[Sporadic juvenile amyotrophic lateral sclerosis with neuronal basophil inclusion bodies--a nosologic entity?]

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.