Lu, M.-K.M.-K.LuFANG-CHIA CHANG et al.2018-09-102018-09-102008http://www.scopus.com/inward/record.url?eid=2-s2.0-41449083857&partnerID=MN8TOARShttp://scholars.lib.ntu.edu.tw/handle/123456789/337681Objective: Movement-related cortical potentials (MRCP; nomenclature of MRCP components according to Shibasaki and Hallett (Shibasaki H, Hallett M. What is the Bereitschaftspotential? Clin Neurophysiol 2006;117:2341-56) were studied in patients with Machado-Joseph disease (MJD) to elucidate the pathophysiology of voluntary movement. Methods: We studied nine genetically proven MJD patients and eight age-matched healthy subjects. Multi-channel electroencephalogram (EEG) recordings were obtained during self-paced fast extensions of the wrist. EEG epochs were time-locked to electromyography (EMG) onset or offset of the voluntary EMG burst and averaged. Results: In the MJD patients, the early Bereitschaftspotential (early BP, -1500 to -500 ms) was not affected but the late BP was reduced over the central midline area and contralaterally to the movement side. The amplitude of the fpMP, a post-movement MRCP component, was also reduced. In addition, the offset cortical potential in the first 500 ms after EMG offset (Moff + 500) was attenuated bilaterally over a wide cortical area. Conclusions: Findings suggest that cortical activations associated with the initiation and termination of a voluntary movement are impaired in MJD patients. Significance: Abnormalities of pre- and post-movement MRCP components provide researchers with pathophysiological insight into voluntary motor dysfunction in MJD. ? 2008 International Federation of Clinical Neurophysiology.Bereitschaftspotential; Cerebello-thalamo-cortical circuit; Machado-Joseph disease (spinocerebellar ataxia type 3); Movement-related cortical potential; Offset cortical potential; Post-movement cortical potential[SDGs]SDG3adult; article; clinical article; contingent negative variation; controlled study; electroencephalogram; electromyography; evoked muscle response; female; human; Machado Joseph disease; male; motor dysfunction; motor performance; pathophysiology; priority journal; voluntary movement; wrist; Adult; Cerebral Cortex; Electroencephalography; Electromyography; Evoked Potentials, Motor; Female; Humans; Machado-Joseph Disease; Male; Motor Activityjournal article10.1016/j.clinph.2008.01.008