TITLE

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

AUTHOR(S)
Kurian, K. M.; Forbes, A. B.; Colviile, S.; Swingler, A. J.
PUB. DATE
January 2009
SOURCE
Journal of Neurology, Neurosurgery & Psychiatry;Jan2009, Vol. 80 Issue 1, p84
SOURCE TYPE
Academic Journal
DOC. TYPE
Article
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.
ACCESSION #
36132269

 

Related Articles

  • Does apoptosis occur in amyotrophic lateral sclerosis? TUNEL experience from human Amyotrophic Lateral Sclerosis (ALS) tissues. Tomik, Barbara; Adamek, Dariusz; Pierzchalski, Piotr; Banares, Steven; Duda, Ateksandra; Partyka, Dorota; Pawlik, Wiestaw; Katuża, Józef; Krajewski, Stan; Szczudlik, Andrzej // Folia Neuropathologica;2005, Vol. 43 Issue 2, p75 

    The role that apoptosis plays in the pathogenesis of amyotrophic lateral sclerosis (ALS) is still unclear From our autopsy samples, we have undertaken an effort to verify if apoptosis in ALS really occurs or if can at least he detected. The study was performed using TUNEL method for screening...

  • Basic facts and figures. Wood-Allum, Clare; Shaw, Pamela // GP: General Practitioner;3/18/2002, p57 

    Focuses on motor neurone disease (MND), one of the three most common neurodegenerative diseases in adulthood. Age groups affected by the disease; Symptoms of amyotrophic lateral sclerosis, a subtype of MND; Other subtypes of the disease.

  • Motor Neurone Disease Australia. BERRY, EMI // Money (Australia Edition);Mar2015, Issue 176, p26 

    The article offers information on the Motor Neurone Disease Australia, which aims to promote optimal care for people with motor neurone disease (MND) and finance MND research.

  • Improving survival in amyotrophic lateral sclerosis: future treatments in a modern service. Hrastelj, James; Robertson, Neil // Journal of Neurology;Jul2015, Vol. 262 Issue 7, p1791 

    An introduction to the journal is presented where the editor discusses various reports published in the issue which includes the discovery of another gene associated with amyotrophic lateral sclerosis (ALS), the association between methylation of the C9orf72 promoter and disease severity and the...

  • A zebrafish model exemplifies the long preclinical period of motor neuron disease. Ramesh, Tennore M.; Shaw, Pamela J.; McDearmid, Jonathan // Journal of Neurology, Neurosurgery & Psychiatry;Nov2014, Vol. 85 Issue 11, p1288 

    A letter to the editor is presented in response to the article "Amyotrophic lateral sclerosis: a long preclinical period?" by A. Eisen and colleagues published in a issue of 2014, is presented.

  • Response to a letter by Dr T Ramesh. Eisen, Andrew // Journal of Neurology, Neurosurgery & Psychiatry;Nov2014, Vol. 85 Issue 11, p1289 

    A response from the author of the article "Amyotrophic lateral sclerosis: a long preclinical period?" that was published in a issue of 2014 is presented.

  • Motor neurone disease. Shaw, Pamela J // BMJ: British Medical Journal (International Edition);04/24/99, Vol. 318 Issue 7191, p1118 

    Discusses the motor neurone disease, one of the most common neurodegenerative diseases of adult onset. Incidence of the disease; Pathogenesis of motor neurone disease; Treatment.

  • Erratum to: Caregiver burden in amyotrophic lateral sclerosis: a cross-sectional investigation of predictors. Burke, Tom; Elamin, Marwa; Galvin, Miriam; Gallagher, Laura; Hardiman, Orla; Pender, Niall // Journal of Neurology;Dec2015, Vol. 262 Issue 12, p2799 

    A correction to the article "Caregiver Burden in Amyotrophic Lateral Sclerosis: A Cross-Sectional Investigation of Predictors," in a 2015 issue of the "Journal of Neurology" is presented.

  • Interpreting change scores on the Amyotrophic Lateral Sclerosis Assessment Questionnaire (ALSAQ-40). Jenkinson, C; Peto, V; Jones, G; Fitzpatrick, R // Clinical Rehabilitation;Jul2003, Vol. 17 Issue 4, p380 

    Objectives: To determine the amount of change over time on the five dimensions of the 40-item Amyotrophic Lateral Sclerosis Assessment Questionnaire (ALSAQ-40) that are subjectively meaningful to patients. Minimally important differences (MID), effect sizes (ES) and standardized response means...

Share

Read the Article

Courtesy of THE LIBRARY OF VIRGINIA

Sorry, but this item is not currently available from your library.

Try another library?
Sign out of this library

Other Topics