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Mixed results for GPi-DBS in the treatment of cranio-facial and cranio-cervical dystonia symptoms

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Abstract

The aim of the study is to determine clinical outcomes in patients undergoing Globus Pallidus Internus Deep Brain Stimulation (GPi-DBS) for cranio-facial and cranio-cervical dystonia (Meige) symptoms. A total of 6 patients seen between 2002 and 2010 with cranio-facial and cranio-cervical dystonia symptoms were identified from the University of Florida Institutional Review Board approved database. Patients were videotaped using a standardized protocol, and tapes were randomized and blindly reviewed by a movement disorders neurologist. The Unified Dystonia Rating Scale improved 31.6 ± 23.2% (range: 3.4–63.2%) at 6 months and 63.7 ± 35.3% (range: 6.3–100%) at 12 months. The Burke–Fahn–Marsden Dystonia Rating Scale improved 45.3 ± 29.5% (range: 4.7–75.0%) at 6 months and 61.8 ± 30.9% (range: 16.6–100%) at 12 months. One patient significantly had a very large improvement with little evidence of residual dystonia. Blepharospasm improved in all patients, whereas speech and swallowing did not improve in this cohort. Two patients improved with unilateral GPi-DBS, although one required a contralateral DBS later in the disease course. Two patients were managed with low frequency stimulation (<100 Hz). Two patients had less than 20% benefit. GPi-DBS for cranio-facial and cranio-cervical symptoms is an effective strategy to manage a subset of patients who remain unresponsive to optimized medical management. Unilateral stimulation may be an option for some patients, but it remains unclear whether response to single-sided stimulation will be sustainable. The mixed results of this GPi-DBS case series highlight the need for a careful re-examination of selection criteria, alternative brain targets, and possibly rescue leads for patients who are non-responders to the GPi target.

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References

  1. Tolosa E, Marti MJ (1988) Blepharospasm-oromandibular dystonia syndrome (Meige’s syndrome): clinical aspects. Adv Neurol 49:73–84

    PubMed  CAS  Google Scholar 

  2. Kraft SP, Lang AE (1988) Cranial dystonia, blepharospasm and hemifacial spasm: clinical features and treatment, including the use of botulinum toxin. CMAJ 139(9):837–844

    PubMed  CAS  Google Scholar 

  3. Kupsch A, Benecke R, Muller J, Trottenberg T, Schneider GH, Poewe W, Eisner W, Wolters A, Muller JU, Deuschl G, Pinsker MO, Skogseid IM, Roeste GK, Vollmer-Haase J, Brentrup A, Krause M, Tronnier V, Schnitzler A, Voges J, Nikkhah G, Vesper J, Naumann M, Volkmann J (2006) Pallidal deep-brain stimulation in primary generalized or segmental dystonia. N Engl J Med 355(19):1978–1990. doi:10.1056/NEJMoa063618

    Article  PubMed  CAS  Google Scholar 

  4. Vidailhet M, Vercueil L, Houeto JL, Krystkowiak P, Benabid AL, Cornu P, Lagrange C, Tezenas du Montcel S, Dormont D, Grand S, Blond S, Detante O, Pillon B, Ardouin C, Agid Y, Destee A, Pollak P (2005) Bilateral deep-brain stimulation of the globus pallidus in primary generalized dystonia. N Engl J Med 352(5):459–467. doi:10.1056/NEJMoa042187

    Article  PubMed  CAS  Google Scholar 

  5. Kumar R, Dagher A, Hutchison WD, Lang AE, Lozano AM (1999) Globus pallidus deep brain stimulation for generalized dystonia: clinical and PET investigation. Neurology 53(4):871–874

    PubMed  CAS  Google Scholar 

  6. Grips E, Blahak C, Capelle HH, Bazner H, Weigel R, Sedlaczek O, Krauss JK, Wohrle JC (2007) Patterns of reoccurrence of segmental dystonia after discontinuation of deep brain stimulation. J Neurol Neurosurg Psychiatry 78(3):318–320. doi:10.1136/jnnp.2006.089409

    Article  PubMed  CAS  Google Scholar 

  7. Markaki E, Kefalopoulou Z, Georgiopoulos M, Paschali A, Constantoyannis C (2010) Meige’s syndrome: a cranial dystonia treated with bilateral pallidal deep brain stimulation. Clin Neurol Neurosurg 112(4):344–346. doi:10.1016/j.clineuro.2009.12.005

    Article  PubMed  Google Scholar 

  8. Ostrem JL, Marks WJ Jr, Volz MM, Heath SL, Starr PA (2007) Pallidal deep brain stimulation in patients with cranial-cervical dystonia (Meige syndrome). Mov Disord 22(13):1885–1891. doi:10.1002/mds.21580

    Article  PubMed  Google Scholar 

  9. Houser M, Waltz T (2005) Meige syndrome and pallidal deep brain stimulation. Mov Disord 20(9):1203–1205. doi:10.1002/mds.20522

    Article  PubMed  Google Scholar 

  10. Blomstedt P, Tisch S, Hariz MI (2008) Pallidal deep brain stimulation in the treatment of Meige syndrome. Acta Neurol Scand 118(3):198–202. doi:10.1111/j.1600-0404.2008.00999.x

    Article  PubMed  CAS  Google Scholar 

  11. Burke RE, Fahn S, Marsden CD, Bressman SB, Moskowitz C, Friedman J (1985) Validity and reliability of a rating scale for the primary torsion dystonias. Neurology 35(1):73–77

    PubMed  CAS  Google Scholar 

  12. Goetz CG, Stebbins GT, Shale HM, Lang AE, Chernik DA, Chmura TA, Ahlskog JE, Dorflinger EE (1994) Utility of an objective dyskinesia rating scale for Parkinson’s disease: inter- and intrarater reliability assessment. Mov Disord 9(4):390–394. doi:10.1002/mds.870090403

    Article  PubMed  CAS  Google Scholar 

  13. Foote KD, Sanchez JC, Okun MS (2005) Staged deep brain stimulation for refractory craniofacial dystonia with blepharospasm: case report and physiology. Neurosurgery 56 (2):E415; discussion E415

    Google Scholar 

  14. Loher TJ, Capelle HH, Kaelin-Lang A, Weber S, Weigel R, Burgunder JM, Krauss JK (2008) Deep brain stimulation for dystonia: outcome at long-term follow-up. J Neurol 255(6):881–884. doi:10.1007/s00415-008-0798-6

    Article  PubMed  CAS  Google Scholar 

  15. Berman BD, Starr PA, Marks WJ Jr, Ostrem JL (2009) Induction of bradykinesia with pallidal deep brain stimulation in patients with cranial-cervical dystonia. Stereotact Funct Neurosurg 87(1):37–44. doi:10.1159/000195718

    Article  PubMed  Google Scholar 

  16. Andrews C, Aviles-Olmos I, Hariz M, Foltynie T (2010) Which patients with dystonia benefit from deep brain stimulation? A metaregression of individual patient outcomes. J Neurol Neurosurg Psychiatry 81(12):1383–1389. doi:10.1136/jnnp.2010.207993

    Article  PubMed  Google Scholar 

  17. Groen JL, Ritz K, Contarino MF, van de Warrenburg BP, Aramideh M, Foncke EM, van Hilten JJ, Schuurman PR, Speelman JD, Koelman JH, de Bie RM, Baas F, Tijssen MA (2010) DYT6 dystonia: mutation screening, phenotype, and response to deep brain stimulation. Mov Disord 25(14):2420–2427. doi:10.1002/mds.23285

    Article  PubMed  Google Scholar 

  18. Martinez-Torres I, Limousin P, Tisch S, Page R, Pinto A, Foltynie T, Bhatia KP, Hariz MI, Zrinzo L (2009) Early and marked benefit with GPi DBS for Lubag syndrome presenting with rapidly progressive life-threatening dystonia. Mov Disord 24(11):1710–1712. doi:10.1002/mds.22656

    Article  PubMed  Google Scholar 

  19. Oyama G, Foote KD, Hwynn N, Jacobson CEt, Malaty IA, Rodriguez RL, Zeilman P, Okun MS (2011) Rescue leads: a salvage technique for selected patients with a suboptimal response to standard DBS therapy. Parkinsonism Relat Disord. doi: 10.1016/j.parkreldis.2011.03.009

  20. Lyons MK, Birch BD, Hillman RA, Boucher OK, Evidente VG (2010) Long-term follow-up of deep brain stimulation for Meige syndrome. Neurosurg Focus 29(2):E5. doi:10.3171/2010.4.FOCUS1067

    Article  PubMed  Google Scholar 

  21. Muta D, Goto S, Nishikawa S, Hamasaki T, Ushio Y, Inoue N, Mita S (2001) Bilateral pallidal stimulation for idiopathic segmental axial dystonia advanced from Meige syndrome refractory to bilateral thalamotomy. Mov Disord 16(4):774–777

    Article  PubMed  CAS  Google Scholar 

  22. Capelle HH, Weigel R, Krauss JK (2003) Bilateral pallidal stimulation for blepharospasm-oromandibular dystonia (Meige syndrome). Neurology 60(12):2017–2018

    PubMed  Google Scholar 

  23. Vercueil L, Pollak P, Fraix V, Caputo E, Moro E, Benazzouz A, Xie J, Koudsie A, Benabid AL (2001) Deep brain stimulation in the treatment of severe dystonia. J Neurol 248(8):695–700

    Article  PubMed  CAS  Google Scholar 

  24. Moro E, Piboolnurak P, Arenovich T, Hung SW, Poon YY, Lozano AM (2009) Pallidal stimulation in cervical dystonia: clinical implications of acute changes in stimulation parameters. Eur J Neurol 16(4):506–512. doi:10.1111/j.1468-1331.2008.02520.x

    Article  PubMed  CAS  Google Scholar 

  25. Kiss ZH, Doig-Beyaert K, Eliasziw M, Tsui J, Haffenden A, Suchowersky O (2007) The Canadian multicentre study of deep brain stimulation for cervical dystonia. Brain 130(Pt 11):2879–2886. doi:10.1093/brain/awm229

    Article  PubMed  Google Scholar 

  26. Hung SW, Hamani C, Lozano AM, Poon YY, Piboolnurak P, Miyasaki JM, Lang AE, Dostrovsky JO, Hutchison WD, Moro E (2007) Long-term outcome of bilateral pallidal deep brain stimulation for primary cervical dystonia. Neurology 68(6):457–459. doi:10.1212/01.wnl.0000252932.71306.89

    Article  PubMed  CAS  Google Scholar 

  27. Kupsch A, Klaffke S, Kuhn AA, Meissner W, Arnold G, Schneider GH, Maier-Hauff K, Trottenberg T (2003) The effects of frequency in pallidal deep brain stimulation for primary dystonia. J Neurol 250(10):1201–1205. doi:10.1007/s00415-003-0179-0

    Article  PubMed  CAS  Google Scholar 

  28. Opherk C, Gruber C, Steude U, Dichgans M, Botzel K (2006) Successful bilateral pallidal stimulation for Meige syndrome and spasmodic torticollis. Neurology 66(4):E14. doi:10.1212/01.wnl.0000190257.55434.ab

    Article  PubMed  CAS  Google Scholar 

  29. Fasano A, Mazzone P, Piano C, Quaranta D, Soleti F, Bentivoglio AR (2008) GPi-DBS in Huntington’s disease: results on motor function and cognition in a 72-year-old case. Mov Disord 23(9):1289–1292. doi:10.1002/mds.22116

    Article  PubMed  Google Scholar 

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Acknowledgments

We would like to acknowledge the support of the Tyler’s Hope Center of Excellence for Dystonia and the UF Foundation.

Conflict of interest

The authors declare that they have no conflict of interest.

Disclosures

Dr. Okun received support from NIH, Michael J. Fox Foundation, and National Parkinson Foundation (NPF). Dr. Okun serves as a consultant to NPF. Drs. Okun and Foote prior to 2009–10 have taught DBS programming courses for Medtronic.

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Authors and Affiliations

  1. Department of Neurology, Center for Movement Disorders and Neurorestoration, University of Florida, 101 S Newell Dr., L3-101, Gainesville, FL, 32611, USA

    Natlada Limotai, Criscely Go, Genko Oyama, Nelson Hwynn, Kelly Foote, Irene Malaty, Pam Zeilman, Ramon Rodriguez & Michael S. Okun

  2. Department of Neurology, College of Medicine Tampa, University of South Florida Health, Tampa, FL, USA

    Theresa Zesiewicz

  3. Chulalongkorn Comprehensive Movement Disorders Center, Chulalongkorn University Hospital, Thai Red Cross Society, Bangkok, Thailand

    Natlada Limotai & Roongroj Bhidayasiri

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  1. Natlada Limotai
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  2. Criscely Go
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  3. Genko Oyama
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Correspondence to Michael S. Okun.

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Limotai, N., Go, C., Oyama, G. et al. Mixed results for GPi-DBS in the treatment of cranio-facial and cranio-cervical dystonia symptoms. J Neurol 258, 2069–2074 (2011). https://doi.org/10.1007/s00415-011-6075-0

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  • DOI: https://doi.org/10.1007/s00415-011-6075-0

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