Approximately thirty five percent of pediatric strokes may be attributed to focal cerebral arteriopathy (FCA). FCA is a unifocal and unilateral stenosis of a large intracranial artery of the anterior circulation. Cases have been associated with varicella infection in the year prior to stroke. Acute ischemic strokes caused by FCA have a 25% recurrence risk in one year and therefore need to be monitored closely. Literature suggests that patients with FCA may benefit from treatment with steroids, in addition to anti-thrombotic therapy, making early diagnosis essential for optimizing outcomes. We present a case of FCA in the setting of recent varicella infection.
A 14-year-old unvaccinated female presented to the emergency department with left facial droop, left hemiparesis and speech disturbance. Symptoms started four days prior, worsening on day of arrival. NIH Stroke Scale on presentation was 2 for aphasia. CT head was normal. MRI brain showed restricted diffusion involving the right internal capsule and deep grey matter. MRA showed right internal carotid artery narrowing. Given delayed presentation, she was out of the window for acute interventions and was transferred for higher level of care. History was notable for varicella 7 months ago and COVID-19 5 months ago. Vessel wall imaging showed narrowing in the right internal carotid and A1, consistent with FCA. Patient was started on pulse dose steroids and aspirin.
We present a case arterial ischemic stroke secondary to FCA in a patient with history of varicella infection. Wild type varicella infection has been associated with FCA up to 12 months post-infection and under-vaccination is associated with over 8-fold increase in risk of childhood stroke. Incidence of strokes related to varicella infection has decreased with the availability of vaccinations, highlighting the importance of childhood vaccines.