La neurostimolazione cerebrale profonda del nucleo pallido interno nel trattamento chirurgico della distonia

Dystonia is a clinical syndrome characterized by involuntary sustained patterns and often repetitive contractions of opposing muscles that causes twisting or spasmodic movements or abnormal postures. Primary dystonia with onset in childhood is a genetic disease usually characterized by limb onset dystonia with subsequent generalization. In some patients the causative mutation is GAG deletion in the DYT1 gene. Pharmacological treatment with different drugs including anticholinergic, dopamino agonists, dopamino antagonists, benzodiazepines is often disappointing with the frequent occurrence of side effects due also to the high doses required. In recent years deep brain stimulation in the Gpi has been used for treatment of dystonia with encouraging results both in adults and in the pediatric population. Nevertheless patients selection and long term DBS effects have to be clarified. Here we report our experience of five years on 33 patients affected by progressive dystonia with onset in childhood or adulthood who underwent deep brain stimulation.