BILATERAL ARACHNOID CYSTS OF THE SYLVIAN REGION IN FEMALE SIBLINGS WITH GLUTARIC ACIDURIA TYPE-I - REPORT OF 2 CASES

Two sisters, aged 6 and 21/2 years, presented with macrocephaly and de layed motor development and were found on computerized tomography to h ave bilateral arachnoid cysts of the sylvian region. Cystoperitoneal s hunting of the larger cysts resulted in considerable neurological impr ovement in both children. Subsequent screening of the patients' urine for organic acids showed that the two sisters were suffering from glut aric aciduria type I(GA-I). To the best of the authors' knowledge, thi s is the first report to provide clear radiological and surgical evide nce of the association between GA-I and bilateral arachnoid cysts of t he sylvian region. The role of the shunting procedure used in these ca ses is discussed.