SciELO - Scientific Electronic Library Online

vol.53 issue4Von hippel-lindaus disease: Report of three cases and review of the literatureMyoclonic epilepsy of late onset in trisomy 21 author indexsubject indexarticles search
Home Pagealphabetic serial listing  

Services on Demand




Related links


Arquivos de Neuro-Psiquiatria

Print version ISSN 0004-282X


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.

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.

        · abstract in Portuguese     · text in Portuguese     · Portuguese ( pdf )


Creative Commons License All the contents of this journal, except where otherwise noted, is licensed under a Creative Commons Attribution License