A 7-year-old boy with Duchenne muscular dystrophy and ADHD who developed acute hepatic failure with autoimmune hepatitis during treatment with pemoline (56 mg/day) is reported from the Children's Hospital, Cincinnati, OH. He presented with fever, vomiting, and jaundice, with thrombocytopenia and petechiae, after a period of decreased appetite. Ten-fold elevations of serum alanine aminotransferase (399 U/L) and aspartate aminotransferase (367 U/L) had been noted prior to pemoline treatment and 8 months prior to admission with hepatic failure. These may be ascribed to the muscle disease but could be consistent with pre-existing liver disease. On admission, these enzymes were markedly elevated, 1816 and 1313 U/L, respectively, and the total and direct bilirubin levels were 104 and 56 mcmol/L, respectively. Tests for hepatitis A and B were negative, but an autoimmune antibody panel, liver biosy findings, and coexistent diabetes were consistent with autoimmune hepatitis. After treatment with methylprednisolone, signs of encephalopathy gradually resolved, and at 2 year follow-up enzyme levels were moderately elevated (197 and 133, respectively) but one half the initial levels before pemoline administration. 
COMMENT. Since reports of acute liver failure associated with pemoline, the drug is no longer recommended as first-line therapy for ADHD. Pemoline has been linked to hepatic failure in 4 reported and a number of unreported cases. The liver toxicity is dose-dependent or due to an immune mediated hypersensitivity. The above case suggests an autoimmune process due to the drug, since pemoline withdrawal and treatment with prednisone resulted in a prompt response that was sustained after steroids were tapered. An analysis of pemoline associated hepatic failure (Shevell M, Schreiber R, Jan 1997) suggested that only one of the 4 reported cases appeared justified. Abbott Laboratories subsequently issued a warning and notice of further cases. (Ped Neur Briefs March 1997).