Document Detail


Peripheral neuropathy in CADASIL.
MedLine Citation:
PMID:  15827866     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
BACKGROUND: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL) is a hereditary cerebral microangiopathy associated with mutations in the Notch 3 gene. The clinical phenotype is characterized by cerebral impairment even though typical microvascular changes are diffuse. OBJECTIVE: To assess peripheral neuropathy in patients with CADASIL. PATIENTS AND METHODS: We enrolled eleven CADASIL patients with variable phenotype including clinical signs of peripheral nerve involvement. In all patients electromyography and nerve conduction velocities were performed. Peripheral nerve biopsy was performed in three cases. RESULTS: We found sensory motor neuropathy in 7/11 patients. Nerve biopsy revealed axonal and demyelinated findings. CONCLUSION: Our findings suggest that peripheral neuropathy may be part of the CADASIL phenotype.
Authors:
Francesco Sicurelli; Maria Teresa Dotti; Nicola De Stefano; Alessandro Malandrini; Mauro Mondelli; Silvia Bianchi; Antonio Federico
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Publication Detail:
Type:  Case Reports; Comparative Study; Journal Article     Date:  2005-04-15
Journal Detail:
Title:  Journal of neurology     Volume:  252     ISSN:  0340-5354     ISO Abbreviation:  J. Neurol.     Publication Date:  2005 Oct 
Date Detail:
Created Date:  2005-10-17     Completed Date:  2006-02-06     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0423161     Medline TA:  J Neurol     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  1206-9     Citation Subset:  IM    
Affiliation:
Dept. of Neurological and Behavioural Sciences, University of Siena, Viale Bracci 2, 53100 Siena, Italy.
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MeSH Terms
Descriptor/Qualifier:
Action Potentials
Adult
Aged
Axons / pathology
Biopsy
CADASIL / diagnosis,  pathology*
Case-Control Studies
Electromyography
Electrophysiology
Female
Humans
Male
Middle Aged
Muscle, Skeletal / innervation
Myelin Sheath / pathology
Nerve Fibers / pathology
Neural Conduction
Peripheral Nervous System Diseases / pathology*,  physiopathology,  surgery
Phenotype
Prospective Studies
Sural Nerve / pathology,  physiology,  surgery

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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