Congenital ocular motor apraxia. Case reports and literature review

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.