Reversible cerebral vasoconstriction syndrome: rare or underrecognized in children?
Reversible cerebral vasoconstriction syndrome (RCVS) is a clinicoradiological diagnosis comprising 'thunderclap' headaches and reversible segmental vasoconstriction of cerebral arteries, occasionally complicated by ischaemic or haemorrhagic stroke. We report a case of RCVS in a 13-year-old male with severe thunderclap headaches and no focal neurological signs. Brain imaging showed multiple posterior circulation infarcts; cerebral computed tomography, magnetic resonance imaging, and catheter angiography showed multifocal irregularity and narrowing, but in different arterial segments. Laboratory studies did not support a diagnosis of vasculitis. Symptoms resolved over 3 weeks; magnetic resonance angiography 3 months later was normal and remained so after 2 years. We highlight the typical clinical features of RCVS in this case and suggest that the diagnosis should be considered in children with thunderclap headaches or stroke syndromes where headache is a prominent feature, especially if cerebrovascular imaging studies appear to be evolving or discrepant.
Neurosciences Unit, University College London Institute of Child Health, London, UK. beckyprobert@msn.com
Rebecca
R
Probert
Dawn E
DE
Saunders
Vijeya
V
Ganesan
D000293:Adolescent; D002404:Catheterization; D002533:Cerebral Angiography; D002536:Cerebral Arteries / Q000000981:diagnostic imaging / Q000473:pathology* / Q000503:physiopathology*; D002544:Cerebral Infarction / Q000150:complications* / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology; D003937:Diagnosis, Differential; D006261:Headache / Q000188:drug therapy / Q000209:etiology* / Q000628:therapy; D006801:Humans; D018810:Magnetic Resonance Angiography; D008297:Male; D020521:Stroke / Q000150:complications / Q000175:diagnosis; D013577:Syndrome; D014057:Tomography, X-Ray Computed; D014661:Vasoconstriction; D020301:Vasospasm, Intracranial / Q000150:complications* / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology