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A patient with myoclonus- dystonia syndrome was treated by implanting electr odes in the internal segment of the globus pallidus (GPi) and applying deep brai n stimulation. Surgery was done in two sessions. The most affected limb was trea ted first and the other limb one year later. Neuronal recordings showed that mos t pallidal neurones discharged in bursts at a relatively low firing rate (mean ( SD), 46 (18) Hz) compared with cells in the GPi in patients with Parkinson’ s d isease. Neurones modified the rate and mode of discharge with dystonic postures and rapid involuntary contractions of limb muscles. Neurological examination at 24 months after surgery showed a decline of 47.8% and 78.5% in the Burke- F ahn- Marsden and disability rating scales, respectively.
A patient with myoclonus-dystonia syndrome was treated by implanting electr odes in the internal segment of the globus pallidus (GPi) and applying deep brai n stimulation. Surgery was done in two sessions. The most affected limb was trea ted first and the other limbs One year later. Neuronal recordings showed that mos t pallidal neurones discharged in bursts at a relatively low firing rate (mean (SD), 46 (18) Hz) compared with cells in the GPi in patients with Parkinson ’sd isease. Neurones modified the rate and mode of discharge with dystonic postures and rapid involuntary contractions of limb muscles. Neurological examination at 24 months after surgery showed a decline of 47.8% and 78.5% in the Burke-F ahn-Marsden and disability rating scales, respectively.