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Citomegalia e malformações do sistema nervoso central: relato de dois casos associados à agenesia do corpo caloso e encefalopatia cística

Cytomegaly and central nervous system malformations: report of two cases associated with agenesia of the corpus callosum and cystic encephalopathy

Resumos

Dois casos de citomegalia do sistema nervoso central em lactentes, comprovados pelo encontro de inclusões virais típicas no tecido nervoso, associados à agenesia da comissura do corpo caloso e encefalopatía cística simétrica pós-infarto, são estudados sob o ponto de vista anátomo-clínico. O autor acredita que a agenesia tenha sido causada por transformação cística da lâmina reuniens e do sulco médio ocluido inoportunamente no período embrionário. Discute a possibilidade dessa malformação cerebral ter sido ocasionada pela virose, não excluindo, contudo, a hipótese de uma casual ocorrência da citomegalia num encéfalo previamente anômalo. Atribui a encefalopatia cística simétrica coexistente à uma redistribuição (desvio) do fluxo sangüíneo cerebral através do que denomina de sistema de irrigação prioritária. Este sistema, provavelmente, atua independentemente dos mecanismos de auto-regulação do fluxo sangüíneo cerebral e entra em ação em situações de emergência, com a finalidade de suprir os centros neurovegetativos homeostáticos de maior importância vital imediata, preservando-os em detrimento — se prolongado o desvio do fluxo sangüíneo — para os demais territórios do sistema nervoso central excluídos do circuito circulatório. Essas áreas, submetidas a uma isquemia relativa, manifestam infartos com características simétricas e sequelas subseqüentes, se o indivíduo sobreviver tempo suficiente para a reabsorpção dos detritos teciduais e substituição por áreas císticas, dada a extensão da necrose.


Two cases of cytomegaly of the central nervous system of infants, disclosing typical viral inclusions in the brain tissues, associated with agenesia of the corpus callosum and cystic symmetrical post-infarction encephalopathy are reported. It is suggested that the callosal defect was caused by cystic degeneration occuring within the lamina reuniens and the prematurely fused sulcus medianus, in early intrauterine life. The possibility of cytomegalic etiology of the malformation is discussed. However the occurrence of viral disease in a previous abnormal brain cannot be discarded. The cystic symmetrical encephalopathy is explained through a redistribution (shunting) of the cerebral blood flow, brought about by the existence of a peculiar mechanism which he calls — priority blood supplying system. This proposed system probably acts independently of the cerebral blood flow autoregulatory mechanisms in emergency states, shunting the blood to the vitaly more important homeostatic neurovegetative centers. This mechanism, in spite of its actual effectiveness, if prolonged, may be deleterious to the excluded territories and produce irreversible damages. The morphologic expression of these damages are the multiple symmetrical brain infarctions. Following occasional survival, the tissue debris of the destroyed structures in the involved areas, are slowly and progressively removed by reabsorption and replaced by unusual symmetrical cavitations.


Citomegalia e malformações do sistema nervoso central. Relato de dois casos associados à agenesia do corpo caloso e encefalopatia cística

Cytomegaly and central nervous system malformations. Report of two cases associated with agenesia of the corpus callosum and cystic encephalopathy

Paulo F. L. Becker

Professor Adjunto do Departamento de Patologia (Prof. Fritz Köberle), Faculdade de Medicina de Ribeirão Preto (USP), São Paulo, Brasil

RESUMO

Dois casos de citomegalia do sistema nervoso central em lactentes, comprovados pelo encontro de inclusões virais típicas no tecido nervoso, associados à agenesia da comissura do corpo caloso e encefalopatía cística simétrica pós-infarto, são estudados sob o ponto de vista anátomo-clínico. O autor acredita que a agenesia tenha sido causada por transformação cística da lâmina reuniens e do sulco médio ocluido inoportunamente no período embrionário. Discute a possibilidade dessa malformação cerebral ter sido ocasionada pela virose, não excluindo, contudo, a hipótese de uma casual ocorrência da citomegalia num encéfalo previamente anômalo. Atribui a encefalopatia cística simétrica coexistente à uma redistribuição (desvio) do fluxo sangüíneo cerebral através do que denomina de sistema de irrigação prioritária. Este sistema, provavelmente, atua independentemente dos mecanismos de auto-regulação do fluxo sangüíneo cerebral e entra em ação em situações de emergência, com a finalidade de suprir os centros neurovegetativos homeostáticos de maior importância vital imediata, preservando-os em detrimento — se prolongado o desvio do fluxo sangüíneo — para os demais territórios do sistema nervoso central excluídos do circuito circulatório. Essas áreas, submetidas a uma isquemia relativa, manifestam infartos com características simétricas e sequelas subseqüentes, se o indivíduo sobreviver tempo suficiente para a reabsorpção dos detritos teciduais e substituição por áreas císticas, dada a extensão da necrose.

SUMMARY

Two cases of cytomegaly of the central nervous system of infants, disclosing typical viral inclusions in the brain tissues, associated with agenesia of the corpus callosum and cystic symmetrical post-infarction encephalopathy are reported. It is suggested that the callosal defect was caused by cystic degeneration occuring within the lamina reuniens and the prematurely fused sulcus medianus, in early intrauterine life. The possibility of cytomegalic etiology of the malformation is discussed. However the occurrence of viral disease in a previous abnormal brain cannot be discarded. The cystic symmetrical encephalopathy is explained through a redistribution (shunting) of the cerebral blood flow, brought about by the existence of a peculiar mechanism which he calls — priority blood supplying system. This proposed system probably acts independently of the cerebral blood flow autoregulatory mechanisms in emergency states, shunting the blood to the vitaly more important homeostatic neurovegetative centers. This mechanism, in spite of its actual effectiveness, if prolonged, may be deleterious to the excluded territories and produce irreversible damages. The morphologic expression of these damages are the multiple symmetrical brain infarctions. Following occasional survival, the tissue debris of the destroyed structures in the involved areas, are slowly and progressively removed by reabsorption and replaced by unusual symmetrical cavitations.

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Departamento de Patologia — Faculdade de Medicina — Caixa Postal 301 — 14100 Ribeirão Preto, SP — Brasil.

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Datas de Publicação

  • Publicação nesta coleção
    09 Abr 2013
  • Data do Fascículo
    Mar 1975
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