Dystonie (maladie) | EPFL Graph Search

Les dystonies sont un groupe de maladies caractérisées par des troubles moteurs, regroupant la dystonie idiopathique de torsion (DIT) ou dystonie généralisée, les autres dystonies héréditaires, le blépharospasme, la dysphonie spasmodique, la dystonie secondaire à des lésions cérébrales et les dystonies en foyer. La dystonie frappe hommes et femmes, jeunes et vieux et les gens de toute origine. Elle n'est pas un trouble psychologique et elle n'affecte pas les facultés intellectuelles. La dystonie n'est pas mortelle, mais elle est chronique et s'accompagne d'incapacité et de douleur, souvent insupportable. Le traitement est difficile et limité à minimiser les symptômes de la maladie, car il n'existe aucun remède connu. Le traitement le plus utilisé est l'injection de toxine botulique, qui bloque l’influx nerveux entre le nerf et le muscle, entraînant ainsi une diminution de la force musculaire. Ce traitement est temporaire, et à renouveler régulièrement car les effets de la toxine s'

Defective inhibition and inter-regional phase synchronization in pianists with musician's dystonia: an EEG study

Friedhelm Christoph Hummel

Recent neurophysiological studies have associated focal-task specific dystonia (FTSD) with impaired inhibitory function. However, it remains unknown whether FTSD also affects the inhibition (INH) of long-term overlearned motor programs. Consequently, we investigated in a Go/NoGo paradigm the neural correlates associated with the activation (ACT) and inhibition of long-term overlearned motor memory traces in pianists with musician's dystonia (MD), a form of FTSD, during a relevant motor task under constraint timing conditions with multichannel EEG. In NoGo trials, the movement related cortical potentials showed a positive shift after the NoGo signal related to inhibition and was significantly smaller over sensorimotor areas in musicians with MD. Further, we observed an increase at 850-900 ms in the power of beta oscillations which was significantly weaker for the patient group. The role of the inter-electrode phase coupling in the sensorimotor integration of inhibitory processes turned out to be the most relevant physiological marker: the global phase synchronization during INH exhibited an increase between 230 and 330 ms and 7-8 Hz, increase which was significantly smaller for pianists with MD. This effect was due to a weaker phase synchronization between the supplementary motor cortex and left premotor and sensorimotor electrodes in patients. Thus, our findings support the hypothesis of a deficient phase coupling between the neuronal assemblies required to inhibit motor memory traces in patients with MD. EMG recorded from the right flexor pollicis longus muscle confirmed that patients with MD had a disrupted INH in NoGo trials.

Wiley-Blackwell2009

Inhibitory control of acquired motor programmes in the human brain

Friedhelm Christoph Hummel

An important basis of skilled human behaviour is the appropriate retrieval of acquired and memorized motor programmes ('motor memory traces'). Appropriate retrieval is warranted if motor programmes are only activated if necessary and are, probably more often, inhibited if required by the context of a given situation. It is unknown how this type of inhibition is accomplished in the brain. We studied context-dependent modulation of motor memory traces in 18 volunteers and six patients with focal dystonia. Cortical function was assessed with transcranial magnetic stimulation over the primary motor cortex (M1) and with task-related analysis of oscillatory EEG activity. An activation (ACT) and inhibition (INH) condition were compared. In both, visual cues were presented at 1/s. In ACT, subjects had to respond to these cues with individual finger movements as learned in a preceding training session. In INH, subjects had to observe the cues without retrieval of motor responses. During INH, inhibitory control of the motor memory trace was confirmed by significant amplitude reduction of motor evoked potentials (MEPs) compared with baseline. This was accompanied by a significant increase of 11-13 Hz oscillatory activity over the sensorimotor areas during INH. During active retrieval of the motor memory traces, the reverse was true (increased MEP amplitudes, decreased oscillatory 11-13 Hz activity). In a small sample of dystonic patients (n = 6), the increase of 11-13 Hz oscillatory activity during INH was consistently absent. The present data demonstrate for the first time cortical correlates of appropriate, context-dependent inhibition of motor memory traces. We propose that focal increases of oscillatory activity are instrumental for inhibitory control at the cortical level. This concept is supported by the preliminary observations in dystonic patients who are known to have deficits of inhibitory motor control and in whom these context-dependent focal increases of oscillatory activity were absent.

Oxford University Press2002

Inhibitory control of acquired motor programmes in the human brain

Friedhelm Christoph Hummel

Oxford University Press2002