Document Detail


Neuropathy in Wolfram syndrome.
MedLine Citation:
PMID:  20888932     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
Wolfram syndrome (WFS) is a degenerative disease with neurological and endocrine disorders, characterized by the association of juvenile diabetes mellitus and bilateral optic atrophy. A polyneuropathy was exceptionally described but its characteristics are not well-established. In addition to our observation, we searched all case reports of patients with WFS in the medical literature (more than 600), and selected patients who underwent an EMG: twenty-one patients underwent an EMG, which was considered as abnormal in only 8 cases. The common profile was axonal sensory-motor polyneuropathy, sometimes with marked decrease of motor conduction velocities. This neuropathy could be due to diabetes mellitus, even though microangiopathic and macroangiopathic complications are rare in WFS. Another origin for this neuropathy could be a degenerative process in relationship with WFS.
Authors:
Stéphane Mathis; Thierry Maisonobe; Jean-Philippe Neau
Publication Detail:
Type:  Journal Article     Date:  2010-10-01
Journal Detail:
Title:  European journal of medical genetics     Volume:  54     ISSN:  1878-0849     ISO Abbreviation:  Eur J Med Genet     Publication Date:    2011 Jan-Feb
Date Detail:
Created Date:  2011-02-28     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101247089     Medline TA:  Eur J Med Genet     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  73-5     Citation Subset:  IM    
Copyright Information:
Copyright © 2010 Elsevier Masson SAS. All rights reserved.
Affiliation:
Université de Poitiers, CHU de Poitiers, Clinique Neurologique, 2 rue de la Milétrie, 86021 Poitiers Cedex 05, France.
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