Central Procedures for Primary Dystonia

Abstract

From the wide range of neurological disorders, very few are accessible to surgical procedures. In 1911, Oppenheim reported on “dystonia musculorum deformans” which had previously been considered by Gowers (1888) and Schwalbe (1908) as a psychiatric disorder. Dystono-dyskinetic syndromes (DDS) are conditions in which a pathological process occurring in the basal ganglia induces a decrease in the inhibition of the brain cortex [1,2]. The syndrome is characterized by sustained muscle contraction leading to repetitive twisting movements and abnormal postures (Figure 108-1 ) [3–7] caused by concurrent contractions of agonist and antagonist muscles. On the basis of results obtained with bilateral pallidotomy for treating the most severe forms of primary generalized DDS and the validated efficiency of deep brain stimulation (DBS) in Parkinson’s disease [8–14], the bilateral chronic electrical stimulation of the Globus Pallidus internus (GPi) was introduced to treat the DDS [15–19].