title: Movement-related cortical potentials in patients with Machado-Joseph disease.
abstract: OBJECTIVE
  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 -500ms) 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 500ms 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.
  
  
  CONCLUSIONS
  Abnormalities of pre- and post-movement MRCP components provide researchers with pathophysiological insight into voluntary motor dysfunction in MJD.
authors.affiliation: Neuroscience Laboratory, Department of Neurology, China Medical University Hospital, No. 2, Yuh-Der Road, Taichung 404, Taiwan.
authors.forename: Ming-Kuei
authors.initials: MK
authors.lastname: Lu
authors.forename: Hsu-Tzu
authors.initials: HT
authors.lastname: Shih
authors.forename: Kai-Ju
authors.initials: KJ
authors.lastname: Huang
authors.forename: Ulf
authors.initials: U
authors.lastname: Ziemann
authors.forename: Chon-Haw
authors.initials: CH
authors.lastname: Tsai
authors.forename: Fang-Chia
authors.initials: FC
authors.lastname: Chang
authors.forename: Ya-Chu
authors.initials: YC
authors.lastname: Chen
authors.forename: Yi-Tsung
authors.initials: YT
authors.lastname: Lin
authors.forename: Wei-Shih
authors.initials: WS
authors.lastname: Huang
authors.forename: Cheng-Chun
authors.initials: CC
authors.lastname: Lee
authors.forename: Chin-San
authors.initials: CS
authors.lastname: Liu
mesh_terms: D000328:Adult; D002540:Cerebral Cortex / Q000503:physiopathology*; D004569:Electroencephalography; D004576:Electromyography; D019054:Evoked Potentials, Motor / Q000502:physiology; D005260:Female; D006801:Humans; D017827:Machado-Joseph Disease / Q000503:physiopathology*; D008297:Male; D009043:Motor Activity / Q000502:physiology*