Document Detail

Monomelic neurogenic syndromes: a prospective study.
MedLine Citation:
PMID:  17610902     Owner:  NLM     Status:  MEDLINE    
Monomelic neurogenic syndromes are rare. Their classification and prognostic features have not been addressed in the European population. A prospective study of 17 patients with monomelic neurogenic amyotrophy, of upper or lower limb onset, with progression limited to one limb for three or more years. Clinical and neurophysiological studies were performed in the subsequent 3 or more years. Fifteen patients were of European origin and two were Asian. Those presenting with proximal monomelic weakness or with involvement of the posterior compartment of the lower leg showed no further progression after the initial period of development of the syndrome. Brisk reflexes in wasted muscles did not predict progression. Electromyographic signs of denervation in the opposite limb at presentation did not predict later progression. Transcranial magnetic stimulation (TMS) features of corticospinal dysfunction were a useful predictor of subsequent progression (p=0.01). One patient with lower limb onset developed conduction block with weakness in an upper limb nine years after presentation, and this upper limb weakness responded to IVIg therapy. This adult-onset European group of patients is different as compared with juvenile-onset Asian cases. The clinical syndromes appear heterogeneous, but neurophysiological investigations, in particular TMS, can be helpful in determining prognosis. Multifocal motor neuropathy should be considered when there is progression, even years after onset.
Mamede de Carvalho; Michael Swash
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2007-07-03
Journal Detail:
Title:  Journal of the neurological sciences     Volume:  263     ISSN:  0022-510X     ISO Abbreviation:  J. Neurol. Sci.     Publication Date:  2007 Dec 
Date Detail:
Created Date:  2007-11-06     Completed Date:  2008-02-14     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0375403     Medline TA:  J Neurol Sci     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  26-34     Citation Subset:  IM    
Department of Neurology, St Maria Hospital, Institute of Molecular Medicine, Faculty of Medicine, Lisbon, Portugal.
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MeSH Terms
Disease Progression
Electromyography / methods
Extremities / physiopathology
Middle Aged
Motor Neuron Disease / diagnosis*,  physiopathology*
Neuromuscular Diseases / diagnosis*,  physiopathology*
Prospective Studies
Transcranial Magnetic Stimulation

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