Hirohata S
Department of Internal Medicine, Teikyo University School of Medicine.
Nihon Rinsho. 1999 Feb;57(2):409-12.
Among various rheumatic diseases, systemic lupus erythematosus and Behcet's disease are frequently complicated with serious neurological involvement, called CNS lupus and neuro-Behcet's syndrome (NB), respectively. CNS lupus includes lupus psychosis, such as organic brain syndrome and non-organic psychosis, as well as non-psychotic CNS lupus, such as epilepsy and focal lesions. Anti-ribosomal P antibody is closely associated with lupus psychosis, whereas anti-phospholipid antibodies appear to be involved in some manifestations of non-psychotic CNS lupus. NB includes acute type and chronic progressive type. Acute NB is characterized by acute meningoencephalitis with focal lesions, which respond to steroid therapy. By contrast, chronic progressive NB is characterized by intractable slowly progressive dementia and/or psychosis with persistent elevation of cerebrospinal fluid IL-6 activity, which is resistant to conventional steroid therapy. However, recent studies suggest the efficacy of low dose methotrexate in chronic progressive NB.
在各种风湿性疾病中，系统性红斑狼疮和白塞病常伴有严重的神经系统受累，分别称为中枢神经系统狼疮和神经白塞综合征（NB）。中枢神经系统狼疮包括狼疮精神病，如器质性脑综合征和非器质性精神病，以及非精神病性中枢神经系统狼疮，如癫痫和局灶性病变。抗核糖体P抗体与狼疮精神病密切相关，而抗磷脂抗体似乎与非精神病性中枢神经系统狼疮的某些表现有关。NB包括急性型和慢性进行型。急性NB的特征是伴有局灶性病变的急性脑膜脑炎，对类固醇治疗有反应。相比之下，慢性进行性NB的特征是难治性缓慢进展性痴呆和/或精神病，脑脊液IL-6活性持续升高，对传统类固醇治疗耐药。然而，最近的研究表明低剂量甲氨蝶呤对慢性进行性NB有效。
