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Arquivos de Neuro-Psiquiatria

Print version ISSN 0004-282X

Abstract

OLIVEIRA, José Teotonio De; MOREIRA, Paulo Roberto R.; CARDOSO, Francisco  and  PERPETUO, Francisco Otaviano Lima. Brown-Vialetto-van Laere syndrome: report of two cases. Arq. Neuro-Psiquiatr. [online]. 1995, vol.53, n.4, pp.789-791. ISSN 0004-282X.  https://doi.org/10.1590/S0004-282X1995000500013.

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.

Keywords : Brown-Vialetto-van Laere syndrome; ponto-bulbar palsy with deafness; muscle spinal atrophy; hereditary deafness.

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