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.

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The intra-operative use of neurophysiological techniques allows reliable identification of the sensorimotor region, and constitutes a prerequisite for its anatomical and functional preservation. The present prospective study combines monopolar cortical stimulation (MCS) with the recording of phase reversal of somatosensory evoked potentials (SEP-PR) in a protocol for the intra-operative mapping of the motor cortex. Functional mapping of the motor cortex by SEP-PR and MCS was performed in 70 patients during surgery in and around the motor cortex. The central sulcus was identified by SEP-PR. Cortical motor mapping was then performed by monopolar anodal (400 Hz) stimulation. Motor responses were recorded by needle electrodes placed in the muscles of the contralateral extremities. Surgery was performed under general anaesthesia without muscle relaxants. Intra-operative localization of the central sulcus by SEP-PR was possible in 68 patients (97.14%). Motor evoked potentials (MEP) were elicited following MCS in 67 cases (95.7%). In 3 cases no MEP was recorded, not even after maximal stimulation intensity, the central sulcus being localized by SEP-PR only. On the other hand, MCS allowed localizing the motor cortex in the 2 cases with no recordable SEP-PR. Thus, combining SEP-PR and MCS allowed intra-operative localization of the sensorimotor cortex in 100% of the cases.

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