your clinic for weakness. He is the star baseball player at the local university, but during the season his batting average has been dropping, and he finds he is having trouble standing on his toes, performing overhead shoulder presses, and more recently ascending steps. You give him a script for some blood testing and an EMG/NCS and schedule a follow-up in 2 weeks.

Unfortunately, shortly after returning home he had a fall down a flight of stairs and sustained a compound radius fracture. He was taken to the emergency room where an open reduction and fixation (ORIF) was performed. Shortly after the surgery, he developed tachycardia, tachypnea, and masseter muscle contractions, which then progressed to fever and rigidity.

Which of the following test results is most likely to be seen in this patient?

A 39-year-old male presents with right-sided weakness and aphasia. NIHSS is 22 on physical exam. He was last known well 2.5 hours prior. The past medical history does not reveal any contraindications to tPA. A CT head is done and shows a hyperdense left MCA sign. The tPA is mixed by the nursing staff while you consent his wife for its administration. When you return to the bedside his aphasia has improved but his NIHSS is still 13. The blood pressure is 189/105 and the glucose is 225. What is the most appropriate next step?

Which of the following is seen on muscle biopsy in patients with steroid myopathy?

A 46-year-old female presents to the clinic with right-hand numbness, specifically in the 4th and 5th digits. EMG testing reveals fibrillations on the first dorsal interosseous, and flexor pollicis longus. EMG testing of the abductor pollicis brevis and triceps was unremarkable. These findings are most likely caused by radiculopathy of which of the following nerve roots?

A 65-year-old right-handed man presents with an acute onset of aphasia. CT scan of the head was normal but the MRI revealed multifocal small acute and subacute ischemic strokes in multiple vascular territories. A cerebral angiogram was performed as part of the diagnostic workup. Based on the clinical history and imaging provided what is the most likely diagnosis?

A 5-year-old girl is brought in by her parents because of 1 month of episodes of screaming at night. She has no recollection of these events in the mornings and she is developmentally appropriate and healthy otherwise. What is the best treatment?

An 80-year-old woman with uncontrolled HTN presents to the ED with symptoms upon awakening of left hemi-sensory loss, last seen well 8 hours prior. MRI is done and shows a lateral thalamic lacunar stroke. The small branch affected in this lacune is most often a branch of which major artery?

An 8-month boy is brought to the pediatrician by his parents with the chief complaint of muscle weakness and poor feeding. The boy’s parents recently emigrated from Latin America where they didn’t have good access to medical care. Physical examination is concerning for hepatomegaly and hypotonia. A muscle biopsy was completed and shown below. What is the most likely diagnosis?

A 26-year-old female presents with unilateral vision loss. She says that she noticed symptoms upon awakening yesterday morning and it has gotten progressively worse since then. On neurological examination, she has 20/70 visual acuity of the left eye and 20/20 of the right. There is a left afferent pupillary defect (APD). Extraocular movements are intact but cause pain. What is the most likely etiology of her symptoms?

A 23-year-old female with relapsing-remitting multiple sclerosis (RRMS) that is well-controlled on teriflunomide presents for routine follow-up. Today she expresses interest in having a child. Which of the following statements regarding multiple sclerosis and pregnancy is true?

Which of the following is true regarding IVIG and PLEX therapy?

Which of the following criteria for brain death based on EEG recording is incorrect?

Other than optic neuritis, which of the following is another typical MRI finding in patients with neuromyelitis optica (NMO) that has diagnostic value?

Which of the following medications is associated with the occurrence of reversible cerebral vasoconstriction syndrome (RCVS)?

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

A patient presents with double vision that improves when tilting their head to the right. A lesion is most likely to be present in which of the following cranial nerves?

Which direction are the patient’s eyes moving at the points marked by the green circles?

A pure sensory stroke is most likely to damage which structure?

A 57-year-old male with HIV who is non-compliant with HAART therapy presented to the emergency room with a 4-week history of altered mental status and falls. On neurological examination, the patient was altered, ataxic, and had right upper extremity weakness. An MRI was performed and shown below. Based on the clinical presentation and imaging provided, what is the most appropriate first step in medical management?

Patients with Krabbe disease have a pathologic accumulation of which of the following?

Which of the following does this EEG tracing reveal?