Schmidbauer M, Hainfellner J A, Budka H
Klinischen Institut für Neurologie, Universität Wien.
Wien Med Wochenschr. 1996;146(9-10):201-2.
Among the very few cases of juvenile sporadic amyotrophic lateral sclerosis (ALS), 6 cases with neuronal basophilic inclusion bodies (BI) and clinical features uncommon to "classical" adult sporadic ALS have been reported. We present here two further cases and review the relevant literature. There are clinical and neuropathological similarities of systemic degeneration between juvenile cases with or without BI and juvenile and adult sporadic ALS when the latter is allowed to run a protracted course in patients on respirators. Because of overlapping topology and neuronal cytoskeletal pathology, the nosologic distinction within the ALS spectrum is questionable.
在极少数青少年散发性肌萎缩侧索硬化症（ALS）病例中，已有报道称6例存在神经元嗜碱性包涵体（BI）且具有“经典”成人散发性ALS不常见的临床特征。我们在此报告另外两例病例并回顾相关文献。当允许后者在使用呼吸机的患者中病程延长时，有BI和无BI的青少年病例与青少年及成人散发性ALS之间在全身变性方面存在临床和神经病理学相似性。由于拓扑结构和神经元细胞骨架病理学存在重叠，ALS谱系内的疾病分类区别值得怀疑。
