Abstract
a very small proportion of patients have a specific treatment (levodopa in dopa-responsive dystonia or drugs that prevent copper accumulation in Wilson’s disease). Therapeutic options must be tailored to the needs of individual patients: in focal dystonia, botulinum toxin is a safe and effective treatment (level I and II studies for blepharospasm and cervical dystonia) with a long term follow-up and a sustained beneficial effect. Physiotherapy could be added to botulinum toxin injections but a lot of debate has recently occurred on the available and future strategies. Most of them were based on pathophysiology of dystonia (altered sensorimotor integration, maladaptative plasticity, abnormal learning). Results of the available studies and new strategies will be discussed. In generalized or severe segmental dystonias, medical treatments should always be tried (anticholinergics, benzodiazepines) but deep brain stimulation (DBS) for patients with generalised dystonia demonstrated a dramatic effect. More recently, DBS has been offered as a therapeutic option in segmental and focal dystonia and indications, results and limitations will be discussed. A few secondary dystonia may benefit from DBS such as tardive dystonia and a sub-type of cerebral palsy but discussion will be expanded (from the medical point of view) to other types of genetic or metabolic disorders with dystonia.
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