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[拉斯穆森综合征——难治性癫痫的外科治疗指征:两例报告]

[Rasmussen syndrome--indications for surgical treatment of refractory epilepsy: report of two cases].

作者信息

Daszkiewicz P, Dabrowski D, Bachański M, Grajkowska W

机构信息

Kliniki Neurochirurgii, Instytutu "Pomnik-Centrum Zdrowia Dziecka".

出版信息

Neurol Neurochir Pol. 2000 Nov-Dec;34(6):1251-60.

PMID:11317501
Abstract

AIM

Presentation of two cases of children with Rasmussen's encephalitis, treated by functional hemispherectomy. Two boys aged 4 and 6, with a typical clinical course, typical findings in CT and MRI scans and characteristic pathologic changes in brain specimens. In both cases was performed functional hemispherectomy.

RESULTS

Immediate cessation of seizures in the immediate postoperative period (Engel class I and II). Later, in a 4-months' follow-up period, a worthwhile improvement in psychomotor development and social functioning was noted. Up-to date opinions published in available literature, related to pathogenesis and treatment modalities of Rasmussen's encephalitis are presented.

CONCLUSIONS

  1. Rasmussen's encephalitis is a definite nosologic entity, leading to drug-resistant epilepsy and a progressive psychomotor deterioration; 2) functional hemispherectomy is a viable alternative, which should be considered in the treatment of Rasmussen's encephalitis; 3) Functional; hemispherectomy, in spite of it's aggressiveness, is a relatively safe procedure; 4) in the follow-up time of 4 months, the results are promising.
摘要

目的

介绍两例采用功能性大脑半球切除术治疗的拉斯穆森脑炎患儿。两名男孩,年龄分别为4岁和6岁,具有典型的临床病程、CT和MRI扫描典型表现以及脑标本特征性病理改变。两例均行功能性大脑半球切除术。

结果

术后即刻癫痫发作停止(恩格尔一级和二级)。随后,在4个月的随访期内,观察到精神运动发育和社会功能有显著改善。文中还介绍了现有文献中发表的有关拉斯穆森脑炎发病机制和治疗方式的最新观点。

结论

1)拉斯穆森脑炎是一种明确的疾病实体,可导致药物难治性癫痫和进行性精神运动衰退;2)功能性大脑半球切除术是一种可行的选择,在拉斯穆森脑炎的治疗中应予以考虑;3)功能性大脑半球切除术尽管具有侵袭性,但仍是一种相对安全的手术;4)在4个月的随访期内,结果令人鼓舞。

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Neurol Neurochir Pol. 2000 Nov-Dec;34(6):1251-60.
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