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NEUROLOGY 1998;50:1143-1146
© 1998 American Academy of Neurology

X-linked female band heterotopia-male lissencephaly syndrome

M. J. Berg, MD, G. Schifitto, MD, J. M. Powers, MD, C. Martinez-Capolino, C-T. Fong, MD, G. J. Myers, MD, L. G. Epstein, MD and C. A. Walsh, MD

From the Departments of Neurology (Drs. Berg, Schifitto, Myers, and Epstein, and C. Martinez-Capolino), Pathology (Dr. Powers), and Pediatrics(Dr. Fong), University of Rochester Medical Center, Rochester, NY; and Division of Neurogenetics (Dr. Walsh), Department of Neurology, Beth Israel Hospital, Harvard Medical School, Boston, MA.

Address correspondence and reprint requests to Dr. Michel J. Berg, University of Rochester Medical Center, Department of Neurology, Box 673, 601 Elmwood Ave., Rochester, NY 14642-8673.

We report a family with band heterotopia in a mother and daughter and lissencephaly in a son (X-linked inheritance pattern). Postmortem examination of the boy revealed classical lissencephaly and, among other findings, simplified and discontinuous inferior olives without inferior olivary heterotopia. The absence of inferior olivary heterotopia may distinguish X-linked lissencephaly from other conditions with classic lissencephaly such as Miller-Dieker syndrome.

Received March 25, 1997. Accepted in final form September 12, 1997.




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