NMJ BinAmir 1

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 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 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 synapsed of the peripheral nervous system is acetylcholine the primary neurotransmitter?

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

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 31-year-old man presents after being found down at the scene of an armed robbery. On physical examination, there is lower extremity weakness and loss of proprioception and vibration on the right side, as well as loss of pain and temperature sensation on the left leg. His sagittal CT spine is shown below. Which is the most likely condition suffered by this man?

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?

In the diagram below, what neurologic information is carried by the tract labeled with the green arrow?

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 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 65-year-old man presents to the emergency room with progressive weakness over the course of several months. Examination shows pure motor weakness with no sensory involvement, decreased reflexes, and diffuse fasciculations. An EMG shows a conduction block of motor neurons outside of compression sites and normal sensory conduction velocities. Which of the following of most appropriate for treatment?

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 37-year-old male presents with sudden onset of tachycardia and hypertension, followed shortly by unilateral rhythmic facial and then arm jerking. The autonomic symptoms of tachycardia and hypertension are most likely to reflect the involvement of which region of the brain during a seizure?

A 38-year-old man presents with a 1-year history of neck pain and left upper extremity weakness. The symptoms started insidiously, and have slowly worsened over time. A sagittal cervical spine MRI is shown below (Left: T2 without contrast. Right: T1 with contrast). Which of the following is the most likely cause of his 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 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 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?

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 78-year-old patient with a history of multiple system atrophy (MSA) presents to your clinic for falls. The patient states that while standing he initially experiences feelings of lightheadedness and tunnel vision before a loss of consciousness. While on the ground he will be “twitching” for 30 seconds before he regains consciousness. Which of the following tests is the most likely to be diagnostic in this case?

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 genes is responsible for familial ALS?

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?

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 65-year-old man presents to the emergency room with progressive weakness over the course of several months. Examination shows pure motor weakness with no sensory involvement, decreased reflexes, and diffuse fasciculations. An EMG shows conduction block of motor neurons outside of compression sites and normal sensory conduction velocities. Which of the following antibodies is associated with this disease?

A 22-year-old female with chronic daily headaches and other neurological symptoms has an MRI performed as part of her diagnostic workup. Her cervical MRI imaging is provided below. In addition to her chronic daily headaches, what are the most likely additional neurological symptoms she experiences?

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 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 autoantibodies are associated with myasthenia gravis?

Which of the following nerves when damaged can lead to scapular winging?

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

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

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 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 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 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?

A healthy 12-year-old female has a brain MRI performed because of a history of medically refractory headaches. Her imaging is shown below. What is the diagnosis?

Anti-GQ1b antibodies are associated with which syndrome?

A 66-year-old male presents to the emergency room as an unrestrained driver in a motor vehicle accident. While initially only complaining of mild neck pain, within 30 minutes of the collision he started to experience bilateral numbness in his hands and significant symmetric upper limb weakness. Soon thereafter, the weakness extended down to his lower extremities. Neurologic examination is remarkable for loss of pain and temperature sensation in his bilateral upper extremities and symmetric bilateral upper extremity greater than lower extremity weakness. Which of the following is the most likely diagnosis?

A 40-year-old previously healthy man arrives in the emergency room with a 1-week history of slowly progressive leg weakness. Symptoms initially started with burning-type pain in his bilateral lower extremities. Neurological examination shows 4-/5 strength in bilateral lower extremities and areflexia of the patella and Achilles. MRI is shown below. Which of the following is the most likely diagnosis?

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

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 CSF findings would be expected in a patient with AIDP?

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?

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

Identify the lesion shown below.

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 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 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 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?

Match the phase of the Valsalva maneuver with its associated physiology.

Which of the following muscles is solely innervated by the C5 nerve root?

Damage to which of the following muscles can lead to scapular winging?

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 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 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?

A 35-year-old male with a past medical history of intravenous drug use presents with back pain, fever, anorexia, and generalized weakness. An MRI of his sagittal spine is shown below. Which of the following is the most likely diagnosis?

The paraspinal muscles are innervated by which of the following?

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 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?

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 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 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?

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

Initiation of urination involves which of the following physiologic processes?

What is the mechanism of action of pregabalin?

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

A 68-year-old man with left leg weakness and pain has an MRI performed to evaluate for a possible etiology. Her imaging is provided below. Based on the location of the lesion, which of the following muscles is most likely weak on examination?

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?

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 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?

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

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

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 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?

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

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 medications do not worsen myasthenia gravis symptoms?

A 63-year-old woman with right leg weakness and pain has an MRI performed to evaluate for a possible cause. Her imaging is provided below. Based on the location of the lesion, which of the following muscles is most likely weak on examination?

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?

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

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?

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?

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

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 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 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 25-year-old patient presents to the emergency room with rapidly progressive lower extremity weakness that started in the feet and spread to the legs, which has also caused difficulty walking. On further inquiry, the patient had recently returned from a hiking trip in the New England area. On examination, flaccidity, and areflexia of lower extremities. Which of the following findings is the most diagnostic for this condition?

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