[Primary cerebral vasculitis in children].

Introduction: Primary cerebral vasculitis in children is a newly recognized, rare inflammatory condition affecting the vessels of the brain. It is characterized by newly acquired neurological deficit(s) with angiographic or histological evidence of central nervous system (CNS) vasculitis, in the absence of other known diseases with these findings.

Material And Methods: We performed a retrospective review of children below 15 years submitted with CNS vasculitis to the department between 1999 and 2008.

Results: Six (two boys, four girls) of ten children with clinical and vascular imaging findings detected by magnetic resonance were diagnosed with primary CNS vasculitis. Age at onset was three to 12 years. Acutely acquired hemiparesis was seen in five children, two had facial palsy. Among other symptoms were headache, ataxia, dysarthria, loss of consciousness and seizures. Only modest changes in blood and spinal fluid values were seen. On magnetic resonance angiography, varying segmental stenoses were found. All had supratentorial lesions, and in two patients infratentorial lesions were also detected by MRI. Monthly treatment with high-dose intravenous corticosteroids for six months was successful in most of the patients. In two patients with progressive CNS vasculitis, the treatment was supplemented by intravenous cyclophosphamide and azathioprin, respectively.

Conclusion: Primary CNS vasculitis is an acutely acquired inflammatory disease with severe neurological deficits and sequelae which may have a fatal outcome. Despite this the prognosis is acceptable since event-free survival can be achieved in almost 70% if early immunosuppressive therapy is initiated.