Available treatment options for dystonia

ABSTRACT

Introduction: Dystonia is a neurologic movement disorder characterized by sustained or intermittent muscle contractions causing abnormal, often repetitive, movements, postures, or both. The etiology of dystonia is diverse and it includes genetic and other causes. Since the advent of botulinum toxin and deep brain stimulation, the treatment of dystonia has markedly improved and is still evolving.

Areas covered: This review gives a historical overview of the treatment options for dystonia. In the absence of pathogenesis-targeted, disease-modifying therapies, the treatment of dystonia remains symptomatic. Botulinum toxin is the first line treatment for some forms of focal and segmental dystonias. Oral medications, including anticholinergics, baclofen, benzodiazepines and antidopaminergic drugs are used primarily in patients with generalized dystonia. Levodopa is an effective treatment for patients with dopa-responsive-dystonia. The use of deep brain stimulation, intrathecal and intraventricular baclofen, as well as ablative surgeries are options for patients with disabling dystonia refractory to other, less invasive, therapies.

Expert opinion: The selected treatment modality should be tailored to the patient`s needs and guided by potential side effects. Refractory cases should be referred to tertiary center for evaluation with a multidisciplinary team and potential surgical treatment.

KEYWORDS:

• Dystonia
• botulinum toxin
• deep brain stimulation

Article highlights

• The treatment of dystonia is symptomatic.

• The first line of treatment is botulinum toxin in patients with focal or segmental dystonia.

• Oral therapies such as anticholinergics, baclofen, benzodiazepines and antidopaminergic drugs, are more often used in generalized dystonias.

• Levodopa should be considered in in patients suspected to have dopa-responsive dystonia.

• The use of intrathecal baclofen is suitable for dystonias secondary to hypoxic-ischemic brain injury or ‘spastic dystonia’.

• Deep brain stimulation targeting globus pallidus or subthalamic nucleus should be considered for the treatment of disabling generalized dystonia.

This box summarizes key points contained in the article.

Declaration of interest

J Jankovic has reciveed research and/or training grants from: Adamas Pharmaceuticals, Inc; Allergan, Inc; Botie Therapies, CHDI Foundation; Civitas/Acorda Therapeutics; Dystonia Coalition; Dystonia Medical Research Foundation; Hoffman-La Roche; Huntington Study group; Kyowa Haako Kirin Pharma, Inc; Medtronic Neuromodulation; Merz Pharmaceuticals; Michael J Fox Foundation for Parkinson Research; Nationa Institutes of Health; Neurocrine Biosciences; NeuroDerm Ltd; Parkinson’s Foundation; Parkinson Study Group; Pfizer, Prothena Biosciences Inc, Psyadon Pharmaceuticals, Inc; Revance Therapeutics, Inc, Sangamo Biosciences, Inc; St Jude Medical; Teva Pharmaceutical Industries Ltd. J Jancovic has servies as a consultant or as an advisory committee member for: Adamas Pharmaceuticals, Inc; Allergan, Inc; Pfizer Inc, Revance Therapeutics, Inc; Teva Pharmaceutical Insustries Ltd. He has also received royalties or other payments from: Cambridge; Elsevier; Future Science Group; Hodder Arnold; Medlink; Neurology; Lippincott Williams ad Wilkins; Wiley-Blackwell. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Additional information

Funding

This paper was not funded