Primary central nervous system vasculitis in childhood: more frequent and variable than thought

Aims: Primary central nervous system (CNS) vasculitis is increasingly described in childhood. Etiology and clinical manifestation is heterogeneous and optimal treatment not yet established.

Methods: Retrospective case series.

Results: Within 1 year three children with primary large vessel vasculitis of the CNS were treated (observed within a population of approximately 700,000 inhabitants/120,000 children).

Patient 1 was a 11-months old girl presenting with subacute supranuclear hemiparesis. Multimodal diagnostic work-up showed vasculitic changes and ischemic lesions in the territory of the contralateral middle and posterior cerebral artery without clues to the etiology of the vasculitis. After a 3 months course of steroids the vessels were normal again and hemiparesis had significantly improved.

Patient 2 was a 6-years old girl with headache and hemichorea. Magnetic resonance angiography revealed stenosis of the contralateral middle cerebral artery. Postvaricella angiopathy was assumed because of positive VZV-PCR in the cerebrospinal fluid. After treatment with aciclovir and steroids for 6 weeks the stenosis and perfusion had improved. Around 2 months later a relapse and another stenosis in the contralateral posterior cerebral artery occurred.

Patient 3 was a 7-years old boy with extensive vasculitis exclusively in the posterior circulation with occlusion of the basilar artery and multiple smaller infarctions of different age in this territory. Steroid treatment led to improvement of the vasculitis changes without further infarction. There was no specific underlying cause identified.

All three patients received ASS as well.

Conclusion: Primary CNS vasculitis is potentially more common in childhood than previously observed. A specific etiology is often missing. In postvaricella angiopathy multiphasic course seems possible. Confinement of childhood CNS vasculitis to the posterior circulation is possible.