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January 1989

Agenesis of the Corpus Callosum and Limbic Malformation in Apert's Syndrome

Author Affiliations

Institute for Basic Research in Developmental Disabilities 1050 Forest Hill Rd Staten Island, NY 10314

Arch Neurol. 1989;46(1):10. doi:10.1001/archneur.1989.00520370012003

To the Editor.  —In the September 1987 issue of the Archives, de Leon et al1 described a case of Apert's syndrome that was characterized most notably by agenesis of the corpus callosum (ACC) and limbic malformations. The subject of ACC is of particular interest to us, since we recently prepared an extensive review of 705 cases, the largest study ever published on this topic. To our knowledge, total or partial ACC is a characteristic feature of only three syndromes (ie, Aicardi's, Andermann's, and Shapiro's), and its frequent, but not universal, occurrence in Apert's syndrome should not be overstated. For example, ACC has been reported in at least 24 cases of fetal alcohol syndrome and nine cases of Dandy-Walker syndrome2; yet to say either is characterized by ACC would be untrue.The presence of severe syndactyly in Apert's syndrome suggests that ACC, mental retardation, and limbic malformations are likely