Deep Brain Stimulation for Dystonia

Dipankar Nandi, Sean O’Riordan, Peter G Bain
European Neurological Review, 2009;4(2):79-82
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Abstract

There is now strong evidence demonstrating that bilateral globus pallidus internus (GPi) stimulation improves motor function and disability in patients with severe primary generalised, segmental or cervical dystonia. The improvement is gradual, typically occurring over a period of about six months, with pain and then phasic components dissipating before the tonic elements of dystonia. Controlled data indicate that the benefits of GPi stimulation are in the order of 40–60% and are sustained at three years. Further work is required to better understand variation in the extent of the response. The procedure is usually performed under general anaesthesia and is well tolerated, although minor and hardwarerelated complications are common. GPi stimulation can also provide useful benefit to selected patients with secondary dystonia, with encouraging results reported for tardive dystonia, myoclonic dystonia and pantothenate-kinase-associated neurodegeneration. Currently, it is not known whether the nucleus ventralis intermedius of the thalamus or GPi is the optimal target for the surgical treatment of writer’s cramp.
Keywords
Primary generalised dystonia, cervical dystonia, secondary dystonia, deep brain stimulation, globus pallidus internus
Disclosure Dipankar Nandi and Peter G Bain receive royalties from Oxford University Press for their book titled Deep Brain Stimulation and are/have been in receipt of grants from Medtronic, a manufacturer of deep brain stimulation equipment. Sean O’Riordan has no conflicts of interest to declare.
Received: March 09, 2009 Accepted July 14, 2009
Correspondence: Peter G Bain, Movement Disorders and Neuromodulation Group, Division of Neurosciences, Imperial College London, Charing Cross Hospital, London, W6 8RF, UK. E: p.bain@ic.ac.uk

Dystonia is a symptom that involves involuntary muscle contractions that are frequently sustained, but can be phasic. These contractions or spasms frequently result in abnormal posturing or repetitive movements in various parts of the body. Dystonia is often accompanied by tremor.

Dystonia has multiple causes but may be categorised by age at onset or by the underlying aetiology as either primary (idiopathic), which may be sporadic or familial, or secondary, the latter often resulting from a neurodegenerative disorder.1 The most commonly inherited form of primary dystonia results from a defect in the DYT-1 gene.2 The specific syndromes of myoclonus-dystonia and dopa-responsive dystonia are classified under the term ‘dystonia plus syndromes’.

Dystonia can also be categorised anatomically according to the part(s) of the body affected by dystonia:

• focal dystonia – involves a single part of the body, for example spasmodic torticollis (cervical dystonia) or blepharospasm or writer’s cramp;
• segmental – involves two or more contiguous regions of the body, for example cervical dystonia or writer’s cramp;
• multifocal – involves two or more non-contiguous regions of the body, for example blepharospasm or dystonia in one foot; and
• generalised – involves both legs and at least one other part of the body.

Levodopa may produce considerable amelioration of symptoms in doparesponsive dystonia. Similarly, alcohol, used judiciously, can be very effective in suppressing myoclonus-dystonia. In general, however, dystonia responds poorly to oral medication, although high doses of anticholinergics or benzodiazepines may have beneficial effects in some cases. Botulinum toxin is the treatment of choice for focal dystonia and is widely used for managing patients with blepharospasm, vocal dystonia and spasmodic torticollis, as well as other focal or segmental dystonias.3,4 However, in some cases tolerance to treatment, mediated through antibodies to botulinum toxin type A or B, may eventually develop.3,4

References:
  1. Trost M, Curr Opin Neurol, 2003;16:495–500.
  2. Németh AH, Brain, 2002;125:695–721.
  3. Jankovic J, Lancet Neurol, 2006;5:864–72.
  4. Albanese A, et al., Eur J Neurol, 2006;13:433–44.
  5. Ford B, et al., J Neurol Neurosurg Psychiatry, 1998;65:472–8.
  6. Krauss JK, et al., J Neurol Neurosurg Psychiatry, 1997;63:642–8.
  7. Krack P, et al., Eur J Neurol, 2001;8:389–99.
  8. Blahak C, et al., J Neurology, 2008;255:178–82.
  9. Capelle HH, Neurology, 2003;60:2017–18.
  10. Coubes P, et al., J Neurosurg, 2004;101:189–94.
  11. Coubes P, et al., Lancet, 2000; 355:w2220–21.
  12. Damier P, et al.; Arch Gen Psychiatry, 2007;64:170–76.
  13. Holloway K, et al., Neuromodulation, 2006;9:253–61.
  14. Hung SW, et al., Neurology, 2007;68:457–9.
  15. Kiss ZH, et al. Brain, 2007;130:2879–86.
  16. Krauss JK, et al., Lancet, 1999;354:837–8.
  17. Krauss JK, et al., J Neurosurg, 2003;98:785–92.
  18. Krauss JK, Acta Neurochir Supp, 2007;97:201–5.
  19. Kupsch A, et al., N Engl J Med, 2006;355:1978–90.
  20. Mueller J, et al., Movement Disorders, 2008;23:131–4.
  21. Vidailhet M, et al., Lancet Neurol, 2007;6:223–9.
  22. Vidailhet M, et al., N Engl J Med, 2005;352:459–67.
  23. Yianni J, et al., Mov Disord, 2003;18:436–42.
  24. Sansur CA, et al., J Neurosurg, 2007;107:998-–1003.
  25. Paluzzi A, et al., Br J Neurosurg, 2006;20:290–95.
  26. Wadia PM, et al., Deep Brain Stimulation, Oxford: Oxford University Press, 2009;106–17.
  27. Fukayama C, et al., J Neurosurg, 2007;107:977–82.
  28. Kleiner-Fisman G, et al., J Neurosurg, 2007;107:29–36.
  29. Yianni J, et al., J Neuropsychiatry Clin Neurosci, 2006;13:738–46.
  30. Krause M, et al., Neurosurgery, 2004;55:1361–70.
  31. Bittar RG, et al., J Clin Neurosci, 2005;12:12–16.
  32. Pillon B, et al., Neurology, 2006;66:1556–8.
  33. Castlenau P, et al., Ann Neurol, 2005;57:738–40.
  34. Trottenberg T, et al., Neurology, 2005;25;64:344–6.
  35. Sako W, et al., Mov Disord, 2008;23:1929–31.
  36. Cif L, et al., Mov Disord, 2004;19:724–7.
  37. Yianni J, et al., J Clin Neurosci, 2004;11:243–5.
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