[完全闭锁状态下的肌萎缩侧索硬化症]

[Amyotrophic lateral sclerosis in totally locked-in state].

作者信息

Oyanagi Kiyomitsu, Mochizuki Yoko, Nakayama Yuki, Hayashi Kentaro, Shimizu Toshio, Nagao Masahiro, Hashimoto Tomoyo, Yamazaki Mineo, Matsubara Shiro, Komori Takashi

机构信息

Department of Brain Disease Research, Shinshu University School of Medicine.

出版信息

Rinsho Shinkeigaku. 2013;53(11):1399-401. doi: 10.5692/clinicalneurol.53.1399.

DOI:10.5692/clinicalneurol.53.1399

PMID:24292003

Abstract

Seven autopsy patients with amyotrophic lateral sclerosis (ALS) in totally locked-in state (TLS) were examined neuropathologically. The patients were composed of 4 males and 3 females, and 3 with familial, 1 sporadic but with mutation in SOD1 gene, and 3 sporadic patients with unremarkable gene mutation. The brains weighed 715, 783, 1,019, 1,050, 1,170, 1,190 or 1,233 g. The tegmentum of the brain stem was markedly degenerated in every patient, and the tracts relating to the somatic sensory and auditory were involved in the lesions.

摘要

对7例处于完全闭锁状态（TLS）的肌萎缩侧索硬化症（ALS）尸检患者进行了神经病理学检查。患者包括4名男性和3名女性，其中3例为家族性，1例为散发性但SOD1基因有突变，3例散发性患者基因突变不明显。大脑重量分别为715、783、1019、1050、1170、1190或1233克。每位患者的脑干被盖均明显退化，与躯体感觉和听觉相关的神经束也有病变。