Esclerose lateral amiotrófica com surdez: relato de um caso e revisão da literatura

Amorim, Annibal Coelho de; Mattos, James Pitagoras de; Lima, Jose Mauro Braz de; Aterino Filho, Cristovão; Lima, Ana Luisa Xavier de

doi:10.1590/S0004-282X1982000200012

Resumos

É relatado um caso de paralisia bulbo-pontina crônica progressiva com surdez, de início precoce, evoluindo de maneira protraída há 9 anos. Pelos achados histopatológicos e eletromiográficos concluem tratar-se de variante da forma juvenil de esclerose lateral amiotrófica, forma de Van Laere.

A case of chronic bulbe-pontine paralysis with deafness, with early onset, coursing along nine years is reported. There seem to be histopathologic and electromiographic evidences on which is concluded to be a variant form of juvenile amyotrophic lateral sclerosis (Van Laere form).

Esclerose lateral amiotrófica com surdez: relato de um caso e revisão da literatura

Amyotrophic lateral sclerosis with deafness (Van Laere form): a case report

Annibal Coelho De Amorim^I; James Pitagoras de Mattos^II; Jose Mauro Braz de Lima^II; Cristovão Aterino Filho^III; Ana Luisa Xavier de Lima^I

^IMédicos residentes. Instituto de Neurologia Deolindo Couto da U.F.R.J. (Serviço do Prof. Bernardo Couto)

^IIProfessores Assistentes. Instituto de Neurologia Deolindo Couto da U.F.R.J. (Serviço do Prof. Bernardo Couto)

^IIIOtorrinolaringologlsta. Instituto de Neurologia Deolindo Couto da U.F.R.J. (Serviço do Prof. Bernardo Couto)

RESUMO

É relatado um caso de paralisia bulbo-pontina crônica progressiva com surdez, de início precoce, evoluindo de maneira protraída há 9 anos. Pelos achados histopatológicos e eletromiográficos concluem tratar-se de variante da forma juvenil de esclerose lateral amiotrófica, forma de Van Laere.

SUMMARY

A case of chronic bulbe-pontine paralysis with deafness, with early onset, coursing along nine years is reported. There seem to be histopathologic and electromiographic evidences on which is concluded to be a variant form of juvenile amyotrophic lateral sclerosis (Van Laere form).

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Trabalho realizado no Instituto de Neurologia Deolindo Couto da U.F.R.J. (Serviço do Prof. Bernardo Couto).

Instituto de Neurologia Deolindo Couto da U.F.R.J. - Av. Venceslau Bras 95 - 22290 Rio de Janeiro, RJ - Brasil.

1. ADAMS, R. D. & VICTOR, M. - Diseases of the periphereal and cranial nerves; Viral Infections of Nervous System. In Principles of Neurology - McGraw Hill Book Company, New York, 1977; pags. 654-818-841.
2. ANTEL, J. P.; RICHMAN; D. P. & ARNARSOW, B. G. W. - Immunogenetics and amyotrophic lateral sclerosis. In Conference on Research Trends in Amyotrophic Lateral Sclerosis - United Kingdom Edition - Academic Press, New York, 1975, pag. 159.
3. ARNOLD, G.; TRIDON, D.; LASENAIRE, M.; P. CARLOS L.; WEBER, M. & BRICHET, B. - A propos d'une observation de sindrome de Fazio-Londe. In Revne d'Oto-neuro-ophtalm 40 (Paris), 1968, 158.
4. BARSY, A. M. & MOUCHETETTE, R. - Sur la sclerose lateral amyotrophique infantile sporadique. In L'Encephale 56, (Paris) 1967, 45.
5. BECKER; P. E. & KOCH, E. - Esclerose lateral amiotrófica com surdez e paralisia bulbar progressiva infantil. In Genética Humana - vol. I, Georg Thieme Verlay, Stuttgart, Ed. Toray S/A (Barcelona), 1969, pag. 415.
6. BOUDIN, M. M.; PEPIN, B. & VERNANT, J. C. - Cas familial de paralysie bulbo-pontine chronique progressive avec surdité. in Rev. Neurol. (Paris). 124, 1971, pag. 90.
7. BRAZ DE LIMA, J. M. - Contribuição para o estudo da esclerose lateral amiotrófica: aspectos clínicos, epidemiológicos e virológicos. Tese, (Rio de Janeiro), 1979.
8. ESPINOSA, R. E.; OKIHIRO, M. M.; MERDER, D. M. & SAYRE, E. P. -Hereditary amyotrophic lateral sclerosis. Neurology (Minneapolis) 12, 1962, 1.
9. FREITAS, M. R. G.; NASCIMENTO, O. J. M. & ARAUJO, E. - Esclerose lateral amiotrófica com surdez. In Anais do VI Congresso Brasileiro de Neurologia, (Rio de Janeiro), 1974, pag. 118.
10. FRIEDMAN, A. P. & FREEDMAN, D. - Amyotrophic lateral sclerosis. In J. Ment. Dis. (U.S.A.) (Ill), 1950, pag. 1.
11. GOMEZ, M. R, - Progressive bulbar paralysis of childhood: Fazio-Londe disease - In Vinken, J. p.; Bryn, G. W. handbook of Clinical Neurology, vol. 27, N. Holland Publishing (Amsterdan), 1975, pag. 103.
12. GOMEZ, M. R.; CHERMONT, V. & BERNSTEIN, J. - Progressive bulbar paralysis in childhood (Fazio-Londe disease): report of a case with pathologic evidence of nuclear atrophy. In Arch Neurol. (Chicago) (6), 1962, 317.
13. GUILLAIN, G. - Jean Martin Charcot (1825-1893). His life, his work - In Eds. Pearce Bailey. P. B. Haeber (cap. 5), 1898, 106.
14. JOHNSON, R. T. - Virological studies of amyotrophic lateral sclerosis - In Conference on Research Trends in Amyotrophic Lateral Sclerosis. United Kingdom Edition, Academic Press, (New York), 1975, pag. 173.
15. KONIGSMARK, B. C. - Hereditary disease of the nervous system with hearing loss. In Vinken J. P. & Bryn, G. W., eds. Handbook of Clinical Neurology (Amsterdam) vol. 22, North Holland Publishing, 1975, pag. 499.
16. KURLAND, G. & BRODY, J. A. - Amyotrophic lateral sclerosis. In Vinken, J. P. & Bryn, G. W. - Handbook of Clinical Neurology, Vol. 22. North Holland Publishing Co, Amsterdan, 1975, pag. 339.
17. MARKAND, O. N. & DALY, D. D. - Juvenile type of slowly progressive bulbar palsy: report of a case. In Neurology (Minneapolis), 1971, 753.
18. MARINESCO, G. - Maladies des Muscles. In Nouveau Traité de Medicine et de Therapeutique, vol. 38, Baillière et Fils, Paris, 1910.
19. MERRIT, H. H. - Amyotrophic lateral sclerosis. In Handbook of Neurology. Oth. Ed., Philadelphia, 1974, pag. 549.
20. NASCIMENTO, O. J. M. - Heredo amiotrofia medular progressiva - Tese, Rio de Janeiro, 1979.
21. ROSE, C. F. - Amyotrophic lateral sclerosis. In Motor Neurone Disease, Harcourt B. Jovanovich, Publishers Grune & Stratton (New York), 1978.
22. ROGER, M. M. J. & PACHE, D. Mme. - A propos des formes lentes de la sclerose lateral amyotrophique: bilan clinique d'un cas de S.L.A. évoluant depuis douze ans. In Rev. Neurol. (Paris), 100, 1959, 234.
23. SZTAJNBERG, M. C. - Doença de Fazio-Londe: aspectos clínicos. Tese, Rio de Janeiro, 1974.
24. STTAL, A. & WENT, L. N. - Juvenile amyotrophic lateral sclerosis: dementia complex in a Dutch family. In Neurology (Minneapolis) 18, 1968, 800.
25. TRILLET, M. M.; GIRARD, P. F.; SCHOTT, B.; RAMEL, P. & WOEHERLE, R. - La paralysie bulbo-pontine chronique progressive avec surdité: a propos d'une observation clinique. In Lyon Med. (223), 1970, 145.
26. VAN LAERE, M. J. - Paralysie bulbo-pontine chronique progressive familiale avec surdité: un cas de sindrome de Klippel-Trénaunay dans la même famile: problemes diagnostique et genetiques. In Rev. Neurol. (Paris) 115, 1966, 289.
27. VEIT, H. - Dynamic view of amyotrophic lateral sclerosis. In J. Nervous and Mental Dis., 106, 1947, 129.
28. VIOLA, M. V. - RNA tumor viruses as causative agents of chronic neurological disease. In Conference on Research Trends in Amyotrophic Lateral Sclerosis. United Kingdon Edition Academic Press, (New York) 1975, pag. 235.

Datas de Publicação

Publicação nesta coleção
20 Ago 2012
Data do Fascículo
Jun 1982

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

[1] 1. ADAMS, R. D. & VICTOR, M. - Diseases of the periphereal and cranial nerves; Viral Infections of Nervous System. In Principles of Neurology - McGraw Hill Book Company, New York, 1977; pags. 654-818-841.

[2] 2. ANTEL, J. P.; RICHMAN; D. P. & ARNARSOW, B. G. W. - Immunogenetics and amyotrophic lateral sclerosis. In Conference on Research Trends in Amyotrophic Lateral Sclerosis - United Kingdom Edition - Academic Press, New York, 1975, pag. 159.

[3] 3. ARNOLD, G.; TRIDON, D.; LASENAIRE, M.; P. CARLOS L.; WEBER, M. & BRICHET, B. - A propos d'une observation de sindrome de Fazio-Londe. In Revne d'Oto-neuro-ophtalm 40 (Paris), 1968, 158.

[4] 4. BARSY, A. M. & MOUCHETETTE, R. - Sur la sclerose lateral amyotrophique infantile sporadique. In L'Encephale 56, (Paris) 1967, 45.

[5] 5. BECKER; P. E. & KOCH, E. - Esclerose lateral amiotrófica com surdez e paralisia bulbar progressiva infantil. In Genética Humana - vol. I, Georg Thieme Verlay, Stuttgart, Ed. Toray S/A (Barcelona), 1969, pag. 415.

[6] 6. BOUDIN, M. M.; PEPIN, B. & VERNANT, J. C. - Cas familial de paralysie bulbo-pontine chronique progressive avec surdité. in Rev. Neurol. (Paris). 124, 1971, pag. 90.

[7] 7. BRAZ DE LIMA, J. M. - Contribuição para o estudo da esclerose lateral amiotrófica: aspectos clínicos, epidemiológicos e virológicos. Tese, (Rio de Janeiro), 1979.

[8] 8. ESPINOSA, R. E.; OKIHIRO, M. M.; MERDER, D. M. & SAYRE, E. P. -Hereditary amyotrophic lateral sclerosis. Neurology (Minneapolis) 12, 1962, 1.

[9] 9. FREITAS, M. R. G.; NASCIMENTO, O. J. M. & ARAUJO, E. - Esclerose lateral amiotrófica com surdez. In Anais do VI Congresso Brasileiro de Neurologia, (Rio de Janeiro), 1974, pag. 118.

[10] 10. FRIEDMAN, A. P. & FREEDMAN, D. - Amyotrophic lateral sclerosis. In J. Ment. Dis. (U.S.A.) (Ill), 1950, pag. 1.

[11] 11. GOMEZ, M. R, - Progressive bulbar paralysis of childhood: Fazio-Londe disease - In Vinken, J. p.; Bryn, G. W. handbook of Clinical Neurology, vol. 27, N. Holland Publishing (Amsterdan), 1975, pag. 103.

[12] 12. GOMEZ, M. R.; CHERMONT, V. & BERNSTEIN, J. - Progressive bulbar paralysis in childhood (Fazio-Londe disease): report of a case with pathologic evidence of nuclear atrophy. In Arch Neurol. (Chicago) (6), 1962, 317.

[13] 13. GUILLAIN, G. - Jean Martin Charcot (1825-1893). His life, his work - In Eds. Pearce Bailey. P. B. Haeber (cap. 5), 1898, 106.

[14] 14. JOHNSON, R. T. - Virological studies of amyotrophic lateral sclerosis - In Conference on Research Trends in Amyotrophic Lateral Sclerosis. United Kingdom Edition, Academic Press, (New York), 1975, pag. 173.

[15] 15. KONIGSMARK, B. C. - Hereditary disease of the nervous system with hearing loss. In Vinken J. P. & Bryn, G. W., eds. Handbook of Clinical Neurology (Amsterdam) vol. 22, North Holland Publishing, 1975, pag. 499.

[16] 16. KURLAND, G. & BRODY, J. A. - Amyotrophic lateral sclerosis. In Vinken, J. P. & Bryn, G. W. - Handbook of Clinical Neurology, Vol. 22. North Holland Publishing Co, Amsterdan, 1975, pag. 339.

[17] 17. MARKAND, O. N. & DALY, D. D. - Juvenile type of slowly progressive bulbar palsy: report of a case. In Neurology (Minneapolis), 1971, 753.

[18] 18. MARINESCO, G. - Maladies des Muscles. In Nouveau Traité de Medicine et de Therapeutique, vol. 38, Baillière et Fils, Paris, 1910.

[19] 19. MERRIT, H. H. - Amyotrophic lateral sclerosis. In Handbook of Neurology. Oth. Ed., Philadelphia, 1974, pag. 549.

[20] 20. NASCIMENTO, O. J. M. - Heredo amiotrofia medular progressiva - Tese, Rio de Janeiro, 1979.

[21] 21. ROSE, C. F. - Amyotrophic lateral sclerosis. In Motor Neurone Disease, Harcourt B. Jovanovich, Publishers Grune & Stratton (New York), 1978.

[22] 22. ROGER, M. M. J. & PACHE, D. Mme. - A propos des formes lentes de la sclerose lateral amyotrophique: bilan clinique d'un cas de S.L.A. évoluant depuis douze ans. In Rev. Neurol. (Paris), 100, 1959, 234.

[23] 23. SZTAJNBERG, M. C. - Doença de Fazio-Londe: aspectos clínicos. Tese, Rio de Janeiro, 1974.

[24] 24. STTAL, A. & WENT, L. N. - Juvenile amyotrophic lateral sclerosis: dementia complex in a Dutch family. In Neurology (Minneapolis) 18, 1968, 800.

[25] 25. TRILLET, M. M.; GIRARD, P. F.; SCHOTT, B.; RAMEL, P. & WOEHERLE, R. - La paralysie bulbo-pontine chronique progressive avec surdité: a propos d'une observation clinique. In Lyon Med. (223), 1970, 145.

[26] 26. VAN LAERE, M. J. - Paralysie bulbo-pontine chronique progressive familiale avec surdité: un cas de sindrome de Klippel-Trénaunay dans la même famile: problemes diagnostique et genetiques. In Rev. Neurol. (Paris) 115, 1966, 289.

[27] 27. VEIT, H. - Dynamic view of amyotrophic lateral sclerosis. In J. Nervous and Mental Dis., 106, 1947, 129.

[28] 28. VIOLA, M. V. - RNA tumor viruses as causative agents of chronic neurological disease. In Conference on Research Trends in Amyotrophic Lateral Sclerosis. United Kingdon Edition Academic Press, (New York) 1975, pag. 235.