ONS Advance Access: Disease Stabilization of DYT1-Positive Primary Generalized Dystonia With Deep Brain Stimulation of the Globus Pallidus Interna: A 15-yr Follow-up

Deep brain stimulation (DBS) is a widely accepted treatment for movement disorders including essential tremor, Parkinson’s disease (PD), and dystonia. DBS of the globus pallidus internus (GPi), for the treatment of dystonia is often effective in reducing the accompanying musculoskeletal deformities.1,2 While many studies point to the efficacy of DBS for dystonia, several authors question the durability and efficacy of such interventions.3 Variability in responses to DBS for dystonia have been reported in several previous series, lending further question to its efficacy as an appropriate intervention in all forms of dystonia.4,5Primary generalized dystonia (PGD) has been shown to more consistently respond to DBS than the secondary type.6 While DBS for PD and essential tremor has substantial support from the medical community, the dystonias are a heterogeneous group of disorders with multiple etiologies, making tracking long-term outcomes difficult.

PGD, a disabling genetic form that presents early in life and is associated with various DYT genes,710 generally displays resistance to pharmacotherapy and often progresses within the first decade of symptom onset.1114 Fortunately, PGD frequently demonstrates robust response to bilateral GPi-DBS.1,14 Until the introduction of DBS as an effective treatment of dystonia, peripheral denervation, thalamotomy, and myelotomy served as irreversible end treatments for severe dystonia.15,16

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