RETINAL DEGENERATION IN HEREDITARY ATAXIA

Deterioration of vision is a not infrequent occurrence in cases of hereditary ataxia. Like the ataxic symptoms it is progressive and may become severe. According to the literature the deterioration of vision has been ascribed to optic atrophy in the majority of cases and only in a few instances are retinal changes or cataract described. It is obvious, however, that the actual findings have often been very slight and that in some cases the ophthalmoscopical picture has been recorded as normal despite a strong reduction in visual acuity (Bell and Carmichael, 1939; van Bogaert, 1948). The present study gives an account of five cases of hereditary ataxia associated with serious loss of vision. The patients have been subjected to careful examination including electro2SLux retinography(E.R.G.) _ in four of the cases. _ ___ This examination has revealed new facts that elucidate the 1O Lux problems associated A with the causation and progression of impairment of vision in this disorder.