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  Vol. 44 No. 9, September 1987 TABLE OF CONTENTS
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Agenesis of the Corpus Callosum and Limbic Malformation in Apert Syndrome (Type I Acrocephalosyndactyly)

Guillermo A. de León, MD; Guillermo de León; Warren D. Grover, MD; Nayere Zaeri, MD; Philip D. Alburger, MD

Arch Neurol. 1987;44(9):979-982.


Abstract

• Agenesis of the corpus callosum and malformation of limbic structures are described in a patient with Apert syndrome, a disorder characterized by acrocephaly, severe syndactyly, and often, mental retardation. Including the present case, malformation of the corpus callosum and/or limbic structures apparently has been reported in a total of ten patients with the syndrome. Complete or partial agenesis of the corpus callosum was found in six patients, septal defects in three, and arhinencephaly and ammonic hypoplasia in one. Since malformation or limbic structures are, to our knowledge, a consistent feature of agenesis of the corpus callosum, it seems that limbic abnormalities could be important for the pathogenesis of mental retardation not only in Apert syndrome, but also in other acallosal patients.



Author Affiliations

From the Neuropathology Laboratory, Department of Neurology, Temple University School of Medicine (Dr de León and Mr de León), and the Departments of Pediatrics (Dr Grover), Anatomic Pathology (Dr Zaeri), and Surgery (Dr Alburger), The St Christopher's Hospital for Children, Philadelphia.


Footnotes

Accepted for publication June 8, 1987.

Reprint requests to Department of Neurology, Temple University Hospital, Broad and Ontario streets, Philadelphia, PA 19140 (Dr de León).



Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us   Add to Digg Digg   Add to Reddit Reddit   Add to Technorati Technorati     What's this?

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

Corpus Callosum Agenesis and Psychosis in Andermann Syndrome
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Arch Neurol 1991;48:1275-1280.
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Agenesis of the Corpus Callosum and Limbic Malformations Revisited
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Arch Neurol 1989;46:1270-1270.
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Agenesis of the Corpus Callosum in Apert Syndrome?
De Leon et al.
Arch Neurol 1989;46:479-479.
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Agenesis of the Corpus Callosum and Limbic Malformation in Apert's Syndrome
Jeret et al.
Arch Neurol 1989;46:10-10.
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