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Long-term effects of bilateral pallidal deep brain stimulation in dystonia: a follow-up between 8 and 16 years

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Observational study to evaluate the long-term motor and non-motor effects of deep brain stimulation (DBS) of the globus pallidus internus (GPi) on medically refractory dystonia.


Dystonia is a chronic disease affecting mainly young patients with a regular life expectancy and lifelong need for therapy. Pallidal DBS is an established treatment for severe isolated dystonia but long-term data are sparse.


We considered 36 consecutive patients with isolated generalized (n = 14) and cervical/segmental (n = 22) dystonia operated at Charité-University Hospital between 2000 and 2007 in a retrospective analysis for long-term outcome of pallidal DBS. In 19 of these patients, we could analyze dystonic symptoms and disability rated by the Burke–Fahn–Marsden Dystonia Rating scale (BFMDRS) at baseline, short-term (ST-FU, range 3–36 months) and long-term follow-up (LT-FU, range 93–197 months). Quality of life and mood were evaluated using the SF36 and Beck Depression Index (BDI) questionnaires.


Patients reached an improvement in motor symptoms of 63.8 ± 5.7% (mean ± SE) at ST-FU and 67.9 ± 6.1% at LT-FU. Moreover, a significant and stable reduction in disability was shown following DBS (54.2 ± 9.4% at ST-FU and 53.8 ± 9.2% at LT-FU). BDI and SF36 had improved by 40% and 23%, respectively, at LT-FU (n = 14). Stimulation-induced adverse events included swallowing difficulties, dysarthria, and bradykinesia. Pulse generator (n = 3) and electrodes (n = 5) were revised in seven patients due to infection.


Pallidal DBS is a safe and efficacious long-term treatment for dystonia with sustained effects on motor impairment and disability, accompanied by a robust improvement in mood and quality of life.

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  1. 1.

    Albanese A et al (2013) Phenomenology and classification of dystonia: a consensus update. Mov Disord 28(7):863–873

  2. 2.

    Fahn S, Bressman S, Marsden CD (1998) Classification of Dystonia. Adv Neurol 78:1–10

  3. 3.

    Ben-Shlomo Y et al (2002) What are the determinants of quality of life in people with cervical dystonia? J Neurol Neurosurg Psychiatry 72(5):608–614

  4. 4.

    Vidailhet M et al (2005) Bilateral deep-brain stimulation of the globus pallidus in primary generalized dystonia. N Engl J Med 352(5):459–467

  5. 5.

    Volkmann J et al (2014) Pallidal neurostimulation in patients with medication-refractory cervical dystonia: a randomised, sham-controlled trial. Lancet Neurol 13(9):875–884

  6. 6.

    Vidailhet M et al (2007) Bilateral, pallidal, deep-brain stimulation in primary generalised dystonia: a prospective 3 year follow-up study. Lancet Neurol 6(3):223–229

  7. 7.

    Volkmann J et al (2012) Pallidal deep brain stimulation in patients with primary generalised or segmental dystonia: 5-year follow-up of a randomised trial. Lancet Neurol 11(12):1029–1038

  8. 8.

    Bereznai B et al (2002) Chronic high-frequency globus pallidus internus stimulation in different types of dystonia: a clinical, video, and MRI report of six patients presenting with segmental, cervical, and generalized dystonia. Mov Disord 17(1):138–144

  9. 9.

    Loher TJ et al (2008) Deep brain stimulation for dystonia: outcome at long-term follow-up. J Neurol 255(6):881–884

  10. 10.

    Sensi M et al (2009) Pallidal stimulation for segmental dystonia: long term follow up of 11 consecutive patients. Mov Disord 24(12):1829–1835

  11. 11.

    Skogseid IM et al (2012) Good long-term efficacy of pallidal stimulation in cervical dystonia: a prospective, observer-blinded study. Eur J Neurol 19(4):610–615

  12. 12.

    Halbig TD et al (2005) Pallidal stimulation in dystonia: effects on cognition, mood, and quality of life. J Neurol Neurosurg Psychiatry 76(12):1713–1716

  13. 13.

    Mueller J et al (2008) Pallidal deep brain stimulation improves quality of life in segmental and generalized dystonia: results from a prospective, randomized sham-controlled trial. Mov Disord 23(1):131–134

  14. 14.

    Silberstein P et al (2003) Patterning of globus pallidus local field potentials differs between Parkinson's disease and dystonia. Brain 126(Pt 12):2597–2608

  15. 15.

    Burke RE et al (1985) Validity and reliability of a rating scale for the primary torsion dystonias. Neurology 35(1):73–77

  16. 16.

    Bhidayasiri R, Tarsy D (2006) Treatment of dystonia. Expert Rev Neurother 6(6):863–886

  17. 17.

    de Carvalho-Aguiar PM, Ozelius LJ (2002) Classification and genetics of dystonia. Lancet Neurol 1(5):316–325

  18. 18.

    Ware JE Jr, Sherbourne CD (1992) The MOS 36-item short-form health survey (SF-36). I. Conceptual framework and item selection. Med Care 30(6):473–483

  19. 19.

    Beck AT et al (1961) An inventory for measuring depression. Arch Gen Psychiatry 4:561–571

  20. 20.

    Horn A, Kuhn AA (2015) Lead-DBS: a toolbox for deep brain stimulation electrode localizations and visualizations. Neuroimage 107:127–135

  21. 21.

    Avants BB et al (2008) Symmetric diffeomorphic image registration with cross-correlation: evaluating automated labeling of elderly and neurodegenerative brain. Med Image Anal 12(1):26–41

  22. 22.

    Ewert S et al (2017) Toward defining deep brain stimulation targets in MNI space: a subcortical atlas based on multimodal MRI, histology and structural connectivity. Neuroimage 170:271–282

  23. 23.

    Cif L et al (2010) Long-term follow-up of DYT1 dystonia patients treated by deep brain stimulation: an open-label study. Mov Disord 25(3):289–299

  24. 24.

    Krause P et al (2016) Long-term results of deep brain stimulation in a cohort of eight children with isolated dystonia. J Neurol 263(11):2319–2326

  25. 25.

    FitzGerald JJ et al (2014) Long-term outcome of deep brain stimulation in generalised dystonia: a series of 60 cases. J Neurol Neurosurg Psychiatry 85(12):1371–1376

  26. 26.

    Andrews C et al (2010) Which patients with dystonia benefit from deep brain stimulation? A metaregression of individual patient outcomes. J Neurol Neurosurg Psychiatry 81(12):1383–1389

  27. 27.

    Isaias IU, Alterman RL, Tagliati M (2008) Outcome predictors of pallidal stimulation in patients with primary dystonia: the role of disease duration. Brain 131(Pt 7):1895–1902

  28. 28.

    Reich MM et al (2019) Probabilistic mapping of the antidystonic effect of pallidal neurostimulation: a multicentre imaging study. Brain 142(5):1386–1398

  29. 29.

    Meoni S et al (2017) Pallidal deep brain stimulation for dystonia: a long term study. J Neurol Neurosurg Psychiatry 88(11):960–967

  30. 30.

    DeLong MR, Crutcher MD, Georgopoulos AP (1985) Primate globus pallidus and subthalamic nucleus: functional organization. J Neurophysiol 53(2):530–543

  31. 31.

    Tisch S et al (2007) Effect of electrode contact location on clinical efficacy of pallidal deep brain stimulation in primary generalised dystonia. J Neurol Neurosurg Psychiatry 78(12):1314–1319

  32. 32.

    Kiss ZH et al (2007) The Canadian multicentre study of deep brain stimulation for cervical dystonia. Brain 130(Pt 11):2879–2886

  33. 33.

    Hung SW et al (2007) Long-term outcome of bilateral pallidal deep brain stimulation for primary cervical dystonia. Neurology 68(6):457–459

  34. 34.

    Alterman RL et al (2007) Lower stimulation frequency can enhance tolerability and efficacy of pallidal deep brain stimulation for dystonia. Mov Disord 22(3):366–368

  35. 35.

    Tsuboi T et al (2019) Importance of the initial response to GPi deep brain stimulation in dystonia: a nine year quality of life study. Parkinsonism Relat Disord 64:249–255

  36. 36.

    Jahanshahi M et al (2014) Pallidal stimulation for primary generalised dystonia: effect on cognition, mood and quality of life. J Neurol 261(1):164–173

  37. 37.

    Stamelou M et al (2012) The non-motor syndrome of primary dystonia: clinical and pathophysiological implications. Brain 135(Pt 6):1668–1681

  38. 38.

    Oh MY et al (2002) Long-term hardware-related complications of deep brain stimulation. Neurosurgery 50(6):1268–1274 (discussion 1274-1276)

  39. 39.

    Joint C et al (2002) Hardware-related problems of deep brain stimulation. Mov Disord 17(Suppl 3):S175–S180

  40. 40.

    Kupsch A et al (2006) Pallidal deep-brain stimulation in primary generalized or segmental dystonia. N Engl J Med 355(19):1978–1990

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The study was supported by a grant of the German Research Foundation (DFG) Grant KFO 247 and the Federal Ministry of Education and research (BMBF) with the network for rare diseases DYSTRACT 01GM1514D. This study has been approved by the local ethics committee and has, therefore, been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.

Author information

Correspondence to Andrea A. Kühn.

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Conflicts of interest

AAK received speaker´s honoraria or consultancies and travel grants from Medtronic, Boston Scientific and Abbott. P. Krause received speaker´s honoraria from Medtronic. S. Völzmann: None. S. Ewert: None. A. Kupsch belongs to the Advisory Board ´Medtronic USA´ and received honoraria from Allergan, Boehringer Ingelheim, Ipsen Pharma, Lundbeck, Medtronic, Merck, Merz Pharmaceuticals, Orion, St. Jude UCB. G.H. Schneider received speaker´s honoraria from Medtronic, Boston Scientific and Abbott.

Ethical standard

This study has been approved by the local ethics committee of the Charité, University Medicine Berlin and has therefore been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki.

Informed consent

All patients gave their written informed consent for long-term followup.

Electronic supplementary material

Below is the link to the electronic supplementary material.

Supplementary file1 Suppl. Table 1: Demographic characteristics and clinical data (mean ± standard error) of the DBS patients with generalized dystonia (1-10) and cervical/segmental dystonia (11-19). None of the patients had any structural brain abnormalities in individual MRI. Note the complete withdrawal of medication after DBS in 10 of 19 patients. BFMDRS Burke–Fahn–Marsden Dystonia Scale for motor impairment (M) and degree of disability (D) at baseline (BL), short-term follow-up (ST-FU) and last long-term follow-up (LT-FU). p.d.: per day; i.r.: if required; *indicates patients that had been included in the national dystonia trial [7, 40] (DOCX 19 kb)

Supplementary file2 Suppl. Table 2: Stimulation parameters of all patients at short-term and long-term follow-up. Patient numbers given here match table 1 with demographic data of all patients. Patient 18 initially was stimulated quadripolar (Vim and Gpi). After lack of benefit of thalamic stimulation, only bilateral pallidal stimulation was selected (DOCX 20 kb)

Supplementary file3 Suppl. Table 3: Overview of the Drop Outs (n=17; 13 CD/SD versus 4 GD patients) and their individual preoperative and short-term (post DBS) scoresavailable retrospectively (range of ST-FU: 3-60months). * Indicate patients with THAP1 gene mutation. Maximum Value in points for TWSTRS=35,TSUI=25, BFMDRS=120. n. a. = not available (DOCX 20 kb)

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Krause, P., Völzmann, S., Ewert, S. et al. Long-term effects of bilateral pallidal deep brain stimulation in dystonia: a follow-up between 8 and 16 years. J Neurol (2020). https://doi.org/10.1007/s00415-020-09745-z

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  • Dystonia
  • Pallidal DBS
  • Long-term effects
  • DBS and quality of life