
Saccades are quick, simultaneous movements of both eyes in the same direction and are abnormal in the condition of oculomotor apraxia (OMA). Most children with OMA must utilize a head thrust to initiate horizontal eye movement away from the straight-ahead gaze position. Typically, vertical eye movements are unaffected by the condition.
The source of OMA is in the brain. The process of initiating eye movements is a complicated neural pathway involving many different structures. Neuroimaging with magnetic resonance imaging (MRI) is commonly performed when evaluating OMA. Findings may be normal or may reveal poor development of regions of the brain, in particular the corpus callosum, cerebellum, and/or fourth ventricle.
Extensive investigations sometimes reveal no associated developmental issues. However, children with OMA often have developmental delays and possess low muscle tone (hypotonia). Speech, reading, and motor delays are common even when neuroimaging studies are normal.
The wide range of clinical entities that have been reported in children with OMA include: agenesis of the corpus callosum, Joubert syndrome, Dandy-Walker malformation, microcephaly, hydrocephalus, vermis hypoplasia, porencephalic cyst, megalocephaly, Krabbe’s leucodystrophy, Pelizaeus Merzbacher disease, Infantile Gaucher disease, GM1 gangliosidosis, Infantile Refsum’s disease, propionic academia, ataxia telangiectasia, Bardet-Biedl syndrome, vermis astrocytoma, vermis cyst, carotid fibromuscular hypoplasia, Cornelia de Lange syndrome, and microphthalmos.
The etiology of OMA is usually not determined and considered idiopathic. However, the condition is sometimes considered secondary and attributed to insults occurring either during the perinatal period or the first 6 months of life. Associations may include perinatal hypoxia, meningitis, periventricular leucomalacia, cerebral palsy, septicemia, anemia, herpes encephalitis, and seizure disorder.
There are no reports of siblings with OMA. It would be extremely rare if other children were born with the same condition.
There is no specific treatment for OMA. Serial ophthalmologic examinations are recommended to monitor for other eye problems associated with OMA.
There are few reports of the long-term prognosis of children born with OMA. The head thrusts associated with OMA typically diminish over time, but tend not to completely disappear. This may represent a true improvement in the disorder or be an adaptive compensatory mechanism to mask the head thrust.
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