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[结节性硬化症伴多发结节所致难治性癫痫的外科治疗:病例报告]

[Surgical treatment for intractable epilepsy caused by tuberous sclerosis complex with multiple tubers: case report].

作者信息

Imoto Hirochika, Fujii Masami, Nomura Sadahiro, Tanaka Nobuhiro, Kajiwara Koji, Fujisawa Hirosuke, Kato Shoichi, Akimura Tatsuo, Suzuki Michiyasu, Arai Nobutaka

机构信息

Department of Neurosurgery, Yamaguchi University School of Medicine, Japan.

出版信息

No Shinkei Geka. 2008 Feb;36(2):155-8.

Abstract

We report a case of intractable epilepsy caused by tuberous sclerosis with multiple tubers that was treated successfully by single resection of a tuber A 2-year-old boy developed tonic spasm-like seizures at the age of 4 months and the frequency of the seizures increased despite therapy with various anticonvulsants. He had no neurological deficits, and his intelligence was normal. MRI showed lesions in the left frontal and the right parietal lobe, and EEG (electroencephalogram) demonstrated sharp waves and spike-and-wave complex bursts over the right parietal region. Subdural electrodes were implanted over these lesions, and ictal low-voltage fast waves were demonstrated on the right parietal lesion. Therefore the right cortical lesion and the epileptogenic cortex were resected. Multiple subpial transaction was also added in the irritative cortex. Histological examination proved that the lesion was a tuberous sclerosis complex (TSC) tuber. Since surgery, the patient has remained seizure-free for 2 years. In patients with multiple TSC tubers in the cortex, we suggest that seizures can be suppressed by single resection of a tuber if the tuber can be identified as epileptogenic before surgery.

摘要

我们报告一例由结节性硬化症伴多个结节引起的难治性癫痫,通过单次切除一个结节成功治愈。一名2岁男孩在4个月大时出现强直性痉挛样发作,尽管使用了各种抗惊厥药物治疗,发作频率仍增加。他没有神经功能缺损,智力正常。磁共振成像(MRI)显示左侧额叶和右侧顶叶有病变,脑电图(EEG)显示右侧顶叶区域有尖波和棘慢复合波暴发。在这些病变上方植入了硬膜下电极,右侧顶叶病变显示出发作期低电压快波。因此,切除了右侧皮质病变和致痫皮质。在刺激性皮质还进行了多次软膜下横切术。组织学检查证实该病变为结节性硬化症复合体(TSC)结节。自手术以来,患者已无癫痫发作2年。对于皮质有多个TSC结节的患者,我们建议,如果在手术前能将结节确定为致痫灶,则单次切除一个结节即可抑制癫痫发作。

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