VALPROATE-INDUCED SYSTEMIC LUPUS-ERYTHEMATOSUS IN A PATIENT WITH PARTIAL TRISOMY OF CHROMOSOME-9 AND EPILEPSY

We report a mentally retarded 30-year-old woman with partial trisomy o f chromosome 9 (46,XX-6,+der(6)t(6,9)pat) who has had epilepsy since a ge 11 months. She had been treated with various combinations of drugs. After 1 year of treatment with valproate (VPA) and ethosuximide (ESM) , the patient developed arthralgias, muscle weakness, fatigue, and fev er. Laboratory examination showed increased sedimentation rate, hyperg ammaglobulinemia, and high titers of antinuclear antibodies (ANA). The possibility of VPA-induced systemic lupus erythematosus (SLE) was con sidered. This diagnosis was supported by detection of antihistone anti bodies and the HLA-DR4 antigen. VPA dosage was tapered and discontinue d, with accompanying resolution of clinical, immunological and hematol ogical signs of SLE 6 weeks after VPA discontinuation. This is the fou rth reported case of VPA-induced SLE.