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[Bilateral operculum syndrome].

作者信息

Lerman-Sagie T, Porat-Alkabetz E, Meir J J, Harel S

机构信息

Child Development Center, Dana Children's Hospital.

出版信息

Harefuah. 1996 Sep;131(5-6):161-3, 215.

PMID:8940497
Abstract

The bilateral operculum syndrome, is a unique developmental syndrome. It is characterized by spastic paralysis of the muscles of the face, pharynx, and of mastication, as well as by epilepsy and mental retardation. Imaging studies show bilateral, structural abnormalities in the frontal, perisylvian region consistent with polymicrogyria. These children are usually diagnosed as suffering from cerebral palsy, but in the bilateral operculum syndrome, intelligence is relatively preserved despite the severe motor involvement. Misdiagnosis may lead to improper estimation of rehabilitation potential preventing appropriate therapy, especially in the field of alternative communication. We present a 3-year-old boy, apparently the first case of this syndrome to be described in Israel.

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