[A female case of isolated angiitis of the central nervous system].

Isolated angiitis of the central nervous system (IACNS) is an extremely rare disorder in childhood. This report presents an 8-year-old female case of IACNS. She showed paresis at the right face and upper limb at the onset, followed by various focal neurological symptoms. Cranial T2-weighted magnetic resonance imaging revealed multiple subcortical high signal lesions, which appeared and disappeared in accordance with her clinical manifestations. The level of her plasma thrombin-antithrombin III complex (TAT) was high during the active phase of the illness and seemed to be a useful marker for the detection of vasculitis in the CNS. Complete remission was achieved by the combination therapy of prednisone and cyclophosphamide.