Peripheral neuropathy in CADASIL.
J Neurol
; 252(10): 1206-9, 2005 Oct.
Article
in En
| MEDLINE
| ID: mdl-15827866
ABSTRACT
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 ANDMETHODS:
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.
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Collection:
01-internacional
Database:
MEDLINE
Main subject:
Peripheral Nervous System Diseases
/
CADASIL
Type of study:
Diagnostic_studies
/
Observational_studies
/
Risk_factors_studies
Limits:
Adult
/
Aged
/
Female
/
Humans
/
Male
/
Middle aged
Language:
En
Journal:
J Neurol
Year:
2005
Document type:
Article
Affiliation country:
Italy