Deep Brain Stimulation for the Dystonias: Evidence, Knowledge Gaps, and Practical Considerations

BACKGROUND: Deep brain stimulation (DBS) of the globus pallidus internus (GPi-DBS) is among the most effective treatment options for dystonias. Because the term "dystonia" is defined by a characteristic phenomenology of involuntary muscle contractions, which may present with a large clinical and pathogenetic heterogeneity, decision making for or against GPi-DBS can be difficult in individual patients. METHODS: A search of the PubMed database for research and review articles, focused on "deep brain stimulation" and "dystonia" was used to identify clinical trials and to determine current concepts in the surgical management of dystonia. Patient selection in previous studies was recategorized by the authors using the new dystonia classification put forward by a consensus committee of experts in dystonia research. The evidence and knowledge gaps are summarized and commented by the authors taking into account expert opinion and personal clinical experience for providing practical guidance in patient selection for DBS in dystonia. RESULTS: The literature review shows that pallidal deep brain stimulation is most effective in patients with isolated dystonia irrespective of the underlying etiology. In contrast, patients with combined dystonias are less likely to benefit from DBS, because the associated neurological symptoms (e.g., hypotonia or ataxia), with the exception of myoclonus, do not respond to pallidal neurostimulation. CONCLUSIONS: It is important to recognize the clinical features of dystonia, because the distinction between isolated and combined dystonia syndromes may predict the treatment response to pallidal deep brain stimulation. The aim of this review is to help guide clinicians with advising patients about deep brain stimulation therapy for dystonia and refering appropriate candidates to surgical centers.