Treatment of Dystonia: Medications, Neurotoxins, Neuromodulation, and Rehabilitation
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REVIEW
Treatment of Dystonia: Medications, Neurotoxins, Neuromodulation, and Rehabilitation Ian O. Bledsoe 1
&
Aaron C. Viser 1 & Marta San Luciano 1
Accepted: 4 October 2020 # The American Society for Experimental NeuroTherapeutics, Inc. 2020
Abstract Dystonia is a complex disorder with numerous presentations occurring in isolation or in combination with other neurologic symptoms. Its treatment has been significantly improved with the advent of botulinum toxin and deep brain stimulation in recent years, though additional investigation is needed to further refine these interventions. Medications are of critical importance in forms of dopa-responsive dystonia but can be beneficial in other forms of dystonia as well. Many different rehabilitative paradigms have been studied with variable benefit. There is growing interest in noninvasive stimulation as a potential treatment, but with limited long-term benefit shown to date, and additional research is needed. This article reviews existing evidence for treatments from each of these categories. To date, there are many examples of incomplete response to available treatments, and improved therapies are needed. Key Words Dystonia . botulinum toxin . neuromodulation . deep brain stimulation . rehabilitation
Introduction Dystonia is a heterogeneous disorder with many etiologies, varied clinical presentations, and diverse treatment responses. Despite the considerable progress in our understanding of this condition, disease-modifying therapies do not exist for most dystonia types. However, the symptomatic treatment of dystonia has improved markedly since the introduction of botulinum toxin (BoNT) and deep brain stimulation surgery (DBS). Treatments for dystonia must be individualized and tailored to each patient with attention to the patient’s age, anatomic distribution of dystonic symptoms, and the potential risk for adverse effects. Treatments may aim to improve abnormal movements, postures, and discomfort as well as
* Ian O. Bledsoe [email protected] Aaron C. Viser [email protected] Marta San Luciano [email protected] 1
Weill Institute for Neurosciences, Movement Disorder and Neuromodulation Center, University of California, San Francisco, 1635 Divisadero St., Suite 520, San Francisco, CA 94115, USA
to manage comorbidities such as mood disorders, contractures, and orthopedic complications. While a robust evidence base is growing in some areas of dystonia therapeutics, including deep brain stimulation and some aspects of neurotoxin treatment, there are notable gaps in welldesigned and controlled trials in other areas. Some of the challenges in designing trials in dystonia include a limited ability of clinical scales to capture true functional change of therapies in some dystonia subtypes and the large heterogeneity in etiology and clinical features of different forms of dystonia. Many studies are uncontrolled, present only short-term follow-up information, and may have relatively small sample sizes. In this review, we will outline the few avai
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