Abstract
A case of 16-year-old male who had congenital blindness, severe mental and physical handicaps and polydipsia, was studied clinically and neuropathologically.
At autopsy, kidneys showed nephrosclerotic changes due to arteriosclerosis and agenesis of vermis were observed in the cerebellum.
Six cases of the congenital polycystic kidney syndrome associated with both congenital blind ness and agenesis of vermis have been reported by Dekaban et al., Heffner et al. and Koya et al. In this case, agenesis of vermis and congenital blindness were similarly recognized. It is inter esting, however, that the renal change was that of arteriosclerotic nephrosclerosis which was not described in the above-mentioned reported cases.
The present case was examined and discussed in connection with the vermic agenesis, and also regarding the renal lesion in relation to the congenital polycystic kidney syndrome observed in the reported cases.
