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: Francesco Fattapposta, Filomena My, Donatella Valente, Rodolfo Quadrini, Carmelo D'Alessio, Giuseppe Amabile
• Journal: Functional neurology (Funct Neurol) Vol. 18 Issue 1 Pg. 29-34 ( 2003) ISSN: 0393-5264 [Print] Italy
• PMID: 12760411 (Publication Type: Case Reports, Journal Article)

Chemical References

• Phenobarbital

Topics

• Adolescent
• Athetosis (diagnosis, drug therapy, physiopathology)
• Chorea (diagnosis, drug therapy, physiopathology)
• Contingent Negative Variation (physiology)
• Electrophysiology
• Event-Related Potentials, P300 (physiology)
• Evoked Potentials, Motor (physiology)
• Humans
• Male
• Movement Disorders (physiopathology)
• Phenobarbital (therapeutic use)