Hereditary Isolated Glucocorticoid Deficiency Associated with Reye's Syndrome• 506

The hallmarks of Reye's Syndrome which include hyperammonemia, elevated liver enzymes and progressive encephalopathy, have been associated with certain inherited metabolic disorders, thus, current recommendations are to screen atypical Reye's Syndrome cases for defects in amino acid, organic acid and carbohydrate metabolism. Hereditary isolated glucocorticoid deficiency(HIGD) is characterized by undetectable cortisol levels, normal mineralocorticoids and elevated ACTH levels while molecular analysis has demonstrated a point mutation in the MC2R gene of the ACTH receptor in some cases. The presenting symptoms of HIGD in early childhood have been hypoglycemia and/or hyperpigmentation. A 3 5/12 year male with expressive language delay and at least one previous unexplained episode of ataxia had a metabolic evaluation which yielded normal levels of plasma carnitine and non diagnostic urine for amino and organic acids. At 3 10/12 years he presented with 24 hours of vomiting followed by diarrhea and seizures. No aspirin had been given. He was lethargic and stimulation produced asymmetric decorticate posturing. Initial glucose was 28 mg/dl, maximal AST 163 U/L (