OPTIC ATROPHY AS THE FIRST SYMPTOM IN HALLERVORDEN-SPATZ-SYNDROME

A 16-year-old boy with the classic or postinfantile type of Hallervord en-Spatz syndrome is described. Bilateral optic atrophy with visual lo ss but without retinal changes was the only presenting symptom. Mild c ognitive impairment, behavioural disturbances and insidious extrapyram idal involvement appeared later. MRI showed marked symmetrical hypoint ensity of the globi pallidi and substantia nigra. This new observation suggests that the occurrence of optic atrophy in a patient with Halle rvorden-Spatz syndrome should be regarded as noncoincidental and stres ses the importance of an accurate neurological work-up in all adolesce nts with any unusual form of progressive optic atrophy.