Aplasia of the cerebellar vermis associated with chronic renal disease. A report of six cases and a review of the literature.
We report six patients with aplasia or hypoplasia of the cerebellar vermis with early symptoms consisting of psychomotor retardation, nystagmus and severely reduced visual acuity due to congenital amaurosis. At age 5.6-12.1 years, five of these patients developed symptoms of chronic renal failure due to renal maldevelopment.


CONCLUSIONS
Children presenting with vermis aplasia should be monitored for associated disorders, especially renal disease.
Keuth
B
B
Klinik und Poliklinik für Kinderheilkunde, Köln, Germany.
Alon
U
U
Fuchshuber
A
A
Michalk
D
D
Querfeld
U
U
D001766:Blindness / Q000209:etiology; D002531:Cerebellum / Q000002:abnormalities*; D002648:Child; D002675:Child, Preschool; D006801:Humans; D008607:Intellectual Disability / Q000150:complications; D007676:Kidney Failure, Chronic / Q000150:complications / Q000209:etiology*; D009759:Nystagmus, Pathologic / Q000209:etiology; D011596:Psychomotor Disorders / Q000209:etiology; D013577:Syndrome