Uveomeningencefalites: registro de dois casos

Sanvito, Wilson Luiz; Tilbery, Charles Peter; Villares, João C. B Arrison

doi:10.1590/S0004-282X1982000100009

Resumos

Após considerações a propósito das manifestações clínicas das uveomeningencefalites, são relatados dois casos com características clínicas ligeiramente diferentes. No caso 1 tratava-se da doença de Harada, tendo o paciente apresentado quadro meningítico agudo acompanhado de confusão mental. O exame do líquido cefalorraquidiano evidenciou um quadro inflamatório do tipo linfo-monocitário e o paciente evoluiu com distúrbios acentuados da memória e uveíte bilateral. Após alguns meses de evolução, a doença deixou como seqüelas uma síndrome de Korsakoff e ambliopia acentuada no olho esquerdo. No caso 2 tratava-se de doença de Behcet, tendo o paciente apresentado dois surtos de meningite do tipo linfomonocitário, lesões herpetiformes na genitalia e uveíte no olho direito que evoluiu para amaurose. São feitas considerações sobre os aspectos etiopatogênicos destas afecções e sobre o controvertido uso dos corticosteróides em seu tratamento.

The uveomeningoencephalitic syndromes are inflamatory diseases, more prevalent in oriental patients, without sexual predilection and with mean age of 30 years. From the clinical point of view they affect the uvea, retina, meninges, the central nervous system and skin and in most cases the onset is followed by three phases: the meningoencephalitic phase, the acute ophthalmic phase and the dermatologic phase. These includes the Harada disease, the Vogt-Koyanagi disease, the Behcet disease and the idiopathic forms. The Vogt-Koyanagi syndrome is a chronic and severa bilateral exudative uveitis associated with whitening of the hair and eyelashes and varying signs of meningeal irritation, less frequent than in Harada's disease. The Harada syndrome is a posterior uveitis accompanied by signs of meningeal irritation, increased protein levels and pleocytosis of the cerebrospinal fluid. The Behcet disease is a relapsing illness, characterized by oral and genital aphtous ulcers and ocular inflamation. In many cases there are additional features; in 10 to 25 percent of the cases there are neurologic involvement, and any portion of the nervous system may be affected. The authors studied two cases of uveome-ningoencephalic syndrome, one case of Vogt-Koyanagi-Harada disease (case 1) and another of Behcet disease (case 2). The case 1 was a white Brazilian, forty-five year-old man, with a acute headache, mental confusion and signs of meningeal irritation. The cerebrospinal fluid was a inflamatory one, with increased lymphocytes and monocytes, and one month after the patient developed bilateral uveit. The patient complicated with amblyopia of the left eye and a Korsakoff syndrome. There was no correlation in the literature of the Korsakoff syndrome and the uveomeningoencephalitic syndrome. The case 2 was a white Brazilian, twenty-four year-old man, with two episodes of meningitis, genital recurrent aphtous ulcers and uveitis in the right eye, with latter blindness of this eye. Some considerations about the etiopathogenic aspects are done and about the controversial use of corticoids in the treatment of these syndromes.

Uveomeningencefalites: registro de dois casos

Uveomeningoencephalitic syndromes: report of two cases

Wilson Luiz Sanvito^I; Charles Peter Tilbery^II; João C. B Arrison Villares^III

^IProf. Pleno. Departamento de Medicina da Faculdade de Ciências Médicas da Santa Casa de São Paulo

^IIProf. Assistente. Departamento de Medicina da Faculdade de Ciências Médicas da Santa Casa de São Paulo

^IIIEx-Residente. Departamento de Medicina da Faculdade de Ciências Médicas da Santa Casa de São Paulo

RESUMO

Após considerações a propósito das manifestações clínicas das uveomeningencefalites, são relatados dois casos com características clínicas ligeiramente diferentes. No caso 1 tratava-se da doença de Harada, tendo o paciente apresentado quadro meningítico agudo acompanhado de confusão mental. O exame do líquido cefalorraquidiano evidenciou um quadro inflamatório do tipo linfo-monocitário e o paciente evoluiu com distúrbios acentuados da memória e uveíte bilateral. Após alguns meses de evolução, a doença deixou como seqüelas uma síndrome de Korsakoff e ambliopia acentuada no olho esquerdo. No caso 2 tratava-se de doença de Behcet, tendo o paciente apresentado dois surtos de meningite do tipo linfomonocitário, lesões herpetiformes na genitalia e uveíte no olho direito que evoluiu para amaurose. São feitas considerações sobre os aspectos etiopatogênicos destas afecções e sobre o controvertido uso dos corticosteróides em seu tratamento.

SUMMARY

The uveomeningoencephalitic syndromes are inflamatory diseases, more prevalent in oriental patients, without sexual predilection and with mean age of 30 years. From the clinical point of view they affect the uvea, retina, meninges, the central nervous system and skin and in most cases the onset is followed by three phases: the meningoencephalitic phase, the acute ophthalmic phase and the dermatologic phase. These includes the Harada disease, the Vogt-Koyanagi disease, the Behcet disease and the idiopathic forms. The Vogt-Koyanagi syndrome is a chronic and severa bilateral exudative uveitis associated with whitening of the hair and eyelashes and varying signs of meningeal irritation, less frequent than in Harada's disease. The Harada syndrome is a posterior uveitis accompanied by signs of meningeal irritation, increased protein levels and pleocytosis of the cerebrospinal fluid. The Behcet disease is a relapsing illness, characterized by oral and genital aphtous ulcers and ocular inflamation. In many cases there are additional features; in 10 to 25 percent of the cases there are neurologic involvement, and any portion of the nervous system may be affected. The authors studied two cases of uveome-ningoencephalic syndrome, one case of Vogt-Koyanagi-Harada disease (case 1) and another of Behcet disease (case 2). The case 1 was a white Brazilian, forty-five year-old man, with a acute headache, mental confusion and signs of meningeal irritation. The cerebrospinal fluid was a inflamatory one, with increased lymphocytes and monocytes, and one month after the patient developed bilateral uveit. The patient complicated with amblyopia of the left eye and a Korsakoff syndrome. There was no correlation in the literature of the Korsakoff syndrome and the uveomeningoencephalitic syndrome. The case 2 was a white Brazilian, twenty-four year-old man, with two episodes of meningitis, genital recurrent aphtous ulcers and uveitis in the right eye, with latter blindness of this eye. Some considerations about the etiopathogenic aspects are done and about the controversial use of corticoids in the treatment of these syndromes.

Texto completo disponível apenas em PDF.

Full text available only in PDF format.

Trabalho da Disciplina de Neurologia do Departamento de Medicina da Faculdade de Ciências Médicas da Santa Casa de São Paulo.

Departamento de Medicina - Faculdade de Medicina da Santa Casa de São Paulo - Rua Cesário Motta Jr. .112 - 01221 São Paulo, SP - Brasil.

1. ALAJOUANINE, Th.; CASTAIGNE, P.; LHERMITTE, F.; CAMBIER, J. & GAUTIER, J. C. - La meningo-encéphalite de la maladie de Behcet. Prèsse Méd. 56:2579, 1961.
2. AWAN, A. M. - Vogt-Koyanagi-Harada syndrome: report of two cases with uveomeningoencephalic syndromes. East Afr. Med. J. 52:544, 1975.
3. BAZEX, A.; BALAS, D.; BAZEX, J.; BEC, P.; SOLIER, M. & SECHEYRON, P. - Maladie de Vogt-Koyanagi-Harada: a propôs de 2 observations. Ann. Dermatol. Venerol. 104:849, 1977.
4. BUGE, A.; ESCOUROLLE, R.; CHOMETTE, G.; RANCUREL, G.; DECHY, H.; HAUW, J. J. & GRAY, F. - Maladie de Behcet avec manifestations neurologiques et fibrose endocardique du coeur droit: etude anatomoclinique d'une observation. Ann. Med. Int. 5:411, 1977.
5. CHAJEK, T. & FAINARU, M. - Behcet's disease: report of 41 cases and a review of the literature. Medicine 54:179, 1975,
6. GODEL, V.; BLÜMENTHAL, M. & REGENBOGEN, L, - Functional evaluation in Harada's disease: a case report. Acta Ophthalmol. 56:314, 1978.
7. HAMZA, M.; ZRIBI, A.; CHADLI, A. & BENAYED, H. - La maladie de Behcet: etude de 22 cases. Nouv. Prèsse Méd. 4:563, 1975.
8. KOZIN, F.; HAÜGHTON, V. & BERNHARD, C. G. - Neuro-Behçet disease: two cases and neuroradiologic findings. Neurology (Minneapolis) 27:1148, 1977.
9. LIMA, J. G. C; ROBORTELLA, M.; ATANES, M.; REIS FILHO, J. B. & NOBREGA, J. A. M. - Sindrome de Vogt-Koyanagi-Harada: relato de um caso. Arq. Neuro-Psiquiat. (São Paulo) 36:67, 1978.
10. MANOR, R. S. - Particular aspects of the Vogt-Koyanagi-Harada syndrome. Ophthalmologica 165:425, 1972.
11. MANOER, R. S.; LIVINI, E. & COHEN, S. - Cell-mediated immunity to human myelin basic protein in Vogt-Koyanagi-Harada syndrome. Invest. Ophthalmol. Visual Sci. 18:204, 1979.
12. MIYAKAWA, T.; MURAYAMA, E.; DESHIMARU, M.; SHIKAI, I. & KOZUMA, S. - Neuro-Behcet's disease showing severe atrophy of the cerebrum. Acta Neuropath.
13. NOVAK, E.; WERNECK, L. C. & MORA, A. H. - Doença de Behçet com envolvimento neurológico. Arq. Neuro-Psiquiat. (São Paulo) 35:146, 1977.
14. O'DUFFY, J. D. & GOLDSTEIN, N. P. - Neurological involvement in seven patients with Behçet's disease. Am. J. Med. 61:170, 1976.
15. OHNO, S. - Study on HL-A antigens in Behçet disease. Acta Societ. Ophthalmol. Japonica 78:1158, 1974.
16. OHNO, S.; CHAR, D. H.; KIMURA, S. & O'CONNOR, G. R. - Vogt-Koyanagi-Harada syndrome. Am. J. Ophthalmol. 83:735, 1977.
17. PERRY, H. D. & FONT, R. L. - Clinical and histopathologic observations in severe Vogt-Koyanagi-Harada syndrome. Am. J. Ophthalmol. 83:242, 1977.
18. PRUDHOMMEAUX, P.; POILPRE, E.; MORIN, P. & LECHEVALIER, B. - Myoclonies d'action au cours d'une uvéeo-névraxite. Oto-Neuro-Opht. 45:145, 1973.
19. ROSSELET, E.: SAUDAN, Y. & JEANNET, M. - Recherche des antigènes HL-A dans la maladie de Behçet. Ophthalmologica 172:116, 1976.
20. SAARI, M. - Vogt-Koyanagi-Harada syndrome: report of a case with many recurrences. Ophthalmologica 169:326, 1974.
21. SCHOENEN, J. & DELWAIDE, P. J. - A propos d´une uvéo-névraxite. Acta Neurol. Belg. 75:267, 1975.
22. TAGAWA, Y.; SUGIURA, S.; YAKURA, H.; WAKISAKA, A. & AIZAWA, M. - HL-A and Vogt-Koyanagi-Harada syndrome. N. Engl. J. Med. 295:173, 1976 (letter).
23. WRIGHT, V. A. & CHAMBERLAIN, M. A. - Syndrome de Behçet. Boletim sobre Doenças Reumáticas (Publicado pela FAPAR com autorização da Arthritis Foundation) 29:1, 1979.
24. YAKURA, H.; WAKISAKA, A.; AIZAWA, M.; ITAKURA, K.; TAGAWA, U. & SUGIURA, S. - HLA-D antigen of Japanese origin (LD-Wa) an its association with Vogt-Koyanagi-Harada syndrome. Tissue Antigens 8:35, 1976.

Datas de Publicação

Publicação nesta coleção
16 Ago 2012
Data do Fascículo
Mar 1982

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

[1] 1. ALAJOUANINE, Th.; CASTAIGNE, P.; LHERMITTE, F.; CAMBIER, J. & GAUTIER, J. C. - La meningo-encéphalite de la maladie de Behcet. Prèsse Méd. 56:2579, 1961.

[2] 2. AWAN, A. M. - Vogt-Koyanagi-Harada syndrome: report of two cases with uveomeningoencephalic syndromes. East Afr. Med. J. 52:544, 1975.

[3] 3. BAZEX, A.; BALAS, D.; BAZEX, J.; BEC, P.; SOLIER, M. & SECHEYRON, P. - Maladie de Vogt-Koyanagi-Harada: a propôs de 2 observations. Ann. Dermatol. Venerol. 104:849, 1977.

[4] 4. BUGE, A.; ESCOUROLLE, R.; CHOMETTE, G.; RANCUREL, G.; DECHY, H.; HAUW, J. J. & GRAY, F. - Maladie de Behcet avec manifestations neurologiques et fibrose endocardique du coeur droit: etude anatomoclinique d'une observation. Ann. Med. Int. 5:411, 1977.

[5] 5. CHAJEK, T. & FAINARU, M. - Behcet's disease: report of 41 cases and a review of the literature. Medicine 54:179, 1975,

[6] 6. GODEL, V.; BLÜMENTHAL, M. & REGENBOGEN, L, - Functional evaluation in Harada's disease: a case report. Acta Ophthalmol. 56:314, 1978.

[7] 7. HAMZA, M.; ZRIBI, A.; CHADLI, A. & BENAYED, H. - La maladie de Behcet: etude de 22 cases. Nouv. Prèsse Méd. 4:563, 1975.

[8] 8. KOZIN, F.; HAÜGHTON, V. & BERNHARD, C. G. - Neuro-Behçet disease: two cases and neuroradiologic findings. Neurology (Minneapolis) 27:1148, 1977.

[9] 9. LIMA, J. G. C; ROBORTELLA, M.; ATANES, M.; REIS FILHO, J. B. & NOBREGA, J. A. M. - Sindrome de Vogt-Koyanagi-Harada: relato de um caso. Arq. Neuro-Psiquiat. (São Paulo) 36:67, 1978.

[10] 10. MANOR, R. S. - Particular aspects of the Vogt-Koyanagi-Harada syndrome. Ophthalmologica 165:425, 1972.

[11] 11. MANOER, R. S.; LIVINI, E. & COHEN, S. - Cell-mediated immunity to human myelin basic protein in Vogt-Koyanagi-Harada syndrome. Invest. Ophthalmol. Visual Sci. 18:204, 1979.

[12] 12. MIYAKAWA, T.; MURAYAMA, E.; DESHIMARU, M.; SHIKAI, I. & KOZUMA, S. - Neuro-Behcet's disease showing severe atrophy of the cerebrum. Acta Neuropath.

[13] 13. NOVAK, E.; WERNECK, L. C. & MORA, A. H. - Doença de Behçet com envolvimento neurológico. Arq. Neuro-Psiquiat. (São Paulo) 35:146, 1977.

[14] 14. O'DUFFY, J. D. & GOLDSTEIN, N. P. - Neurological involvement in seven patients with Behçet's disease. Am. J. Med. 61:170, 1976.

[15] 15. OHNO, S. - Study on HL-A antigens in Behçet disease. Acta Societ. Ophthalmol. Japonica 78:1158, 1974.

[16] 16. OHNO, S.; CHAR, D. H.; KIMURA, S. & O'CONNOR, G. R. - Vogt-Koyanagi-Harada syndrome. Am. J. Ophthalmol. 83:735, 1977.

[17] 17. PERRY, H. D. & FONT, R. L. - Clinical and histopathologic observations in severe Vogt-Koyanagi-Harada syndrome. Am. J. Ophthalmol. 83:242, 1977.

[18] 18. PRUDHOMMEAUX, P.; POILPRE, E.; MORIN, P. & LECHEVALIER, B. - Myoclonies d'action au cours d'une uvéeo-névraxite. Oto-Neuro-Opht. 45:145, 1973.

[19] 19. ROSSELET, E.: SAUDAN, Y. & JEANNET, M. - Recherche des antigènes HL-A dans la maladie de Behçet. Ophthalmologica 172:116, 1976.

[20] 20. SAARI, M. - Vogt-Koyanagi-Harada syndrome: report of a case with many recurrences. Ophthalmologica 169:326, 1974.

[21] 21. SCHOENEN, J. & DELWAIDE, P. J. - A propos d´une uvéo-névraxite. Acta Neurol. Belg. 75:267, 1975.

[22] 22. TAGAWA, Y.; SUGIURA, S.; YAKURA, H.; WAKISAKA, A. & AIZAWA, M. - HL-A and Vogt-Koyanagi-Harada syndrome. N. Engl. J. Med. 295:173, 1976 (letter).

[23] 23. WRIGHT, V. A. & CHAMBERLAIN, M. A. - Syndrome de Behçet. Boletim sobre Doenças Reumáticas (Publicado pela FAPAR com autorização da Arthritis Foundation) 29:1, 1979.

[24] 24. YAKURA, H.; WAKISAKA, A.; AIZAWA, M.; ITAKURA, K.; TAGAWA, U. & SUGIURA, S. - HLA-D antigen of Japanese origin (LD-Wa) an its association with Vogt-Koyanagi-Harada syndrome. Tissue Antigens 8:35, 1976.