Hidranencefalia: estudo clinicopatológico de 6 casos

Ribeiro, Maria Valeriana Moura; Gallina, Richard A.; Sato, Takassu

doi:10.1590/S0004-282X1982000200010

Resumos

Foram estudados 6 casos de hidranencefalia do ponto de vista de sua semiologia clínica, de seus exames complementares e das verificações anatomopatológicas. Os Autores concluem que a transiluminação é de grande utilidade no diagnóstico precoce destes casos. O seguimento dos pacientes e as verificações anatomopatológicas demonstram que a hidranencefalia teve como origem lesões encefaloclásticas (inflamatórias, mecânicas e vasculares) que levaram, antes ou após o nascimento, à destruição total do cerebro com preservação das estruturas sub-tentoriais.

The study of six patients with hydranencephaly is reported. Clinical and necroscopic findings have been performed in correlation to routine laboratory examination. The Authors have concluded that transilumination is an important procedure for early diagnostic of these cases, especially when the patients have a milder or incomplete form of the syndrome. The necroscopic findings suggest that hydranencephaly may be the result of encephaloclastic lesions (inflammatory, mechanical or vascular) that, either before or after birth, lead to complete destruction of the brain, with sparing of sub-tentorial structures.

Hidranencefalia: estudo clinicopatológico de 6 casos

Hydranencephaly: clinicopathological studies in 6 cases

Maria Valeriana Moura Ribeiro^I; Richard A. Gallina^II; Takassu Sato^II

^ILivre-Docente. Departamento de Neuropsiquiatria e Psicologia Médica da Faculdade de Medicina de Ribeirão Preto (Prof. J. Armbrust-Figueiredo)

^IIProfessor Assistente. Departamento de Neuropsiquiatria e Psicologia Médica da Faculdade de Medicina de Ribeirão Preto (Prof. J. Armbrust-Figueiredo)

RESUMO

Foram estudados 6 casos de hidranencefalia do ponto de vista de sua semiologia clínica, de seus exames complementares e das verificações anatomopatológicas. Os Autores concluem que a transiluminação é de grande utilidade no diagnóstico precoce destes casos. O seguimento dos pacientes e as verificações anatomopatológicas demonstram que a hidranencefalia teve como origem lesões encefaloclásticas (inflamatórias, mecânicas e vasculares) que levaram, antes ou após o nascimento, à destruição total do cerebro com preservação das estruturas sub-tentoriais.

SUMMARY

The study of six patients with hydranencephaly is reported. Clinical and necroscopic findings have been performed in correlation to routine laboratory examination. The Authors have concluded that transilumination is an important procedure for early diagnostic of these cases, especially when the patients have a milder or incomplete form of the syndrome.

The necroscopic findings suggest that hydranencephaly may be the result of encephaloclastic lesions (inflammatory, mechanical or vascular) that, either before or after birth, lead to complete destruction of the brain, with sparing of sub-tentorial structures.

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Departamento de Neuropsiquiatria e Psicologia Médica - Faculdade de Medicina de Ribeirão Preto - Caixa Postal 301 - 14100 Ribeirão Preto, SP - Brasil.

1. AICARDI, J.; GOUTTÈRES; F. & HODEBOURG DE VERBOIS, A. - Multicystic encephalomalacia of infants and its relation to abnormal gestation and hidranencephaly. J. Neurol. Sci. 15:357, 1972.
2. ALTSHULER, G. - Toxoplasmosis as a cause of hydranencephaly. Am. J. Dis. Child. 125:251, 1973.
3. ANDRE, M.; PLENAT, F.; FLOQUET, J. & PICARD, L. - Hydranencéphalie Lesions cérébrales majeurs avec comportament neurologique neonatal normal. Arch. Franç. Pediat. 32:915, 1975.
4. APPENZELLER, O.; SNYDER, R. & KORNEFELD, M. - Autonomic failure in hydranencephaly. J. Neurol. Neurosurg. Psychiat, (London) 33:532, 1970.
5. BELL, W. E. & McCORMICK, W. F. - Increased Intracranial Pressure in Children. W. B. Sauders, Philadelphia, 1972.
6. CROME, L. & SYLVESTER, P. E. - Hydranencephaly (Hidrencephaly). Arch. Dis. Child. 33:235, 1958.
7. CROME, L. - Hydrenencephaly. Develop. Med. Child. Neurol. 14:224, 1972.
8. FOWLER, M.; DOW, W. R.; WHITE, T. A. & GREER, C. H. - Congenital hydrocephalus and hydranencephaly in five siblings with autopsy studies: a new disease. Develop. Med. Child Neurol. 14:173, 1972.
9. HALSEY, J. H.; ALLEN, N. & CHAMBERLEIN, H. R. - The morphogenesis of hydranencephaly, J. Neurol. Sci. 12:187, 1971.
10. HOFFMAN, J. & LISS, L. - Hidranencephaly: a case report with autopsy findings in a 7-year-old-girl. Acta Paediat. Scand, 58:297, 1969.
11. LAKE, P. & KURSE, T. - Hydranencephaly presenting as subdural hematoma in an infant. Bull. Los Angeles Neurol. Soc. 35:11, 1970.
12. LEFÉVRE, A. B.; ZACLIS, J. & VALENTE, M. I. - Hidrocefalia ou hidranencefalia: valor da transiluminação do crânio no diagnóstico diferencial. Arq. Neuro-Psiquiat. (São Paulo) 13:325, 1955.
13. LINDENBERG, R. & SWANSON, P. D. - Infantile hydranencephaly: a report of five cases of infarction of both cerebral hemispheries in infancy. Brain 90:839, 1967.
14. LORBER, J. - Hydranencephaly with normal development. Develop. Med. Child Neurol. 7:628, 1965.
15. MUIR, C. S. - Hydranencephaly and allied disorders. A study cerebral defects in Chinese. Arch. Dis. Childh. 34:231, 1959.
16. MYERS, R. E. - Brain pathology following fetal vascular occlusion. An experimental study. Invest. Ophthalm. 8:41, 1969.
17. NEVILLE, B. G. R. - The origins of infantile spasms: evidence from a case of hydranencephaly. Develop. Med. Child Neurol. 14:644, 1972.
18. POSER, C. M.; WALSH, F. C. & SHEINBERG, L. C. - Hydranencephaly. Neurology 5:284, 1955.
19. WEISS, M. H.; YOUNG., H. F. & McFARLAND, D. E. - Hydranencephaly of postnatal origin. J. Neurosurg. 32:715, 1970.

Datas de Publicação

Publicação nesta coleção
16 Ago 2012
Data do Fascículo
Jun 1982

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

[1] 1. AICARDI, J.; GOUTTÈRES; F. & HODEBOURG DE VERBOIS, A. - Multicystic encephalomalacia of infants and its relation to abnormal gestation and hidranencephaly. J. Neurol. Sci. 15:357, 1972.

[2] 2. ALTSHULER, G. - Toxoplasmosis as a cause of hydranencephaly. Am. J. Dis. Child. 125:251, 1973.

[3] 3. ANDRE, M.; PLENAT, F.; FLOQUET, J. & PICARD, L. - Hydranencéphalie Lesions cérébrales majeurs avec comportament neurologique neonatal normal. Arch. Franç. Pediat. 32:915, 1975.

[4] 4. APPENZELLER, O.; SNYDER, R. & KORNEFELD, M. - Autonomic failure in hydranencephaly. J. Neurol. Neurosurg. Psychiat, (London) 33:532, 1970.

[5] 5. BELL, W. E. & McCORMICK, W. F. - Increased Intracranial Pressure in Children. W. B. Sauders, Philadelphia, 1972.

[6] 6. CROME, L. & SYLVESTER, P. E. - Hydranencephaly (Hidrencephaly). Arch. Dis. Child. 33:235, 1958.

[7] 7. CROME, L. - Hydrenencephaly. Develop. Med. Child. Neurol. 14:224, 1972.

[8] 8. FOWLER, M.; DOW, W. R.; WHITE, T. A. & GREER, C. H. - Congenital hydrocephalus and hydranencephaly in five siblings with autopsy studies: a new disease. Develop. Med. Child Neurol. 14:173, 1972.

[9] 9. HALSEY, J. H.; ALLEN, N. & CHAMBERLEIN, H. R. - The morphogenesis of hydranencephaly, J. Neurol. Sci. 12:187, 1971.

[10] 10. HOFFMAN, J. & LISS, L. - Hidranencephaly: a case report with autopsy findings in a 7-year-old-girl. Acta Paediat. Scand, 58:297, 1969.

[11] 11. LAKE, P. & KURSE, T. - Hydranencephaly presenting as subdural hematoma in an infant. Bull. Los Angeles Neurol. Soc. 35:11, 1970.

[12] 12. LEFÉVRE, A. B.; ZACLIS, J. & VALENTE, M. I. - Hidrocefalia ou hidranencefalia: valor da transiluminação do crânio no diagnóstico diferencial. Arq. Neuro-Psiquiat. (São Paulo) 13:325, 1955.

[13] 13. LINDENBERG, R. & SWANSON, P. D. - Infantile hydranencephaly: a report of five cases of infarction of both cerebral hemispheries in infancy. Brain 90:839, 1967.

[14] 14. LORBER, J. - Hydranencephaly with normal development. Develop. Med. Child Neurol. 7:628, 1965.

[15] 15. MUIR, C. S. - Hydranencephaly and allied disorders. A study cerebral defects in Chinese. Arch. Dis. Childh. 34:231, 1959.

[16] 16. MYERS, R. E. - Brain pathology following fetal vascular occlusion. An experimental study. Invest. Ophthalm. 8:41, 1969.

[17] 17. NEVILLE, B. G. R. - The origins of infantile spasms: evidence from a case of hydranencephaly. Develop. Med. Child Neurol. 14:644, 1972.

[18] 18. POSER, C. M.; WALSH, F. C. & SHEINBERG, L. C. - Hydranencephaly. Neurology 5:284, 1955.

[19] 19. WEISS, M. H.; YOUNG., H. F. & McFARLAND, D. E. - Hydranencephaly of postnatal origin. J. Neurosurg. 32:715, 1970.