Unknown Case #3: Bilateral Cerebral Infarcts

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A 20-year-old female presents with bilateral cerebral infarcts of differing ages, including cortical and border zone ischemic infarcts and an old hemorrhagic infarct of the left basal ganglia. Angiography demonstrates stenosis of the bilateral terminal ICAs, M1 segments, and proximal A1 segments. Intracranial vessel wall imaging demonstrates negative remodeling (shrinkage) of the involved arteries with areas of absent to low grade predominantly circumferential vessel wall enhancement without eccentric nodularity. There is no vessel wall enhancement elsewhere. There is no laboratory or clinical evidence of vasculitis, herpes zoster infection, illicit drug use, or atherosclerotic risk factors. Which of the following is true of the most likely diagnosis?

A. A biopsy of the superficial temporal artery and rheumatology consults are recommended

B. Follow-up MRA in 3-4 should be done to evaluate for reversible cerebral vasoconstriction syndrome (RCVS)

C. This diagnosis is associated with both ischemic infarcts and intracranial hemorrhage

D. This patient should start anti-platelet, anti-hypertensive, and lipid-lowering medication

E. This diagnosis if more common in western countries such as the United states than those in east Asia