Quiz 07/24/2022

A 28-year-old male with a known history of neuromyelitis optica spectrum disorder (NMOSD) and a recent diagnosis of acute optic neuritis returns to the emergency room for worsening monocular vision loss despite completing 5 days of high dose (1 gm/day) methylprednisolone therapy. Based on the clinical scenario and level of evidence for efficacy, what is the most appropriate next step in therapy?

A 45-year-old female with a recent diagnosis of multiple sclerosis, based on MRI and CSF analysis, is referred to your clinic to discuss possible disease-modifying therapies. You review the clinical history with her and she states that for the past 18 months she has experienced progressive right upper and lower extremity weakness. She denies any periods of transient improvement or dramatic worsening of symptoms. Based on this clinical history, what is the most appropriate disease-modifying therapy for this patient?

Which of the following is the mechanism of action for natalizumab?

A 36-year-old woman was recently started on natalizumab for management of her multiple sclerosis. Over the next several months, she develops progressive weakness, incoordination, and speech disturbances before passing away six months after initiating natalizumab. Testing for which of the following pathogens should have been performed prior to natalizumab initiation to prevent this outcome?

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?

A previously healthy 25-year-old female presents to the emergency room with lower extremity weakness, numbness, and urinary retention. Lower extremity hyperreflexia and a sensory level at T10 were appreciated on examination. A contrasted cervical/thoracic and lumbar MRI was performed and shown below. Lumbar puncture was performed after imaging was completed and showed an elevated protein concentration, normal glucose, 3 WBCs, and oligoclonal bands. Rheumatologic studies are negative. Based on the most likely diagnosis, what is the most appropriate next step in medical management?

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

The lesion shown below is most consistent with which of the following pathologies?

A 50-year-old male presents to the emergency room with a three-day history of double vision and difficulty walking. Neurological examination reveals decreased eye movements. Strength in the limbs is relatively preserved but all reflexes are absent. There is also marked limb clumsiness. Which of the following is the most likely diagnosis?

Alemtuzumab is a monoclonal antibody therapy that targets which of the following proteins?

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

Which of the following comorbidities would prohibit the use of dalfampridine?

A 26-year-old female presents with unilateral vision loss. She says that she noticed symptoms upon awakening yesterday morning and it has gotten progressively worse since then. On neurological examination, she has 20/70 visual acuity of the left eye and 20/20 of the right. There is a left afferent pupillary defect (APD). Extraocular movements are intact but cause pain. What is the most likely etiology of her symptoms?

Which of the following medications used in the management of multiple sclerosis is the most teratogenic?

A 42-year-old woman with multiple sclerosis presents to the ER after having worsening confusion over a few days with worsening weakness and vision loss. She was on natalizumab for years and her most recent JCV antibody titer was negative. She has missed several months of infusions due to her helping a sick relative. Her MRI shows multiple new T2 hyperintensities with enhancement in the periventricular and subcortical white matter. What is the most likely diagnosis?

This brain biopsy is consistent with which of the following pathologies?

A 57-year-old male with HIV who is non-compliant with HAART therapy presented to the emergency room with a 4-week history of altered mental status and falls. On neurological examination, the patient was altered, ataxic, and had right upper extremity weakness. An MRI was performed and shown below. Based on the clinical presentation and imaging provided, what is the most appropriate first step in medical management?

A patient with relapsing-remitting multiple sclerosis presents to the emergency department with a new headache, confusion, and vision loss. A T2 sequence from their MRI is shown below. Lumbar puncture is positive for JCV in the CSF. What is the mechanism of action of the disease-modifying therapy most likely to be attributed to this clinical scenario?

Other than optic neuritis, which of the following is another typical MRI finding in patients with neuromyelitis optica (NMO) that has diagnostic value?

A 26-year-old female presents with unilateral vision loss. She says that she noticed the symptoms upon awakening yesterday morning and has gotten progressively worse since then. On neurological examination, she has 20/70 visual acuity of the left eye and 20/20 of the right. There is a left afferent pupillary defect (APD). Extraocular movements are intact but cause pain. What is the most appropriate treatment?

A 33-year-old patient presents in the clinic for evaluation of bilateral leg weakness, and urinary incontinence. He first started noticing right leg weakness about 2 years ago and then a few months later felt similar symptoms developing in his left foot. These symptoms gradually worsened over time to the point that he had to start using a cane because of frequent falls. Imaging of the whole neuroaxis was performed. MRI of the brain showed several periventricular and juxtacortical T2 hyperintensities with no enhancement. A lumbar puncture was performed and it had unique CSF oligoclonal bands. What is the most likely diagnosis?

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

The lesion shown below (blue arrow) is most consistent with which of the following pathologies?

A 13-year-old male with a recent history of acute disseminated encephalomyelitis (ADEM) presents to the clinic for post-hospital follow-up. He has made a near-complete recovery from his illness but his parents want to know what is his risk of recurrence. What is the most accurate statement you can share with the parents?

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?

A 32-year-old woman with a known history of relapsing-remitting multiple sclerosis currently on ocrelizumab presents to the emergency room with the concern of having a demyelinating attack. An MRI brain with contrast shows a single, ovoid, juxtacortical white matter lesion with contrast enhancement that was not present on her last MRI scan. Which of the following is the most appropriate next step in medical management?

A 36-year-old female with bilateral lower extremity weakness, hyperreflexia, and difficulty emptying her bladder gets an urgent MRI with contrast of her thoracic spine (T2 sagittal (left) and axial (right)). Which of the following is the most likely diagnosis?

Which of the following is a side effect of glatiramer acetate?

A patient recently diagnosed with multiple sclerosis was started on an oral disease-modifying therapy (DMT) that works on nuclear factor erythroid-derived 2-related factor (Nrf2)-dependent and independent pathways. One week later they call you complaining of flushing of the skin. What is the most appropriate next step in management?

Which of the following statements regarding Alexander’s disease is incorrect?

A 23-year-old female with relapsing-remitting multiple sclerosis (RRMS) that is well-controlled on teriflunomide presents for routine follow-up. Today she expresses interest in having a child. Which of the following statements regarding multiple sclerosis and pregnancy is true?

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?

A 24-year-old woman presents to the ER with a one-day history of diplopia. On examination, the patient has right internuclear ophthalmoplegia. On further discussion, she describes an episode of left lower extremity numbness about 2 years ago that resolved after a few weeks on its own. An MRI of her brain and spinal cord was ordered. Findings included a T2 hyperintense contrast-enhancing lesion in the pons, as well as non-contrast enhancing T2 hyperintense lesions in periventricular and juxtacortical regions as well as in the thoracic spinal cord. What is the next step to confirm her diagnosis?

Aquaporin-4 (AQP4) channels are found on which cell type?

Which of the following is a common side effect of dimethyl fumarate?

Which of the following is not a risk factor for the development of natalizumab-associated PML?

Which of the following medications requires blood pressure and pulse monitoring for at least 6 hours after the first dose to assess for first-dose-related bradycardia?

Which of the following medications are known to develop neutralizing antibodies (NAbs) against it with chronic use?

A 47-year-old man with a history of bilateral cataract surgery at the age of 20 presents to the clinic for evaluation of progressive unsteadiness and cognitive decline. His father and older brother reportedly had similar symptoms in their early adulthood as well. On exam, there is notable dysmetria and gait ataxia. There were also abnormal soft-tissue masses appreciated near the Achilles tendons bilaterally. Which of the following is most likely to be abnormal in this patient blood?

A 65-year-old right-handed man presents with an acute onset of aphasia. CT scan of the head was normal but the MRI revealed multifocal small acute and subacute ischemic strokes in multiple vascular territories. A cerebral angiogram was performed as part of the diagnostic workup. Based on the clinical history and imaging provided what is the most likely diagnosis?