A 53-year-old woman comes to the physician because of a 3-year history of increasing weakness of her extremities and neck pain that is worse on coughing or sneezing. She first noticed weakness of her right upper extremity 3 years ago, which progressed to her right lower extremity 2 years ago, her left lower extremity 1 year ago, and her left upper extremity 6 months ago. She has had difficulty swallowing and speaking for the past 5 months. Vital signs are within normal limits. Examination shows an ataxic gait. Speech is dysarthritic. Muscular examination shows spasticity and muscle strength is decreased in all extremities. There is bilateral atrophy of the sternocleidomastoid and trapezius muscles. Deep tendon reflexes are 4+ bilaterally. Plantar response shows an extensor response bilaterally. Sensation is decreased below the C5 dermatome bilaterally. Which of the following is the most likely cause of this patient's symptoms?
ACerebral glioblastoma multiforme
BForamen magnum meningioma
CCerebellar astrocytoma
DAmyotrophic lateral sclerosis
EMultiple sclerosis
A 62-year-old woman is brought to the physician because of 6 months of progressive weakness in her arms and legs. During this time, she has also had difficulty swallowing and holding her head up. Examination shows pooling of oral secretions. Muscle strength and tone are decreased in the upper extremities. Deep tendon reflexes are 1+ in the right upper and lower extremities, 3+ in the left upper extremity, and 4+ in the left lower extremity. Sensation to light touch, pinprick, and vibration are intact. Which of the following is the most likely diagnosis?
AGuillain-Barré syndrome
BAmyotrophic lateral sclerosis
CSpinal muscular atrophy
DMyasthenia gravis
ESyringomyelia
A 29-year-old man comes to the emergency department because of progressively worsening fatigue and shortness of breath for the past 2 weeks. His only medication is insulin. Examination shows elevated jugular venous distention and coarse crackles in both lungs. Despite appropriate life-saving measures, he dies. Gross examination of the heart at autopsy shows concentrically thickened myocardium and microscopic examination shows large cardiomyocytes with intracellular iron granules. Examination of the spinal cord shows atrophy of the lateral corticospinal tracts, spinocerebellar tracts, and dorsal columns. Which of the following is the most likely underlying cause of this patient's condition?
ACTG trinucleotide repeat expansion on chromosome 19
BSOD1 gene mutation on chromosome 21
CSMN1 gene mutation on chromosome 5
DGAA trinucleotide repeat expansion on chromosome 9
EDystrophin gene mutation on the X chromosome
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