Acta Neurochirurgica

, Volume 152, Issue 6, pp 1007–1014

Regional cerebral blood flow changes induced by deep brain stimulation in secondary dystonia

  • Zinovia Kefalopoulou
  • Anna Paschali
  • Elli Markaki
  • John Ellul
  • Elisabeth Chroni
  • Pavlos Vassilakos
  • Constantine Constantoyannis
Clinical Article



The purpose of the present study is to investigate the effect of deep brain stimulation (DBS) on regional cerebral blood flow (rCBF) in cases of secondary dystonia as well as to correlate the rCBF changes with clinical outcomes.


Six patients with medically intractable secondary dystonia who underwent DBS surgery were included in this study. Burke–Fahn–Mardsen Dystonia Rating Scale (BFMDRS) was used for the assessment of dystonia, before and after surgery. Single photon emission computed tomography (SPECT) of the brain was performed postoperatively in the two stimulation states (ON-DBS and OFF-DBS) and the changes of rCBF in the three following brain regions of interest (ROIs): primary motor cortex, premotor and supplementary motor cortex, and prefrontal cortex were evaluated.


Two patients exhibited excellent response to DBS, two patients got moderate benefit after the procedure, and in two patients, no clinical improvement was achieved. A mean improvement of 49.1% (0–90.7%) in BFMDRS total scores was found postoperatively. Brain SPECT data analysis revealed an overall decrease in rCBF in the investigated ROIs, during the ON-DBS state. Clinical improvement was significantly correlated with the observed decrease in rCBF in the presence of DBS.


When conservative treatment fails to relieve severely disabled patients suffering from secondary dystonia, DBS may be a promising therapeutic alternative. Moreover, this study indicates a putative role of brain SPECT imaging as a postoperative indicator of clinical responsiveness to DBS.


Deep brain stimulation Regional cerebral blood flow Secondary dystonia SPECT Dystonia 


  1. 1.
    Albanese A, Barnes MP, Bhatia KP, Fernandez-Alvarez E, Filippini G, Gasser T, Krauss JK, Newton A, Rektor I, Savoiardo M, Valls-Sole J (2006) A systematic review on the diagnosis and treatment of primary (idiopathic) dystonia and dystonia plus syndromes: report of an EFNS/MDS-ES Task Force. Eur J Neurol 13:433–444CrossRefPubMedGoogle Scholar
  2. 2.
    Asanuma K, Carbon-Correll M, Eidelberg D (2005) Neuroimaging in human dystonia. J Med Invest 52(Suppl):272–279CrossRefPubMedGoogle Scholar
  3. 3.
    Bhatia KP, Marsden CD (1994) The behavioural and motor consequences of focal lesions of the basal ganglia in man. Brain 117(Pt 4):859–876CrossRefPubMedGoogle Scholar
  4. 4.
    Bhidayasiri R (2006) Dystonia: genetics and treatment update. Neurologist 12:74–85CrossRefPubMedGoogle Scholar
  5. 5.
    Ceballos-Baumann AO, Passingham RE, Warner T, Playford ED, Marsden CD, Brooks DJ (1995) Overactive prefrontal and underactive motor cortical areas in idiopathic dystonia. Ann Neurol 37:363–372CrossRefPubMedGoogle Scholar
  6. 6.
    Chen CC, Kuhn AA, Trottenberg T, Kupsch A, Schneider GH, Brown P (2006) Neuronal activity in globus pallidus interna can be synchronized to local field potential activity over 3-12 Hz in patients with dystonia. Exp Neurol 202:480–486CrossRefPubMedGoogle Scholar
  7. 7.
    Cif L, El Fertit H, Vayssiere N, Hemm S, Hardouin E, Gannau A, Tuffery S, Coubes P (2003) Treatment of dystonic syndromes by chronic electrical stimulation of the internal globus pallidus. J Neurosurg Sci 47:52–55PubMedGoogle Scholar
  8. 8.
    Constantoyannis C, Kagadis GC, Ellul J, Kefalopoulou Z, Chroni E (2008) Nucleus ventralis oralis deep brain stimulation in postanoxic dystonia. Mov Disord 24:306–308CrossRefGoogle Scholar
  9. 9.
    Coubes P, Cif L, El Fertit H, Hemm S, Vayssiere N, Serrat S, Picot MC, Tuffery S, Claustres M, Echenne B, Frerebeau P (2004) Electrical stimulation of the globus pallidus internus in patients with primary generalized dystonia: long-term results. J Neurosurg 101:189–194CrossRefPubMedGoogle Scholar
  10. 10.
    Detante O, Vercueil L, Thobois S, Broussolle E, Costes N, Lavenne F, Chabardes S, Lebars D, Vidailhet M, Benabid AL, Pollak P (2004) Globus pallidus internus stimulation in primary generalized dystonia: a H215O PET study. Brain 127:1899–1908CrossRefPubMedGoogle Scholar
  11. 11.
    Eidelberg D, Moeller JR, Antonini A, Kazumata K, Nakamura T, Dhawan V, Spetsieris P, deLeon D, Bressman SB, Fahn S (1998) Functional brain networks in DYT1 dystonia. Ann Neurol 44:303–312CrossRefPubMedGoogle Scholar
  12. 12.
    Eltahawy HA, Saint-Cyr J, Giladi N, Lang AE, Lozano AM (2004) Primary dystonia is more responsive than secondary dystonia to pallidal interventions: outcome after pallidotomy or pallidal deep brain stimulation. Neurosurgery 54:613–619, discussion 619-621CrossRefPubMedGoogle Scholar
  13. 13.
    Fahn S, Bressman SB, Marsden CD (1998) Classification of dystonia. Adv Neurol 78:1–10PubMedGoogle Scholar
  14. 14.
    Gsell W, De Sadeleer C, Marchalant Y, MacKenzie ET, Schumann P, Dauphin F (2000) The use of cerebral blood flow as an index of neuronal activity in functional neuroimaging: experimental and pathophysiological considerations. J Chem Neuroanat 20:215–224CrossRefPubMedGoogle Scholar
  15. 15.
    Hallett M (2006) Pathophysiology of dystonia. J Neural Transm 94:485–488CrossRefGoogle Scholar
  16. 16.
    Hartmann A, Pogarell O, Oertel WH (1998) Secondary dystonias. J Neurol 245:511–518CrossRefPubMedGoogle Scholar
  17. 17.
    Jinnah HA, Hess EJ (2006) A new twist on the anatomy of dystonia: the basal ganglia and the cerebellum? Neurology 67:1740–1741CrossRefPubMedGoogle Scholar
  18. 18.
    Katsakiori P, Kefalopoulou Z, Markaki E, Paschali A, Ellul J, Chroni E, Constantoyannis C (2009) DBS for secondary dystonia. Results on 8 patients. Acta Neurochir 151:473–478CrossRefGoogle Scholar
  19. 19.
    Krauss JK, Loher TJ, Weigel R, Capelle HH, Weber S, Burgunder JM (2003) Chronic stimulation of the globus pallidus internus for treatment of non-dYT1 generalized dystonia and choreoathetosis: 2-year follow up. J Neurosurg 98:785–792CrossRefPubMedGoogle Scholar
  20. 20.
    Kumar R, Dagher A, Hutchison WD, Lang AE, Lozano AM (1999) Globus pallidus deep brain stimulation for generalized dystonia: clinical and PET investigation. Neurology 53:871–874PubMedGoogle Scholar
  21. 21.
    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 355:1978–1990CrossRefPubMedGoogle Scholar
  22. 22.
    Kupsch A, Kuehn A, Klaffke S, Meissner W, Harnack D, Winter C, Haelbig TD, Kivi A, Arnold G, Einhaupl KM, Schneider GH, Trottenberg T (2003) Deep brain stimulation in dystonia. J Neurol 250(Suppl 1):I47–I52PubMedGoogle Scholar
  23. 23.
    Liu X, Yianni J, Wang S, Bain PG, Stein JF, Aziz TZ (2006) Different mechanisms may generate sustained hypertonic and rhythmic bursting muscle activity in idiopathic dystonia. Exp Neurol 198:204–213CrossRefPubMedGoogle Scholar
  24. 24.
    Marsden CD, Obeso JA, Zarranz JJ, Lang AE (1985) The anatomical basis of symptomatic hemidystonia. Brain 108(Pt 2):463–483CrossRefPubMedGoogle Scholar
  25. 25.
    Meunier S, Lehericy S, Garnero L, Vidailhet M (2003) Dystonia: lessons from brain mapping. Neuroscientist 9:76–81CrossRefPubMedGoogle Scholar
  26. 26.
    Mueller J, Skogseid IM, Benecke R, Kupsch A, Trottenberg T, Poewe W, Schneider GH, Eisner W, Wolters A, Muller JU, Deuschl G, Pinsker MO, Roeste GK, Vollmer-Haase J, Brentrup A, Krause M, Tronnier V, Schnitzler A, Voges J, Nikkhah G, Vesper J, Naumann M, Volkmann J (2008) Pallidal deep brain stimulation improves quality of life in segmental and generalized dystonia: results from a prospective, randomized sham-controlled trial. Mov Disord 23:131–134CrossRefPubMedGoogle Scholar
  27. 27.
    Ostrem JL, Starr PA (2008) Treatment of dystonia with deep brain stimulation. Neurotherapeutics 5:320–330CrossRefPubMedGoogle Scholar
  28. 28.
    Playford ED, Passingham RE, Marsden CD, Brooks DJ (1998) Increased activation of frontal areas during arm movement in idiopathic torsion dystonia. Mov Disord 13:309–318CrossRefPubMedGoogle Scholar
  29. 29.
    Pralong E, Pollo C, Villemure JG, Debatisse D (2007) Opposite effects of internal globus pallidus stimulation on pallidal neurones activity. Mov Disord 22:1879–1884CrossRefPubMedGoogle Scholar
  30. 30.
    Pretto TE, Dalvi A, Kang UJ, Penn RD (2008) A prospective blinded evaluation of deep brain stimulation for the treatment of secondary dystonia and primary torticollis syndromes. J Neurosurg 109:405–409CrossRefPubMedGoogle Scholar
  31. 31.
    Schaltenbrand G, Wahren A (1982) Stereotaxic atlas of the human brain. Thieme, New YorkGoogle Scholar
  32. 32.
    Thobois S, Ballanger B, Xie-Brustolin J, Damier P, Durif F, Azulay JP, Derost P, Witjas T, Raoul S, Le Bars D, Broussolle E (2008) Globus pallidus stimulation reduces frontal hyperactivity in tardive dystonia. J Cereb Blood Flow Metab 28:1127–1138CrossRefPubMedGoogle Scholar
  33. 33.
    Tisch S, Rothwell JC, Limousin P, Hariz MI, Corcos DM (2007) The physiological effects of pallidal deep brain stimulation in dystonia. IEEE Trans Neural Syst Rehabil Eng 15:166–172CrossRefPubMedGoogle Scholar
  34. 34.
    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 248:695–700CrossRefPubMedGoogle Scholar
  35. 35.
    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 352:459–467CrossRefPubMedGoogle Scholar
  36. 36.
    Vitek JL (2002) Pathophysiology of dystonia: a neuronal model. Mov Disord 17(Suppl 3):S49–S62CrossRefPubMedGoogle Scholar
  37. 37.
    Zhang JG, Zhang K, Wang ZC, Ge M, Ma Y (2006) Deep brain stimulation in the treatment of secondary dystonia. Chin Med J (Engl) 119:2069–2074Google Scholar

Copyright information

© Springer-Verlag 2010

Authors and Affiliations

  • Zinovia Kefalopoulou
    • 1
  • Anna Paschali
    • 2
  • Elli Markaki
    • 1
  • John Ellul
    • 3
  • Elisabeth Chroni
    • 3
  • Pavlos Vassilakos
    • 2
  • Constantine Constantoyannis
    • 1
    • 4
  1. 1.Functional Neurosurgery Unit, Department of NeurosurgeryMedical School of PatrasPatrasGreece
  2. 2.Department of Nuclear MedicineMedical School of PatrasPatrasGreece
  3. 3.Department of NeurologyMedical School of PatrasPatrasGreece
  4. 4.Functional Neurosurgery Unit, Department of Neurosurgery, School of MedicineUniversity of PatrasRionGreece

Personalised recommendations