2020 Volume 36 Issue 2 Pages 153-158
Reversible cerebral vasoconstriction syndrome (RCVS) presents neurologically with reversible segmental vasospasms and is characterized primarily by complaints of sudden, severe (‘thunderclap’) headaches. Although disease prognosis is often good, some reports have described poorer outcomes in patients with comorbid stroke or other cerebrovascular injury. Here, we report a pediatric case of RCVS complicated by stroke resulting in lasting neurological sequelae.
A 13-year-old boy suddenly developed severe headaches, vomiting, disordered articulation, and left-sided arm and leg paralysis. Cranial magnetic resonance imaging revealed a cerebral infarction spanning the right globus pallidus to the corona radiata. Cranial magnetic resonance angiography indicated multifocal vascular wall irregularities and stenosis. Cerebral angiography indicated multi-segmental vasoconstriction. RCVS was diagnosed from these findings and clinical course; both improved upon oral verapamil administration, but the patient still experienced left arm rigidity and sensory paralysis.
We examined previously reported cases of RCVS complicated with cerebral infarction; pediatric cases were more likely to be male and to develop multifocal infarctions.
RCVS should be considered in the differential diagnosis of pediatric patients complaining of sudden, severe headaches. Furthermore, clinicians should continue to perform imaging exams regularly while observing patient symptoms and neurological findings.