Quiz 06/22/2022 (3)

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?

A 56-year-old male comes to the clinic with a burning and tingling sensation in his feet and hands for the past 4 months. He has a history of morbid obesity and underwent gastric bypass surgery last year. On physical exam, he has 3+ ankle and patellar reflexes. He also has a positive Babinski and positive Romberg sign. There is impaired position and vibration sense in his big toe bilaterally. This patient’s condition is most likely due to a deficiency of which of the following?

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?

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

A 65-year-old female presents with a complaint of burning and tingling of her feet. Symptoms started in her toes two years ago and now extend up to her ankles. Neurological examination showed normal strength and intact sensation to light touch and vibration but an impaired sensation to pinprick in the first toes bilaterally. If performed, which of the following studies would likely be abnormal?

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

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?

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 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?

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?

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?

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?

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 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?

Anti-GQ1b antibodies are associated with which syndrome?

What is the mechanism of action of pregabalin?

A 37-year-old female presents to the emergency room with diplopia and ambulation issues. Other than having an upper respiratory tract infection two weeks ago, there is no other significant past medical history. On physical exam, the patient has a decreased range of motion of the extraocular muscles bilaterally, diffuse areflexia, and marked dysmetria on finger-to-nose and alternating movements testing. Which of the following autoantibodies is most likely associated with this disease?

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

Which of the following forms of Charcot-Marie-Tooth (CMT) disease causes axonal polyneuropathy?

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 strength with both ankle dorsiflexion and foot inversion. Weakness of which of the following muscles is responsible for the patient’s symptoms?

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 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 40-year-old male is referred to the clinic for a one-year history of progressive weakness of the upper and lower extremities. Past medical history is impressive for thrombocytopenia, anemia, and hepatosplenomegaly. Neurological examination shows 2/5 strength in the distal lower limbs bilaterally with diminished ankle and patellar reflexes. The sensory exam shows diminished vibratory and pinprick sensation in both legs. He also has a markedly orange discoloration of his tonsils. Genetic testing is performed which reveals a mutation in the ABCA1 gene. Which of the following is the most likely diagnosis?

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?

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