Title
Movement-related desynchronization-synchronization (ERD/ERS) in patients with Unverricht-Lundborg disease.
Abstract
We studied changes in event-related desynchronization/synchronization (ERD/ERS) patterns in patients with Unverricht–Lundborg disease (ULD), presenting with prominent action myoclonus. We analyzed the movement-related ERD/ERS in α and β frequency bands in 15 patients using self-paced finger extension as a motor paradigm and we compared the results with those obtained in 12 healthy volunteers. In all ULD patients, α- and β-ERD regularly occurred with onset and location similar to that found in healthy controls, but the desynchronization of α activity was significantly greater than in controls (C3: −64.4±9.8% vs. −49.7±14.8%; p=0.004). Moreover, in the patients, both α- and β-ERD spread toward frontal electrodes. In controls, the post-movement β-ERS regularly occurred; it was absent in eight patients with severe action myoclonus, while, in seven patients with mild or moderate myoclonus, the β-peak was significantly smaller with respect to that measured in controls (55.6±15.1% vs. 153.9±99.8%, p=0.006). The failure of β-ERS well-correlated with motor impairment resulting from action myoclonus, whereas SSEPs and long-loop reflexes performed to detect signs of cortical hyperexcitability showed inconsistent changes. In ULD patients, ERD/ERS changes indicate an increased activation of motor cortex during movement planning and a great reduction of post-excitatory inhibition of motor cortex. The changes involving β-ERS had a significant relationship with the functional disability in individual patients and might play a pathogenic role in the motor dysfunction.
Year
DOI
Venue
2006
10.1016/j.neuroimage.2006.06.036
NeuroImage
Keywords
Field
DocType
Action myoclonus,ERD/ERS,EEG,Progressive myoclonus epilepsies
Myoclonus,Motor impairment,Developmental psychology,Action myoclonus,Psychology,Reflex,Movement planning,Motor cortex,Electroencephalography,Unverricht–Lundborg disease
Journal
Volume
Issue
ISSN
33
1
1053-8119
Citations 
PageRank 
References 
1
0.43
0
Authors
8
Name
Order
Citations
PageRank
E Visani110.43
P Agazzi210.43
L Canafoglia310.43
F Panzica410.43
C Ciano510.43
V Scaioli610.43
G Avanzini710.43
S Franceschetti810.43