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Clinical Pediatrics
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Congenital Ocular Motor Apraxia

Case Reports and Literature Review

Rhandy PeBenito, MD

Stanley S. Lamm Institute for Child Neurology and Developmental Medicine and the Department of Pediatrics, The Long Island College Hospital, Brooklyn, New York

Joan B. Cracco, MD

Department of Neurology (Child Neurology), Health Science Center at Brooklyn, State University of New York, Brooklyn, New York

Two children with congenital ocular motor apraxia (C-OMA) associated with congenital malformations of the central nervous system (CNS) are presented, and the literature is reviewed. C-OMA is an abnormality of ocular motility characterized by defective or absent voluntary horizontal gaze, associated with a characteristic head thrust. Although not rare, it is infrequently recognized. C-OMA is not a specific disease entity, but a sign. It may be associated with other static congenital CNS disorders and must be distinguished from acquired ocular motor apraxias (A-OMA) seen in progressive brain disorders and in certain serious systemic diseases. Therefore, when movements typical of C-OMA are observed, a careful systemic and neurologic examination should be performed, including immunoglobulin screening, cranial computerized tomography scanning, and adequate follow-up. Close relatives also should be examined.

Clinical Pediatrics, Vol. 27, No. 1, 27-31 (1988)
DOI: 10.1177/000992288802700105
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