[A comparative immunohistochemical study of ubiquitin-positive skein-like inclusions in anterior horn neurons in subgroups of adult-onset motor neuron diseases].
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.
Matsumoto
S
S
Department of Neurology, Kitano Hospital & Neurological Center.
Kusaka
H
H
Ito
H
H
Yamasaki
M
M
Imai
T
T
D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D000690:Amyotrophic Lateral Sclerosis / Q000473:pathology; D000870:Anterior Horn Cells / Q000166:cytology* / Q000378:metabolism / Q000502:physiology; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D002479:Inclusion Bodies / Q000378:metabolism*; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000378:metabolism / Q000473:pathology*; D009410:Nerve Degeneration; D014452:Ubiquitins / Q000032:analysis*
D014452:Ubiquitins