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胼胝体切开术治疗与结节性硬化症相关的耐药性痉挛。

Corpus callosotomy for drug-resistant spasms associated with tuberous sclerosis complex.

机构信息

Department of Child Neurology, Comprehensive Epilepsy Center, Seirei Hamamatsu General Hospital, Hamamatsu 430-8558, Japan.

Epilepsy and Surgery, Comprehensive Epilepsy Center, Seirei Hamamatsu General Hospital, Hamamatsu 430-8558, Japan.

出版信息

Epilepsy Behav. 2019 Sep;98(Pt A):228-232. doi: 10.1016/j.yebeh.2019.06.007. Epub 2019 Aug 5.

DOI:10.1016/j.yebeh.2019.06.007
PMID:31394351
Abstract

BACKGROUND

Corpus callosotomy (CC) has recently been adopted for the treatment of drug-resistant epileptic spasms and tonic spasms. In the present study, we investigated CC outcomes among patients with drug-resistant epileptic spasms or tonic spasms associated with tuberous sclerosis complex (TSC).

METHODS

We retrospectively collected data from seven patients (3 women, 4 men) with diagnosed TSC and who were treated using CC at Seirei Hamamatsu General Hospital in Japan. All patients had experienced drug-resistant epileptic spasms (<3 s of muscular contraction) or tonic spasms (>3 s) prior to CC, which were confirmed via video-electroencephalogram monitoring.

RESULTS

All patients exhibited multiple bilateral cortical tubers on brain magnetic resonance imaging. The main seizure types were epileptic spasms in four, tonic spasms in one, and both seizure types in two patients. Patients underwent total CC between the ages of 25 months and 21.5 years. Additional resection or disconnection was performed in two patients. The follow-up period after CC ranged between 9 months and 3.5 years. Three patients achieved remission from spasms following CC alone. Two other patients became free from spasms several months after CC but required an additional focus disconnection or medical treatment. The remaining two patients continued to show spasms or asymmetrical tonic seizures.

CONCLUSION

Total CC resulted in freedom from drug-resistant epileptic or tonic spasms in several patients with TSC. Stepwise progression from CC to additional resection or disconnection surgery may aid in the treatment of spasms secondary to TSC.

摘要

背景

胼胝体切开术(CC)最近被用于治疗耐药性癫痫痉挛和强直痉挛。在本研究中,我们调查了与结节性硬化症(TSC)相关的耐药性癫痫痉挛或强直痉挛患者的 CC 结果。

方法

我们回顾性收集了日本滨松圣心综合医院接受 CC 治疗的 7 名(3 名女性,4 名男性)诊断为 TSC 的患者的数据。所有患者在 CC 之前均经历过耐药性癫痫痉挛(肌肉收缩<3 秒)或强直痉挛(>3 秒),这是通过视频-脑电图监测确认的。

结果

所有患者的脑磁共振成像均显示多个双侧皮质结节。主要的发作类型是 4 例癫痫痉挛,1 例强直痉挛,2 例两种发作类型。患者在 25 个月至 21.5 岁之间接受了完全 CC。两名患者进行了额外的切除或切断。CC 后随访时间为 9 个月至 3.5 年。三名患者在单独接受 CC 后痉挛缓解。另外两名患者在 CC 后几个月内停止了痉挛,但需要进行额外的病灶切断或药物治疗。其余两名患者继续出现痉挛或不对称性强直发作。

结论

完全 CC 使数名 TSC 患者的耐药性癫痫或强直痉挛得到缓解。从 CC 到额外切除或切断手术的逐步进展可能有助于治疗 TSC 引起的痉挛。

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