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Arquivos de Neuro-Psiquiatria

Print version ISSN 0004-282X

Abstract

ABRAHAM, Ronaldo et al. Aicardi's syndrome: report of two cases. Arq. Neuro-Psiquiatr. [online]. 1986, vol.44, n.4, pp.364-372. ISSN 0004-282X.  https://doi.org/10.1590/S0004-282X1986000400007.

Aicardi's syndrome is a clinical entity characterized by agenesis of the corpus callosum, severe mental subnormality, seizures (most freque tly spasms in flexion), characteristic electroencephalographic changes (burst-supression pattern), typical chorioretinal lacunae, present only in females. Other associated findings are rib and vertebral dysplasias; frequently found are cortical heterotopias. This syndrome is of hereditary origin, now considered a probable X-linked dominant trait with male lethality. The authors describe two cases of this syndrome with the full clinical picture.

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