[Congenital oculomotor apraxia. 2 cases with an electro-oculography study]

Congenital oculomotor apraxia is characterized by the inability of the young child to produce horizontal saccades when his head is fixed; two cases are reported. In a free condition this trouble is compensated by thrust head movements allowing the child to lead his gaze to the stimulus or to reach it by successive hypometric jerks released by movement. A blink usually precedes the eye movement probably allowing the child to escape from an excessive fixation. This apraxia is combined with a motor and psychological development delay. The evolution of all the troubles is usually good with minimal sequelaes (difficulty for reading). Without any anatomical argument, it may be assumed that a dysfunction of area 8 and of the fronto-mesencephalic pathway is responsible for this abnormality. The resemblance of this disturbance to an earlier phylogenic period of the oculomotor development (inferior mammalian) and its spontaneous good evolution evoke more a maturation delay of the supranuclear control system of saccades than a true disease.