Abstract

DE BENITO, L et al. Comments on Cogan's syndrome (congenital ocular motor apraxia) based on a case. Arch Soc Esp Oftalmol [online]. 2004, vol.79, n.4, pp.189-191. ISSN 0365-6691.

Case: A 4-month-old girl was seen by a paediatrician for psychomotor delay and defective visual fixation. In order to shift gaze direction, she had to jerk her head in order to drag her eyes in the direction she wanted to look. She also showed non-specific pyramidal impairment. Herpes human virus type 6 (HHV-6) was amplified by PCR. No other findings could explain her troubles with fixation. She was diagnosed with congenital ocular motor apraxia (C.O.M.A.). Discussion: When C.O.M.A. is suspected, other causes of defective fixation and abnormal cephalic movements must be ruled out through the use of physical examination, electrophysiology and neuroimaging: troubles with ocular motility, poor vision or neurological processes. There have been cases associated with neurological misdevelopment, systemic diseases, metabolic deficits and chromosomic abnormalities.

Keywords : Cogan’s syndrome; congenital ocular motor apraxia; head thrust; saccadic movements.