A 30-year-old man with long-standing localization-related epilepsy and
mental retardation had seizures that were partially controlled with v
alproate (VPA) 500 mg four times daily. Routine examination showed sev
ere thrombocytopenia with mild leukopenia and chronic low-grade hemoly
tic anemia. Pertinent laboratory results included positive ANA, rheuma
toid factor, anti-NIA, circulating immune complexes, and antihistone a
ntibody.The patient was treated with high dosage prednisone with parti
al improvement, but continued to have exacerbations at lower dosages.
Fourteen months later, VPA was discontinued, and rapid improvement ens
ued. Prednisone was subsequently discontinued, and the patient has now
maintained normal platelet counts for 18 months.