title: Preprogramming motor dysfunction in paroxysmal kinesigenic choreoathetosis.
abstract: Paroxysmal kinesigenic choreoathetosis (PKC) is characterized by abnormal involuntary movements precipitated by sudden movement. As a result, a possible impairment of cerebral organization of voluntary motor activity is hypothesized in PKC. We examined a 14-year-old boy affected by a sporadic form of PKC, adopting a multimodal psychophysiological approach, including P300, contingent negative variation (CNV) and a specific paradigm for the study of movement related potentials (MRPs). Recordings were made before and after phenobarbital therapy. No changes were observed in the non-motor parameters (P300 and early wave of the CNV), whereas the premotor CNV component and the electrophysiological components, reflecting the preprogramming activity of a voluntary motor act, showed selective modifications induced by the anticonvulsant therapy. Our PKC patient presents a disorder of temporal organization of a voluntary motor response to a stimulus. Both a clinical improvement and normalization of motor-related electrophysiological anomalies were observed during phenobarbital (PB) therapy.
authors.affiliation: Department of Neurology and Otolaryngology, University "La Sapienza", Rome, Italy. francesco.fattapposta@uniroma1.it
authors.forename: Francesco
authors.initials: F
authors.lastname: Fattapposta
authors.forename: Filomena
authors.initials: F
authors.lastname: My
authors.forename: Donatella
authors.initials: D
authors.lastname: Valente
authors.forename: Rodolfo
authors.initials: R
authors.lastname: Quadrini
authors.forename: Carmelo
authors.initials: C
authors.lastname: D'Alessio
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Amabile
mesh_terms: D000293:Adolescent; D001264:Athetosis / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology*; D002819:Chorea / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology*; D003265:Contingent Negative Variation / Q000502:physiology; D004594:Electrophysiology; D018913:Event-Related Potentials, P300 / Q000502:physiology; D019054:Evoked Potentials, Motor / Q000502:physiology; D006801:Humans; D008297:Male; D009069:Movement Disorders / Q000503:physiopathology; D010634:Phenobarbital / Q000627:therapeutic use
chemical_list: D010634:Phenobarbital