The relationship between Bunina bodies, skein-like inclusions and neuronal loss in amyotrophic lateral sclerosis.
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.
van Welsem
M E
ME
Laboratoire de Neuropathologie Raymond Escourolle, Hôpital de la Salpêtrière, 47 Boulevard de l'Hôpital, 75651 Paris Cedex 13, France.
Hogenhuis
J A
JA
Meininger
V
V
Metsaars
W P
WP
Hauw
J-J
JJ
Seilhean
D
D
D000328:Adult; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000378:metabolism / Q000473:pathology* / Q000503:physiopathology; D001933:Brain Stem / Q000378:metabolism / Q000473:pathology* / Q000503:physiopathology; D002452:Cell Count; D016923:Cell Death / Q000502:physiology*; D055316:Cystatin C; D015891:Cystatins / Q000378:metabolism; D003599:Cytoskeleton / Q000378:metabolism / Q000473:pathology*; D005260:Female; D006801:Humans; D007002:Hypoglossal Nerve / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology; D007150:Immunohistochemistry; D002479:Inclusion Bodies / Q000378:metabolism / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009046:Motor Neurons / Q000378:metabolism / Q000473:pathology*; D013116:Spinal Cord / Q000378:metabolism / Q000473:pathology* / Q000503:physiopathology; D013223:Statistics as Topic; D025801:Ubiquitin / Q000378:metabolism
C525030:CST3 protein, human; D055316:Cystatin C; D015891:Cystatins; D025801:Ubiquitin