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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 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 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?
Which of the following CSF findings would be expected in a patient with AIDP?
Which of the following forms of Charcot-Marie-Tooth (CMT) disease causes axonal polyneuropathy?
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?
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. What is the most likely diagnosis for his symptoms?
Which of the following is the genetic mutation commonly seen in patients with hereditary neuropathy with liability to pressure palsy (HNPP)?
Concentrically-layered Schwann-cell processes (“onion bulbs”) are seen surrounding nerve fibers on nerve biopsy in which of the following diseases?
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?
Anti-GQ1b antibodies are associated with which syndrome?
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 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?