Torsion Dystonias Treatment & Management

Updated: Feb 15, 2016
  • Author: Priyantha Herath, MD, PhD; Chief Editor: Selim R Benbadis, MD  more...
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Treatment

Medical Care

Available therapies for dystonia include oral medications or subcutaneous botulinum toxin injections, surgical procedures, and physical and/or rehabilitation therapies. [28, 29] Therapy for most people with dystonia is symptomatic, directed at controlling the intensity of the dystonic contractions.

  • Although no curative treatment for dystonia is available, treatment of the underlying disorder may help reverse symptoms in patients with secondary forms of dystonia (eg, from Wilson disease or DRD).

  • Early diagnosis and start of treatment for dystonia, though not proven to alter its course or increase the likelihood for remission, may improve quality of life and alleviate the disability of patients with dystonia.

  • Overall, about 40% of patients improve with oral therapy. Adverse effects of the particular agents used can limit the benefits.

  • Overall, the goals of therapy should be directed at increasing movement, alleviating pain, preventing contractures, restoring functional abilities, and minimizing adverse effects from medical therapy. [30]

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Surgical Care

Surgical care is reserved for patients with severe symptoms in whom drug therapy fails. In general, it should be considered in patients with generalized dystonia because these patients are severely affected, because their condition is most likely to be refractory to therapy, or because they have unfavorable responses to medical therapy primarily due to adverse effects related to their need for increasing doses or to drug interactions from polypharmacy. Careful patient selection is one of the most important aspects of ensuring a successful surgical outcome.

  • Thalamotomy was originally the preferred surgery for dystonia. [31] However, GPI pallidal deep brain stimulation (DBS) has produced remarkable improvement in dystonic symptoms associated with Parkinson disease. In light of this, pallidotomy or thalamotomy is no longer considered appropriate.

  • With the development of high-frequency stimulation as an alternative to the creation of surgical lesions, surgical procedures have become safer and adverse effects are easier to control than before. As the disease progresses, stimulation may be varied. [32]

  • Over the past few years, DBS of the globus pallidus interna (GPI) has gained widespread acceptance as an effective treatment for primary generalized dystonia. [33, 34, 35] {ref36

  • GPI DBS is becoming popular in patients with primary dystonia because of its effectiveness and safety. It can be proposed at the initial phase of the disease to limit the functional consequences and to improve the prognosis for functional recovery. The consensus is that the secondary forms are less responsive than primary forms, yet responses in secondary forms do occur. [39]

  • Selective peripheral denervation with partial rhizotomy performed by an experienced surgeon may have a role in cervical dystonia that does not respond to other therapies. [41]

  • Myectomy may be beneficial for blepharospasm and minimally effective for cervical dystonia. Problems include weakness and disfigurement.

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