Matsumoto S, Kusaka H, Ito H, Yamasaki M, Imai T
Department of Neurology, Kitano Hospital & Neurological Center.
Rinsho Shinkeigaku. 1993 Nov;33(11):1125-30.
This study investigated the expression of ubiquitin in anterior horn cells of various subgroups of adult-onset motor neuron diseases (MNDs). Ubiquitin-positive skein-like inclusions (SLIs) were found in all 11 cases of sporadic amyotrophic lateral sclerosis (ALS) patients, two patients with lower MND, 3 sporadic cases of ALS with dementia, 3 sporadic ALS cases with long-term use of respirators, and two cases of sporadic ALS with Lewy body-like hyaline inclusions. This result suggests that a similar pathomechanism is involved in the degeneration of the lower motor neurons in these subgroups. SLIs were not detected in two cases of adult-onset MND with basophilic inclusions. This may indicate that adult-onset MND with basophilic inclusions is a distinct nosological entity of classical ALS and some relationship to juvenile ALS with basophilic inclusions is suggested.
本研究调查了泛素在成年发病的运动神经元病（MNDs）各亚组前角细胞中的表达情况。在11例散发性肌萎缩侧索硬化症（ALS）患者、2例下运动神经元病患者、3例散发性ALS合并痴呆患者、3例长期使用呼吸机的散发性ALS患者以及2例伴有路易体样透明包涵体的散发性ALS患者的所有病例中均发现了泛素阳性的丝状包涵体（SLIs）。这一结果表明，这些亚组中运动神经元的变性涉及相似的发病机制。在2例伴有嗜碱性包涵体的成年发病MND患者中未检测到SLIs。这可能表明，伴有嗜碱性包涵体的成年发病MND是经典ALS的一个独特病种，并提示其与伴有嗜碱性包涵体的青少年ALS存在某种关联。
