Antoine J C, Steck A, Michel D
Service de Neurologie, Hôpital de Bellevue, Saint-Etienne, France.
Rev Neurol (Paris). 1993;149(8-9):496-9.
A 75 year old woman died of predominantly motor peripheral neuropathy with amyotrophy and fasciculations progressing to tetraplegia and death within 19 months. There was a mild distal sensory loss. At electrophysiology, the pattern was initially demyelinating and later became axonal. Nerve biopsy disclosed severe myelinated and unmyelinated fiber loss with wallerian degeneration. The remaining fibers had demyelination widening of the external myelin lamellae and intense hypermyelination. Serum contained an anti-MAG monoclonal IgM reacting with SGPG. Two siblings without monoclonal gammopathy had died of definite amyotrophic lateral sclerosis. This family association is discussed.
一名75岁女性死于以运动为主的周围神经病变，伴有肌萎缩和肌束震颤，19个月内进展为四肢瘫痪并死亡。存在轻度远端感觉丧失。电生理检查显示，最初为脱髓鞘模式，后来变为轴索性。神经活检显示严重的有髓和无髓纤维丧失以及华勒变性。剩余纤维有髓鞘板层外部脱髓鞘增宽和强烈的髓鞘过度增生。血清中含有一种与SGPG反应的抗MAG单克隆IgM。两名无单克隆丙种球蛋白病的兄弟姐妹死于明确的肌萎缩侧索硬化症。讨论了这种家族关联。
