Treatment of chorea with levetiracetam.

Editor, We read with interest the case report by D’Amelio et al. titled “Levetiracetam in the treatment of vascular chorea: a case report” [1]. The authors describe a 74-year-old patient with hemichorea from a presumed vascular etiology that markedly improved with levetiracetam. We also report the case of a woman who experienced acute chorea that was successfully treated with levetiracetam (LEV, Keppra). The patient is a 42-year-old woman who was referred to an academic movement disorders center for evaluation of involuntary, continuous flowing movements that she had developed suddenly in her extremities, trunk, and neck 5 years ago. The patient had suffered from insulin–dependent diabetes mellitus (IDDM) since age 5. She denied that the severity of the movements fluctuated with her blood glucose levels. Previous attempts to treat the involuntary movements with carbamazepine (Tegretol), baclofen (Lioresal), tizanidine (Zanaflex), and quetiapine (Seroquel) had failed. The patient’s medical history was remarkable for diabetic retinopathy. She had one brother who had been diagnosed with IDDM at age 6. Neither of her parents had childhood diabetes, although her father developed adult– onset diabetes. Her family history was negative for movement disorders or other neurological diseases. The patient was employed full time as a secretary. The patient’s physical and neurological examinations were unremarkable, with the exception of moderate to severe involuntary movements of her extremities, trunk, and neck that were consistent with chorea. Laboratory tests as part of an inflammatory and genetic work–up for chorea were normal, including sedimentation rate, antistreptolysin–O–titer, antinuclear antibodies, anticardiolipin antibodies, angiotensin–1–converting enzyme, ceruloplasmin, Huntington’s disease genetic test, thyroid function tests, CPK, C–reactive protein concentration, rheumatoid factor, complement 3/complement 4, and lupus anticoagulant. Magnetic resonance imaging of the brain revealed an infarct in the right cerebellum. Carotid ultrasound and electrocardiogram were normal. The patient was treated with LEV, starting with 250 mg once a day and increasing to 250 mg twice daily 4 days later. Within 7 days, she noticed a moderate improvement in the chorea. LEV was increased to 250 mg in the morning and 500 mg in the afternoon, with almost complete resolution of chorea. There were no side effects from LEV. The etiology of the acute generalized chorea that our patient experienced is unclear and may have been caused by an autoimmune mechanism. LEV (S–enantiomer of alpha–ethyl–2–oxo–1–pyrrolidine acetamide) is an antiepileptic medication that is approved as monotherapy or as an adjunct to treat partial–onset seizures at doses of 1,000– 3,000 mg/day. Preliminary animal studies and additional case reports indicate that LEV may reduce fever-induced chorea [2], paroxysmal choreoathetosis [3], and levodopainduced dyskinesia [4]. The case report by D’Amelio et al. and our report further indicate that LEV may be effective in treating chorea from a variety of etiologies. We agree that controlled trials are needed to evaluate its safety and efficacy for this purpose.