Ubiquitin-positive inclusion in anterior horn cells in subgroups of motor neuron diseases: A comparative study of adult-onset amyotrophic lateral sclerosis, juvenile amyotrophic lateral sclerosis and werdnig-hoffmann disease

Sadayuki Matsumoto, Satoshi Goto, Hirofumi Kusaka, Terukuni Imai, Nobuyuki Murakami, Yoshio Hashizume, Haruo Okazaki, Asao Hirano

Research output: Contribution to journalArticlepeer-review

40 Scopus citations

Abstract

This report concerns the expression of ubiquitin in anterior horn cells of various subgroups of adult and infantile motor neuron disease (MNDs); immunohistochemical techniques were employed. Ubiquitin-positive skein-like inclusions (SLIs) were found in all cases of adult-onset amyotrophic lateral sclerosis (ALS), including 16 cases with sporadic ALS, two cases of familial ALS with posterior column degeneration and Lewy body-like hyaline incusions (LBHIs), two sporadic ALS cases with LBHIs, and three cases of sporadic ALS with dementia. SLIs were not found in anterior horn cells of 5 cases with Werdnig-Hoffmann disease (WHD). However, granular ubiquitin-positive deposits were seen in ballooned neurons of WHD patients. No ubiquitinated materials were found in the perikarya of two sporadic juvenile ALS patients with basophilic inclusions (BIs), but granular ubiquitin-immunoreactive deposits were occasionally observed in the BIs. These results suggest that ubiquitin-positive SLIs are characteristic features of various forms of adult-onset ALS and that aggregated ubiquitinated granules are characteristic of ballooned neurons of WHD. Ubiquitinated structures and their distribution patterns may reflect degenerative processes of anterior horn cells, and may be useful for classifying subgroups of motor neuron diseases.

Original languageEnglish (US)
Pages (from-to)208-213
Number of pages6
JournalJournal of the Neurological Sciences
Volume115
Issue number2
DOIs
StatePublished - Apr 1993

Keywords

  • Amyotrophic lateral sclerosis
  • Anterior horn cell
  • Juvenile amyotrophic lateral sclerosis
  • Ubiquitin
  • Werdnig-Hoffmann disease

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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