Pseudotumor cerebri in a boy with 11-beta-hydroxylase deficiency--a possible relation to rapid steroid withdrawal

A boy who was known to suffer from adrenogenital syndrome due to 11-beta-hydroxylase deficiency was treated with appropriate steroid replacement, which fully compensated for the deficiency. An intercurrent febrile illness with anorexia and vomiting necessitated an abrupt discontinuation of steroids. He presented with classic signs of pseudotumor cerebri 3 days following steroid withdrawal. Our studies imply that a combination of steroid withdrawal and dehydration with increased sodium urinary excretion, caused rapid fluid shifts within the brain, resulting in intracranial hypertension. At present, when a considerable number of children are on long-term steroids for various reasons, pseudotumor cerebri should be considered in the list of complications during abrupt steroid withdrawal.