RT Journal Article
SR Electronic
T1 Differentiating lower motor neuron syndromes
JF Journal of Neurology, Neurosurgery & Psychiatry
JO J Neurol Neurosurg Psychiatry
FD BMJ Publishing Group Ltd
SP 474
OP 483
DO 10.1136/jnnp-2016-313526
VO 88
IS 6
A1 Nidhi Garg
A1 Susanna B Park
A1 Steve Vucic
A1 Con Yiannikas
A1 Judy Spies
A1 James Howells
A1 William Huynh
A1 José M Matamala
A1 Arun V Krishnan
A1 John D Pollard
A1 David R Cornblath
A1 Mary M Reilly
A1 Matthew C Kiernan
YR 2017
UL http://jnnp.bmj.com/content/88/6/474.abstract
AB 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.