Atypical angiitis of the central nervous system: A case report

Abstract

We report a 51-year-old woman with atypical angiitis of the central nervous system (CNS). She complained of a sudden onset of severe throbbing headache followed by progressive weakness of left upper limb. On admission, computed tomography (CT) scanning showed a subcortical hematoma in the right frontoparietal lobes. Cerebral angiography showed multiple segmental narrowing in the small- or medium-sized vessels suggesting an angiitis. Serologic findings were normal except for an elevation of anticentromere antibodies. She had once experienced transient Raynaud's phenomenon. The history of Raynaud's phenomenon and an elevation of anticentromere antibodies strongly suggested a relation to some rheumatologic disorders. However, this case does not exactly meet any type of rheumatologic disorder in the literature. Follow-up angiography after 2 months of steroid therapy revealed marked improvement of the angiitis. We discuss the etiology of angiitis of the CNS and the value of the repeated angiography.