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M. Michael Cohen, Jr, DMD, PhD
Department of Oral Biology and Department of Pediatrics Dalhousie University Halifax, Nova Scotia, Canada B3H 3J5

Arch Neurol. 1989;46(12):1270.

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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 . . . [Full Text PDF of this Article]

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