Neuropathology of amyotrophic lateral sclerosis with extra-motor system degeneration: Characteristics and differences in the molecular pathology between ALS with dementia and Guamanian ALS

Akito Ikemoto, Asao Hirano, Ichiro Akiguchi

Research output: Contribution to journalArticle

17 Citations (Scopus)

Abstract

Amyotrophic lateral sclerosis (ALS) is classified into distinct subtypes mainly based on clinicopathological features, in addition to epidemiologic and genetic backgrounds. In addition to sporadic ALS with classical pathology, characteristics in the clinical features, in the histological findings and their topographical distribution, and in the molecular pathology, especially the intracytoplasmic neuronal inclusions, enable us to identify the following subtypes: ALS with dementia (ALS-D), ALS in the Western Pacific, ALS with multi-system degeneration, familial ALS, and superoxide dismutase 1-linked ALS. These subtypes not infrequently exhibit various types of extra-motor system degeneration, and even multisystem pathology. Some of the subtypes (for instance ALS-D or familial ALS) can be deduced, to a certain extent, from characteristic neuronal inclusions such as ALS-type ubiquitinated inclusions, Lewy body-like hyaline inclusions (LBHIs), or ubiquitinated intracytoplasmic neuronal inclusions as typically seen in the dentate fascia. The purpose of this article is to clarify the molecular pathogenesis of the cerebral cortex in ALS-D and Guamanian ALS and parkinsonism-dementia complex (PDC), in order to elucidate the relationship and distinction between these two subtypes. As indicated previously, investigations on ubiquitin-immunoreactivity in the hippocampus further support the view that the pathology of G-ALS/PDC may be that of a tau-related tangle disorder, whilst ALS-D has a feature of the motor neuron disease type-frontotemporal dementia. (ALS 2000; 1:97-104)

Original languageEnglish (US)
Pages (from-to)97-104
Number of pages8
JournalAmyotrophic Lateral Sclerosis
Volume1
Issue number2
DOIs
StatePublished - 2000

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Molecular Pathology
Amyotrophic Lateral Sclerosis
Parkinsonian Disorders
Pathology
Dementia
Neuropathology
Lewy Bodies
Frontotemporal Dementia
Motor Neuron Disease
Hyalin
Inclusion Bodies
Dentate Gyrus
Ubiquitin
Cerebral Cortex
Hippocampus

Keywords

  • amyotrophic lateral sclerosis
  • extra-motor system
  • molecular pathology
  • neuronal inclusions
  • ubiquitin

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology

Cite this

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abstract = "Amyotrophic lateral sclerosis (ALS) is classified into distinct subtypes mainly based on clinicopathological features, in addition to epidemiologic and genetic backgrounds. In addition to sporadic ALS with classical pathology, characteristics in the clinical features, in the histological findings and their topographical distribution, and in the molecular pathology, especially the intracytoplasmic neuronal inclusions, enable us to identify the following subtypes: ALS with dementia (ALS-D), ALS in the Western Pacific, ALS with multi-system degeneration, familial ALS, and superoxide dismutase 1-linked ALS. These subtypes not infrequently exhibit various types of extra-motor system degeneration, and even multisystem pathology. Some of the subtypes (for instance ALS-D or familial ALS) can be deduced, to a certain extent, from characteristic neuronal inclusions such as ALS-type ubiquitinated inclusions, Lewy body-like hyaline inclusions (LBHIs), or ubiquitinated intracytoplasmic neuronal inclusions as typically seen in the dentate fascia. The purpose of this article is to clarify the molecular pathogenesis of the cerebral cortex in ALS-D and Guamanian ALS and parkinsonism-dementia complex (PDC), in order to elucidate the relationship and distinction between these two subtypes. As indicated previously, investigations on ubiquitin-immunoreactivity in the hippocampus further support the view that the pathology of G-ALS/PDC may be that of a tau-related tangle disorder, whilst ALS-D has a feature of the motor neuron disease type-frontotemporal dementia. (ALS 2000; 1:97-104)",
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