[Isolated angitis of the central nervous system--case report and review]

Isolated angitis of the central nervous system (IACNS) is rare condition with inflammation limited to vessels supplying the brain. This IACNS has been a poorly characterized and infrequently reported illness since it was first described as a separate entity in 1959. However, a patient with IACNS has not been reported in Japan. A patient, 39-year old-male, with IACNS limited to small and middle vessels is described. Recurrent, transient consciousness disturbances, focal myoclonus, papilloedema and temporal lobe epilepsy were observed during disease course. CSF finding of this patient showed lymphocytosis with marked increased protein. Carotid and vertebral angiogram showed irregular luminal outline in branches of all arteries. The most specific finding is that of alternating areas of focal stenosis and ectasia giving a "sausage" pattern. These characteristic findings showed in the branch of external carotid arteries. There was no evidence of systemic vasculitis by systemic angiography. Biopsy of temporal artery showed lymphocyte infiltration, fibrinoid necrosis without giant cell and granuloma. Formation of A-V malformation in the branch of external carotid artery was also observed. This histopathological finding was compatible with necrotizing angitis, not granulomatous angitis. Isolated angitis of the central nervous system was diagnosed. In spite of his administration of corticosteroid, cerebrospinal fluid abnormalities has not responded markedly. Abnormal findings of carotid and vertebral angiogram also has not changed. After 7 years from his onset, his neurologic signs and symptoms were well controlled with administration of anti-epileptic drugs.