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 Volume 59, Number 12, December 24, 2002
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Neurology 2002;59:1968-1971
© 2002 American Academy of Neurology
Brief Communications

MRI shows abnormal white matter maturation in classical holoprosencephaly

A.J. Barkovich, MD, E.M. Simon, MD, O.A. Glenn, MD, N.J. Clegg, PhD, S.L. Kinsman, MD, M. Delgado, MD and J.S. Hahn, MD

From the Departments of Diagnostic Radiology (Drs. Barkovich and Glenn) and Neurology and Pediatrics (Dr. Barkovich), University of California, San Francisco; Department of Radiology (Dr. Simon), Children’s Hospital of Philadelphia, PA; and The Carter Centers for Brain Research in Holoprosencephaly and Related Malformations (Drs. Barkovich, Simon, Clegg, Kinsman, Delgado, and Hahn), Baltimore, MD, Dallas, TX, and Stanford, CA.

Address correspondence and reprint requests to Dr. A.J. Barkovich, Division of Neuroradiology, L 371, University of California, San Francisco, 505 Parnassus Avenue, Box 0628, San Francisco, CA 94143-0628; e-mail: jimb{at}radiology.ucsf.edu

In an attempt to assess white matter maturation in holoprosencephaly (HPE), MRI scans of 47 patients with HPE were retrospectively reviewed. White matter maturation was delayed in 25/47 patients, including 24/29 patients with classic HPE who were <=12 months old at the time of imaging. Of interest, all six patients with the middle interhemispheric (MIH) variant of HPE had normal myelination, regardless of age. This study provides further evidence that the classic and MIH variants of HPE have different underlying causes.




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M. C. Patterson
Holoprosencephaly: The face predicts the brain; the image predicts its function
Neurology, December 24, 2002; 59(12): 1833 - 1834.
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