Quiz 05/31/2022

This muscle biopsy is most consistent with which myopathic disease? 

Which of the following brainstem auditory evoked potential (BAEP) results is most consistent with brain death?

Which of the following is the genetic mutation commonly seen in patients with hereditary neuropathy with liability to pressure palsy (HNPP)?

A 45-year-old man with a past medical history of IV drug use presents with a 3-day history of back pain and fever with numbness and tingling in his arms bilaterally. He is now unable to write his name or button his shirt. His cervical MRI is shown below. Which of the following is the most likely diagnosis?

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

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

A 20-year-old female presents to the clinic with weakness and numbness in the right leg. She states that earlier in the day she was taking a long nap on her right side, and when she awoke, her entire leg felt like pins and needles. When she got up, she felt that she was dragging her right leg on the floor. She has had similar experiences in other limbs over the years but symptoms would slowly improve over weeks. Nerve conduction studies showed a right common peroneal nerve conduction block and mildly decreased conduction velocities of all nerves tested. Her mother reports that her father had similar episodes before he passed away from an unrelated illness. Which of the following is the most likely diagnosis?

Which of the following correctly identifies the cranial nerve(s) that transverse through the structure (blue arrow) shown below?

A 45-year-old man with a past medical history of cardiac arrhythmia requiring pacemaker placement is referred to the clinic with a twenty-five-year history of slowly-progressive bilateral ptosis and diplopia. On examination, he has bilateral ptosis, decreased range of motion of the extraocular muscles, and mild proximal weakness in his upper and lower extremities. Which of the following is the most likely diagnosis?

A 27-year-old female with a history of cataracts and an unknown family history was referred to the neurology clinic for excessive daytime sleepiness. On examination facial weakness and grip myotonia were appreciated. Genetic testing revealed a mutation in the DMPK gene. Based on her diagnosis, which of the following tests should be performed annually?

A 26-year-old male suffered a spinal cord injury after falling from a ladder while hanging Christmas lights. In the ICU, he is experiencing episodes of sudden extreme increases in blood pressure, bradycardia, and diaphoresis. This condition is seen most commonly in lesions of which spinal cord levels?

A 67 y.o. female with HTN and atrial fibrillation on coumadin was in the Neuro ICU in a comatose state after a large intracranial hemorrhage. After a couple of days of monitoring, she had a new finding on her physical exam that prompted stat imaging, which showed signs of uncal herniation. Surgery was not performed due to goals of care preferences, and she, unfortunately, passed away from her ailment. An autopsy was requested. The image below shows compression on a cranial nerve from the herniation. Through which anatomic structure does this nerve pass to exit the skull?

A 24-year-old male presents to the clinic with numbness in the hands and feet. He has been experiencing these symptoms since his late teens, but the numbness has gotten progressively worse. Additionally, he has been stumbling and losing his balance more frequently in the past 18 months, and he received a new pair of glasses because he was having trouble with his vision at night. On physical exam, he has decreased sensation to vibration and pinprick in his bilateral lower extremities and palpable, enlarged nerves. Laboratory studies reveal an elevated level of phytanic acid. What is the most likely diagnosis?

The pathologic findings on the image shown below can be secondary to bilateral mononeuropathies involving which of the following nerves?

Which of the following medications, when taken alongside statin therapy, increases the risk of statin-induced myopathy?

Parents bring their 3-year-old son to the clinic for difficulty walking. On examination, the patient has a waddling gait and enlarged calf muscles. When rising from a seated position he has to rely extensively on his arms to stand up. Which of the following is the most likely inheritance pattern of the genetic disorder affecting this patient?

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 62-year-old woman with low back pain presents to the emergency department complaining of 4 weeks of a strange sensation when wiping with toilet paper, as well as urinary and fecal incontinence. On exam, she has 5/5 strength throughout her limbs and normal deep tendon reflexes. What is the location of her lesion?

A 30-year-old woman has visual evoked potentials performed that show a right eye P100 latency of 140 msec and a left eye P100 latency of 103 msec. Which of the following is the most likely cause?

Anti-GQ1b antibodies are associated with which syndrome?

A 20-year-old male presents with severe burns on his hands after accidentally touching a stove. Neurology was consulted because the family noted that the patient did not initially pull away from the hot stove as one would expect. Physical exam shows decreased sensation of pain and temperature along the back and arms, with intact strength. T2 sagittal MRI of the cervical spine is shown below. Which of the following is commonly associated with the pathology shown?

Which of the following transverses through the structure (blue arrow) shown below?

A 27-year-old previously healthy female is seen at the clinic for 5 days of leg weakness. The weakness has progressively been getting worse and now she has difficulty rising from a chair. Her past medical history is unremarkable except for a few days of an upset stomach and diarrhea that occurred two weeks ago. On physical exam, strength is diminished in the bilateral lower extremities. Deep tendon reflexes are 1+ in the lower extremities. A lumbar puncture is ordered. Testing of the cerebral spinal fluid (CSF) in this patient is most likely to show which of the following?

A 42-year-old woman presents to the clinic with a 2-month history of distal symmetric hand weakness that started after hospitalization for severe abdominal pain. Before this hospitalization, she was drinking alcohol with her friends when she acutely developed severe abdominal pain, nausea, restlessness, altered mental status, and hallucinations. She was noted to have dark urine at the time of admission. Since then, her GI symptoms and mentation have returned to baseline, but her weakness has persisted. On exam, she has 3/5 strength of the intrinsic muscles of the hand bilaterally. Conduction velocities of the bilateral upper extremities were unremarkable. Needle EMG revealed fibrillations and decreased recruitment of motor unit action potentials in muscles innervated by the radial and ulnar nerves. Which of the following is the most likely cause of the patient’s symptoms?

A 58-year-old male with a prolonged history of myasthenia gravis was recently discharged 4 months ago from the hospital with his second myasthenic crisis requiring transient intubation. Since that hospitalization, he was on prednisone, tapered down to 40 mg a day, cyclosporine and pyridostigmine. He comes to the ED today after having a generalized tonic-clonic seizure. On arrival to the ED, the patient is post-ictal and has vision deficits on exam. MRI of the brain shows T2 hyperintensities on the posterior cerebral hemispheres. Which of the following is the likely etiology of this clinical presentation?

A 36-year-old man with no significant past medical history presents with lower back pain, lower extremity weakness, and urinary incontinence. A non-contrast enhancing lesion was found on lumbar MRI (T1 sequence without contrast image shown below). What is the most likely diagnosis?

A neurology consult is placed on a full-term newborn male after being delivered by an uncomplicated vaginal delivery with good APGAR scores. The physical exam reveals a midline lesion in his lumbar region. Based on the pathology shown below, which of the following would most likely also be present on head imaging?

A 4-month-old boy is brought by his parents due to difficulty in feeding, and episodes of apnea. Physical examination reveals tongue fasciculations, a bulging anterior fontanelle, and an abducens nerve palsy. MRI reveals an Arnold Chiari malformation type II. What additional defect is this child likely to have?

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 47-year-old female presents to the clinic with the chief complaint of slowly progressive proximal muscle weakness. Electromyography showed short duration, low amplitude motor unit action potentials. A muscle biopsy was performed and shown below. A diagnosis is made and the patient is started on a therapeutic agent. What is the most likely medication initiated?

You are evaluating a 1-year-old who has had delayed motor development and diffuse weakness since birth and presents now to the hospital for a first-time seizure. The physical exam is notable for 3/5 deltoid abduction, 4-/5 hip abduction, 4-/5 hip flexion, and 4-/5 hip extension. He is unable to walk but is meeting cognitive milestones. An MRI brain is done and shows diffuse scattered T2 FLAIR white matter hyperintensities.

If genetic testing is performed, a mutation in which of the following genes would be most expected?

A 5-year-old male presents because of muscle stiffness. His symptoms are worse during the colder winter months and with gym class at school. He sometimes becomes so stiff he can hardly breathe. Examination shows no muscle atrophy or hypertrophy but myotonia is present. Upon further questioning, you learn he has an older brother with similar symptoms. What is the likely dysfunctional gene in this patient?

A 72 y.o. female presents to the clinic with complaints of decreased interest in eating, and has lost weight. She states foods have an unusual taste and are not appealing. On detailed examination, she is found to have normal extraocular movements, normal facial muscle symmetry, and normal facial sensation and swallowing. On further detailed examination, she has a loss of taste sensation in the right anterior two-thirds of her tongue. Of the following, which is the most likely area of injury?

A 48-year-old male with a history of a motor vehicle accident and right leg tibial plateau fracture was referred to a neurologist because of right leg weakness. He states he often catches his right foot while walking and stubs his toes when he walks barefoot. On examination, there is decreased ankle dorsiflexion and eversion of the foot. Weakness of which of the following muscles is responsible for the patient’s symptoms?

A 57-year-old male undergoes a cervical decompression laminoplasty for myelopathy, and on postoperative day #2, he complains of severe left shoulder pain. On examination, he has weakness with abduction of the left arm at the shoulder and flexion of the left arm at the elbow. This patient’s lesion localizes to which nerve root?

A 60-year-old woman with a history of diabetes is presenting to the neurology clinic with a 4-month history of burning/tingling in her bilateral toes. Neurological examination revealed 5/5 strength in all muscle groups, normal reflexes, and intact sensation to light touch in the bilateral lower extremities. There was mildly decreased sensation to pinprick in the 1^st toe bilaterally. Which of the following tests is the most likely to be diagnostic for this patient?

Which of the following features on EMG is suggestive of chronic denervation?

A 6-year-old female is seen in the clinic for decreased balance and numbness in her fingers and toes. Her father, who is with her at the clinic visit, states that she was developmentally normal until 2 years of age, at which time she began to have frequent falls. During the past year, she has also developed slurred speech. The neurological exam reveals truncal ataxia, ataxic gait, scanning speech, and decreased sensation to vibration and pinprick in her extremities. The ocular exam reveals irregular capillary dilation of the retina and skin. What is the most likely diagnosis?

Congenital muscular dystrophies are most often associated with which of the following pathophysiologies?

A 25-year-old female with no significant past medical history presents with a two-day history of progressive bilateral lower extremity weakness and numbness. On physical exam, she has 3/5 strength in hip flexion and knee extension bilaterally, along with a loss of sensation. She is up to date on all vaccinations. She is afebrile. Sagittal (left) and axial (right) T2 thoracic MRI images are shown below. Which of the following is the most likely diagnosis?

Which of the following is the most sensitive test for myasthenia gravis (MG)?

A 37-year-old female with a past medical history of chronic fatigue and heat intolerance presents with right arm weakness. A cervical spine MRI is performed and shown below (Left: T2 sagittal. Right: T2 axial). Which of the following is the most likely diagnosis?

Which of the following nerves is a terminal branch of the posterior cord?

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

Identify the structure (blue arrow) shown below:

Which of the following dermatomes lies closest to the T2 dermatome, relative to the topographical position on the skin?

A 57-year-old male with recently diagnosed small cell lung cancer (SCLC) is presenting to the electromyography lab with a one-month history of muscle cramps and muscle pain. Percussive myotonia is appreciated on examination. Electromyography showed myokymic discharges. Autoantibodies against which of the following channels are likely present in this case?

Which of the following is the relay center for sensory information of the body?

A 65-year-old female comes to the clinic complaining of weakness in his extremities. Symptoms are more prominent in the legs than in the arms. Oddly, when he tries to exert himself, he notes an improvement in his strength. Neurodiagnostic testing reveals low amplitude compound muscle action potentials (CMAPs) with an increase in amplitude on high frequency (50 Hz) rapid repetitive stimulation. Which of the following malignancies is most closely associated with this patient’s neurologic syndrome?

The trochlear nucleus sends nerve fibers to which of the following muscles?

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

Concentrically-layered Schwann-cell processes (“onion bulbs”) are seen surrounding nerve fibers on nerve biopsy in which of the following diseases?

A four-year-old boy with no significant past medical history is brought to your clinic due to difficulty walking. His mother states that he began walking at 12 months of age, but in the past few months, he seems to be more fatigued and weak when walking. On physical exam, his calves seem to be abnormally large. This disorder is caused by what mutation?

You are asked to evaluate a patient in the neurointensive care unit who is comatose and has had a somatosensory evoked potential performed. Which of the following is most suggestive of a poor prognosis for this patient?

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?

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

A patient with a history of a right shoulder anterior dislocation comes to the clinic with the chief complaint of right arm numbness. On examination, there is a loss of sensation to light touch on the lateral portion of the upper arm. Based on the sensory disturbance, which of the following nerves is most likely injured?

A 63-year-old female presents with a 6-month history of difficulty walking and muscle weakness. Neurologic examination reveals normal bulk but muscle fasciculations, bilateral extensor plantar responses, muscle spasticity, and hyperreflexia. Pathology at which of the following structures could explain these symptoms?

A 33-year-old female presents to the clinic for evaluation of a left lower extremity foot drop. On physical examination, there is weakness in dorsiflexion. Ankle eversion, inversion, and plantar flexion strength are normal. Hip abduction strength is normal as well. Which of the following is the most likely etiology for the patient’s symptoms?

A 32-year-old female presents to the emergency room after a motor vehicle accident as a restrained back seat passenger. Her only complaint on arrival is back pain. She has bruises across her abdomen and chest and a normal neurological exam. Thoracic spine imaging was completed and is shown below. Which of the following is the most likely diagnosis?

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?

A 40-year-old male presents to the clinic with difficulty swallowing. On additional questioning, he reports difficulty walking, falls, and dysarthria. Examination shows bilateral facial weakness, fasciculations, and gynecomastia. A CAG trinucleotide repeat on which of the following genes is the cause of this disease?

Which of the following autoantibodies are associated with myasthenia gravis?

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

A patient with medically refractory epilepsy secondary to mesial temporal sclerosis recently had a right hemispheric anterior temporal lobectomy. He is complaining of a post-operative visual field deficit. Which of the following is most likely to be appreciated on the exam?

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?

Of the following, which extraocular muscle is not innervated by the oculomotor nerve?

Which of the following is the thalamic relay center for proprioception of the face?

What is the typical brainstem auditory evoked response (BAER) finding with cortical hearing loss?

Based on the findings on the muscle biopsy (trichrome stain) shown below, which of the following is most likely seen on examination of this patient?

A 30-year-old male presents to the clinic with a fifteen-year history of progressive weakness and sensory loss in his lower extremities. Examination shows hammertoes, high arches on his feet, 4/5 ankle dorsiflexion strength, and a mild decrease in sensation to pinprick in the 1st toes bilaterally. Upon questioning he mentions that his father had similar-looking feet and started to use a cane in his fifties. What is the most likely diagnosis for his symptoms?

This muscle biopsy is most consistent with which myopathic disease?

A 47-year-old female presents to the clinic with the chief complaint of slowly progressive proximal muscle weakness. Electromyography showed short duration, low amplitude motor unit action potentials. A muscle biopsy was performed and shown below. A diagnosis is made and the patient is started on a therapeutic agent. What is the most likely medication initiated?

You are asked to see a 64-year-old man with hypertension, hyperlipidemia, and peripheral vascular disease who is post-operative day 1 from an aortic aneurysm repair. On exam, he exhibits bilateral lower extremity weakness, as well as loss of pain and temperature sensation below the umbilicus. Light touch and proprioception are intact in the lower extremities. What is the most likely diagnosis?

A 75-year-old man with hypertension and diabetes presents to the emergency department with a sudden onset of loss of sensation in the buttocks, perineum, and inner surfaces of both thighs, and urinary and fecal incontinence. On physical exam, he has loss of pain and light touch in the area described, as well as a loss of sphincter tone, and hyperreflexia in the lower extremities. Strength is mildly decreased in the distal lower extremities, without atrophy. What structure is most likely injured?

A 30-year-old male presents to the clinic with a fifteen-year history of progressive weakness and sensory loss in his lower extremities. Examination shows hammertoes, high arches on his feet, 4/5 ankle dorsiflexion strength, and a mild decrease in sensation to pinprick in the 1st toes bilaterally. Upon questioning he mentions that his father had similar-looking feet and started to use a cane in his fifties. Which of the following genetic mutations may be the cause of the patient’s symptoms?

A thin 28-year-old male presents to the clinic with complaints of foot drop. On exam, he has a right foot drop as well as numbness on the lateral aspect of the right lower leg. You suspect compression of a nerve at the fibular head. Malfunction of which of the following nerves causes the foot drop itself?

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

A 6-month-old male is admitted to the ED for acute respiratory failure. On examination, he has generalized weakness, diffuse hypotonia, and areflexia. He was admitted to the pediatric Intensive Care Unit and intubated. A chest X-ray showed a narrow chest. EMG/NCS was performed showing axonal motor polyneuropathy with tongue fasciculations and signs of chronic denervation. What is the most likely pathologic etiology causing this presentation?

Oculomotor nerve palsy is most likely to be seen secondary to an aneurysm in which of the following arteries?

Which of the following cranial nerve(s) transverses through the structure (blue arrow) shown below?

A 45-year-old male presents with left-sided neck and shoulder pain. On examination, there is right arm abduction weakness and decreased bicep and brachioradialis reflexes. An EMG was ordered and showed fibrillations of the deltoids, supraspinatus, and biceps muscles. EMG testing of the triceps, first dorsal interosseous, abductor policies brevis, and rhomboid muscles were normal. These findings are most likely due to radiculopathy of which of the following nerve roots?

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

A 57-year-old male with recently diagnosed small cell lung cancer (SCLC) is presenting to the electromyography lab with a one-month history of muscle cramps and muscle pain. Percussive myotonia is appreciated on examination. Electromyography showed myokymic discharges. Autoantibodies against which of the following channels are likely present in this case?

A 27-year-old presents to the clinic with finger extension, wrist extension, shoulder abduction, and shoulder adduction weakness. There is also numbness along the lateral and posterior arm. Which of the following is the most likely cause of the patient’s symptoms?

Eteplirsen is an effective treatment in patients with Duchenne muscular dystrophy (DMD) that have which of the following dystrophin mutations?

Which of the following genes is responsible for familial ALS?

A 65-year-old woman with a history of a known right posterior communicating artery aneurysm comes to the emergency department with focal neurological symptoms. Brain MRI shows interval enlargement of the aneurysm. Which of the following clinical symptoms is most likely?

A 56-year-old male with a past medical history of smoking presents to the clinic with the chief complaint of a 1-month history of progressive muscle weakness. Interestingly, he notes that his strength seems to improve with repeated use. The physical examination is notable for proximal muscle weakness of the bilateral lower extremities and areflexia in all extremities. Serology and electrodiagnostic testing confirm the diagnosis. What is the first-line therapy for symptomatic management?

A 40-year-old male presents with a one-day history of numbness of his right arm. This has never happened before, but he does report an episode of left leg weakness that self-resolved two years ago. The neurological examination confirms a sensory disturbance of his right arm. Sagittal MRI of the cervical spine is shown below (Left: T1, Middle: T1 with contrast, Right: T2). Which of the following is the most likely diagnosis?

A 35-year-old man developed a right clavicle fracture after an accidental fall from a ladder while hanging Christmas lights. He was treated conservatively with a figure-of-eight bandage and sling. He presents to your clinic 2 weeks after the accident. He states ever since the accident he has had right arm and shoulder pain, as well as numbness extending from his triceps to his middle dorsal aspect of the forearm, wrist, and dorsal aspect of digits 1,2 and 3. On examination, his strength is 5/5 throughout all extremities except 4/5 to abduction of the deltoid, extension of the triceps, and wrist extension.

Which nerve or cord, if injured by the clavicle fracture, would explain his symptoms?

A 26-year-old woman presents to the emergency department with third-degree burns covering the entire palmar aspect of her right hand. She claims that she was cooking dinner and touched the stove, but states that she “didn’t notice it was hot”. On neurologic exam, she is unable to distinguish between hot and cold sensations on her hands, arms, shoulders, chest, and back. She also fails to respond to pinprick sensation in the same distribution. She denies any major recent traumatic injuries. Which of the following is the most likely cause of her symptoms?

A 65-year-old male with a history of alcoholic cirrhosis presents to the emergency department with dark black coffee-ground emesis. He is found to have a systolic blood pressure of 76/40 and is tachycardic to 120 BPM. Which nerve is most responsible for carrying the afferent fibers from carotid sinus baroreceptors that cause the reflex increase in heart rate seen in this patient?

Which of the following medications is effective in the treatment of myotonia?

This muscle biopsy is most consistent with which myopathic disease?