Rasmussen syndrome: An Argentinean experience in 32 patients
Rasmussen syndrome: An Argentinean experience in 32 patients
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DOI：
10.1016/j.seizure.2013.02.003
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发表时间：
2013-06-01
影响因子：
3
通讯作者：
Bartuluchi, Marcelo
中科院分区：
文献类型：
--
作者：
Caraballo, Roberto H.;Fortini, Sebastian;Bartuluchi, Marcelo
目的：材料与方法：回顾性分析1990 - 2012年32例Rasmussen综合征患者的临床特征、治疗方法及演变过程。癫痫发作的平均和中位年龄分别为6.5岁和7岁。28例患者的部分持续性癫痫开始于平均9.5岁。26例患者在癫痫发作后的头两年内发生固定性轻偏瘫。发作期脑电图显示多灶性起源，但局限于所有患者的受影响的半球。轻度局灶性萎缩累及颞-岛区，伴同侧角和侧裂扩大。在T2和Flair图像中分别观察到25例和17例患者的异常皮质和/或皮质下高信号。在5名患者中记录了基底节T2高信号和萎缩。25例患者使用了与免疫球蛋白相关的皮质类固醇。手术治疗25例。经过平均随访13年（范围，2-20）良好的手术效果-恩格尔类I -观察23 25例operated.Conclusion：皮质类固醇和静脉注射免疫球蛋白治疗应考虑在疾病的早期阶段。RS患者对手术切除受累半球反应良好。(C)2013年英国癫痫协会。由爱思唯尔有限公司出版。保留所有权利。
Purpose: The aim of this study is to analyze the electroclinical features, treatment, and evolution of patients with Rasmussen syndrome (RS).Materials and methods: We conducted a retrospective, descriptive study in 32 consecutive patients with RS followed between 1990 and 2012.Results: Twenty boys and 12 girls were included in the study. The mean and median ages at onset of the seizures were 6.5 and 7 years, respectively. Twenty-eight cases had epilepsia partialis continua that had started at a mean age of 9.5 years. Fixed hemiparesis occurred within the first two years after seizure onset in 26 patients. The ictal EEG showed a multifocal origin, but confined to the affected hemisphere in all patients. Mild focal atrophy involved the temporo-insular region associated with enlargement of the ipsilateral horn and Sylvian fissure. An abnormal cortical and/or subcortical hyperintense signal was observed in T2 and Flair images in 25 and 17 patients, respectively. T2 hyperintensity and atrophy in the basal ganglia was documented in five patients. Corticosteroids associated with immunoglobulins were used in 25 patients. Surgical treatment was performed in 25 patients. After a mean follow-up of 13 years (range, 2-20) good surgical outcome - Engel class I - was observed in 23 of 25 patients operated.Conclusion: Corticosteroid and intravenous immunoglobulin treatment should be considered in the early stages of the disease. Patients with RS had a good response to surgical excision of the affected hemisphere. (C) 2013 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.