Kawai Kensuke, Shimizu Hiroyuki, Yagishita Akira, Maehara Taketoshi, Tamagawa Kimiko
Department of Neurosurgery, Tokyo Metropolitan Neurological Hospital, Tokyo, Japan.
J Neurosurg. 2004 Aug;101(1 Suppl):7-15. doi: 10.3171/ped.2004.101.2.0007.
Epilepsy in patients with bihemispheric malformations of cortical development (MCD) is typically medically intractable. Focal resection has been reported to be ineffective. Corpus callosotomy has been advocated as a treatment option, but the results have been reported only in several case reports. The authors describe a series of 10 patients with bihemispheric MCDs who underwent total corpus callosotomy.
The MCDs in these patients included lissencephaly, band heterotopia, perisylvian polymicrogyria, and tuberous sclerosis. Preoperatively all patients suffered disabling drop attacks or intense head drop seizures that caused frequent physical injuries. The follow-up period ranged from 1.4 to 5.8 years (median 3.2 years). Seizure outcome, parental assessment of daily function, and parental satisfaction with outcome were assessed postoperatively. Drop attacks disappeared completely during the entire follow-up period in eight patients and decreased to less than 10% of baseline in one. Other types of seizures were resolved completely in one patient and decreased in seven. Overall daily function improved and parents were satisfied with the surgery-related results in all patients except one who experienced a recurrence of drop attacks. There were no signs of significant and persistent neurological deficits in any case.
Results of total corpus callosotomy in patients with bihemispheric MCDs were favorable in most cases. The procedure was particularly effective against drop attacks causing physical injuries and impaired quality of life in these patients.
双侧大脑皮质发育畸形(MCD)患者的癫痫通常药物治疗难以控制。据报道,局灶性切除无效。胼胝体切开术已被提倡作为一种治疗选择,但仅在几例病例报告中报道过其结果。作者描述了一组10例接受完全胼胝体切开术的双侧MCD患者。
这些患者的MCD包括无脑回畸形、带状异位、外侧裂周围多小脑回畸形和结节性硬化症。术前,所有患者均患有致残性跌倒发作或剧烈的头部下垂性癫痫,导致频繁身体受伤。随访期为1.4至5.8年(中位数3.2年)。术后评估癫痫发作结果、家长对日常功能的评估以及家长对结果的满意度。8例患者在整个随访期内跌倒发作完全消失,1例患者跌倒发作减少至基线的10%以下。其他类型的癫痫在1例患者中完全缓解,7例患者减少。除1例跌倒发作复发的患者外,所有患者的总体日常功能均有所改善,家长对手术相关结果满意。所有病例均未出现明显且持续的神经功能缺损迹象。
大多数情况下,双侧MCD患者完全胼胝体切开术的结果良好。该手术对这些患者中导致身体受伤和生活质量受损的跌倒发作特别有效。