Neurofibromatose e doença arterial oclusiva intracraniana extensa (doença de Moya-Moya): registro de um caso

Gracia, C. M.; Bittencourt, P. C. T.; Mazer, S.; Bittencourt, P. R. M.

doi:10.1590/S0004-282X1986000400011

Resumos

O caso de urna paciente de 33 anos com neurofibromatose e lesões vasculares intracranianas do tipo Moya-Moya é discutido com relação a aspectos clínicos e angiográficos. Revisão da literatura mostra que esta rara associação produz lenta e progressiva deterioração intelectual e motora em adolescentes e adultos jovens. Não há linhas específicas de tratamento e o prognóstico é variável, alguns pacientes parecendo cessar de progredir na sua deterioração por pelo menos alguns anos.

A 33-year-old female with neurofibromatosis and intracranial vascular lesions of the Moya-Moya type is reported. Clinical and angiographic aspects of both syndromes are discussed with respect to earlier reports of this rare association, with the conclusion that it produces slow intelectual and motor deterioration in adolescents or young adults. There are no specific guidelines to therapy, and the prognosis is mixed, some patients ceasing to progress at least for a few years.

Neurofibromatose e doença arterial oclusiva intracraniana extensa (doença de Moya-Moya): registro de um caso

Neurofibromatosis with Moya-Moya disease: report of one case

C. M. Gracia^I; P. C. T. Bittencourt^II; S. Mazer^III; P. R. M. Bittencourt^I

^IUnidades de Neurologia Clínica

^IIe Disciplina de Neurologia do Hospital de Clínicas da Universidade Federal do Paraná

^IIIe Tomografia Axial Computadorizada do Hospital Nossa Senhora das Graças; Serviço de Angiografia

RESUMO

O caso de urna paciente de 33 anos com neurofibromatose e lesões vasculares intracranianas do tipo Moya-Moya é discutido com relação a aspectos clínicos e angiográficos. Revisão da literatura mostra que esta rara associação produz lenta e progressiva deterioração intelectual e motora em adolescentes e adultos jovens. Não há linhas específicas de tratamento e o prognóstico é variável, alguns pacientes parecendo cessar de progredir na sua deterioração por pelo menos alguns anos.

SUMMARY

A 33-year-old female with neurofibromatosis and intracranial vascular lesions of the Moya-Moya type is reported. Clinical and angiographic aspects of both syndromes are discussed with respect to earlier reports of this rare association, with the conclusion that it produces slow intelectual and motor deterioration in adolescents or young adults. There are no specific guidelines to therapy, and the prognosis is mixed, some patients ceasing to progress at least for a few years.

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Unidade de Neurologia Clinica, Hospital N.S. das Graças - Rum Alcides Munhoz, %3S -80510 - Curitiba, PR - Brasil.

1. CANALE, D.J. & BEBIN J. - Von Recklinghausen disease of nervous system. In P.J. Vinken & G.W. Bruin: Handbook of Clinical Neurology 14:132. North-Holland Publ. Co., Amsterdam, 1972.
2. ERICKSON, R.P.; WOOLLISCROFT, J. & ALLEN, R.J. - Familial occurrence of intracranial arterial occlusive disease (Moya-Moya) in neurofibromatosis. Clin. Genetics 18:191, 1980.
3. FYE, K.H.; JACOBS, R.P. & ROER, R.L. - Vascular manifestations of von Recklinghausen's disease. Western J. Med. 122:110, 1975.
4. GEBARSKI S.S.; GABRIELSEN, T.O.; KNAKE, J.E. & LATACK, J.T. - Posterior circulation intracranial arterial occlusive disease in neurofibromatosis. Amer. J-Neuroradiol. 4:1245, 1982.
5. HALONEN, H.; HALONEN, V.; DONNER, M.; IVANANEN, M.; VUOLIO, M. & MAKINEN, J. - Occlusive disease of intracranial main arteries with collateral networks in children. Neuropaediatrie 4:187, 1973.
6. HILAL, S.K.; SOLOMON, G.E.; GOLD, A.P. & CARTER, S. - Primary cerebral arterial occlusive disease in children. Radiology 99:87, 1971.
7. LAMAS, E.; LOBATO, E.D.; CABELLO, A. & ABAD, J.M. - Multiple intracranial arterial occlusive disease (Moya-Moya disease) in patient with neurofibromatosis-Acta neurochir. 45:133, 1978.
8. LEONE, R.G.; SCHATZKI, S.C. & WOLPW, E.R. - Neurofibromatosis with extensive intracranial arterial occlusive disease. Amer. J. Neuroradiol. 3:572, 1982.
9. SALYER, W.R. & SALYER, D.C. - The vascular lesion of neurofibromatosis-Angiology 25:510, 1974.
10. SASAKI, O.; ISHII, R.; KOIKE, T. & TANAKA, R. - Multiple cerebral occlusive disease associated with neurofibromatosis. No To Shinkei 36:159, 1984.
11. STOCKMAN, J.A.; NIGRO, M.A. & MISHKIN, M.M. - Occlusion of large vessels in sickle cell anemia. New Engl. J. Med. 287:846, 1972.
12. SUZUKI, J. & KODAMA, N. - Moya-Moya disease: a review. Stroke 14:104, 1983.
13. SUZUKI, J. & TAKAKU, A. - Cerebral vascular Moya-Moya disease: a disease showing abnormal net-like vessels in base of brain. Arch. Neurol. 20:288, 1969.
14. TABOADA, D.; AL.ONSO, A. & MORE, J. - Occlusion of the cerebral arteries in Recklinghausen's disease. Neuroradiology 18:281, 1979.
15. TOMSICK, T.A.; LUKIN, R.R.; CHAMBERS, A.A. & BENTON, C. - Neurofibromatosis and arterial occlusive disease. Neuroradiology 11:229, 1976.
16. VIGNES, B.; GUBERT, J.P.; MARTIN, G. & LABRUNE, B. - Maladie de Moya-Moya et maladie de Recklinghausen: a propos d'une observation. Ann. Péd. 21:469, 1973.
17. ZOCHOED, D. - Von Recklinghausen's vasculopathy. Amer. J. med. Sei. 287:64, 1984.

Datas de Publicação

Publicação nesta coleção
29 Jun 2011
Data do Fascículo
Dez 1986

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

[1] 1. CANALE, D.J. & BEBIN J. - Von Recklinghausen disease of nervous system. In P.J. Vinken & G.W. Bruin: Handbook of Clinical Neurology 14:132. North-Holland Publ. Co., Amsterdam, 1972.

[2] 2. ERICKSON, R.P.; WOOLLISCROFT, J. & ALLEN, R.J. - Familial occurrence of intracranial arterial occlusive disease (Moya-Moya) in neurofibromatosis. Clin. Genetics 18:191, 1980.

[3] 3. FYE, K.H.; JACOBS, R.P. & ROER, R.L. - Vascular manifestations of von Recklinghausen's disease. Western J. Med. 122:110, 1975.

[4] 4. GEBARSKI S.S.; GABRIELSEN, T.O.; KNAKE, J.E. & LATACK, J.T. - Posterior circulation intracranial arterial occlusive disease in neurofibromatosis. Amer. J-Neuroradiol. 4:1245, 1982.

[5] 5. HALONEN, H.; HALONEN, V.; DONNER, M.; IVANANEN, M.; VUOLIO, M. & MAKINEN, J. - Occlusive disease of intracranial main arteries with collateral networks in children. Neuropaediatrie 4:187, 1973.

[6] 6. HILAL, S.K.; SOLOMON, G.E.; GOLD, A.P. & CARTER, S. - Primary cerebral arterial occlusive disease in children. Radiology 99:87, 1971.

[7] 7. LAMAS, E.; LOBATO, E.D.; CABELLO, A. & ABAD, J.M. - Multiple intracranial arterial occlusive disease (Moya-Moya disease) in patient with neurofibromatosis-Acta neurochir. 45:133, 1978.

[8] 8. LEONE, R.G.; SCHATZKI, S.C. & WOLPW, E.R. - Neurofibromatosis with extensive intracranial arterial occlusive disease. Amer. J. Neuroradiol. 3:572, 1982.

[9] 9. SALYER, W.R. & SALYER, D.C. - The vascular lesion of neurofibromatosis-Angiology 25:510, 1974.

[10] 10. SASAKI, O.; ISHII, R.; KOIKE, T. & TANAKA, R. - Multiple cerebral occlusive disease associated with neurofibromatosis. No To Shinkei 36:159, 1984.

[11] 11. STOCKMAN, J.A.; NIGRO, M.A. & MISHKIN, M.M. - Occlusion of large vessels in sickle cell anemia. New Engl. J. Med. 287:846, 1972.

[12] 12. SUZUKI, J. & KODAMA, N. - Moya-Moya disease: a review. Stroke 14:104, 1983.

[13] 13. SUZUKI, J. & TAKAKU, A. - Cerebral vascular Moya-Moya disease: a disease showing abnormal net-like vessels in base of brain. Arch. Neurol. 20:288, 1969.

[14] 14. TABOADA, D.; AL.ONSO, A. & MORE, J. - Occlusion of the cerebral arteries in Recklinghausen's disease. Neuroradiology 18:281, 1979.

[15] 15. TOMSICK, T.A.; LUKIN, R.R.; CHAMBERS, A.A. & BENTON, C. - Neurofibromatosis and arterial occlusive disease. Neuroradiology 11:229, 1976.

[16] 16. VIGNES, B.; GUBERT, J.P.; MARTIN, G. & LABRUNE, B. - Maladie de Moya-Moya et maladie de Recklinghausen: a propos d'une observation. Ann. Péd. 21:469, 1973.

[17] 17. ZOCHOED, D. - Von Recklinghausen's vasculopathy. Amer. J. med. Sei. 287:64, 1984.