Quiz 06/22/2022 (4)

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 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?

Which of the following genes is responsible for familial ALS?

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 is the mechanism of action of riluzole?

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?