Extensive brain calcification in two children with bilateral Coats' disease

We report two children with bilateral Coats' disease associated with cerebr al calcifications in the basal ganglia and deep white matter, asymptomatic at the time of their discovery. Cerebellar ataxia developed secondarily in one of them. Both children were born small for date and had febrile convuls ive seizures. Three similar patients have been previously reported, two of them in the same sibship: the third reported patient died of aplastic anemi a. Bilateral Coots' disease in children should prompt systematic CT scan in search of cerebral calcifications. If present, neurological and genetic pr ognosis should be cautious.