| Clinical and pathological heterogeneity in a series of 31 patients with IgM-related neuropathy. | |
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MedLine Citation:
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PMID: 22632783 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
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BACKGROUND: IgM-related neuropathy generally presents as a late-onset demyelinating polyneuropathy with predominant sensory loss and ataxia. Sporadic cases with atypical presentation have been described. PATIENTS AND METHODS: We report clinical and pathological findings from 31 patients with IgM-related neuropathy followed in our Institute of Neurology over a 20-year period. RESULTS: Typical presentation with predominant sensory ataxic neuropathy was observed in 18/31 patients. In the remaining 13/31 (42%), we observed an atypical phenotype, characterized by multiple mononeuropathy or polyneuropathy with predominant motor impairment; one patient had polyneuropathy with predominant small-fibre involvement. Uncommon pathological findings consisting in inflammatory infiltrates, focal axonal loss or light chain deposition were observed in 8 patients, all with atypical clinical phenotype. Almost all patients with atypical phenotype improved with immunosuppressive therapy. CONCLUSIONS: A significant proportion of patients with IgM-related neuropathy presents with atypical clinical features. In these patients, sural nerve biopsy helps clarify heterogeneous disease mechanisms and identify patients who might benefit from immunosuppressive therapy. |
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Authors:
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M Luigetti; A Conte; N Montano; A Del Grande; F Madia; M Lo Monaco; L Laurenti; M Sabatelli |
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Publication Detail:
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Type: JOURNAL ARTICLE Date: 2012-5-24 |
Journal Detail:
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Title: Journal of the neurological sciences Volume: - ISSN: 1878-5883 ISO Abbreviation: - Publication Date: 2012 May |
Date Detail:
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Created Date: 2012-5-28 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 0375403 Medline TA: J Neurol Sci Country: - |
Other Details:
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Languages: ENG Pagination: - Citation Subset: - |
Copyright Information:
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Copyright © 2012 Elsevier B.V. All rights reserved. |
Affiliation:
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Institute of Neurology, Catholic University of the Sacred Heart, Rome, Italy. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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