Clinical features and neurophysiological follow-up in a case of Brown-Vialetto-Van Laere syndrome.
Neuromuscul Disord
; 15(8): 565-8, 2005 Aug.
Article
em En
| MEDLINE
| ID: mdl-16009552
ABSTRACT
Brown-Vialetto-Van Laere syndrome is a rare disease of unknown origin commonly considered as part of the large group of motor neuron diseases. The course is quite variable it may be quickly fatal or protracted, with relapsing phases followed by periods of arrest and even partial improvement. We describe a case of Brown-Vialetto-Van Laere syndrome with strong family history for sensorineural hearing impairment. The patient came to our medical attention for severe respiratory failure and leg weakness. The clinical conditions partially improved with recovery of spontaneous respiration and mild increase in muscle strength. The neurophysiological studies performed on our patient showed evidence of nerve damage with subsequent improvement. Our study raises the possibility that the disorder is due to primary nerve damage, which can better justify the intermittent course of the disease, the partial clinical regression and the neurophysiological improvement, never detected in typical motor neuron disorders.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Doença dos Neurônios Motores
/
Doenças dos Nervos Cranianos
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Perda Auditiva Neurossensorial
Tipo de estudo:
Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
Limite:
Female
/
Humans
/
Middle aged
Idioma:
En
Ano de publicação:
2005
Tipo de documento:
Article