Local field potentials and oscillatory activity of the internal globus pallidus in myoclonus-dystonia.

The pathophysiology of myoclonus-dystonia (M-D), an autosomal dominantly inherited movement disorder characterized by myoclonic jerks and dystonic contractions, is largely unknown. In the present study, local field potential (LFP) activities in the globus pallidus internus (GPi) from two genetically proven M-D patients are investigated. Coherence analysis between GPi LFP activity and electromyographic muscle activity (EMG) and synchronization of GPi neuronal activity using event-related spectral perturbation (ERSP) in a go-no-go paradigm were studied. Significant increased coherence in the 3 to 15 Hz frequency band was detected between GPi LFP activity and several muscles, with the LFP leading the muscles. The ERSP analysis revealed synchronization in the 3 to 15 Hz frequency band within the GPi before the imperative cue of the go-no-go task and desynchronization in the same band after the cue. The LFP recordings of the GPi in M-D show that the low-frequency band previously described in dystonia is also involved in the dystonia plus syndrome M-D. The 3 to 15 Hz synchronization in the go-no-go paradigm has not been described previously and may point to the existence of (myoclonus-)dystonia specific oscillatory activity in the GPi.