Cogan (2) syndrome (oculomotor apraxia syndrome; wieacker syndrome)    263

General: X-linked; oculomotor apraxia and muscle atrophy; prevalent in males; corpus callosum can be hypoplastic.

Ocular: Rapid and frequent blinking; conjugate palsy; congenital oculomotor apraxia with patient unable to move eyes voluntarily to one side but with otherwise normal ocular movements; patient fixes objects by head tilt and turning, which causes further ocular deviation via the vestibular reflex; compensation for this overshoot is accomplished by some jerky eye movements with final fixation possible and gradual return of the head to the primary position; may be associated with abnormal electroretinographic responses.

Clinical: Slow progression, predominantly distal muscle atrophy; congenital contracture of feet; dyspraxia of face and tongue muscles; mild mental retardation.

Borchert MS, et al. Congenital oculomotor apraxia: findings with magnetic resonance imaging. J Clin Neural Ophthalmol 1987; 7:104.

Cogan DG. Type of congenital ocular motor apraxia presenting jerky head movement. Jackson Memorial Lecture. Trans Am Acad Ophthalmol Otolaryngol 1952; 56:853.

Magni R, et al. Electroretinographic findings in congenital oculomotor apraxia (Cogan's syndrome). Doc Ophthalmol 1994; 86:259-266.

Vassella F, et al. Cogan's congenital oculomotor apraxia in two successive generations. Dev Med Child Neurol 1972; 14:788.