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Cited by 88Amyotrophic Lateral Sclerosis ResearchNeurogenetic and Muscular Disorders ResearchHereditary Neurological Disorders

The DOI matches its Crossref record; 20 of the 20 references it cites were found in a registry or an index.

Cause of death and clinical grading criteria in a cohort of amyotrophic lateral sclerosis cases undergoing autopsy from the Scottish Motor Neurone Disease Register

Kurian, K M, Forbes, R B, Colville, S, Swingler, R J·2009·Journal of Neurology, Neurosurgery & Psychiatry
DOI 10.1136/jnnp.2008.149708
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Abstract

Background The Scottish Motor Neurone Disease Register is a population based register of amyotrophic lateral sclerosis/motor neurone disease (ALS/MND) in Scotland, with high case ascertainment levels. Objective To investigate the cause of death by autopsy and assess grading criteria in a cohort of cases of ALS from the Scottish MND Register. Methods The records of 44 patients undergoing autopsy were reviewed to determine the cause of death, clinical assessment (El Escorial and modified World Federation of Neurology criteria) during life and neuropathological autopsy findings. Results In a cohort of 44 cases undergoing autopsy between 1989 and 1998, the cause of death could be directly or indirectly (bronchopneumonia, aspiration/pneumonia and respiratory failure) attributed to MND in 32/44 (73%) cases. The clinical diagnosis of MND was confirmed at autopsy in 44/44 (100%) cases, 3/44 (7%) cases showed coexistent neurodegenerative disease and 5/44 (11%) were familial MND cases. Conclusions Within our cohort, MND contributes to death in the majority of cases and there is excellent clinicopathological correlation, irrespective of the clinical grading criteria used. However, the autopsy rate is low (4%) and further larger studies are required to identify heterogeneity within the disease.

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Crossref · 10.1136/jnnp.2008.149708

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Metadata

Authors
Kurian, K. M., Forbes, R. B., Colville, S., & Swingler, R. J.
Affiliations
  • Ninewells Hospital (GB)
  • Craigavon Area Hospital (GB)
Corresponding author
Kurian, K M
ORCID
Venue
Journal of Neurology, Neurosurgery & Psychiatry
Publisher
BMJ
Published
2008-12-16
Volume
80
Issue
1
Pages
84-87
ISSN
0022-3050 (ISSN-L)
DOI
10.1136/jnnp.2008.149708
OpenAlex
W2046129246
Cited by
88
References
20
Citations by year
2026: 4 · 2025: 1 · 2024: 3 · 2023: 3 · 2022: 6 · 2021: 9 · 2020: 13 · 2019: 2 · 2018: 3 · 2017: 7 · 2016: 7 · 2015: 2 · 2014: 6 · 2013: 5 · 2012: 4
FWCI
2.82
Type
journal article
Language
en
Open access
closed
Locations
  • Journal of Neurology Neurosurgery & Psychiatry (publishedVersion)
  • PubMed (publishedVersion)
  • Explore Bristol Research (submittedVersion)
  • Bristol Research (University of Bristol) (submittedVersion)
Topics
  • Health Sciences › Medicine › Clinical Neurology › Amyotrophic Lateral Sclerosis Research
  • Health Sciences › Medicine › Genetics (clinical) › Neurogenetic and Muscular Disorders Research
  • Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Hereditary Neurological Disorders
Keywords
amyotrophic lateral sclerosis, motor neuron disease, causes of death, autopsy findings, clinicopathological correlation, El Escorial criteria, respiratory failure, aspiration pneumonia, bronchopneumonia
MeSH terms
Adult, Aged, Aged, 80 and over, Amyotrophic Lateral Sclerosis, Autopsy, Brain Diseases, Bronchopneumonia, Cause of Death, Female, Heart Diseases, Humans, Male, Middle Aged, Registries, Respiratory Insufficiency, Scotland, Cohort Studies, Young Adult

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