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Dystonia

  • Maria Fiorella ContarinoEmail author
  • Joachim K. Krauss
Chapter
  • 32 Downloads

Abstract

Dystonic syndromes can be complex and present with different characteristics. Both accurate clinical diagnosis and characterization of dystonia can be very challenging and require expertise, but are crucial for an appropriate medical and surgical treatment.

Oral treatment, above all with anticholinergics, is the first choice in generalized forms of dystonia. However, the effects of medical treatment are often disappointing and limited by the onset of side effects. Although botulinum neurotoxin (BoNT) is effective and safe for focal or segmental dystonia, some patients do not respond to this treatment at all, or loose treatment effect over time.

Deep brain stimulation has become a cornerstone and an accepted treatment modality for treatment resistant patients with dystonia. The target of choice is the posteroventral lateral globus pallidus internus (GPi).

As opposed to Parkinson’s disease or tremors, programming of deep brain stimulation (DBS) in dystonia poses specific issues, since the effect on dystonic symptoms may be considerably delayed. Furthermore there is no uniform agreement on how chronic stimulation settings should be programmed, and pulse width and frequency varies between centers. Since often higher intensities for stimulation are needed, rechargeable implantable pulse generators may be considered.

Solid evidence shows that isolated idiopathic and inherited forms of dystonia respond consistently better to DBS compared to acquired combined forms, although occasionally good to excellent results can also be obtained in the latter. Unfortunately, a smaller fraction of patients does not respond to DBS as expected. Moreover, although quality of life is usually improved after DBS also in the long term, some patients can be less satisfied about the outcome despite sizable motor improvement. This calls for a broader preoperative evaluation and postoperative management, which includes also psychological and social aspects.

Given the complexity of these relative rare disorders, DBS treatment should be performed by experienced teams.

Keywords

Dystonia Deep brain stimulation Globus pallidus 

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Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  • Maria Fiorella Contarino
    • 1
    • 2
    Email author
  • Joachim K. Krauss
    • 3
  1. 1.Department of NeurologyLeiden University Medical CenterLeidenThe Netherlands
  2. 2.Department of NeurologyHaga Teaching HospitalThe HagueThe Netherlands
  3. 3.Department of NeurosurgeryHannover Medical SchoolHannoverGermany

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