Agenesis of the Corpus Callosum and Limbic Malformations Revisited

To the Editor.  —In the September 1987 issue of the Archives, de León et al¹ reported a case of Apert syndrome with agenesis of the corpus callosum and limbic malformations. Recently, in a letter to the editor, Jeret et al² 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 al² 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 malformations³ 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.⁵Malformations do not have to occur universally in a condition to be of significance; they may be