Background: Congenital ocular motor apraxia (COMA) is characterized by the inability to generate volitional horizontal saccadic eye movements in the absence of other focal neurologic abnormalities. Subjects: We report on two children (ages 5 months and 3 years) whose COMA did not adhere to these classic criteria. The children were followed up clinically with serial ocular motor examinations and neuroimaging over a period of 3 years. Results: In the first child horizontal COMA was associated initially with neonatal communicating hydrocephalus. Two and one half years after the first signs of COMA, a fourth ventricle medulloblastoma appeared. The second child, who recovered from a periventricular hemorrhage caused by perinatal asphyxia, manifested vertical COMA and compensatory vertical head thrusts. Conclusions: COMA may be associated with hydrocephalus, pontocerebellar tumor, and periventricular hemorrhage. These rare variants of COMA emphasize that the eye movement deficits may arise from several locations, cerebral as well as pontocerebellar, in the neuronal pathways generating saccades.
Bibliographical noteFunding Information:
From the Depaf~tment of Ophthalmology and Visual Sciences,~ the Mallinckrodt Institute of Radiology, b and the Department of Neurology, c St. Louis Children's Hospital, Washington University School of Medicine, St. Louis, Missouri. Supported by National Institutes of Health program project grant No. O1 NS 17763-I 2 (L. T.), National lnstitutes of Health grant No. R O1 EYI O2 14-O1A 3 (L. T.), a McDonnell Center for Higher Brain Function Research Grant (I.A.), Knights Templar Eye Foundation Research Grant (I.d.), and in part by National Institutes of Health grant No. EY02687 and an unrestricted grant to the Department of Ophthalmology and Visual Sciences from Research to prevent Blindness, Inc. Reprint requests: L. Tychsen, MD, St. Louis Children's Hospital, Washington University School of Medicine, One Children's Place, Room 2 South 89, St. Louis, MO 63110. Copyright © 1997 by the American Assodation for Pediatric Ophthalmology and Strabismzar. 1091-8531/97 $5.00 + 0 75/1/84884