Long-term consequences of Reye syndrome: a sibling-matched, controlled study of neurologic, cognitive, academic, and...

Sixteen survivors of Reye syndrome treated at Yale-New Haven Hospital between January, 1977, and December, 1978, received neurologic, neuromaturational, cognitive, educational, and psychiatric assessments; 12 had siblings who were also evaluated. In the RS cohort, 13 of 16 patients were in stage 3/5 coma and intracranial pressure was monitored in 12 of 16 for 4.7 days. Blood ammonia concentration was greater than 300 micrograms/ml in 13 of 16 patients with a mean peak 438. Abnormalities on neurologic examination were noted in eight RS children and in none of the siblings. No significant differences emerged on psychometric testing of RS with siblings (12 children) or on group differences (16 RS children as a group compared to 12 siblings as controls). A significant difference was noted for those four children with onset of RS under age 7 years compared to their siblings (IQ 108 vs 134). The sibling IQ-RS IQ difference was significantly correlated with age of onset of RS. Individual Full Scale IQ scores and the sibling-RS IQ differences were also correlated with severity of RS. Similar findings were observed for educational testing. Eleven of the RS children received a psychiatric diagnosis (attention deficit disorder or anxiety reaction) compared to two of the control children. Five of the RS children had experienced a significant recent life stress. As a group, children with RS remain remarkably intact; however, those with most severe RS or who were very young when affected may have some sequelae.