Oculomotor-corpus callosum dysplasia.

An infant with congenital bilateral ophthalmoplegia with levator and pupillary sparing is presented. The eyes are fixed in a divergent position with no apparent motility. The baby is otherwise clinically normal and is developing in a normal fashion except for delayed growth pattern. Visual attention is present and he fixates with either eye. Computed tomography demonstrates an associated dysplasia of the corpus callosum and an abnormal ventricular system. Neuroendocrine studies performed at one year of age demonstrate subnormal levels of growth hormone. It is postulated that this represents an embryodysgenesis involving the developing mesencephalic tegmentum (oculomotor nuclei) and the diencephalic lamina reuniens (corpus callosum). It is the first reported case of congenital ophthalmoplegia with corpus callosum dysplasia. The "embryodysgenic" relationship with other forebrain-ocular anomalies has been alluded to and remains speculative.