Shimizu N, Sugita K, Ishii M, Kawazoe Y, Miyamoto H, Niimi H
Department of Pediatrics, School of Medicine, University of Chiba.
No To Hattatsu. 1994 Jan;26(1):38-43.
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.
中枢神经系统孤立性血管炎（IACNS）在儿童期是一种极为罕见的疾病。本报告介绍了一例8岁女性IACNS病例。她起病时表现为右侧面部和上肢无力，随后出现各种局灶性神经症状。头颅T2加权磁共振成像显示多个皮质下高信号病灶，这些病灶随着她的临床表现出现和消失。在疾病活动期，她的血浆凝血酶 - 抗凝血酶III复合物（TAT）水平升高，似乎是检测中枢神经系统血管炎的一个有用指标。通过泼尼松和环磷酰胺联合治疗实现了完全缓解。
