Congenital ocular motor apraxia: An inability to unlock the vestibulo-ocular reflex

Abstract

The authors report ten cases of congenital ocular motor apraxia. Two patients had Cockayne's syndrome. One patient had succinic semialdehyde dehydrogenase deficiency. One patient had vermian hypoplasia of the cerebellum. Four patients had precocious head movements with abnormal limb tremor in the first two days of life.Based on their findings the authors postulate that head thrusts are the result of a failure to unlock the vestibulo-ocular reflex from the object of regard rather than a strategy to assist horizontal gaze.