Síndrome de Brown-Vialleto-Van Laere: relato de dois casos

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

doi:10.1590/S0004-282X1995000500013

Resumos

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.

síndrome de Brown-Vialetto-van Laere; paralisia ponto-bulbar com surdez; atrofia muscular espinhal; surdez hereditária

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.

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

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

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.

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.

Texto completo disponível apenas em PDF.

Full text available only in PDF format.

Aceite: 8-agosto-1995.

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

1. Alberca R, Montero C, Ibafíez A, Segura Dl, Miranda-Nieves G. Progressive bulbar paralysis associated with neural deafness: a nosological entity. Arch Neurol 1980, 37: 214-216.
2. Brucher JM, Dom R, Lombaert A, Carton H. Progressive ponto-bulbar palsy with deafness: clinical pathological study of two cases. Arch Neurol 1981, 38: 186-190.
3. Francis DA, Ponsford JR, Wiles CM, Thomas PK, Duchen LW. Brown-Vialetto-van Laere syndrome. Neuropathol Appl Neurobiol 1993, 19:91-94.
4. Gallai V, Hockaday JM, Hughes JT, Lane DJ, Oppenheimer DR, Rushworth G. Ponto-bulbar palsy with deafness (Brown-Vialetto-van Laere syndrome): a report on three cases. J Neurol Sei 1981,50: 259-275.
5. Gourie-Devi M, Suresh TG. Madras pattern of motor neuron disease in South India. J Neurol Neurosurg Psychiatry 1988, 51:773-777.
6. Harding AE. Inherited neuronal atrophy and degeneration predominantly of lower motor neurons. In Dyck PJ (ed).Peripheral neuropathy. Ed3. Philadelphia: Saunders, 1993:1051-1065.
7. Hawkins SA, Nevin NC, Harding AE. Ponto bulbar palsy and neurosensory deafness (Brown-Vialetto-van Laere syndrome) with possible autosomal dominance inheritance. J Med Genet 1990,27:176-179.
8. Lombaert A, Dom R, Carton H, Brucher JM. Progressive ponto-bulbar palsy with deafness; a clinico-pathological study. Acta Neurol Belg 1976, 76:309-314.
9. Luxon LM. Diseases of the eigth cranial nerve. J In Dyck PJ. (ed) Peripheral neuropathy. Ed 3: Philadelphia: Saunders, 1993:837-868.
10. Piccolo G, Marchioni E, Maurelli M, Simonetti F, Bizzetti F, Savoldi F. Recovery from respiratory muscle failure in a sporadic case of Brown-Vialetto-van Laere syndrome with unusually late onset. J Neurol 1992, 239:355-356.
11. Rosemberg S, Lancellotti CLP, Arita F, Campos C. Progressive bulbar paralysis of childhood (Fazio-Londe disease) with deafness: case report with clinicopathologic correlation. Eur Neurol 1982, 21:84-89.

Datas de Publicação

Publicação nesta coleção
08 Dez 2010
Data do Fascículo
Dez 1995

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

[1] 1. Alberca R, Montero C, Ibafíez A, Segura Dl, Miranda-Nieves G. Progressive bulbar paralysis associated with neural deafness: a nosological entity. Arch Neurol 1980, 37: 214-216.

[2] 2. Brucher JM, Dom R, Lombaert A, Carton H. Progressive ponto-bulbar palsy with deafness: clinical pathological study of two cases. Arch Neurol 1981, 38: 186-190.

[3] 3. Francis DA, Ponsford JR, Wiles CM, Thomas PK, Duchen LW. Brown-Vialetto-van Laere syndrome. Neuropathol Appl Neurobiol 1993, 19:91-94.

[4] 4. Gallai V, Hockaday JM, Hughes JT, Lane DJ, Oppenheimer DR, Rushworth G. Ponto-bulbar palsy with deafness (Brown-Vialetto-van Laere syndrome): a report on three cases. J Neurol Sei 1981,50: 259-275.

[5] 5. Gourie-Devi M, Suresh TG. Madras pattern of motor neuron disease in South India. J Neurol Neurosurg Psychiatry 1988, 51:773-777.

[6] 6. Harding AE. Inherited neuronal atrophy and degeneration predominantly of lower motor neurons. In Dyck PJ (ed).Peripheral neuropathy. Ed3. Philadelphia: Saunders, 1993:1051-1065.

[7] 7. Hawkins SA, Nevin NC, Harding AE. Ponto bulbar palsy and neurosensory deafness (Brown-Vialetto-van Laere syndrome) with possible autosomal dominance inheritance. J Med Genet 1990,27:176-179.

[8] 8. Lombaert A, Dom R, Carton H, Brucher JM. Progressive ponto-bulbar palsy with deafness; a clinico-pathological study. Acta Neurol Belg 1976, 76:309-314.

[9] 9. Luxon LM. Diseases of the eigth cranial nerve. J In Dyck PJ. (ed) Peripheral neuropathy. Ed 3: Philadelphia: Saunders, 1993:837-868.

[10] 10. Piccolo G, Marchioni E, Maurelli M, Simonetti F, Bizzetti F, Savoldi F. Recovery from respiratory muscle failure in a sporadic case of Brown-Vialetto-van Laere syndrome with unusually late onset. J Neurol 1992, 239:355-356.

[11] 11. Rosemberg S, Lancellotti CLP, Arita F, Campos C. Progressive bulbar paralysis of childhood (Fazio-Londe disease) with deafness: case report with clinicopathologic correlation. Eur Neurol 1982, 21:84-89.