Forma de heidenhain da doença de creutzfeldt-jakob: relato de um caso

Pinto Junior, Luciano Ribeiro; Lancellotti, Carmem Lucia Penteado; Onozuka, Angela Pires; Landivar, Alejandro Peralta; Sanvito, Wilson Luiz

doi:10.1590/S0004-282X1986000100008

Resumos

Registro de um caso da doença de Creutzfeldt-Jakob, forma de Heidenhain, que se caracteriza por comprometimento visual importante. As manifestações iniciais consistiram de distúrbios visuais. A paciente evoluiu para franca demência, ao lado de manifestações pirâmido-extrapiramidais. Além dos dados clínicos o EEG mostrou atividade periódica característica da doença. O estudo anátomo-patológico confirmou o diagnóstico clínico mostrando alteração esponjosa, degeneração neuronal e astrogliose. Além do quadro clínico, eletrencefalográfico e anátomo-patológico são discutidos os aspectos etiopatogênicos da doença.

A case of Creutzfeldt-Jakob disease, Heidenhain's form is reported. The first clinical manifestations were cortical blindness and visual agnosia. The patient here concerned, a woman aged sixty three, during the clinical course of the disease showed mental deterioration and pyramido-extrapyramidal manifestations. She died after five months. The electroencephalographic findings showed periodic activity. The anatomopatological examination showed neuronal degeneration, status spongiosus and proliferation of astroglial cells. The clinical, electroencephalographic, pathological and etiopathogenical aspects are discussed.

Luciano Ribeiro Pinto Junior^I; Carmem Lucia Penteado Lancellotti^II; Angela Pires Onozuka^II; Alejandro Peralta Landivar^III; Wilson Luiz Sanvito^I

^INeurologista - Departamento de Medicina (Disciplina de Neurologia) e Departamento de Anatomia Patológica, Faculdade de Ciências Médicas da Santa Casa de São Paulo

^IIPatologista - Departamento de Medicina (Disciplina de Neurologia) e Departamento de Anatomia Patológica, Faculdade de Ciências Médicas da Santa Casa de São Paulo

^IIIResidente - Departamento de Medicina (Disciplina de Neurologia) e Departamento de Anatomia Patológica, Faculdade de Ciências Médicas da Santa Casa de São Paulo

RESUMO

Registro de um caso da doença de Creutzfeldt-Jakob, forma de Heidenhain, que se caracteriza por comprometimento visual importante. As manifestações iniciais consistiram de distúrbios visuais. A paciente evoluiu para franca demência, ao lado de manifestações pirâmido-extrapiramidais. Além dos dados clínicos o EEG mostrou atividade periódica característica da doença. O estudo anátomo-patológico confirmou o diagnóstico clínico mostrando alteração esponjosa, degeneração neuronal e astrogliose. Além do quadro clínico, eletrencefalográfico e anátomo-patológico são discutidos os aspectos etiopatogênicos da doença.

SUMMARY

A case of Creutzfeldt-Jakob disease, Heidenhain's form is reported. The first clinical manifestations were cortical blindness and visual agnosia. The patient here concerned, a woman aged sixty three, during the clinical course of the disease showed mental deterioration and pyramido-extrapyramidal manifestations. She died after five months. The electroencephalographic findings showed periodic activity. The anatomopatological examination showed neuronal degeneration, status spongiosus and proliferation of astroglial cells. The clinical, electroencephalographic, pathological and etiopathogenical aspects are discussed.

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

1. BERNOULLI, C. ; SIEGFRIED, J. & BAUMGARTNER, G. - Danger of accidental person-to-person transmission of Creutzfeldt-Jakob disease by surgery. Lancet 1:478, 1977.
2. BROWN, P. & CATHALA, F. - Creutzfeldt-Jakob disease in France: I. Retrospective study of the Paris area during the ten-year period 1968-1977. Ann. Neurol. 5:189, 1979.
3. BROWN, P.; CATHALA, F. & GAJDUSEK, D.C. - Creutzfeldt-Jakob disease in France: III. Epidemiological study of 170 patients dying during the decade 1968-1977. Ann. Neurol. 6:438, 1979.
4. BROWN, P.; CATHALA, F.; SADOWSKY, D. & GAJDUSEK, D.C. - Creutzfeldt-Jakob disease in France: II. Clinical Characteristics of 124 consecutive verified cases during the decade 1968-1977. Ann. Neurol. 6:430, 1979.
5. BUGE, A.; ESCOUROLLE, R. ; BRION, S. ; RANCUREL, G. ; HAUWN, J.J.; MEHAUT, M. ; GRAY, F. & GAJDUSEK, D.C. - Maladie de Creutzeldt-Jakob familiale. Étude clinique et anatomique de trois cas huit répartis sur trois générations. Transmission au singe écureuil. Rev. neurol. (Paris) 134:165, 1978.
6. CATHALA, F. - Le Kuru et la tremblante modèles d'étude pour la maladie de Creutzfeldt-Jakob. Bull. Acad. nat. Méd. 164:737, 1980.
7. CATHALA, F.; BROWN, P.; CASTAIGNE, P. & GAJDUSEK, D.C. - La maladie de Creutzfeldt-Jakob en France continentale: étude retrospective de 1968 a 1977. Rev. neurol. (Paris) 135:439, 1979.
8. CATHALA, F.; MOREAU-DOBOIS, M.C. & BROWN, P. - Maladie de Creutzfeldt-Jakob: nouvelles acquisitions sur la biologie des virus non conventionnels. Path. Biol. 28:545, 1980.
9. CHOU, S.M. ; PAYNE, W.N. ; GIBBS, C.J. & GAJDUSEK, D.C. - Transmission and scanning electron microscopy of spongiform change in Creutzfeldt-Jakob disease. Brain 103:885, 1980.
10. COURT, L. - Pathologie expérimentale des encephalopathies spongiformes subaigues. Bull. Acad. nat. Méd. 164:742, 1980.
11. FURLAN, A.J.; HENRY, C.E.; SWEENEY, P.J. & MITSUMOTO, H. - Focal EEG abnormalities in Heidenhain's variant of Jakob-Creutzfeldt disease. Arch. Neurol. 38:312, 1981.
12. GALVEZ, S.; MASTERS, C. & GAJDUSEK, D.C. - Descriptive epidemiology of Creutzfeldt-Jakob disease in Chile. Arch. Neurol. 37:11, 1980.
13. HAUW, J.J. & ESCOUROLLE, R. - Aspects neuropathologiques des encéphalopathies spongiformes transmissibles humaines et expérimentales. Bull. Acad. nat. Méd. 164:746, 1980.
14. KAMIN, M. & PATTEN, B.M. - Creutzfeldt-Jakob disease: possible transmission to humans by consumption of wild animal brains. Amer. J. Med. 76:142, 1984.
15. KIRSCHBAUM, W.R. - Jakob-Creutzfeldt Disease. Elsevier, Amsterdam, 1968.
16. MANUELIDIS, E.E.; ANGELO, J.N.; GORGACZ, E.J. & MANUELIDIS, L. - Transmission of Creutzfeldt-Jakob disease to Syrian hamster. Lancet 1:479, 1977.
17. MASTERS, C.L.; HARRIS, J.O.; GAJDUSEK, D.C.; GIBBS, C.J. BERNOULLI, C. & ASHER, D.M. - Creutzfeldt-Jakob disease: patterns of worldwide occurrence and the significance of familial and sporadic clustering. Ann. Neurol. 5:177, 1979.
18. MASTERS, C.L. & RICHARDSON JR., E.P. - Subacute spongiform encephalopathy (Creutzfeldt-Jakob disease): the nature and progression of spongiform change. Brain 101:333, 1978.
19. MISHRA, D.N.; SHUKLA, G.D.; SINGH, D. & MATREJA, V.S. - Creutzfeldt-Jakob disease. J. ind. med. Assoc. 76:15, 1981.
20. NEVIN, S.; Mc MENEMEY, W.H.; BEHRMAN, S. & JONES, D.P. - Subacute spongiform encephalopathy - a subacute form of encephalopathy to vascular dysfunction (spongiform cerebral atrophy). Brain 83:519, 1960.
21. PATY, J.; BONNAUD, E.; LATINVILLE, D.; BRENOT, Ph.; VITAL, CI.; HENRY, P. & FAURE, J.M.A. - Correlations électrophysiologiques, cliniques et anatomiques dans un cas de maladie de Creutzfeldt-Jakob (forme de Heidenhain). Rev. neurol. (Paris) 134:223, 1978.
22. REUCK, J.; COSTER, W.; OTTE, G. & VANDER EECKEN, H. - Papova virus-like particles in a Nigral Lype of Creutzfeldt-Jakob disease. J. Neurol. 213:179, 1976.
23. SANVITO, W.L.; GUIDUGLI NETO, J.; NEGRISOLI, M.C.B.; DUARTE, M.I.S. & MELLO, A.CP. - Doença de Creutzfeldt-Jakob: considerações clínicas, eletrencefalográficas e anátomo-patológicas a propósito de um caso. Arq. Neuro-psiquiat. (São Paulo) 29:103, 1971.
24. SCHOENE, W.C.; MASTERS, CL.; CLARENCE JR., J.G.; GAJDUSEK, D.C.; TYLER, H.R.; MOORE, F.D. & DAMMIN, G.J. - Transmissible spongiform encephalopathy (Creutzfeldt-Jakob disease). Atypical clinical and pathological findings. Arch. Neurol. 38:473, 1981.
25. SOTELO, J.; GIBBS JR., C.J. & GAJDUSEK, D.C. - Autoantibodies against axonal neurofilaments in patients with Kuru and Creutzfeldt-Jakob disease. Science 210:190, 1980.

Forma de heidenhain da doença de creutzfeldt-jakob relato de um caso

Heidenhain form of Creutzfeldt-Jakob disease: a case report

Datas de Publicação

Publicação nesta coleção
29 Jun 2011
Data do Fascículo
Mar 1986

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

[1] 1. BERNOULLI, C. ; SIEGFRIED, J. & BAUMGARTNER, G. - Danger of accidental person-to-person transmission of Creutzfeldt-Jakob disease by surgery. Lancet 1:478, 1977.

[2] 2. BROWN, P. & CATHALA, F. - Creutzfeldt-Jakob disease in France: I. Retrospective study of the Paris area during the ten-year period 1968-1977. Ann. Neurol. 5:189, 1979.

[3] 3. BROWN, P.; CATHALA, F. & GAJDUSEK, D.C. - Creutzfeldt-Jakob disease in France: III. Epidemiological study of 170 patients dying during the decade 1968-1977. Ann. Neurol. 6:438, 1979.

[4] 4. BROWN, P.; CATHALA, F.; SADOWSKY, D. & GAJDUSEK, D.C. - Creutzfeldt-Jakob disease in France: II. Clinical Characteristics of 124 consecutive verified cases during the decade 1968-1977. Ann. Neurol. 6:430, 1979.

[5] 5. BUGE, A.; ESCOUROLLE, R. ; BRION, S. ; RANCUREL, G. ; HAUWN, J.J.; MEHAUT, M. ; GRAY, F. & GAJDUSEK, D.C. - Maladie de Creutzeldt-Jakob familiale. Étude clinique et anatomique de trois cas huit répartis sur trois générations. Transmission au singe écureuil. Rev. neurol. (Paris) 134:165, 1978.

[6] 6. CATHALA, F. - Le Kuru et la tremblante modèles d'étude pour la maladie de Creutzfeldt-Jakob. Bull. Acad. nat. Méd. 164:737, 1980.

[7] 7. CATHALA, F.; BROWN, P.; CASTAIGNE, P. & GAJDUSEK, D.C. - La maladie de Creutzfeldt-Jakob en France continentale: étude retrospective de 1968 a 1977. Rev. neurol. (Paris) 135:439, 1979.

[8] 8. CATHALA, F.; MOREAU-DOBOIS, M.C. & BROWN, P. - Maladie de Creutzfeldt-Jakob: nouvelles acquisitions sur la biologie des virus non conventionnels. Path. Biol. 28:545, 1980.

[9] 9. CHOU, S.M. ; PAYNE, W.N. ; GIBBS, C.J. & GAJDUSEK, D.C. - Transmission and scanning electron microscopy of spongiform change in Creutzfeldt-Jakob disease. Brain 103:885, 1980.

[10] 10. COURT, L. - Pathologie expérimentale des encephalopathies spongiformes subaigues. Bull. Acad. nat. Méd. 164:742, 1980.

[11] 11. FURLAN, A.J.; HENRY, C.E.; SWEENEY, P.J. & MITSUMOTO, H. - Focal EEG abnormalities in Heidenhain's variant of Jakob-Creutzfeldt disease. Arch. Neurol. 38:312, 1981.

[12] 12. GALVEZ, S.; MASTERS, C. & GAJDUSEK, D.C. - Descriptive epidemiology of Creutzfeldt-Jakob disease in Chile. Arch. Neurol. 37:11, 1980.

[13] 13. HAUW, J.J. & ESCOUROLLE, R. - Aspects neuropathologiques des encéphalopathies spongiformes transmissibles humaines et expérimentales. Bull. Acad. nat. Méd. 164:746, 1980.

[14] 14. KAMIN, M. & PATTEN, B.M. - Creutzfeldt-Jakob disease: possible transmission to humans by consumption of wild animal brains. Amer. J. Med. 76:142, 1984.

[15] 15. KIRSCHBAUM, W.R. - Jakob-Creutzfeldt Disease. Elsevier, Amsterdam, 1968.

[16] 16. MANUELIDIS, E.E.; ANGELO, J.N.; GORGACZ, E.J. & MANUELIDIS, L. - Transmission of Creutzfeldt-Jakob disease to Syrian hamster. Lancet 1:479, 1977.

[17] 17. MASTERS, C.L.; HARRIS, J.O.; GAJDUSEK, D.C.; GIBBS, C.J. BERNOULLI, C. & ASHER, D.M. - Creutzfeldt-Jakob disease: patterns of worldwide occurrence and the significance of familial and sporadic clustering. Ann. Neurol. 5:177, 1979.

[18] 18. MASTERS, C.L. & RICHARDSON JR., E.P. - Subacute spongiform encephalopathy (Creutzfeldt-Jakob disease): the nature and progression of spongiform change. Brain 101:333, 1978.

[19] 19. MISHRA, D.N.; SHUKLA, G.D.; SINGH, D. & MATREJA, V.S. - Creutzfeldt-Jakob disease. J. ind. med. Assoc. 76:15, 1981.

[20] 20. NEVIN, S.; Mc MENEMEY, W.H.; BEHRMAN, S. & JONES, D.P. - Subacute spongiform encephalopathy - a subacute form of encephalopathy to vascular dysfunction (spongiform cerebral atrophy). Brain 83:519, 1960.

[21] 21. PATY, J.; BONNAUD, E.; LATINVILLE, D.; BRENOT, Ph.; VITAL, CI.; HENRY, P. & FAURE, J.M.A. - Correlations électrophysiologiques, cliniques et anatomiques dans un cas de maladie de Creutzfeldt-Jakob (forme de Heidenhain). Rev. neurol. (Paris) 134:223, 1978.

[22] 22. REUCK, J.; COSTER, W.; OTTE, G. & VANDER EECKEN, H. - Papova virus-like particles in a Nigral Lype of Creutzfeldt-Jakob disease. J. Neurol. 213:179, 1976.

[23] 23. SANVITO, W.L.; GUIDUGLI NETO, J.; NEGRISOLI, M.C.B.; DUARTE, M.I.S. & MELLO, A.CP. - Doença de Creutzfeldt-Jakob: considerações clínicas, eletrencefalográficas e anátomo-patológicas a propósito de um caso. Arq. Neuro-psiquiat. (São Paulo) 29:103, 1971.

[24] 24. SCHOENE, W.C.; MASTERS, CL.; CLARENCE JR., J.G.; GAJDUSEK, D.C.; TYLER, H.R.; MOORE, F.D. & DAMMIN, G.J. - Transmissible spongiform encephalopathy (Creutzfeldt-Jakob disease). Atypical clinical and pathological findings. Arch. Neurol. 38:473, 1981.

[25] 25. SOTELO, J.; GIBBS JR., C.J. & GAJDUSEK, D.C. - Autoantibodies against axonal neurofilaments in patients with Kuru and Creutzfeldt-Jakob disease. Science 210:190, 1980.