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 a...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.展开更多
文摘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.