Variants of congenital ocular motor apraxia: associations with hydrocephalus, pontocerebellar tumor, and a deficit of vertical saccades.
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.


METHODS
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.
Anteby
I
I
Department of Ophthalmology and Visual Sciences, St. Louis Children's Hospital, Washington University School of Medicine, Missouri 63110, USA.
Lee
B
B
Noetzel
M
M
Tychsen
L
L
D001072:Apraxias / Q000150:complications* / Q000151:congenital / Q000503:physiopathology*; D001237:Asphyxia / Q000150:complications; D002543:Cerebral Hemorrhage / Q000150:complications; D002551:Cerebral Ventricle Neoplasms / Q000150:complications / Q000175:diagnosis; D002675:Child, Preschool; D006801:Humans; D006849:Hydrocephalus / Q000150:complications / Q000000981:diagnostic imaging; D007223:Infant; D007231:Infant, Newborn; D007232:Infant, Newborn, Diseases; D008279:Magnetic Resonance Imaging; D008297:Male; D008527:Medulloblastoma / Q000150:complications / Q000175:diagnosis; D009801:Oculomotor Muscles / Q000503:physiopathology*; D014057:Tomography, X-Ray Computed