A comparative immunohistochemical study of ubiquitin-positive skein-like inclusions in anterior horn neurons in subgroups of adult-onset motor neuron diseases
Matsumoto, S.; Kusaka, H.; Ito, H.; Yamasaki, M.; Imai, T.
Rinsho Shinkeigaku 33(11): 1125-1130
1993
ISSN/ISBN: 0009-918X PMID: 8124868 Document Number: 406234
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.