title: Oxcarbazepine-responsive paroxysmal kinesigenic dyskinesia in Wilson disease.
abstract: A 22-year-old man presented with a 2-year history of tremor of the upper limbs associated to behavioral disorders. A magnetic resonance imaging of the brain showed hyperintensity in the right frontoparietal region, basal ganglia, particularly in the caudate nucleus, midbrain, and pons in T2 sequences, fluid-attenuated inversion recovery, and diffusion. Serum ceruloplasmin levels were 4 mg/dL (range, 20-45 mg/dL), and 24-hour urine cooper excretion was increased up to 223 µg (10-40 µg/24 hours). Slit lamp examination demonstrated the presence of a Kayser-Fleischer ring and penicillamine treatment started. Four months later, he developed episodes of paroxysmal dystonic posturing of his left arm, which increased in frequency reaching 2 or 3 attacks per hour. They were triggered by voluntary movements and forced him to adopt an abnormal flexion of the left forearm over the left bicep and were preceded by a tightening sensation of the left forearm muscles. Episodes completely remitted with oxcarbazepine.
authors: Department of Neurology, University of Buenos Aires, Argentina. fmicheli@fibertel.com.ar
authors: Federico
authors: F
authors: Micheli
authors: Lorena
authors: L
authors: Tschopp
authors: Maria Graciela
authors: MG
authors: Cersosimo
mesh_terms: D002220:Carbamazepine / Q000031:analogs & derivatives* / Q000627:therapeutic use; D004421:Dystonia / Q000139:chemically induced / Q000175:diagnosis* / Q000188:drug therapy; D006527:Hepatolenticular Degeneration / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D008297:Male; D009069:Movement Disorders / Q000175:diagnosis / Q000188:drug therapy; D000078330:Oxcarbazepine; D010396:Penicillamine / Q000009:adverse effects*; D055815:Young Adult
chemical_list: D002220:Carbamazepine; D010396:Penicillamine; D000078330:Oxcarbazepine