Quiz 07/11/2022

A 71-year-old male with a history of mild ankle weakness and balance issues for the last 6 years is referred to the lab for a nerve conduction study (NCS). While performing the testing the patient states that a few of his siblings, as well as his father, had similar issues. Examination showed high arches and hammertoes. Based on the clinical history and NCS, what is the most likely diagnosis?

A 5-month-old female is brought to the emergency room by her parents because of an episode concerning for possible seizure. On arrival, the patient appears back to her clinical baseline neurologically. Several hypomelanotic lesions are appreciated while performing a skin check. The mother notes that her older brother has similar skin findings. An EEG is performed and shown below. A typical event was captured that correlated with an electrodecrement of cortical activity on EEG.

Based on the child’s diagnosis, what is the most appropriate therapy?

A 5-year-old male presents because of muscle stiffness. His symptoms are worse during the colder winter months and with gym class at school. He sometimes becomes so stiff he can hardly breathe. Examination shows no muscle atrophy or hypertrophy but myotonia is present. Upon further questioning, you learn he has an older brother with similar symptoms. What is the likely dysfunctional gene in this patient?

Which of the following is classically seen on electromyography in a patient with an acute neuronal injury?

What is the typical brainstem auditory evoked response (BAER) finding with cortical hearing loss?

A 6-year-old Amish female presents to the emergency department with a 2-month history of gradual cognitive decline and jerking limb movements. A routine EEG was obtained (see below). Which of the following diseases is consistent with these EEG findings?

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?

The electrical fields that generate the cortical activity appreciated on electroencephalogram (EEG) are the result of which of the following?

This muscle biopsy is most consistent with which myopathic disease?

A neurology consult was placed for a 35-year-old male who was admitted overnight after a motor vehicle accident that occurred earlier in the day. He experienced multiple bone fractures, including the right humerus at the spiral groove. The primary team is concerned about a radial nerve injury. Which of the following findings are most likely to be found on EMG/NCS?