The relationship between Bunina bodies, skein-like inclusions and neuronal loss in amyotrophic lateral sclerosis.

Abstract:

:Specific pathological hallmarks have been described in amyotrophic lateral sclerosis (ALS), which include motor neuronal loss, Bunina bodies (BBs) and skein like inclusions (SLIs). We investigated the relation between these three lesions in the cervical and lumbar anterior horns and the hypoglossal nuclei of 20 ALS patients and 9 controls using a quantitative light microscopy study. Immunohistochemistry with anti-cystatin C and anti-ubiquitin was used to detect the BBs and SLIs, respectively. A significant relation between the severity of neuronal loss and the proportion of SLI-containing neurons was found in the spinal cord, whereas no relation was found with BBs. We therefore propose that BBs and SLIs participate in two different steps of the cascade leading to neuronal loss.

journal_name

Acta Neuropathol

journal_title

Acta neuropathologica

authors

van Welsem ME,Hogenhuis JA,Meininger V,Metsaars WP,Hauw JJ,Seilhean D

doi

10.1007/s00401-001-0507-3

subject

Has Abstract

pub_date

2002-06-01 00:00:00

pages

583-9

issue

6

eissn

0001-6322

issn

1432-0533

journal_volume

103

pub_type

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