Arai Nobutaka, Umitsu Reiko, Komori Takashi, Hayashi Masaharu, Kurata Kiyoko, Nagata Jinro, Tamagawa Kimiko, Mizutani Toshio, Oda Masaya, Morimatsu Yoshio
Department of Clinical Neuropathology, Tokyo Metropolitan Institute for Neuroscience, Tokyo, Japan.
Pathol Int. 2003 Jun;53(6):345-52. doi: 10.1046/j.1440-1827.2003.01480.x.
Microdysgenesis (MD) is a neuropathological term that implies a variety of minor developmental abnormalities of the brain. Recently, MD has been used for pathological diagnosis of cerebral tissues surgically resected from epileptic patients. However, criteria or consensus on pathological diagnosis of MD is still vague and controversial because of the lack of control studies. Therefore, this study paid special attention to the presence of white matter neurons with perineuronal glial satellitosis (WMN-GS) and perivascular glial satellitosis (PVGS) in the white matter, which are occasionally observed in epileptic foci, in order to clarify whether they could be handled as definite findings of MD. The materials included 80 autopsied whole brains ranging from normal subjects to patients with cerebrovascular disorder, neurodegenerative diseases and malformations. In each case, the presence of WMN-GS and/or PVGS was searched in 10 gyri in all five lobes (rostral frontal lobe, caudal frontal lobe, parietal lobe, temporal lobe and oc-cipital lobe) and evaluated. Statistically significant, WMN-GS and/or PVGS preferentially appeared in a diseased group consisting of neuronal migration disorder and related conditions, such as polymicrogyria, nodular heterotopia or tuberous sclerosis, leading to a suggestive conclusion that the presence of WMN-GS and/or PVGS could be a peculiar form of MD possibly derived from neuronal migrational arrest or related events, even if they appear alone without any other gross abnormalities.
微小发育异常（MD）是一个神经病理学术语，意味着大脑存在多种轻微的发育异常。最近，MD已被用于对癫痫患者手术切除的脑组织进行病理诊断。然而，由于缺乏对照研究，MD病理诊断的标准或共识仍然模糊且存在争议。因此，本研究特别关注白质中伴有神经元周围胶质卫星现象（WMN-GS）和血管周围胶质卫星现象（PVGS）的白质神经元的存在情况，这些现象偶尔会在癫痫病灶中观察到，以明确它们是否可被视为MD的明确表现。研究材料包括80个尸检全脑，涵盖正常受试者以及患有脑血管疾病、神经退行性疾病和畸形的患者。在每个病例中，在所有五个脑叶（额前叶、额后叶、顶叶、颞叶和枕叶）的10个脑回中搜索WMN-GS和/或PVGS的存在情况并进行评估。具有统计学意义的是，WMN-GS和/或PVGS优先出现在由神经元迁移障碍及相关病症组成的疾病组中，如多小脑回、结节性异位症或结节性硬化症，从而得出一个具有提示性的结论：WMN-GS和/或PVGS的存在可能是MD的一种特殊形式，可能源于神经元迁移停滞或相关事件，即使它们单独出现且无任何其他明显异常。
