Only 8 cases of congenital ocular motor apraxia have been reported. All have been described as occurring in males.' The distinguishing features of this syndrome include: (1) absent or defective voluntary horizontal eye movements with retention of normal random versions; (2) characteristic jerking movements of the head to break fixation; (3) absent or defective quick phase of the optokinetic response with; (4) controversion of eyes when the head is turned on the vertical axis; (5) reading difficulties.
Acquired forms of this syndrome were described as early as 1908 by Wilson, and in 1909 by Balint; however, recognition of the congenital variety dates back only to 1952, when Cogan reported 4 cases in males.2 There have been at least 10 acquired cases reported3 and 8 of the congenital variety.1,4 Cogan has knowledge of other unreported cases.5 Thus, the condition may not be rare, but only relatively unrecognized.
CAMPBELL WL, ZELLER R. Congenital Ocular Motor Apraxia in Females. Arch Ophthalmol. 1961;66(5):643-645. doi:10.1001/archopht.1961.00960010645007