Childhood primary angiitis of the central nervous system: two biopsy-proven cases.

Primary angiitis of the central nervous system is a rare idiopathic vasculitis predominantly affecting the central nervous system. The literature includes 10 histologically confirmed cases in childhood. We identify two additional cases, one presenting with both uveitis and cerebrospinal fluid neutrophilic pleocytosis, which has not been reported previously, and demonstrate the importance of biopsy in suspected cases.