A mentally retarded 30-year-old woman with partial trisomy of chromosome 9, suffering from epilepsy since age 11 months, developed systemic lupus erythematosus after one year of treatment with valproate (VPA) and ethosuximide (ESM) at the Clinica Neurologica, Universita di Roma Tor Vergata, Italy. When prednisone 1 mg/kg/day was administered and VPA gradually discontinued, clinical remission occurred within 10 days. The patient was maintained on ESM without relapse. 
COMMENT. This was the fourth reported case of VPA-induced systemic lupus erythematosus. It presented with arthralgia, fever, and fatigue, after prolonged treatment. It resolved rapidly after discontinuing the drug.