APLASIA OF THE CEREBELLAR VERMIS ASSOCIATED WITH CHRONIC RENAL-DISEASE - A REPORT OF 6 CASES AND A REVIEW OF THE LITERATURE

We report six patients with aplasia or hypoplasia of the cerebellar ve rmis with early symptoms consisting of psychomotor retardation, nystag mus and severely reduced visual acuity due to congenital amaurosis. At age 5.6-12.1 years, five of these patients developed symptoms of chro nic renal failure due to renal maldevelopment. Conclusion Children pre senting with vermis aplasia should be monitored for associated disorde rs, especially renal disease.