Other Syndromes Frequently Associated with Callosal Agenesis

  • Guy Geoffroy
Part of the Advances in Behavioral Biology book series (ABBI, volume 42)


More than 50 disorders associated with agenesis of the corpus callosum (ACC) have been reported in medical literature. In most cases, the association appears to be a chance occurrence. Some of the cases have chromosomal abnormalities, others have central nervous malformation or metabolic disease and a few have peroxysomal disease or apparently unrelated systemic malformations (see Tables 1 and 2). A number of cases without concomitant pathology have also been described. Clinicians are at present considering the possibility that inherited metabolic disease may be responsible for cerebral malformations (Dobyns, 1989; Kolodny, 1989). In this context, we recently examined a brother and sister similarly afflicted with a complex neurological syndrome and an unusual metabolic abnormality of lipids. Both had agenesis of the cerebellar vermis.


Corpus Callosum Zellweger Syndrome Hypothalamic Dysfunction Apert Syndrome Hypohidrotic Ectodermal Dysplasia 
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Copyright information

© Plenum Press, New York 1994

Authors and Affiliations

  • Guy Geoffroy
    • 1
  1. 1.Service de Neurologie, Département de PédiatrieHôpital Sainte-Justine pour les EnfantsMontréalCanada

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