Oscillatory cortical drive to isometrically contracting muscle in Unverricht-Lundborg type progressive myoclonus epilepsy (ULD)

Objective: We investigated with whole-scalp magnetoencephalography (MEG) oscillatory cortical drive to isometrically contracting muscle in 8 genetically verified, and thus etiologically homogeneous, Unverricht-Lundborg type progressive myoclonus epilepsy (ULD) patients suffering from cortical myoclonus and generalized tonic-clonic seizures. The results were compared with those of 8 healthy control subjects. Methods: Cortical MEG signals were measured simultaneously with surface electromyography (EMG) during isometric contraction of the left and right first dorsal interosseus muscles. Cortex-muscle coherence and cross-correlograms between MEG and EMG signals were calculated as indicators of oscillatory cortical drive to muscle. The cortical areas involved in the maximum cortex-muscle coherence were also identified. Results: In patients, the strengths of the dominant coherent peaks were 2–4 fold compared with the healthy controls. Whereas the coherence was found strictly in the contralateral primary motor cortex in controls, additional coherent activity was observed ipsilaterally in 5 out of 8 patients. Conclusions: The remarkably increased MEG-EMG coherence in ULD patients suggests altered oscillatory cortical drive to the muscle during isometric contraction. We suggest that the enhanced cortex-muscle coherence in ULD patients reflects reduced inhibition in the motor cortex, and may contribute to disturbed voluntary movements.