Neurostimulation of the ventral intermediate thalamic nucleus in inherited myoclonus-dystonia syndrome.

We report on the effects of bilateral neurostimulation of the ventral intermediate thalamic nucleus (VIM) in a patient with medically intractable and progressing inherited myoclonus dystonia syndrome (IMDS). Postoperatively, the patient improved by approximately 80% on the modified version of a myoclonus score without any significant change in the dystonic symptoms. This suggests that neurostimulation of the VIM may be an effective treatment for myoclonus in pharmacologically intractable IMDS.