Extra-motor abnormalities in amyotrophic lateral sclerosis: another layer of heterogeneity

McCombe, P. A., Wray, N. R. and Henderson, R. D. (2017) Extra-motor abnormalities in amyotrophic lateral sclerosis: another layer of heterogeneity. Expert Review of Neurotherapeutics, 17 6: 561-577. doi:10.1080/14737175.2017.1273772


Author McCombe, P. A.
Wray, N. R.
Henderson, R. D.
Title Extra-motor abnormalities in amyotrophic lateral sclerosis: another layer of heterogeneity
Journal name Expert Review of Neurotherapeutics   Check publisher's open access policy
ISSN 1744-8360
1473-7175
Publication date 2017-06-01
Year available 2017
Sub-type Critical review of research, literature review, critical commentary
DOI 10.1080/14737175.2017.1273772
Open Access Status Not yet assessed
Volume 17
Issue 6
Start page 561
End page 577
Total pages 17
Place of publication Abingdon, Oxfordshire, United Kingdom
Publisher Taylor & Francis
Language eng
Formatted abstract
Introduction: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease defined by the presence of muscle weakness. The motor features of disease are heterogeneous in site of onset and progression. There are also extra-motor features in some patients. The genetic basis for extra-motor features is uncertain. The heterogeneity of ALS is an issue for clinical trials.

Areas covered: This paper reviews the range and prevalence of extra-motor features associated with ALS, and highlights the current information about genetic associations with extra-motor features.

Expert commentary: There are extra-motor features of ALS, but these are not found in all patients. The most common is cognitive abnormality. More data is required to ascertain whether extra-motor features arise with progression of disease. Extra-motor features are reported in patients with a range of causative genetic mutations, but are not found in all patients with these mutations. Further studies are required of the heterogeneity of ALS, and genotype/phenotype correlations are required, taking note of extra-motor features.
Keyword Amyotrophic lateral sclerosis
Autonomic disturbance
Cognitive impairmemt
Motor neuron disease
Sensory disturbance
Q-Index Code C1
Q-Index Status Provisional Code
Institutional Status UQ

Document type: Journal Article
Sub-type: Critical review of research, literature review, critical commentary
Collections: UQ Centre for Clinical Research Publications
HERDC Pre-Audit
Institute for Molecular Bioscience - Publications
Admin Only - UQCCR
 
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