title: Hippocampal and neocortical ubiquitin-immunoreactive inclusions in amyotrophic lateral sclerosis with dementia.
abstract: Amyotrophic lateral sclerosis (ALS) patients with dementia were found to have ubiquitin-immunoreactive (IR) inclusions in the dentate granule cells of the hippocampus. These inclusions were also present in some patients with minor cognitive changes but otherwise typical ALS. Ubiquitin-IR inclusions were also found in neurons of superficial layers of the frontal and temporal cortex and in the entorhinal cortex in patients with ALS and dementia. These ubiquitin-IR inclusions were non-argyrophilic, and were not labelled by antibodies which identify Alzheimer's neurofibrillary tangles and Pick bodies, nor were they typical of cortical Lewy bodies. Our findings indicate that ubiquitin-IR inclusions in small neurons of the hippocampus, entorhinal area and neocortex are a characteristic feature of degeneration of non-motor cortex in ALS, and are particularly associated with cognitive impairment and dementia of frontal lobe type.
authors.affiliation: Department of Neurology, Institute of Psychiatry, London, UK.
authors.forename: G
authors.initials: G
authors.lastname: Wightman
authors.forename: V E
authors.initials: VE
authors.lastname: Anderson
authors.forename: J
authors.initials: J
authors.lastname: Martin
authors.forename: M
authors.initials: M
authors.lastname: Swash
authors.forename: B H
authors.initials: BH
authors.lastname: Anderton
authors.forename: D
authors.initials: D
authors.lastname: Neary
authors.forename: D
authors.initials: D
authors.lastname: Mann
authors.forename: P
authors.initials: P
authors.lastname: Luthert
authors.forename: P N
authors.initials: PN
authors.lastname: Leigh
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000544:Alzheimer Disease / Q000378:metabolism / Q000473:pathology; D000690:Amyotrophic Lateral Sclerosis / Q000378:metabolism / Q000473:pathology*; D002540:Cerebral Cortex / Q000378:metabolism / Q000473:pathology*; D003704:Dementia / Q000378:metabolism / Q000473:pathology*; D005260:Female; D006624:Hippocampus / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D013194:Staining and Labeling; D014452:Ubiquitins / Q000276:immunology / Q000378:metabolism*
chemical_list: D014452:Ubiquitins