Panencefalite esclerosante subaguda em adulto: registro de um caso

Kouyoumdjian, João Aris

doi:10.1590/S0004-282X1985000300011

Resumos

É apresentado caso de PEES em um paciente de 29 anos de idade, o que constitui fato incomum nessa rara afecção. O caso apresentado preenchia todos os critérios diagnósticos clássicos não sendo necessária biópsia cerebral. Os casos de início tardio em nada diferem dos casos de apresentação usual exceto talvez pelo curso fatal mais rápido. Os achados tomográficos de neurocisticer-cose no caso descrito foram coincidentes, provavelmente.

A case of SSPE in a 29-year-old man is presented. The clinical picture was typical with progressive dementia associated with periodic myoclonus. The electroencephalogram showed periodic complexes; the serum and cerebrospinal fluid (CSF) measles antibodies obtained by complement fixation were 1:256 and 1:32 respectively; the CSF gamma globulin was raised (32%) with an oligoclonal distribution. The fundoscopic examination demonstrated a retinal change in maculae and the computed tomography showed cysts and calcifications characteristic of Cysticercus cellulosae. The latter findings probably were not implicated with the SSPE because neurocysticercosis is a very common disease in this region of the country and frequently there are asymptomatic cysts and/or calcifications on CT. The essential clinical and epidemiologic picture of SSPE are discussed and probably the cases with an adult onset had a more rapid outcome to death.

Panencefalite esclerosante subaguda em adulto: registro de um caso

Adult onset of subacute sclerosing panencephalitis: a case report

João Aris Kouyoumdjian

Chefe da Disciplina. Faculdade de Medicina de São José do Rio Preto (FMSJRP)

RESUMO

É apresentado caso de PEES em um paciente de 29 anos de idade, o que constitui fato incomum nessa rara afecção. O caso apresentado preenchia todos os critérios diagnósticos clássicos não sendo necessária biópsia cerebral. Os casos de início tardio em nada diferem dos casos de apresentação usual exceto talvez pelo curso fatal mais rápido. Os achados tomográficos de neurocisticer-cose no caso descrito foram coincidentes, provavelmente.

SUMMARY

A case of SSPE in a 29-year-old man is presented. The clinical picture was typical with progressive dementia associated with periodic myoclonus. The electroencephalogram showed periodic complexes; the serum and cerebrospinal fluid (CSF) measles antibodies obtained by complement fixation were 1:256 and 1:32 respectively; the CSF gamma globulin was raised (32%) with an oligoclonal distribution. The fundoscopic examination demonstrated a retinal change in maculae and the computed tomography showed cysts and calcifications characteristic of Cysticercus cellulosae. The latter findings probably were not implicated with the SSPE because neurocysticercosis is a very common disease in this region of the country and frequently there are asymptomatic cysts and/or calcifications on CT. The essential clinical and epidemiologic picture of SSPE are discussed and probably the cases with an adult onset had a more rapid outcome to death.

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Trabalho da Disciplina de Neurologia da Faculdade de Medicina de São José do Rio Preto (FMSJRP).

Departamento de Medicina Integrada, Disciplina de Neurologia, Faculdade de Medicina de São José do Rio Preto - Av. Brig. Faria Lima, 5.416 - 15.100, São José do Rio Preto, SP - Brasil.

1. ANDRIOLA, M. & KARLSBERG, R.O. - Maculopathy in subacute sclerosing panencephalitis. Amer. J. Dis. Child. 124:187, 1972.
2. CANELAS, H.M.; LEFRÈVE, A.B.; TOGNOLA, W.A.; FONSECA, L.C.; JULIÂO, O.F.; ASSIS, J.L.; JORGE, F.B. & XAVIER-LIMA, A. - Subacute sclerosing leucoencephalitis: an epidemiological, clinical and biochemical study of 31 cases. Arq. Neuro-Psiquiat. (São Paulo) 25:255, 1967.
3. CAPE, C.A.; MARTINEZ, A.J.; ROBERTSON, J.T.; HAMILTON, R. & JABBOUR, J.T. - Adult onset of subacute sclerosing panencephalitis. Arch. Neurol. 28:124, 1973.
4. CERNESCTJ, C. & MILEA, S. - Further epidemiological investigations on subacute sclerosing panencephalitis in Romania. Rev. roum. Méd-Virol. 31:173, 1980.
5. COBB, W. - The periodic events of subacute sclerosing leucoencephalitis. Electro-enceph. clin. Neurophysiol. 21:278, 1966.
6. HLADY, J.; NORMAN, M.G.; THIESSEN, P.N.; DUNN, H.G. & TINGLE, A. - Clinical-pathological conference: seizures in a 15-year-old. J. Pediatrics 101:641, 1982.
7. JABBOUR, J.T.; GARCIA, J.H.; LEMMI, H.; RAGLAND, J.; DUENAS, D.A. & SEVER, J.L. - Subacute sclerosing panencephalitis. A multidisciplinary study of eight cases. J. amer. med. Assoc 207:2248, 1969.
8. JOHANNES, R.S. & SEVER, J.L. - Subacute sclerosing panencephalitis. Ann. Rev. Med. 26:589, 1975.
9. MARKAND, O.N. & PANSZI, J.G. - The electroencephalogram in subacute sclerosing panencephalitis. Arch. Neurol. 32:719, 1975.
10. MAYSTRE, H. - La panencéphalite sclérosante subaiguS de l'adulte. Arch. Neurol. Neurochir. Psychiat 127:31, 1980.
11. MODLIN, J.F.; HALSEY, N.A.; EDDINS, D.L.; CONRAD, J.L.; JABBOUR, J.T.; CHIEN, L. & ROBINSON, H. - Epidemiology of subacute sclerosing panencephalitis. J. Pediatrics 94:231, 1979.
12. MOODIE, J.W.; MACKENZIE, D.J.M. & KIPPS, A. - Subacute sclerosing panencephalitis in Southern Africa. Recent additions to the SSPE registry. South African med. J. 58:964, 1980.
13. MURATA, R.; FUJII, K. & YOSHIDA, R. - Computed tomographic findings in subacute sclerosing panencephalitis. FoL psychiat. neurol. jap. 33:153, 1979.
14. RISK, W.S. & HADDAD, F.S. - The variable natural history of subacute sclerosing panencephalitis. A study of 118 cases from the Middle East. Arch. Neurol. 36:610, 1979.
15. ROBERTSON, W.C.; CLARK, D.B. & MARKESBERY, W.R. - Review of 38 cases of subacute sclerosing panencephalitis: effect of amantadine on the natural course of the disease. Ann. Neurol. 8:422, 1980.
16. SOBCZYK, W.; KULCZYCKI, J. & HORBOWSKA - Meales antibodies in the saliva of children with subacute sclerosing panencephalitis. J. Neurol. 228:219, 1982.

Datas de Publicação

Publicação nesta coleção
13 Ago 2012
Data do Fascículo
Set 1985

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

[1] 1. ANDRIOLA, M. & KARLSBERG, R.O. - Maculopathy in subacute sclerosing panencephalitis. Amer. J. Dis. Child. 124:187, 1972.

[2] 2. CANELAS, H.M.; LEFRÈVE, A.B.; TOGNOLA, W.A.; FONSECA, L.C.; JULIÂO, O.F.; ASSIS, J.L.; JORGE, F.B. & XAVIER-LIMA, A. - Subacute sclerosing leucoencephalitis: an epidemiological, clinical and biochemical study of 31 cases. Arq. Neuro-Psiquiat. (São Paulo) 25:255, 1967.

[3] 3. CAPE, C.A.; MARTINEZ, A.J.; ROBERTSON, J.T.; HAMILTON, R. & JABBOUR, J.T. - Adult onset of subacute sclerosing panencephalitis. Arch. Neurol. 28:124, 1973.

[4] 4. CERNESCTJ, C. & MILEA, S. - Further epidemiological investigations on subacute sclerosing panencephalitis in Romania. Rev. roum. Méd-Virol. 31:173, 1980.

[5] 5. COBB, W. - The periodic events of subacute sclerosing leucoencephalitis. Electro-enceph. clin. Neurophysiol. 21:278, 1966.

[6] 6. HLADY, J.; NORMAN, M.G.; THIESSEN, P.N.; DUNN, H.G. & TINGLE, A. - Clinical-pathological conference: seizures in a 15-year-old. J. Pediatrics 101:641, 1982.

[7] 7. JABBOUR, J.T.; GARCIA, J.H.; LEMMI, H.; RAGLAND, J.; DUENAS, D.A. & SEVER, J.L. - Subacute sclerosing panencephalitis. A multidisciplinary study of eight cases. J. amer. med. Assoc 207:2248, 1969.

[8] 8. JOHANNES, R.S. & SEVER, J.L. - Subacute sclerosing panencephalitis. Ann. Rev. Med. 26:589, 1975.

[9] 9. MARKAND, O.N. & PANSZI, J.G. - The electroencephalogram in subacute sclerosing panencephalitis. Arch. Neurol. 32:719, 1975.

[10] 10. MAYSTRE, H. - La panencéphalite sclérosante subaiguS de l'adulte. Arch. Neurol. Neurochir. Psychiat 127:31, 1980.

[11] 11. MODLIN, J.F.; HALSEY, N.A.; EDDINS, D.L.; CONRAD, J.L.; JABBOUR, J.T.; CHIEN, L. & ROBINSON, H. - Epidemiology of subacute sclerosing panencephalitis. J. Pediatrics 94:231, 1979.

[12] 12. MOODIE, J.W.; MACKENZIE, D.J.M. & KIPPS, A. - Subacute sclerosing panencephalitis in Southern Africa. Recent additions to the SSPE registry. South African med. J. 58:964, 1980.

[13] 13. MURATA, R.; FUJII, K. & YOSHIDA, R. - Computed tomographic findings in subacute sclerosing panencephalitis. FoL psychiat. neurol. jap. 33:153, 1979.

[14] 14. RISK, W.S. & HADDAD, F.S. - The variable natural history of subacute sclerosing panencephalitis. A study of 118 cases from the Middle East. Arch. Neurol. 36:610, 1979.

[15] 15. ROBERTSON, W.C.; CLARK, D.B. & MARKESBERY, W.R. - Review of 38 cases of subacute sclerosing panencephalitis: effect of amantadine on the natural course of the disease. Ann. Neurol. 8:422, 1980.

[16] 16. SOBCZYK, W.; KULCZYCKI, J. & HORBOWSKA - Meales antibodies in the saliva of children with subacute sclerosing panencephalitis. J. Neurol. 228:219, 1982.