Abstract

Background and purposePrimary generalized dystonia (PGD) is a medically refractory progressive disease of the brain causing near total handicap of affected patients. The aim of the study was to assess the efficacy and safety of bilateral pallidal stimulation in patients with PGD. Material and methodsThe study population is composed of 5 patients with PGD. The formal objective assessment included the Burke-Fahn-Marsden dystonia rating scale (BFMDRS). All stereotactic procedures were performed in general anaesthesia using a Leksell G stereotactic head frame without electrophysiological guidance. Immediately after insertion of deep brain stimulation (DBS) leads, the internal pulse generators (Itrel II or Soletra) were implanted subcutaneously in the chest wall or abdominal region. ResultsThere were no complications related to the stereotactic procedures. The hardware-related complications (two broken DBS leads) were replaced successfully. There were no infections or erosions of implanted hardware. It has been observed that in the long-term follow-up period primary set bipolar stimulation mode lost its benefit achieved previously. Various stimulation combinations were investigated. Monopolar cathodal or especially multi-contact cathodal stimulation was the most effective one. The efficacy of bilateral pallidal stimulation was proved by the objective validated BFMDRS at long-term follow-up. ConclusionsResponse to DBS may improve with the number of activated cathodal contacts within the globus pallidus internus.

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