Saccades, vergence and combined eye movements in a young subject with Congenital Central Hypoventilation Syndrome (CCHS).

PURPOSE: To study saccades, vergence and combined eye movements in a case of Congenital Central Hypoventilation Syndrome.

METHODS

A 16-year-old girl with congenital partial third cranial nerve palsy, with ptosis and divergent strabismus dependent on viewing distance, participated in this study. A first operation for her ptosis was done seven years ago and an operation for her strabismus was done four years ago. The day of our oculomotor test, the patient had a residual exotropia of 12 prism diopters, mild amblyopia of the left eye and no binocular vision. LEDs on a table placed at eye level were used to stimulate saccades, pure vergence along the median plane and combined saccade-vergence movements. Horizontal eye movements from both eyes were recorded simultaneously with a photoelectric device (Bouis Oculometer).

RESULTS

The binocular coordination of saccades in this subject was unstable and more variable than normal, and there was divergent post-saccadic drift, i.e., in the direction of her residual deviation. The patient had difficulty making movements in space, particularly vergence eye movements. Pure saccades and combined movements showed abnormally long latencies and marked hypometrias. The velocity of pure saccades was normal. In contrast, the velocity of saccades in the combined movements was abnormally slow.

CONCLUSION

The long latency and the low accuracy of the eye movements in natural space indicate a general deficit in the central circuitry that controls the initiation and programming of all these types of eye movements. The absence of pure vergence and the slowness of the saccades in the combined movements could be due to a brainstem deficit specific to the vergence oculomotor system.