Chenevier F, Renoux C, Marignier R, Durand-Dubief F, Hermier M, Streichenberger N, Vukusic S, Confavreux C
Service de Neurologie A and the European Database for Multiple Sclerosis Coordinating Centre, Hôpital Neurologique Pierre Wertheimer, Lyon-Bron Cedex, France.
J Neurol Neurosurg Psychiatry. 2009 Oct;80(10):1159-61. doi: 10.1136/jnnp.2008.154567.
Primary angiitis of the central nervous system (PACNS) is a rare inflammatory disease restricted to the CNS of unknown cause. Clinical presentation and evolution are highly variable with potentially fluctuating signs and symptoms. Brain imaging often shows supratentorial ischaemic lesions. Definite diagnosis is established by brain biopsy. Treatment usually combines glucocorticosteroids and cyclophosphamide. A case of PACNS is reported here, which was proved by a brain biopsy and characterised by unusually prominent involvement of the posterior cerebral fossa. Successful treatment with mycophenolate mofetil in combination with steroids is described.
中枢神经系统原发性血管炎（PACNS）是一种罕见的炎症性疾病，病因不明，仅累及中枢神经系统。临床表现和病程高度多变，体征和症状可能波动。脑部影像学检查常显示幕上缺血性病变。通过脑活检确诊。治疗通常联合使用糖皮质激素和环磷酰胺。本文报告一例经脑活检证实的PACNS病例，其特征为后颅窝受累异常突出。描述了霉酚酸酯联合类固醇治疗成功的案例。
