Quiz 07/23/2022

A 4-year-old boy is brought to the pediatric neurology clinic with 4 months history of progressive dysarthria, ataxia, and abnormal limb movements. His mother says that the patient’s right side of the mouth seems to be pulled to the right and his left arm always seems to be pulled up towards his chest. An MRI was performed and shown below. What is the likely diagnosis?

A 3-day-old male infant is evaluated in the NICU for fever and focal seizures. He was born at 39 weeks gestation via uncomplicated vaginal delivery and had good APGAR scores. Brain imaging shows periventricular calcifications and severe ventriculomegaly. Auditory testing also demonstrates bilateral hearing impairment. What is the most likely cause of the patient’s symptoms?

A 30-year-old woman has visual evoked potentials performed that show a right eye P100 latency of 140 msec and a left eye P100 latency of 103 msec. Which of the following is the most likely cause?

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 43-year-old man with obesity and hypertension presents with complaints of excessive daytime sleepiness, gasping awakenings, and witnessed periods of not breathing during sleep. A polysomnogram is performed and confirms the presence of sleep-disordered breathing. What feature would point to a central rather than obstructive cause?

An otherwise healthy 17-year-old female presents to the emergency department with right arm weakness. The symptoms started insidiously over the weekend and haven’t improved over the last 3 days. Examination shows 4/5 strength of the right upper extremity. A brain MRI is performed and shows a T2 hyperintense lesion in the subcortical white matter with contrast enhancement. No other lesions are seen. Upon further questioning, she states that she has never experienced any similar episodes of transient neurologic symptoms before. Which of the following is the correct diagnosis?

Which of the following is the most likely cause of the findings seen on this gross pathology image?

Which of the following is true regarding IVIG and PLEX 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 an appropriate test to assess for dorsolateral frontal lobe dysfunction?

Socially inappropriate behavior, disinhibition, echopraxia, and utilization behavior are typically seen in which of the following?

A 57-year-old navy veteran with a history of PTSD presents with a 2-year history of progressive, asymmetric weakness. The patient states that symptoms started in his left arm. Six months later he started to have falls due to tripping over his right foot. His neurological examination shows weakness of the intrinsic hand muscles of the left arm, ⅖ strength on dorsiflexion of the right ankle, and no sensory disturbances. Reflexes were mute in the left upper extremity and there were several beats of clonus at the right ankle. Fasciculations were present in the tongue, right gastrocnemius, and left tibialis anterior. An EMG is recommended for diagnosis. Which of the following EMG findings are most likely seen in this patient?

Which of the following is associated with a “delta brush” pattern on EEG?

Paraphasic and semantic errors in speech can occur secondary to a lesion in which of the following regions?

A 56-year-old man with idiopathic Parkinson’s disease presents to the emergency department for hypertensive emergency and tachycardia. You suspect that this may be an adverse effect related to one of his medications. Patients that are prescribed MAO-B Inhibitors should be counseled on avoiding which of the following?