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December 1989

Agenesis of the Corpus Callosum and Limbic Malformations Revisited

Author Affiliations

Department of Oral Biology and Department of Pediatrics Dalhousie University Halifax, Nova Scotia, Canada B3H 3J5

Arch Neurol. 1989;46(12):1270. doi:10.1001/archneur.1989.00520480012006

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

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