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Ubiquitin Immunohistochemistry Suggests Classic Motor Neuron Disease, Motor Neuron Disease With Dementia, and Frontotemporal Dementia of the Motor Neuron Disease Type Represent a Clinicopathologic Spectrum

ABSTRACTOne of the characteristic pathologic changes in classic motor neuron disease (MND) is the presence of ubiquitin-immunoreactive (ub-ir) inclusions in the cytoplasm of lower motor neurons. In addition, cases of MND with dementia (MND-d) also have ub-ir neuronal cytoplasmic inclusions and dystr... Full description

Journal Title: Journal of neuropathology and experimental neurology 2005-08, Vol.64 (8), p.730-739
Main Author: Mackenzie, Ian R. A
Other Authors: H. Feldman, Howard
Format: Electronic Article Electronic Article
Language: English
Subjects:
Quelle: Alma/SFX Local Collection
Publisher: Hagerstown, MD: American Association of Neuropathologists, Inc
ID: ISSN: 0022-3069
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recordid: cdi_proquest_miscellaneous_68488523
title: Ubiquitin Immunohistochemistry Suggests Classic Motor Neuron Disease, Motor Neuron Disease With Dementia, and Frontotemporal Dementia of the Motor Neuron Disease Type Represent a Clinicopathologic Spectrum
format: Article
creator:
  • Mackenzie, Ian R. A
  • H. Feldman, Howard
subjects:
  • Adult
  • Aged
  • Aged, 80 and over
  • Biological and medical sciences
  • Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases
  • Dementia - classification
  • Dementia - complications
  • Dementia - metabolism
  • Dementia - pathology
  • Diseases of the nervous system
  • Female
  • Humans
  • Immunohistochemistry - methods
  • Inclusion Bodies - metabolism
  • Inclusion Bodies - pathology
  • Male
  • Medical sciences
  • Middle Aged
  • Motor Neuron Disease - complications
  • Motor Neuron Disease - metabolism
  • Motor Neuron Disease - pathology
  • Nerve Degeneration - complications
  • Nerve Degeneration - metabolism
  • Nerve Degeneration - pathology
  • Neurites - metabolism
  • Neurofibrillary Tangles - metabolism
  • Neurofibrillary Tangles - pathology
  • Neurology
  • Neurons - metabolism
  • Neurons - pathology
  • Postmortem Changes
  • Radiotherapy. Instrumental treatment. Physiotherapy. Reeducation. Rehabilitation, orthophony, crenotherapy. Diet therapy and various other treatments (general aspects)
  • Ubiquitin - metabolism
ispartof: Journal of neuropathology and experimental neurology, 2005-08, Vol.64 (8), p.730-739
description: ABSTRACTOne of the characteristic pathologic changes in classic motor neuron disease (MND) is the presence of ubiquitin-immunoreactive (ub-ir) inclusions in the cytoplasm of lower motor neurons. In addition, cases of MND with dementia (MND-d) also have ub-ir neuronal cytoplasmic inclusions and dystrophic neurites in extramotor neocortex and hippocampus. Although this extramotor pathology is a highly sensitive marker for dementia in MND, similar changes are found in a subset of patients with frontotemporal dementia (FTD) with no motor symptoms (FTD-MND type). The purpose of this study is to more fully describe and compare the pattern of ub-ir pathology in these 3 conditions. We performed ubiquitin immunohistochemistry on postmortem tissue, representing a wide range of neuroanatomic structures, in cases of classic MND (n = 20), MND-d (n = 15), and FTD-MND type (n = 15). We found the variety of morphologies and the anatomic distribution of ub-ir pathology to be greater than previously documented. Moreover, the degree of overlap suggests that MND, MND-d, and FTD-MND type represent a spectrum of clinical disease with a common pathologic substrate. The only finding restricted to a specific subgroup of patients was the presence of ub-ir neuronal intranuclear inclusions in some cases of familial FTD.
language: eng
source: Alma/SFX Local Collection
identifier: ISSN: 0022-3069
fulltext: fulltext
issn:
  • 0022-3069
  • 1554-6578
url: Link


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descriptionABSTRACTOne of the characteristic pathologic changes in classic motor neuron disease (MND) is the presence of ubiquitin-immunoreactive (ub-ir) inclusions in the cytoplasm of lower motor neurons. In addition, cases of MND with dementia (MND-d) also have ub-ir neuronal cytoplasmic inclusions and dystrophic neurites in extramotor neocortex and hippocampus. Although this extramotor pathology is a highly sensitive marker for dementia in MND, similar changes are found in a subset of patients with frontotemporal dementia (FTD) with no motor symptoms (FTD-MND type). The purpose of this study is to more fully describe and compare the pattern of ub-ir pathology in these 3 conditions. We performed ubiquitin immunohistochemistry on postmortem tissue, representing a wide range of neuroanatomic structures, in cases of classic MND (n = 20), MND-d (n = 15), and FTD-MND type (n = 15). We found the variety of morphologies and the anatomic distribution of ub-ir pathology to be greater than previously documented. Moreover, the degree of overlap suggests that MND, MND-d, and FTD-MND type represent a spectrum of clinical disease with a common pathologic substrate. The only finding restricted to a specific subgroup of patients was the presence of ub-ir neuronal intranuclear inclusions in some cases of familial FTD.
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subjectAdult ; Aged ; Aged, 80 and over ; Biological and medical sciences ; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases ; Dementia - classification ; Dementia - complications ; Dementia - metabolism ; Dementia - pathology ; Diseases of the nervous system ; Female ; Humans ; Immunohistochemistry - methods ; Inclusion Bodies - metabolism ; Inclusion Bodies - pathology ; Male ; Medical sciences ; Middle Aged ; Motor Neuron Disease - complications ; Motor Neuron Disease - metabolism ; Motor Neuron Disease - pathology ; Nerve Degeneration - complications ; Nerve Degeneration - metabolism ; Nerve Degeneration - pathology ; Neurites - metabolism ; Neurofibrillary Tangles - metabolism ; Neurofibrillary Tangles - pathology ; Neurology ; Neurons - metabolism ; Neurons - pathology ; Postmortem Changes ; Radiotherapy. Instrumental treatment. Physiotherapy. Reeducation. Rehabilitation, orthophony, crenotherapy. Diet therapy and various other treatments (general aspects) ; Ubiquitin - metabolism
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descriptionABSTRACTOne of the characteristic pathologic changes in classic motor neuron disease (MND) is the presence of ubiquitin-immunoreactive (ub-ir) inclusions in the cytoplasm of lower motor neurons. In addition, cases of MND with dementia (MND-d) also have ub-ir neuronal cytoplasmic inclusions and dystrophic neurites in extramotor neocortex and hippocampus. Although this extramotor pathology is a highly sensitive marker for dementia in MND, similar changes are found in a subset of patients with frontotemporal dementia (FTD) with no motor symptoms (FTD-MND type). The purpose of this study is to more fully describe and compare the pattern of ub-ir pathology in these 3 conditions. We performed ubiquitin immunohistochemistry on postmortem tissue, representing a wide range of neuroanatomic structures, in cases of classic MND (n = 20), MND-d (n = 15), and FTD-MND type (n = 15). We found the variety of morphologies and the anatomic distribution of ub-ir pathology to be greater than previously documented. Moreover, the degree of overlap suggests that MND, MND-d, and FTD-MND type represent a spectrum of clinical disease with a common pathologic substrate. The only finding restricted to a specific subgroup of patients was the presence of ub-ir neuronal intranuclear inclusions in some cases of familial FTD.
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24Neurites - metabolism
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31Radiotherapy. Instrumental treatment. Physiotherapy. Reeducation. Rehabilitation, orthophony, crenotherapy. Diet therapy and various other treatments (general aspects)
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titleUbiquitin Immunohistochemistry Suggests Classic Motor Neuron Disease, Motor Neuron Disease With Dementia, and Frontotemporal Dementia of the Motor Neuron Disease Type Represent a Clinicopathologic Spectrum
authorMackenzie, Ian R. A ; H. Feldman, Howard
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abstractABSTRACTOne of the characteristic pathologic changes in classic motor neuron disease (MND) is the presence of ubiquitin-immunoreactive (ub-ir) inclusions in the cytoplasm of lower motor neurons. In addition, cases of MND with dementia (MND-d) also have ub-ir neuronal cytoplasmic inclusions and dystrophic neurites in extramotor neocortex and hippocampus. Although this extramotor pathology is a highly sensitive marker for dementia in MND, similar changes are found in a subset of patients with frontotemporal dementia (FTD) with no motor symptoms (FTD-MND type). The purpose of this study is to more fully describe and compare the pattern of ub-ir pathology in these 3 conditions. We performed ubiquitin immunohistochemistry on postmortem tissue, representing a wide range of neuroanatomic structures, in cases of classic MND (n = 20), MND-d (n = 15), and FTD-MND type (n = 15). We found the variety of morphologies and the anatomic distribution of ub-ir pathology to be greater than previously documented. Moreover, the degree of overlap suggests that MND, MND-d, and FTD-MND type represent a spectrum of clinical disease with a common pathologic substrate. The only finding restricted to a specific subgroup of patients was the presence of ub-ir neuronal intranuclear inclusions in some cases of familial FTD.
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pmid16106222
doi10.1097/01.jnen.0000174335.27708.0a
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