Congenital ocular motor apraxia: a neurodevelopmental and neuroradiological study

Abstract

Since its first description in 1952 neither the pathogenesis nor the localization of congenital ocular motor apraxia (COMA) have been cleared. There are reports indicating that COMA is more variable in its clinical expression and neuroradiological findings than previously assumed. In eight children with COMA neurological examination, developmental testing and neuroradiological investigation by CT or MRI were carried out. Ataxia was found in five, developmental delay in six of the patients. CT or MRI were normal in four children, the others had variable malformations such as hypoplasia of the cerebellum or corpus callosum, heterotopias of gray matter and maturational delays in the basal ganglia. No correlations between the clinical symptoms and the neuroradiological findings were found. The authors suggest that COMA is not an entity but rather a symptom, variably associated with malformations and maturational disorders of the brain.

Key Words:

• congenital ocular motor apraxia
• neuroimaging
• neurological and developmental disorders