Síndrome de Brown-Vialleto-Van Laere. Relato de dois casos

 

Brown-Vialetto-van Laere syndrome: report of two cases

 

 

José Teotonio De Oliveira; Paulo Roberto R. Moreira; Francisco Cardoso; Francisco Otaviano Lima Perpétuo

Serviço de Neurologia do Hospital das Clínicas da Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, MG

 

 

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RESUMO

A síndrome de Brown-Vialetto-van Laere é entidade degenerativa hereditária ou esporádica, rara, caracterizada por surdez neuro-sensorial seguida ou acompanhada por uma variedade de alterações de nervos cranianos, podendo ainda ocorrer acometimento de corno anterior e vias ópticas. Relatamos dois casos e discutimos o diagnóstico diferencial e a relação da síndrome com as atrofias espinhais e surdez hereditária.

Palavras-chave:síndrome de Brown-Vialetto-van Laere, paralisia ponto-bulbar com surdez, atrofia muscular espinhal, surdez hereditária.

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SUMMARY

Brown-Vialetto-van Laere syndrome is a rare hereditary or sporadic degenerative disorder characterised by progressive sensoryneural deafness, followed or accompanied by cranial nerve palsies. The anterior horn cells and the optic pathways may be involved in some cases. We report two cases, and comment the differential diagnosis and the relationships of this syndrome to the muscle spinal atrophies and the hereditary deafness.

Key-words: Brown-Vialetto-van Laere syndrome, ponto-bulbar palsy with deafness, muscle spinal atrophy, hereditary deafness.

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Aceite: 8-agosto-1995.

 

 

Dr. José Teotonio de Oliveira - Av. Pasteur 89 sala 1107 - 30150-290 Belo Horizonte MG - Brasil.