[Angiitis of the autonomic central nervous system].

Autonomic central nervous system (CNS) angiitis was classified with primary angiitis of the CNS (PACNS) or CNS angiitis secondary to systemic vasculitis. Mean onset age of PACNS was about 50 years. Typical symptoms were a chronic progressive headache and encephalopathy. Cerebrospinal fluid analysis was compatible with aseptic meningitis. Findings from imaging were varied and included arterial stenosis. Exclusion diagnosis of PACNS mimics and a brain biopsy were required for a definite diagnosis. Some collagen diseases could cause secondary CNS vasculitis. Patients with secondary CNS vasculitis were required more extensive immunosuppression therapy.