Agenesis of the corpus callosum and limbic malformations revisited.

Abstract

To the Editor. —In the September 1987 issue of theArchives, de Leon et al 1 reported a case of Apert syndrome with agenesis of the corpus callosum and limbic malformations. Recently, in a letter to the editor, Jeret et al 2 responded that the case for such central nervous system abnormalities in the Apert syndrome should not be overstated. It seems to me, however, that Jeret et al 2 do the opposite; they understate it and, at the same time, overstate the need for chromosomal analysis in the Apert syndrome. We have had a long-standing interest in children with multiple malformations 3 and craniosynostosis, 4 especially Apert syndrome. 5,6 At least 30 cases of Apert syndrome are known to be associated with agenesis of the corpus callosum or limbic malformations, or both. 5 Malformations do not have to occur universally in a condition to be of significance ; they may be