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Concentrically-layered Schwann-cell processes (“onion bulbs”) are seen surrounding nerve fibers on nerve biopsy in which of the following diseases?
Which of the following dermatomes are correctly matched with their associated anatomical location?
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 3-year-old girl is admitted to the hospital with a 2-day history of constipation, lethargy, and a weak cry. Repetitive stimulation performed at 20 Hz revealed incremental response. What is the likely cause of this patient’s illness?
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 CSF findings would be expected in a patient with AIDP?
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 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?
Which of the following dermatomes lies closest to the T2 dermatome, relative to the topographical position on the skin?
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?
Which of the following forms of Charcot-Marie-Tooth (CMT) disease causes axonal polyneuropathy?
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 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 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?
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?
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?
Which of the following is true regarding IVIG and PLEX therapy?
Which of the following is the most sensitive test for myasthenia gravis (MG)?
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?
A 65-year-old female with 40 pack-year history presents with a 2-week history of generalized weakness that improves with repeated testing. You are concerned for Lambert-Eaton myasthenic syndrome (LEMS). Which of the following statements is incorrect regarding LEMS and/or myasthenia gravis (MG)?
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 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 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?
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 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 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?
Anti-GQ1b antibodies are associated with which syndrome?
Which of the following nerves is a terminal branch of the posterior cord?
What is the mechanism of action of pregabalin?
Damage to which of the following muscles can lead to scapular winging?
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?
The pathologic findings on the image shown below can be secondary to bilateral mononeuropathies involving which of the following nerves?
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 autoantibodies are associated with myasthenia gravis?
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?
The paraspinal muscles are innervated by 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?
An EMG/NCS is performed for the evaluation of subacute left upper extremity weakness in an adult female. There are fibrillation potentials in the deltoid and biceps on EMG and decreased median and radial sensory amplitudes on NCS. Which portion of the brachial plexus was injured?
A 67-year-old male wakes up with a painful, right-sided, itchy rash coming across his abdomen like a band involving the umbilicus. His primary care provider is concerned that the patient has shingles. For this patient, re-activation of the varicella-zoster virus occurred at the dorsal root ganglion at which thoracic level?
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 56-year-old female presents to the clinic with right posterior leg pain. On examination, there is decreased sensation of the right posterior calf and weakness in right ankle plantarflexion. EMG testing shows fibrillations of the gastrocnemius and gluteus maximus muscles. EMG testing of the tibialis anterior, gluteus medius, and tensor fascia lata were normal. What is the most likely etiology of this patient’s symptoms?
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 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 1st toe bilaterally. Which of the following tests is the most likely to be diagnostic for this patient?
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?
Which of the following medications do not worsen myasthenia gravis 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?
Which of the following muscles is solely innervated by the C5 nerve root?
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?