Inage Yukiko, Halliday William C, Go Cristina, Ochi Ayako, Akiyama Tomoyuki, Akiyama Mari, Widjaja Elysa, Otsubo Hiroshi
Division of Pathology, The Hospital for Sick Children, Ontario, Canada M5G 1X8.
Brain Dev. 2012 Feb;34(2):118-23. doi: 10.1016/j.braindev.2011.03.009. Epub 2011 Apr 13.
Epileptic spasms in older children have increasingly been recognized as a distinct seizure type and subset of these patients are considered for surgical resection. This study compares histopathology and magnetic resonance imaging (MRI), especially focusing the difference between the cortical grey matter and the subcortical white matter to understand the extensive epileptic brain in patients with epileptic spasms. We examined 22 patients consisting of 11 patients with epileptic spasms and 11 with partial seizures. Scalp video electroencephalography (EEG) showed interictal generalized epileptiform discharges (9 patients with epileptic spasms vs. 1 with partial seizures) and ictal generalized epileptiform discharges (10 vs. 3). We found MRI abnormalities in a single lobe (6 vs. 7) and multiple lobes (2 vs. 1). Surgical resections were performed across multiple lobes (9 vs. 2), comparing within a single lobe (2 vs. 9), (p<0.001). Histopathology showed abnormal cortical organizations as FCD (2 vs. 5) and microdysgenesis (4 vs. 4), normal (4 vs. 1). Two patients with epileptic spasms showed hyaline proteoplasmic astrocytopathy. There were heterotopic neurons (10 vs. 10), cluster of oligodendroglia (8 vs. 7), balloon cells (2 vs. 5) and blurred myelination (1 vs. 4), in the white matter. Seizure-free outcomes were seen in seven patients with epileptic spasms (64%) and four with partial seizures (36%). The multilobar epileptogenic zones existed in patients with epileptic spasms, compared with the focal epileptogenic zone in patients with partial seizures. There was no difference of MRI and histopathology findings in cortex and subcortical white matter between two groups.
大龄儿童的癫痫性痉挛越来越被认为是一种独特的癫痫发作类型，其中一部分患者会考虑进行手术切除。本研究比较了组织病理学和磁共振成像（MRI），尤其关注皮质灰质和皮质下白质之间的差异，以了解癫痫性痉挛患者广泛受累的癫痫脑。我们检查了22例患者，其中11例患有癫痫性痉挛，11例患有部分性癫痫发作。头皮视频脑电图（EEG）显示发作间期广泛性癫痫样放电（9例癫痫性痉挛患者对1例部分性癫痫发作患者）和发作期广泛性癫痫样放电（10例对3例）。我们发现单叶MRI异常（6例对7例）和多叶MRI异常（2例对1例）。多叶手术切除（9例对2例）与单叶手术切除（2例对9例）相比，（p<0.001）。组织病理学显示皮质组织异常为局灶性皮质发育不良（FCD，2例对5例）和微小发育异常（4例对4例），正常（4例对1例）。2例癫痫性痉挛患者表现为透明蛋白性星形细胞病。白质中有异位神经元（10例对10例）、少突胶质细胞簇（8例对7例）、气球样细胞（2例对5例）和髓鞘模糊（1例对4例）。7例癫痫性痉挛患者（64%）和4例部分性癫痫发作患者（
