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Differentiating lower motor neuron syndromes.

Authors :
Garg N
Park SB
Vucic S
Yiannikas C
Spies J
Howells J
Huynh W
Matamala JM
Krishnan AV
Pollard JD
Cornblath DR
Reilly MM
Kiernan MC
Source :
Journal of neurology, neurosurgery, and psychiatry [J Neurol Neurosurg Psychiatry] 2017 Jun; Vol. 88 (6), pp. 474-483. Date of Electronic Publication: 2016 Dec 21.
Publication Year :
2017

Abstract

Lower motor neuron (LMN) syndromes typically present with muscle wasting and weakness and may arise from pathology affecting the distal motor nerve up to the level of the anterior horn cell. A variety of hereditary causes are recognised, including spinal muscular atrophy, distal hereditary motor neuropathy and LMN variants of familial motor neuron disease. Recent genetic advances have resulted in the identification of a variety of disease-causing mutations. Immune-mediated disorders, including multifocal motor neuropathy and variants of chronic inflammatory demyelinating polyneuropathy, account for a proportion of LMN presentations and are important to recognise, as effective treatments are available. The present review will outline the spectrum of LMN syndromes that may develop in adulthood and provide a framework for the clinician assessing a patient presenting with predominantly LMN features.<br />Competing Interests: Competing interests: None declared.<br /> (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.)

Details

Language :
English
ISSN :
1468-330X
Volume :
88
Issue :
6
Database :
MEDLINE
Journal :
Journal of neurology, neurosurgery, and psychiatry
Publication Type :
Academic Journal
Accession number :
28003344
Full Text :
https://doi.org/10.1136/jnnp-2016-313526