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A 4-year-old male with no significant past medical history presents to the emergency room after experiencing a first-time seizure. An EEG is obtained and captures a seizure arising from the left hemisphere and polymorphic delta slowing on the same side. Over the next several months the child continues to experience focal-onset seizures despite being on 2 maximally-dosed seizure medications. His brain MRI, below, shows asymmetric left-hemispheric atrophy. His clinic presentation and imaging are most consistent with which of the following diseases?
Gaucher disease is caused by a homozygous mutation in the GBA gene which encodes the enzyme beta-glucocerebrosidase. Which of the following neurologic disorders is also linked to mutations, both homozygous and heterozygous, in the GBA gene?
A 50-year-old male has noted winging of his right scapular and pain for the past four months following a hyperextension injury. Based on the EMG data outlined below where is this patient’s pathological lesion?
The F wave response is an:
A 27-year-old woman presents with recurrent episodes of tinnitus, vertigo, and hearing loss. She denies any associated headaches. Episodes typically last between 2 and 6 hours in duration. MRI of the brain with and without contrast did not reveal any pathology. Physical examination is unremarkable except for sensorineural hearing loss appreciated in the right ear. Which of the following is the most likely diagnosis?
A 65-year-old woman with a past medical history of hypertension, hyperlipidemia, and migraine headaches previously well-controlled on propranolol, presents to the clinic with new headache characteristics. The patient states that she recently has a new scalp tenderness. On further history, she endorses jaw soreness after eating. What is the most appropriate initial diagnostic test?
Socially inappropriate behavior, disinhibition, echopraxia, and utilization behavior are typically seen in which of the following?
Which of the following medications is approved only for moderate to severe Alzheimer’s Disease?
An 18-year-old woman presents as a new patient to your clinic for “seizures”. On review of her medical records, you find that she has had numerous ED visits and inpatient admissions for generalized shaking spells. Several of them have been captured on video EEG recording and there was no EEG correlate with the behavior, suggesting that they are psychogenic non-epileptic attacks/spells (PNEA/PNES). She says she is seeing you today because “I need someone to refill my seizure medications.” Which of the following basic principles of medical ethics could be best applied to this scenario?
A 49-year-old male with a chronic history of myalgias was referred to the EMG lab with progressive muscle weakness. Based on the EMG table shown below what is the most likely diagnosis?
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 34-year-old male presents with personality changes that started after a motor vehicle accident-related traumatic brain injury. His family has noticed that since the accident, the patient no longer actively seeks out hobbies that he once enjoyed. He will often forget to eat meals unless prompted, even if the food is placed in front of him. Which of the following medications would most likely help with his behavior?
A 6-year-old girl presents to the clinic with frequent episodes of awakening from sleep in extreme fear. Sometimes she has a brief twitching of the limbs with this. She has these events in clusters on some nights, lasting seconds to minutes. On further questioning, you discover the mother had a similar problem when she was around this age, which slowly got better over time. Both patient and mother are otherwise healthy with no intellectual deficits or symptomatic features. Which of the following gene mutations do they both likely share?
Which of the following is the mechanism of action of memantine?
A 50-year-old female presents with altered mental status. Her family states that over the past couple of weeks, the patient has had progressive memory loss, mood changes, and poor judgment. On examination, the patient has involuntary arm movements and an exaggerated startle response. Brain MRI, DWI sequence, is shown below. Which of the following is the most likely diagnosis?
Identify the lesion shown in the image below.
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?
Which of the following blood vessels that supply the thalamus is the only one to branch off of the posterior communicating artery (PCOM) instead of the posterior cerebral artery (PCA)?
A 28-year-old caucasian male was last seen well at 9:00 AM when he got into a verbal argument with his girlfriend. When the girlfriend returned from work at 5:00 PM, she found the patient on the floor of the garage altered, and states he “smelled unusual”. In the emergency department, the patient is disoriented but arousable with much effort. He complains of a headache and blurred vision, and he appears uncomfortable with a respiratory rate of 40. You send stat labs and they are listed below. What treatment should you administer immediately?
ABG: 7.18 pH, PCO2 = 28, PO2 = 118
Electrolytes: Na 135 mEq/L, K 5.0 mEq/L, Cl 105 mEq/L, HCO3 15 mEq/L
A 19-year-old boy presents with his mother for complaints of frequent eye blinking and shoulder-shrugging spells occurring multiple times a day. He also has frequent episodes of humming a three-note tune randomly throughout the day. He has had these problems since he was 17. He had an MRI brain and EEG that were negative. He takes no medications. The symptoms do not impact his activities of daily living, but he does feel embarrassed by the symptoms. He went to cognitive behavioral therapy for this for a year and has not seen a benefit. Which of the following is a first-line medication for this patient?
Identify the pattern seen in the 5th second of this EEG recording.
A 68-year-old female presents to your clinic with the chief complaint of daytime somnolence. She endorses an unpleasant feeling in her lower extremities while trying to go to bed which prevents her from sleeping. Moving her limbs gives transient relief. What is the most appropriate first diagnostic test?
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?
Identify the lesion shown in the image below.
How long ago did the ischemic injury seen on this neuronal tissue micropathology slide occur?
Which of the following medications is the most effective in the management of PTSD-associated nightmares?
Which one of the following pairs regarding antipsychotic drugs and their adverse effects is incorrect?
A 23-year-old female with a past medical history of depression presents with right arm paralysis that started after she found out her boyfriend died from a motor vehicle accident. Her physical exam shows no effort during muscle strength testing and positive withdrawal when a noxious stimulus is applied. The rest of the neurological examination, including reflexes, was normal. MRI of the brain and cervical spine were unremarkable. Which of the following is the most likely diagnosis?
What is the mechanism of action of dabigatran?
Which of the following EEG artifacts is prominently seen on this EEG?