Patient Selection Criteria for Deep Brain Stimulation for Dystonia

2020 ◽  
pp. 175-184
Author(s):  
Laura S. Surillo Dahdah ◽  
Rasheda El-Nazer ◽  
Richard B. Dewey ◽  
Padraig O’Suilleabhain ◽  
Shilpa Chitnis

Dystonia is defined as a movement disorder characterized by sustained or intermittent muscle contractions causing abnormal, often repetitive, movements, postures, or both. Dystonic movements are typically patterned and twisting and may be tremulous. Dystonia is often initiated or worsened by voluntary action and associated with overflow muscle activation. A recent revision now classifies dystonia into two axes: (1) clinical characteristics (age at onset, temporal pattern, body distribution, whether focal, segmental, or generalized; and associated features) and (2) etiology, whether idiopathic/genetic or secondary to other neurological/medical diseases. Pharmacological treatments for dystonia remain generally unsatisfactory and consist of various combinations of levodopa, anticholinergics, muscle-relaxing drugs as well as botulinum toxin injections in focal and segmental dystonia. Overall in outcomes are poor because of limited efficacy and the potential for significant side effects such as sedation and cognitive impairment. A humanitarian-device exemption from the Food and Drug Administration was issued for the treatment of medically refractory symptoms of generalized dystonia with the use of DBS. Bilateral GPi DBS surgery is effective for both generalized and focal dystonia including cervical dystonia and tardive dystonia. DBS may be the best available treatment for disabling symptoms of generalized, cervical, tardive, and other dystonia that have failed to respond to oral drugs and botulinum toxin injections (when applicable) as long as contractures have not developed, because in this situation, DBS will be ineffective. Rigorous patient selection and careful management of comorbidities are essential for favorable outcomes.

2020 ◽  
Author(s):  
Laura M. Scorr ◽  
Stewart A. Factor ◽  
Sahyli Perez Parra ◽  
Rachel Kaye ◽  
Randal C Paniello ◽  
...  

Objective: To better characterize oromandibular dystonia (OMD) to facilitate early diagnosis and test the hypothesis that botulinum toxin treatment alleviates symptoms, regardless of etiology, to provide guidance on treatment strategies. Methods: To better characterize this condition we utilize a three-pronged approach. First, we provide a comprehensive summary of the worlds literature encompassing 1157 cases in 27 separate manuscripts. Next, we describe the clinical features of 727 OMD subjects enrolled by the Dystonia Coalition (DC), an international multicenter database. Finally, we provide details of the treatment approach and response from two expert centers where large numbers of OMD patients are followed. Cases from expert centers were utilized to analyze whether response to botulinum toxin varied by etiology of OMD. Results: In all cohorts, typical age at onset was in the 50s and approximately 70% of cases were female. Although the literature OMD more commonly described as a focal dystonia, analysis of the DC database revealed it more commonly appears as part of a segmental dystonia. Expert center review of 173 cases revealed botulinum toxin injections improved symptom severity by more than 50% in approximately 78% of subjects. Among the patients at expert centers, analysis revealed that treatment response did not vary by etiology. Conclusions: Botulinum toxin injections are an effective treatment for OMD, regardless of etiology. By providing a more comprehensive description of OMD and the therapeutic efficacy of botulinum toxin for this type of dystonia, we hope to improve clinical recognition to aid in timely diagnosis and inform treatment strategies.


1992 ◽  
Vol 50 (4) ◽  
pp. 426-432 ◽  
Author(s):  
Luiz A. F. Andrade ◽  
Henrique B. Ferraz

Dystonia may be classified by age of onset (childhood, adolescence, adult onset), body distribution of the abnormal movements (focal, segmental, unilateral, multifocal and generalized) and etiology (idiopathic and symptomatic). We studied 76 patients with idiopathic dystonia among 122; cases of dystonic syndrome (62.3% of the total). There were 48 female and 28 male patients. Adult-onset focal dystonia was the most frequent feature (37 patients). The onset of generalized dystonia was more frequently seen under the age of 20, whereas focal and segmental dystonia usually started over this age. Postural tremor of the hands was observed in 19.7% of the patients. Spasmodic torticollis was the most prevalent form of dystonia overall. Except for writer's cramp, which occurred more frequently in males, and generalized dystonia, which was equally divided between sexes, all other forms were more frequent in females. Our data suggest that differences in racial origin, social and economical status and environmental factors do not account for a different manifestation in dystonia pattern.


2021 ◽  
Vol 12 ◽  
Author(s):  
Laura M. Scorr ◽  
Stewart A. Factor ◽  
Sahyli Perez Parra ◽  
Rachel Kaye ◽  
Randal C. Paniello ◽  
...  

Objective: The goal of this study is to better characterize the phenotypic heterogeneity of oromandibular dystonia (OMD) for the purpose of facilitating early diagnosis.Methods: First, we provide a comprehensive summary of the literature encompassing 1,121 cases. Next, we describe the clinical features of 727 OMD subjects enrolled by the Dystonia Coalition (DC), an international multicenter cohort. Finally, we summarize clinical features and treatment outcomes from cross-sectional analysis of 172 OMD subjects from two expert centers.Results: In all cohorts, typical age at onset was in the 50s and 70% of cases were female. The Dystonia Coalition cohort revealed perioral musculature was involved most commonly (85%), followed by jaw (61%) and tongue (17%). OMD more commonly appeared as part of a segmental dystonia (43%), and less commonly focal (39%) or generalized (10%). OMD was found to be associated with impaired quality of life, independent of disease severity. On average, social anxiety (LSA score: 33 ± 28) was more common than depression (BDI II score: 9.7 ± 7.8). In the expert center cohorts, botulinum toxin injections improved symptom severity by more than 50% in ~80% of subjects, regardless of etiology.Conclusions: This comprehensive description of OMD cases has revealed novel insights into the most common OMD phenotypes, pattern of dystonia distribution, associated psychiatric disturbances, and effect on QoL. We hope these findings will improve clinical recognition to aid in timely diagnosis and inform treatment strategies.


1996 ◽  
Vol 105 (1) ◽  
pp. 33-42 ◽  
Author(s):  
Bruce J. Davidson ◽  
Christy L. Ludlow

The purpose was to examine whether physiological changes can be found in laryngeal muscles following repeated treatment with botulinum toxin injections in spasmodic dysphonia. Seven patients whose treatment consisted of multiple unilateral thyroarytenoid injections were examined more than 6 months following their most recent botulinum toxin injection by fiberoptic laryngoscopy and electromyography. Comparisons were made between injected and contralateral noninjected muscles' motor unit characteristics, muscle activation patterns, and vocal fold movement characteristics. The results demonstrated that motor unit characteristics differed between injected and noninjected muscles and that these differences were greater in patients less than 12 months since last injection. Motor unit duration differences were reduced and motor unit amplitude and numbers of turns were increased in muscles sampled over 1 year after injection. These results suggest that while the physiologic effects of botulinum toxin are reversible, the reinnervation process continues past 12 months following injection.


2021 ◽  
Vol 0 (0) ◽  
Author(s):  
Buket Tuğan Yıldız ◽  
Deniz Tuncel Berktaş

AbstractBackground and objectivesBotulinum toxin injection has been used for many years for various indications such as cervical dystonia, blepharospasm, oromandibular dystonia, hemifacial spasm in neurology. Botulinum toxin injections have been made in our clinic for about ten years. We want to report our experiences about botulinum toxin treatment in neurologic disease.MethodsIn this study, the data of the patients receiving BoNT injection between January 2018 and December 2019 was retrospectively analyzed. Age, gender, botulinum toxin indications, and mean dose, duration of efficacy, side effects noted for each injection were recorded.ResultsThere were 122 patients who received botulinum toxin injections between January 2018 and December 2019. Of the 122 patients identified, 28 had cervical dystonia, 61 had HFS, 21 had blepharospasm, 4 had generalized dystonia, 1 had hemidystonia following thalamic bleeding, 1 had tardive dystonia, 4 had migraine, 1 had bruxism and 1 had both migraine and bruxism.ConclusionBoNT injection is a treatment that has been used for various indications in neurology for almost 40 years. Side effects are limited and temporary with appropriate injections. We also had a wide range of indication profiles and high numbers of patients to whom we administered the BoNT treatment.


2004 ◽  
Vol 171 (4S) ◽  
pp. 452-452 ◽  
Author(s):  
Apostolos Apostolidis ◽  
Roshni Popat ◽  
Yiangos Yiangou ◽  
Preston A. Baecker ◽  
Anthony Ford ◽  
...  

Author(s):  
Selly Marisdina ◽  
Henry Sugiharto ◽  
A Pradian

Back Ground: Hemifacial spasm is one of movement disorder case that commonly found in daily clinical practice. Epidemiological data are very limited, the average prevalence is 11 per 100,000 population, 14.5 per 100,000 in women and 7.4 per 100,000 in men. In Germany, the estimated prevalence is 8000 to 9000 peoples.1 The incidence of women is more than that of men with a ratio of 2:1. Based on Yaltho and Jankovic study in 2011, out of 215 patients, the ratio of men to women was 1:1.8.2 One study in Indonesia also reported that most of the subjects were female (64.7%).3 Treatment with botulinum toxin injections is preferred to microvascular decompression surgery therapy, but this injection is only effective in a few months and quite expensive. This study is the first study to assess the effectiveness of dry needling on clinical improvement of hemifacial spasm compared to standard therapy of botulinum toxin injection.Methods: The study design was quasi experimental. Total of 24 subjects were divided into two groups. The first group underwent dry needling intervention while the other had botulinum injection. Clinical severity before and after treatment in both groups was assessed using Jankovic and HFS7 scores.Results: In dry needling group there were significant differences between Jankovic and HFS7 score at baseline and at week 1, 2, 3 and 4. While in botox group significant differences were also Jankovic and HFS7 score at baseline and at weeks 2 and 4. There were also a significant difference of Jankovic and HFS7 score when we compared dry needling group to botulinum toxin group.Conclusion: Dry needling can be an alternative treatment for hemifacial spasm, although clinical improvements based on Jankovic and HFS7 scores in dry needling group were not as effective as those with botulinum toxin injections.


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