Baba Shimpei, Okanishi Tohru, Nishimura Mitsuyo, Kanai Sotaro, Itamura Shinji, Suzuki Takayuki, Masuda Yosuke, Enoki Hideo, Fujimoto Ayataka
Department of Child Neurology, Seirei-Hamamatsu General Hospital, Hamamatsu, Shizuoka, Japan.
Department of Child Neurology, Seirei-Hamamatsu General Hospital, Hamamatsu, Shizuoka, Japan.
Brain Dev. 2018 Sep;40(8):719-723. doi: 10.1016/j.braindev.2018.03.007. Epub 2018 Apr 3.
Polymicrogyria, a malformation of the cerebral cortex, frequently causes epilepsy. Diffuse bilateral polymicrogyria (DBP) is related to poor epilepsy prognosis, but most patients with DBP are not good candidates for resective epilepsy surgery and effectiveness of corpus callosotomy (CC), a palliative surgery, for patients without resective epileptogenic cortices, has not been established in DBP. Because CC might be effective against DBP-related epilepsy, we conducted total CC in three pediatric DBP cases.
Case 1. A girl developed epilepsy at 3 months of age, with focal versive seizures and epileptic spasms. The electroencephalogram (EEG) showed a suppression-burst pattern. Total CC was performed at 6 months of age. Case 2. A female infant developed epilepsy on the day of birth, exhibiting epileptic spasms, generalized tonic-clonic seizures, and eye-deviating seizures. She had a history of clusters of tonic seizures. Total CC was performed at 1 year and 2 months of age. After CC, the epileptic focus of the tonic seizures was identified; a secondary resective surgery was conducted. Case 3. A girl developed multiple types of seizures at 3 years of age. Frequent atypical absence status was refractory to antiepileptic drugs. Total CC was conducted at 8 years of age.
Case 1: Frequencies of both seizure types decreased. The background EEG changed to continuous high-voltage slow waves. Case 2: Clusters of tonic seizures were well-controlled. Case 3: Atypical absence seizures completely disappeared.
CC could be effective for patients with DBP, whose habitual seizures include epileptic spasms and absence seizures.
多小脑回畸形是一种大脑皮质发育畸形，常导致癫痫。双侧弥漫性多小脑回畸形（DBP）与癫痫预后不良有关，但大多数DBP患者并非切除性癫痫手术的合适人选，对于没有可切除的致痫皮质的患者，姑息性手术胼胝体切开术（CC）在DBP中的有效性尚未确立。由于CC可能对DBP相关癫痫有效，我们对3例儿童DBP患者进行了全胼胝体切开术。
病例1。一名女孩在3个月大时出现癫痫，伴有局灶性旋转性发作和癫痫性痉挛。脑电图（EEG）显示为抑制-爆发模式。在6个月大时进行了全胼胝体切开术。病例2。一名女婴在出生当天出现癫痫，表现为癫痫性痉挛、全身强直-阵挛发作和眼球偏斜发作。她有强直性发作簇的病史。在1岁2个月时进行了全胼胝体切开术。胼胝体切开术后，确定了强直性发作的癫痫病灶；进行了二次切除性手术。病例3。一名女孩在3岁时出现多种类型的发作。频繁的非典型失神状态对抗癫痫药物难治。在8岁时进行了全胼胝体切开术。
病例1：两种发作类型的频率均降低。背景脑电图变为持续的高电压慢波。病例2：强直性发作簇得到良好控制。病例3：非典型失神发作完全消失。
胼胝体切开术对DBP患者可能有效，其习惯性发作包括癫痫性痉挛和失神发作。
