Document Detail


Clinical and electrophysiologic features of oculopharyngeal muscular dystrophy: lack of evidence for an associated peripheral neuropathy.
MedLine Citation:
PMID:  20181517     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
OBJECTIVE: To describe the clinical and electrophysiologic features of an unselected population of patients diagnosed clinically or genetically with oculopharyngeal muscular dystrophy (OPMD), and to discern any association with a peripheral neuropathy. METHODS: Patients with a clinical or genetic diagnosis of OPMD were retrospectively identified and characterized in terms of clinical and electrophysiologic features. RESULTS: Fourteen patients who met the design criteria were identified and included. All had progressive ptosis and dysphagia, and most had ophthalmoparesis and proximal limb weakness. The electromyographic findings were similar to findings in other dystrophic diseases. Nine out of 10 patients had normal sural sensory nerve action potentials (mean amplitude 14.2 muV, range 0-22 muV). CONCLUSIONS: The electrophysiologic findings associated with OPMD are similar to changes noted in other dystrophic diseases. These findings argue against an association between OPMD and peripheral neuropathy. SIGNIFICANCE: OPMD may not be associated with a peripheral neuropathy, as has been previously suggested.
Authors:
Lyell K Jones; C Michel Harper
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Publication Detail:
Type:  Journal Article     Date:  2010-02-23
Journal Detail:
Title:  Clinical neurophysiology : official journal of the International Federation of Clinical Neurophysiology     Volume:  121     ISSN:  1872-8952     ISO Abbreviation:  Clin Neurophysiol     Publication Date:  2010 Jun 
Date Detail:
Created Date:  2010-05-24     Completed Date:  2010-06-28     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  100883319     Medline TA:  Clin Neurophysiol     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  870-3     Citation Subset:  IM    
Copyright Information:
Copyright 2010 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.
Affiliation:
Department of Neurology, Mayo Clinic, Rochester, MN, USA. lyell@mayo.edu
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MeSH Terms
Descriptor/Qualifier:
Adult
Aged
Electromyography
Female
Humans
Male
Middle Aged
Muscle Weakness / complications,  physiopathology
Muscle, Skeletal / physiopathology*
Muscular Dystrophy, Oculopharyngeal / complications*,  diagnosis,  physiopathology
Neural Conduction / physiology*
Patient Selection
Peripheral Nerves / physiopathology*
Peripheral Nervous System Diseases / complications*,  physiopathology
Retrospective Studies
Comments/Corrections
Comment In:
Clin Neurophysiol. 2010 Jun;121(6):803-4   [PMID:  20194049 ]

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