Bilateral arachnoid cysts of the temporal fossa in four children with glutaric aciduria type I.

Glutaric aciduria type I is an uncommon inborn error of metabolism. It is a serious disease, often with a fatal outcome. This study reports the presence of bilateral temporal fluid collections, probably bilateral arachnoid cysts, in association with glutaric aciduria type I. The CT and, when available, MR studies from five patients with this disorder were reviewed. Four of the patients had findings consistent with bilateral arachnoid cysts of the temporal fossa. This is a rare occurrence, with only 11 such cases reported in the literature. The observed association between temporal fluid collections and glutaric aciduria type I suggests that patients with bilateral arachnoid cysts should be investigated for this metabolic disorder.