1116 REYES' SYNDROME: EVIDENCE FOR ABNORMAL POLYAMINE (PA) METABOLISM

Abstract

Four children, ages 1-16, were admitted with a history of a prodrome viral illness and, after an interval, pernicious vomiting and the onset of disordered psychomotor activity. Abnormal liver function tests including blood ammonia were present in all during the course of their illness. Patients sampled for PAs were staged with respect to CNS status, I-IV, after Huttenlocher Serial serum-free PAs, namely spermine (SPM) and spermidine (SPD) were determined from serum sample aliquots by radioimmunoassay. All patients had elevated SPD values (mean peak value of 2.06 nanomol/ml) which declined rapidly to a post-admission value of 0.45 nanomol/ml in the 3 surviving children. Highest SPD value (4.20 nanomol/ml) was observed in the patient with the most severe neurological score (III) on admission. SPM levels, by contrast, were much lower and there was a 1-2 day delay in SPM peak development in the 3 surviving children. Clinical improvement paralleled the decline in serum PAs. PA administered I.V. to man causes prompt and persistent vomiting. Specific PA binding sites have been identified on animal brain cell membranes. SPM has been demonstrated to markedly reduce oxygen consumption by brain tissue. In vitro Na, K-ATPase activity is also depressed by PA addition. Parenteral PA doses to experimental animals may cause somnolence, ataxia, coma and death.

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