Movement-related cortical potentials in patients with Machado-Joseph disease.
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.
Lu
Ming-Kuei
MK
Neuroscience Laboratory, Department of Neurology, China Medical University Hospital, No. 2, Yuh-Der Road, Taichung 404, Taiwan.
Shih
Hsu-Tzu
HT
Huang
Kai-Ju
KJ
Ziemann
Ulf
U
Tsai
Chon-Haw
CH
Chang
Fang-Chia
FC
Chen
Ya-Chu
YC
Lin
Yi-Tsung
YT
Huang
Wei-Shih
WS
Lee
Cheng-Chun
CC
Liu
Chin-San
CS
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*