Hart Y M, Cortez M, Andermann F, Hwang P, Fish D R, Dulac O, Silver K, Fejerman N, Cross H, Sherwin A
Montreal Neurological Institute and Hospital, PQ, Canada.
Neurology. 1994 Jun;44(6):1030-6. doi: 10.1212/wnl.44.6.1030.
We treated 19 patients with Rasmussen's syndrome (chronic encephalitis and epilepsy)--a rare progressive disorder of unknown etiology causing focal epilepsy, hemiparesis, and intellectual deterioration--with intravenous immunoglobulins, high-dose steroids, or both, to control seizures and improve the end point of the disease. Ten of 17 patients receiving steroids, and eight of nine patients receiving immunoglobulins, had some reduction of seizure frequency in the short term. Improvement in hemiparesis was slight. The effect of these drugs in ameliorating the end point of the disease in the long term remains unknown, and further multicenter studies with standardized protocols are warranted.
我们用静脉注射免疫球蛋白、大剂量类固醇或两者兼用治疗了19例患有拉斯穆森综合征（慢性脑炎和癫痫）的患者，该综合征是一种病因不明的罕见进行性疾病，可导致局灶性癫痫、偏瘫和智力衰退，目的是控制癫痫发作并改善疾病的终点。接受类固醇治疗的17例患者中有10例，接受免疫球蛋白治疗的9例患者中有8例在短期内癫痫发作频率有所降低。偏瘫的改善很轻微。这些药物对长期改善疾病终点的效果尚不清楚，因此有必要开展进一步的标准化方案多中心研究。
