Familial congenital ocular motor apraxia

Congenital ocular motor apraxia (COMA) is a unique ocular motor disorder which is characterized by a deficit in initiation of voluntary horizontal eye movement with reserved reflex eye movement. Although a portion of cases with COMA were found to be associated with other abnormalities, COMA in most patients is an isolated disorder. The most characteristic appearance of these patients is compensatory head thrusts which usually become less evident with increasing age. Since Cogan first described COMA in 1952, many cases have been reported. The majority of these occurred sporadically with only a few exceptions. We report on 4 patients with COMA. Two of them were siblings, and the other 2 patients were father and daughter. The ocular motility status is described in detail.