Quiz 07/21/2022 (2)

What is the pathophysiology that leads to the pathology shown with a black arrow in the image below?

A mutation in which of the following genes would be most likely in a patient with numerous cavernous malformations seen on head imaging?

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?

Cool limbs during a nerve conduction study will have which of the following effects on the data recorded?

A 17-year-old woman with no significant past medical history except for an upper respiratory tract infection 2 weeks ago presents with a one-day history of fever, headache, and altered mental status. On examination, the patient also had right-sided ataxia. An MRI brain was ordered and T2 sequence images are shown below. The lesions showed minimal lesional enhancement with contrast administration (image not shown). What is the most likely diagnosis?

Which of the following is the primary source of norepinephrine in the brain?

Which of the following is the mechanism of action of memantine?

Which of the following is not a known side effect of chronic phenytoin use?

You have recently diagnosed an asymptomatic 45-year-old man with Huntington’s disease based on genetic testing which was requested based on a family history of the disease. The patient was informed of these findings in the clinic with his wife by his side. On the following day, you receive a phone call from the patient’s 23-year-old son asking what the results of the testing were. What is the most appropriate response to this question?

A 33-year-old female presents to the clinic for evaluation of a left lower extremity foot drop. On physical examination, there is weakness in dorsiflexion. Ankle eversion, inversion, and plantar flexion strength are normal. Hip abduction strength is normal as well. Which of the following is the most likely etiology for the patient’s symptoms?

Which of the following brain metastases has the highest risk of intracranial hemorrhage?

Patients with Alzheimer’s disease usually have a loss of cholinergic neurons from which of the following regions?

The vessel imaging shown below is consistent with which of the following?

A 35-year-old woman with a past medical history of ocular and oral dryness presents with a 5-month history of progressive difficulties with ambulation. On exam, there is diminished vibratory sensation along the plantar/dorsal surfaces of her feet, 1+ ankle reflexes bilaterally, and a positive Romberg sign. Strength is normal. A tissue biopsy confirms the diagnosis. Which of the following is the most likely diagnosis?

A 56-year-old female with a past medical history of IgA deficiency presents to the emergency room with a 4-day history of progressive, ascending lower extremity weakness. Neurological examination reveals flaccid paralysis of the bilateral lower extremities and areflexia. Imaging of the lumbar spine shows contrast enhancement of the cauda equina and CSF studies show elevated proteins and a normal WBC count. What is the most appropriate next step in therapy?

You are caring for a 38-year-old man with schizophrenia since his early twenties. He has had many years of uncontrollable irregular facial movements including tongue writhing, lip smacking, and frequent blinking. His psychiatrist stopped the medication suspected to cause this problem a year ago, yet he is still having the symptoms and they are especially bothersome. Which of the following medications is FDA approved for the treatment of this problem?

A 53-year-old male presents with a 3-month history of hypersexuality, visual agnosia, excessive chewing, and lip-smacking. Past medical history is notable for HSV encephalitis 6 months ago, which was treated with IV acyclovir. Based on the clinical history and examination, which of the following would most likely be appreciated on a brain MRI?

Nursing staff in the neonatal intensive care unit noticed that a 32-week gestational age newborn with a complicated delivery and low initial Apgar scores and intracranial hemorrhage developed abnormal movements on the 2nd day of life. The movements were described as rhythmic, high-frequency movements of the left limb. There were no associated autonomic changes appreciated with events. The child was placed on continuous EEG recording and an event was captured which had no EEG correlate. What is the most likely diagnosis?

A 67-year-old man presents urgently to the emergency room with an acute onset of left-sided weakness and numbness. A CT head was performed and shown below. What is the most likely etiology of the patient’s symptoms?

A 16-year-old male is seen in the clinic for burning and tingling sensations in his upper hands and feet. Additionally, he is a soccer player and states that he does not sweat during physical activity. On physical exam, he has diffuse red-blue papules in his gluteal region, around his navel and nipples, and on his buccal mucosa. There is no family history of skin lesions. This patient likely has a defect in which protein?

Identify the pathological abnormality shown in the image below.

A 68-year-old male presents with complaints of insomnia for 2 years. On further questioning, he also endorses a 2 years period of feelings of guilt about his sleeping, considerably low appetite (current BMI = 17), and low energy. He used to enjoy checkers but has lost interest in most activities these days. He denies suicidal ideation. He has previously declined medications to help with this, but he says he is ready to try something. Which of the following medications is most appropriate?

Patients with neurofibromatosis type 1 (NF1) classically have which of the following associated with their disease?

A 35-year-old male with balance issues is referred for an EMG/NCS. Distal sensory loss of the feet, high-arched feet, and diffuse hyporeflexia are appreciated on the exam. His nerve conduction studies are shown below. Based on the available information what is the most likely diagnosis?

Vasogenic edema would be best appreciated on which of the following MRI sequences?

A 40-year-old male with a history of headaches, seizures, and recurrent strokes has a brain biopsy performed to aid in diagnosis. Electron microscopy of his brain tissue shows granular osmiophilic material in the basal lamina of small cutaneous arterioles. What is the cause of this patient’s disease?

A 67-year-old male wakes up with a painful, right-sided, itchy rash coming across his abdomen like a band involving the umbilicus. His primary care provider is concerned that the patient has shingles. For this patient, re-activation of the varicella-zoster virus occurred at the dorsal root ganglion at which thoracic level?

Anti-Ma2 autoantibodies are most commonly associated with which of the following tumors?

A 65-year-old female presents to the Emergency Room with a 2-month history of altered mental status and jerking. On examination, you appreciate an exaggerated startle response. Her MRI was shown below. Which of the following statements is true?

Which of the following arteries is the first branch of the internal carotid after it enters the cranial cavity?

A 16-year-old male is brought to the emergency room by his parents for generalized weakness. The patient states that he woke up in the morning that day with flaccid paralysis of his arms and legs which was so significant that he couldn’t get out of bed. In typical teenage boy fashion, he wasn’t very worried about this at the time and went back to bed. It wasn’t until 4 hours later when he woke up again with the same degree of weakness that he became concerned and screamed for his parents to help. The patient denies any recent drug or alcohol use. The only interesting thing from the previous night was that he partook in and won a pizza-eating contest with his friends. The patient’s father admits to having had similar episodes to this when he was younger. If the patient’s symptoms are due to a genetic disorder, a mutation in which of the following genes is most likely responsible?

A 7-year-old girl presents with 2 weeks of a right-sided unilateral throbbing headache. She has otherwise been healthy with no other symptoms and has no past medical history except for a light pink birthmark around her right eye. What is the most appropriate next step?

A 72-year-old man with a history of right-sided carotid stenosis was transferred from an outside hospital to your institution to have a carotid stent placed after experiencing a mild right-hemispheric ischemic stroke. The patient was sent to the angiography lab and had a carotid stent placed, however, it was done so on the unaffected (left) side. What is this an example of?

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

A 65-year-old female comes to the clinic complaining of weakness in his extremities. Symptoms are more prominent in the legs than in the arms. Oddly, when he tries to exert himself, he notes an improvement in his strength. Neurodiagnostic testing reveals low amplitude compound muscle action potentials (CMAPs) with an increase in amplitude on high frequency (50 Hz) rapid repetitive stimulation. Which of the following malignancies is most closely associated with this patient’s neurologic syndrome?

Which of the following is the mechanism of action of ocrelizumab?

A 64-year-old man with idiopathic Parkinson’s disease being treated with carbidopa-levodopa is experiencing significant wearing-off despite increasing frequency and dose of levodopa-carbidopa, which is now producing significant peak dose dyskinesia. Which of the following would be a reasonable next agent to prescribe to reduce this wearing-off effect?

A 7-year-old boy with a history of right facial port-wine stain and mild developmental delay presents to the emergency room for seizures. An MRI brain is performed and the T1 with contrast sequence is shown below. What is the most likely diagnosis?

This muscle biopsy is most consistent with which myopathic disease?

The medulla develops from which of the five primary vesicles of the brain?