Dystonia

Ailsa Snaith; Derick Wade

. 2014 Feb 28;2014:1211.

Dystonia

Ailsa Snaith ^1,^#, Derick Wade ^2,^#

PMCID: PMC3938056 PMID: 25347760

Abstract

Introduction

Dystonia is usually a lifelong condition with persistent pain and disability. Focal dystonia affects a single part of the body; generalised dystonia can affect most or all of the body. It is more common in women, and some types of dystonia are more common in people of Ashkenazi descent.

Methods and outcomes

We conducted a systematic review and aimed to answer the following clinical questions: What are the effects of drug treatments, surgical treatments, and physical treatments for focal and generalised dystonia? We searched: Medline, Embase, The Cochrane Library, and other important databases up to September 2013 (Clinical Evidence reviews are updated periodically; please check our website for the most up-to-date version of this review). We included harms alerts from relevant organisations such as the US Food and Drug Administration (FDA) and the UK Medicines and Healthcare products Regulatory Agency (MHRA).

Results

We found 19 studies that met our inclusion criteria. We performed a GRADE evaluation of the quality of evidence for interventions.

Conclusions

In this systematic review, we present information relating to the effectiveness and safety of the following interventions: acupuncture, amantadine, baclofen, benzatropine, biofeedback, botulinum toxins, bromocriptine, carbamazepine, carbidopa/levodopa, clonazepam, clozapine, deep brain stimulation of thalamus and globus pallidus, diazepam, gabapentin, haloperidol, lorazepam, myectomy (for focal dystonia), occupational therapy, ondansetron, physiotherapy, pregabalin, procyclidine, selective peripheral denervation (for focal dystonia), speech therapy, tizanidine, trazodone hydrochloride, and trihexyphenidyl.

Key Points

Dystonia is characterised by involuntary muscle contractions, resulting in abnormal postures and twisting of body parts.

It is often a lifelong condition, with persistent pain and disability.
Focal dystonia affects a single part of the body; generalised dystonia can affect most or all of the body.
It is more common in women, and some types of dystonia are more common in people of Ashkenazi descent.

Botulinum toxin is effective at relieving cervical dystonia in adults.

Botulinum A toxin and botulinum B toxin are both effective treatments for focal dystonia.
We don't know whether botulinum toxins are effective for generalised dystonia.

Although we assessed other treatments, we primarily found evidence for botulinum toxin, and it is currently the mainstay of treatment for focal dystonia.

We don't know whether any other drug treatments (amantadine, baclofen, benzatropine, bromocriptine, carbamazepine, carbidopa/levodopa, clonazepam, clozapine, diazepam, gabapentin, haloperidol, lorazepam, ondansetron, pregabalin, procyclidine, tizanidine, trazodone hydrochloride, and trihexyphenidyl) are effective for either focal or generalised dystonia.

We don't know whether deep brain stimulation of thalamus and globus pallidus is effective for either focal or generalised dystonia. We don't know whether any other surgical interventions (selective peripheral denervation or myectomy) are effective for focal dystonia.

Most people will see a physiotherapist after diagnosis, but there is no consistent approach to treatment. We don't know whether any other physical treatments (acupuncture, biofeedback, occupational therapy, or speech therapy) are effective for either focal or generalised dystonia.

About this condition

Definition

Dystonia is a neurological disorder characterised by involuntary, abnormal muscle contractions that result in sustained abnormal postures, twisting, or both, and repetitive movements of body parts. It arises from dysfunction of the motor control system within the central nervous system. Dystonia is most simply classified by location: focal dystonia involves a single body part; multifocal dystonia involves two or more unrelated body parts; segmental dystonia affects two or more adjacent parts of the body; hemidystonia involves the arm and leg on the same side of the body; and generalised dystonia affects most or all of the body. For the purpose of this review we have classified dystonia into focal dystonia and generalised/other dystonia. However, studies in which dystonia has been classified according to other classification systems are also covered. In addition to focal and generalised dystonia, classification may also be based on age at onset (early onset or late onset), or according to the cause of the dystonia: primary dystonia where dystonia is the only sign and no cause can be identified; dystonia-plus syndrome where dystonia is associated with other pathology (e.g., dopa-responsive dystonia and myoclonus dystonia); heredodegenerative dystonia where dystonia is a sign associated with neurological conditions, such as Parkinson's disease and Huntington's disease; and secondary dystonia where a cause (usually environmental) can be identified, such as head injury and use of drugs (e.g., neuroleptic drugs and metoclopramide). Certain dystonias may also be classified as task specific; examples of task-specific focal hand dystonia include writer's cramp, typist's cramp, and musician's cramp (affects, for example, pianists and flautists). Diagnosis: the clinical diagnosis of dystonia is based on the hallmark features of the abnormal, involuntary, and prolonged muscle contractions with consistent directionality that lead to an abnormal posture of the area affected. There is no definitive diagnostic test for dystonia. Investigation typically involves history and clinical examination, laboratory tests, and imaging, to establish severity and potential cause. Laboratory tests and neuro-imaging may help to rule out metabolic or structural causes. Genetic testing, electrophysiological tests, and tissue biopsy may also be considered. The goal of accurate diagnosis is to facilitate treatment choice.

Incidence/ Prevalence

Dystonia occurs worldwide, with prevalence estimates varying widely depending on study methodology. In the US, the prevalence of focal dystonia has been reported as 30 per 100,000 people. Cervical dystonia (torticollis or 'wry neck') is the most common adult form of focal dystonia, with a prevalence in Europe of 5.7 per 100,000. Other frequently occurring focal dystonias are blepharospasm (forceful eyelid closures), which affects 3.6 per 100,000 people, and limb dystonias (e.g., writer's cramp), which affect 1.4 per 100,000. In the US, the prevalence of generalised dystonia has been reported as 0.2–6.7 per 100,000 population; generalised dystonia affects more people of Ashkenazi descent. In Europe, the prevalence of primary dystonia has been estimated at 15.2 per 100,000. Studies identified to have rigorous methodology estimated the prevalence of early-onset (at <20 years of age) dystonia to be 11.1 per 100,000 for dystonia in people of Ashkenazi descent from the New York area, 60 per 100,000 for late-onset (at >20 years of age) dystonia in the overall population of Northern England, and 300 per 100,000 for late-onset dystonia in the Italian population (aged 50 years or older). Dystonia occurs more frequently in women.

Aetiology/ Risk factors

The pathophysiology of dystonia remains unclear. Dystonia may occur because of abnormal neurochemical transmission in the basal ganglia, brainstem, or both, resulting in abnormal execution of motor control. Focal dystonias have been associated with loss of inhibition, abnormal plasticity in the motor cortex, and impairments in spatial and temporal discrimination. There is debate on the extent to which psychological factors cause dystonia, although they can undoubtedly exacerbate it. Dystonia can be classified as primary (where underlying cause is unknown) or secondary (related to known disorders). The primary disorders may be further classified as hereditary or sporadic. Currently, 19 types of dystonia can be distinguished on a genetic basis, six of which are primary dystonias (DYT1, 2, 4, 6, 7, and 13). The remainder are secondary dystonia, dystonia-plus syndromes, and paroxysmal dystonias.

Prognosis

Dystonia is often a lifelong disorder, once it has started, although a small minority experience complete remission. Most people with dystonia have a normal life expectancy, but with continued symptoms. The presence and severity of symptoms are unpredictable, as symptoms may fluctuate over time (e.g., stressful situations may make symptoms worse) or may disappear or stabilise for a time. Regardless of the cause, dystonic contractions may have a chronic course and may lead to severe persistent pain and disability. Also, embarrassment caused by the symptoms may lead to social withdrawal. Prognosis seems to depend on a number of factors, including age at onset, distribution, and cause. Focal dystonia may become generalised over time. Dystonia with a later age of onset has a lower likelihood of spreading compared with dystonia beginning in childhood. Similarly, dystonia starting in the neck is less likely to spread than dystonia starting in the limbs.

Aims of intervention

To improve quality of life by minimising: immediate symptoms (movement, posture, pain); limitation of activities; pain; and social consequences, with minimal adverse effects of treatment.

Outcomes

Neurological disability: In dystonia clinical trials, outcome is usually measured using disease-specific rating scales: Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS), Tsui Scale, Cervical Dystonia Severity Scale (CDSS), Jankovic Rating Scale (JRS), and Blepharospasm Disability Index (BSDI; see table 1 ). Quality of life; adverse effects of treatment.

Table 1.

Commonly used rating scales for dystonia.

Scale	Feature	Interpretation	Range*
Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS)	Three subscales, assessed by clinician: (1) movement disorder severity (range 0–35) (2) disability (range 0–30) (3) pain (range 0–20)	A decrease in TWSTRS-total or subscale score indicates an improvement in the person's dystonia. Dystonia trials frequently use TWSTRS-total or the individual TWSTRS-severity, TWSTRS-pain, or TWSTRS-disability scales as the primary outcome	0–85
Tsui Scale	Clinician-assessed scale of impairment that grades severity of postural deviance (rotatocollis, antecollis, retrocollis, head tilt, and elevation of shoulder), acknowledges the presence or absence of head tremor, and includes whether the movements are continuous or intermittent		0–25
Cervical Dystonia Severity Scale (CDSS)	Uses a protractor and wall chart to rate the severity of the head's deviation from neutral in each of the three planes of motion (rotation, laterocollis, anterocollis/retrocollis)
Jankovic Rating Scale (JRS)	Includes two categories: severity and frequency, each with 5 rating classes of 0–4 points		0–8
Blepharospasm Disability Index (BSDI)	Disease-specific self-assessment scale consisting of 6 × 5-point items assessing vehicle driving, reading, watching TV, shopping, getting about on foot, and doing everyday activities	0 = no interference in these activities and 30 = severe interference	0–30
Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS)	Assessment of severity and frequency of dystonia in 9 body areas (including eyes, mouth, speech or swallowing, neck, right and left arms, trunk, and right and left legs)	0 = no dystonia and 120 = maximum severity	0–120
Writer’s Cramp Rating Scale (WCRS)	Assessment of writing posture (elbow, wrist, and fingers), movements (latency and tremor), and speed of writing	0 = no impairment and 30 = marked impairment	0–30

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*Higher score indicates greater severity in all scales.

Methods

Clinical Evidence search and appraisal September 2013. The following databases were used to identify studies for this systematic review: Medline 1966 to September 2013, Embase 1980 to September 2013, and The Cochrane Database of Systematic Reviews 2013, issue 2 (1966 to date of issue). Additional searches were carried out in the Database of Abstracts of Reviews of Effects (DARE) and the Health Technology Assessment (HTA) Database. We also searched for retractions of studies included in the review. Titles and abstracts identified by the initial search, run by an information specialist, were first assessed against predefined criteria by an evidence scanner. Full texts for potentially relevant studies were then assessed against predefined criteria by an evidence analyst. Studies selected for inclusion were discussed with an expert contributor. All data relevant to the review were then extracted by an evidence analyst. Study design criteria for inclusion in this review were: RCTs and published systematic reviews of RCTs in English, including open studies and containing more than 20 individuals (with any split per arm), of whom at least 80% were followed up. There was no minimum length of follow-up. We included RCTs and systematic reviews of RCTs where harms of an included intervention were assessed, applying the same study design criteria for inclusion as we did for benefits. In addition we use a regular surveillance protocol to capture harms alerts from organisations such as the FDA and the MHRA, which are added to the reviews as required. To aid readability of the numerical data in our reviews, we round many percentages to the nearest whole number. Readers should be aware of this when relating percentages to summary statistics such as relative risks (RRs) and odds ratios (ORs). We have performed a GRADE evaluation of the quality of evidence for interventions included in this review (see table). The categorisation of the quality of the evidence (high, moderate, low, or very low) reflects the quality of evidence available for our chosen outcomes in our defined populations of interest. These categorisations are not necessarily a reflection of the overall methodological quality of any individual study, because the Clinical Evidence population and outcome of choice may represent only a small subset of the total outcomes reported, and population included, in any individual trial. For further details of how we perform the GRADE evaluation and the scoring system we use, please see our website (www.clinicalevidence.com).

Table.

GRADE Evaluation of interventions for Dystonia.

Important outcomes	Neurological disability, Quality of life
Studies (Participants)	Outcome	Comparison	Type of evidence	Quality	Consistency	Directness	Effect size	GRADE	Comment
What are the effects of drug treatments for focal dystonia?
at least 14 (at least 1029)	Neurological disability	Botulinum A toxin versus placebo in cervical dystonia in adults	4	0	0	–1	0	Moderate	Directness point deducted for including only people who had previously responded to onabotulinumtoxinA in 1 RCT
3 (308)	Neurological disability	Botulinum B toxin versus placebo in cervical dystonia in adults	4	0	0	0	0	High
3 (252)	Neurological disability	Botulinum A toxin versus botulinum B toxin in cervical dystonia in adults	4	0	0	–2	0	Low	Directness points deducted for not reporting doses in 1 study and population differences between studies in previous experience with botulinum A toxin
1 (31)	Neurological disability	Low-dose (100 U Botox/250 U Dysport) versus high-dose (>200 U Botox/960 U Dysport) botulinum A toxin in cervical dystonia in adults	4	–2	0	–1	0	Very low	Quality points deducted for sparse data and incomplete reporting of results; directness point deducted for no direct comparison between groups
1 (92)	Neurological disability	Low-dose (2500–5000 U) versus high-dose (10,000 U) botulinum B toxin in cervical dystonia in adults	4	–1	–1	0	0	Low	Quality point deducted for sparse data; consistency point deducted for differing results with different outcome measures
1 (66)	Neurological disability	Botulinum A toxin versus trihexyphenidyl in cervical dystonia in adults	4	–2	0	–2	0	Very low	Quality points deducted for sparse data and incomplete reporting; directness points deducted for differences in disease severity between groups and short cycle intervals between injections affecting generalisability of results
1 (92)	Neurological disability	Botulinum B toxin in botulinum A toxin-resistant adults versus respondent adults	4	–2	0	0	0	Low	Quality points deducted for sparse data and incomplete reporting of results
1 (40)	Neurological disability	Botulinum A toxin versus placebo in people with writer's cramp	4	–1	–1	0	0	Low	Quality point deducted for sparse data; consistency point deducted for differing results with different outcome measures
What are the effects of physical treatments for focal dystonia?
1 (40)	Neurological disability	Physiotherapy plus biofeedback plus drug treatment versus drug treatment alone	4	–2	0	–1	0	Very low	Quality points deducted for sparse data, results after crossover, and unequal observation periods; directness point deducted for including only people who had previously responded to botulinum A toxin
1 (20)	Neurological disability	Physiotherapy plus relaxation versus no physiotherapy plus relaxation	4	–1	0	–1	0	Low	Quality point deducted for sparse data; directness point deducted for including a subset of participants who were also receiving botulinum toxin
1 (20)	Quality of life	Physiotherapy plus relaxation versus no physiotherapy plus relaxation	4	–1	0	–1	0	Low	Quality point deducted for sparse data; directness point deducted for including a subset of participants who were also receiving botulinum toxin
1 (31)	Quality of life	Voice therapy plus botulinum A toxin versus sham voice therapy plus botulinum A toxin versus botulinum A toxin-only for laryngeal dystonia (adductor spasmodic dysphonia)	4	–3	0	0	0	Very low	Quality points deducted for sparse data, incomplete reporting of results, selection bias, and botulinum toxin dose inconsistencies
What are the effects of surgical treatments for generalised dystonia?
1 (40)	Neurological disability	Deep brain stimulation versus sham treatment	4	–2	0	–1	0	Very low	Quality points deducted for sparse data and no long-term results; directness point deducted for inclusion of mixed population of people with focal and generalised dystonia
1 (less than 40)	Quality of life	Deep brain stimulation versus sham treatment	4	–2	–1	–1	0	Very low	Quality points deducted for sparse data and no long-term results; consistency point deducted for lack of consistent benefit in different elements of quality of life; directness point deducted for inclusion of people with focal dystonia, affecting generalisability of results

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We initially allocate 4 points to evidence from RCTs, and 2 points to evidence from observational studies. To attain the final GRADE score for a given comparison, points are deducted or added from this initial score based on preset criteria relating to the categories of quality, directness, consistency, and effect size. Quality: based on issues affecting methodological rigour (e.g., incomplete reporting of results, quasi-randomisation, sparse data [<200 people in the analysis]). Consistency: based on similarity of results across studies. Directness: based on generalisability of population or outcomes. Effect size: based on magnitude of effect as measured by statistics such as relative risk, odds ratio, or hazard ratio.

Glossary

High-quality evidence: Further research is very unlikely to change our confidence in the estimate of effect.
Low-quality evidence: Further research is very likely to have an important impact on our confidence in the estimate of effect and is likely to change the estimate.
Moderate-quality evidence: Further research is likely to have an important impact on our confidence in the estimate of effect and may change the estimate.
Very low-quality evidence: Any estimate of effect is very uncertain.

Disclaimer

The information contained in this publication is intended for medical professionals. Categories presented in Clinical Evidence indicate a judgement about the strength of the evidence available to our contributors prior to publication and the relevant importance of benefit and harms. We rely on our contributors to confirm the accuracy of the information presented and to adhere to describe accepted practices. Readers should be aware that professionals in the field may have different opinions. Because of this and regular advances in medical research we strongly recommend that readers' independently verify specified treatments and drugs including manufacturers' guidance. Also, the categories do not indicate whether a particular treatment is generally appropriate or whether it is suitable for a particular individual. Ultimately it is the readers' responsibility to make their own professional judgements, so to appropriately advise and treat their patients. To the fullest extent permitted by law, BMJ Publishing Group Limited and its editors are not responsible for any losses, injury or damage caused to any person or property (including under contract, by negligence, products liability or otherwise) whether they be direct or indirect, special, incidental or consequential, resulting from the application of the information in this publication.

Contributor Information

Ailsa Snaith, Oxford Radcliffe Hospitals Trust Oxford, UK.

Derick Wade, Oxford Centre for Enablement, Oxford, UK.

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BMJ Clin Evid. 2014 Feb 28;2014:1211.

Botulinum toxins for focal dystonia (e.g., botulinum A toxin, botulinum B toxin)

Summary

Botulinum toxin is effective at relieving cervical dystonia symptoms in adults.

Botulinum A toxin and botulinum B toxin are both effective.

We found most evidence for botulinum toxin, and it is the mainstay of modern treatment for focal dystonia.

NOTE: We found no clinically important results from RCTs about botulinum A toxin in the treatment of people with focal dystonia of other body sites (eyelid, larynx, and hand). We found no clinically important results about other botulinum toxins, apart from botulinum A toxin and botulinum B toxin, in the treatment of focal dystonia.

Benefits and harms

Botulinum A toxin versus placebo in cervical dystonia in adults:

We found one systematic review (search date 2003) and three subsequent RCTs.

Neurological disability

Botulinum A toxin compared with placebo Botulinum A toxin is more effective at improving cervical dystonia at up to 20 weeks, as assessed by an improvement in Tsui Scale, Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS), Cervical Dystonia Severity Scale (CDSS), physician- and patient-rated scores, and the proportion of people reporting pain relief (moderate-quality evidence).

Ref (type)	Population	Outcome, Interventions	Results and statistical analysis	Effect size	Favours
Neurological disability
Systematic review	113 adults with cervical dystonia 3 RCTs in this analysis	Improvement of at least 3 points on Tsui Scale 3–6 weeks 32/56 (57%) with botulinum A toxin 13/57 (23%) with placebo	OR 4.25 95% CI 2.00 to 9.05 P = 0.002 NNT 4 95% CI 3 to 6	Moderate effect size	botulinum A toxin
Systematic review	353 adults with cervical dystonia 6 RCTs in this analysis	Any improvement in Tsui Scale or Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) 0–12 weeks 97/174 (56%) with botulinum A toxin 31/179 (17%) with placebo	OR 5.47 95% CI 3.52 to 8.48 P = 0.002	Large effect size	botulinum A toxin
Systematic review	510 adults with cervical dystonia 11 RCTs in this analysis	Any improvement in subjective patient-related scales 0–16 weeks 161/273 (59%) with botulinum A toxin 46/237 (19%) with placebo	OR 6.58 95% CI 4.55 to 9.54 P = 0.00001 NNT 3 95% CI 3 to 3	Large effect size	botulinum A toxin
Systematic review	350 adults with cervical dystonia 4 RCTs in this analysis	Physicians reporting improvement 0–16 weeks 123/197 (62%) with botulinum A toxin 46/153 (30%) with placebo	OR 4.17 95% CI 2.70 to 6.44 P <0.00001 NNT 3 95% CI 3 to 5	Moderate effect size	botulinum A toxin
Systematic review	162 adults with cervical dystonia 5 RCTs in this analysis	Proportion of people reporting pain relief time frame not reported 60/84 (71%) with botulinum A toxin 9/78 (12%) with placebo	OR 11.92 95% CI 6.32 to 22.5 P <0.00001 NNT 2 95% CI 2 to 3	Large effect size	botulinum A toxin
RCT	116 adults with cervical dystonia	Mean change in TWSTRS-Total score from baseline at week 4 –15.6 with botulinum A toxin –6.7 with placebo	P <0.001 Intention-to-treat analysis	Effect size not calculated	botulinum A toxin
RCT 3-armed trial	233 adults with cervical dystonia	Mean change in TWSTRS-Total score from baseline at week 4 –9.9 with incobotulinumtoxinA (120 U) –2.2 with placebo	Adjusted mean difference –7.5 95% CI –10.4 to –4.6 P <0.001 Intention-to-treat analysis	Effect size not calculated	botulinum A toxin
RCT 3-armed trial	233 adults with cervical dystonia	Mean change in TWSTRS-Total score from baseline at week 4 –10.9 with incobotulinumtoxinA (240 U) –2.2 with placebo	Adjusted mean difference –9.0 95% CI –12.0 to –5.9 P <0.001 Intention-to-treat analysis	Effect size not calculated	botulinum A toxin
RCT 3-armed trial	233 adults with cervical dystonia	Mean change in TWSTRS-Total score from baseline at week 8 –6.9 with incobotulinumtoxinA (120 U) +0.4 with placebo	Adjusted mean difference –7.1 95% CI –10.1 to –4.2 P <0.001 Intention-to-treat analysis	Effect size not calculated	botulinum A toxin
RCT 3-armed trial	233 adults with cervical dystonia	Mean change in TWSTRS-Total score from baseline at week 8 –8.2 with incobotulinumtoxinA (240 U) +0.4 with placebo	Adjusted mean difference –8.6 95% CI –11.5 to –5.8 P <0.001 Intention-to-treat analysis	Effect size not calculated	botulinum A toxin
RCT 3-armed trial	233 adults with cervical dystonia	Mean change in TWSTRS-Total score from baseline up to 20 weeks –3.6 with incobotulinumtoxinA (120 U) +1.7 with placebo	Adjusted mean difference –5.2 95% CI –7.4 to –3.0 P <0.001 Intention-to-treat analysis	Effect size not calculated	botulinum A toxin
RCT 3-armed trial	233 adults with cervical dystonia	Mean change in TWSTRS-Total score from baseline up to 20 weeks –4.6 with incobotulinumtoxinA (240 U) +1.7 with placebo	Adjusted mean difference –6.4 95% CI –8.6 to –4.2 P <0.001 Intention-to-treat analysis	Effect size not calculated	botulinum A toxin
RCT	170 adults with cervical dystonia and who previously responded to onabotulinumtoxinA injections; see further information on studies	Cervical Dystonia Severity Scale (CDSS) at week 6 Mean –1.81 points with known responders to onabotulinumtoxinA Mean –0.31 points with placebo	P = 0.012 Intention-to-treat analysis	Effect size not calculated	botulinum A toxin
RCT	170 adults with cervical dystonia and who previously responded to onabotulinumtoxinA injections; see further information on studies	Improved Physician Global Assessment Scale scores at week 6 62% with known responders to onabotulinumtoxinA 42% with placebo	P = 0.022	Effect size not calculated	botulinum A toxin

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Quality of life

No data from the following reference on this outcome.

Adverse effects

Ref (type)	Population	Outcome, Interventions	Results and statistical analysis	Effect size	Favours
Adverse effects
Systematic review	421 adults with cervical dystonia 6 RCTs in this analysis	Proportion of people with any adverse effect (neck weakness, dysphagia, voice changes/hoarseness, dry mouth/sore throat) 131/226 (58%) with botulinum A toxin 89/195 (46%) with placebo	OR 2.10 95% CI 1.32 to 3.25 P = 0.002 NNH 6 95% CI 4 to 15	Small effect size	placebo
Systematic review	605 adults with cervical dystonia 10 RCTs in this analysis	Proportion of people with neck weakness 62/339 (18%) with botulinum A toxin 9/266 (3%) with placebo	OR 4.86 95% CI 2.55 to 9.25 P <0.00001 NNH 8 95% CI 7 to 10	Moderate effect size	placebo
Systematic review	210 adults with cervical dystonia 2 RCTs in this analysis	Proportion of people with voice changes/hoarseness 15/120 (13%) with botulinum A toxin 4/90 (4%) with placebo	OR 2.62 95% CI 0.98 to 7.01 P = 0.05		Not significant
Systematic review	401 adults with cervical dystonia 6 RCTs in this analysis	Proportion of people with dry mouth/sore throat 42/216 (19%) with botulinum A toxin 15/185 (8%) with placebo	OR 2.54 95% CI 1.42 to 4.55 P = 0.002 NNH 10 95% CI 7 to 21	Moderate effect size	placebo
RCT 3-armed trial	233 adults with cervical dystonia	Proportion of people with dysphagia 8/78 (10%) with incobotulinumtoxinA (120 U) 13/81 (16%) with incobotulinumtoxinA (240 U) 2/74 (3%) with placebo	Significance not assessed
RCT 3-armed trial	233 adults with cervical dystonia	Proportion of people with neck pain 4/78 (5%) with incobotulinumtoxinA (120 U) 10/81 (12%) with incobotulinumtoxinA (240 U) 1/74 (1%) with placebo	Significance not assessed
RCT 3-armed trial	233 adults with cervical dystonia	Proportion of people with muscle weakness 5/78 (6%) with incobotulinumtoxinA (120 U) 8/81 (10%) with incobotulinumtoxinA (240 U) 1/74 (1%) with placebo	Significance not assessed
RCT	170 adults with cervical dystonia and who previously responded to onabotulinumtoxinA injections;	Proportion of people with treatment-related dysphagia 7% with known responders to onabotulinumtoxinA 0% with placebo Absolute numbers not reported	P = 0.03	Effect size not calculated	placebo
RCT	170 adults with cervical dystonia and who previously responded to onabotulinumtoxinA injections;	Proportion of people with treatment-related neck pain 1% with known responders to onabotulinumtoxinA 4% with placebo Absolute numbers not reported	Reported as not significant		Not significant

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No data from the following reference on this outcome.

Botulinum B toxin versus placebo in cervical dystonia in adults:

We found one systematic review (search date 2003).

Neurological disability

Botulinum B toxin compared with placebo Botulinum B toxin is more effective at 4 to 8 weeks at improving Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS)-total score and patient and physician assessments of global improvement in symptoms (high-quality evidence).

Ref (type)	Population	Outcome, Interventions	Results and statistical analysis	Effect size	Favours
Neurological disability
Systematic review	122 adults with cervical dystonia for at least 1 year and previously treated with botulinum A toxin, Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS)-total score >20 and TWSTRS-severity >10 Data from 1 RCT	Improvement of at least 20% of TWSTRS-total score 4 weeks 60/92 (65%) with botulinum B toxin 8/30 (27%) with placebo	OR 4.69 95% CI 2.06 to 10.69 P = 0.0002 NNT 3 95% CI 2 to 6	Moderate effect size	botulinum B toxin
Systematic review	122 adults with cervical dystonia for at least 1 year and previously treated with botulinum A toxin, TWSTRS-total score >20 and TWSTRS-severity >10 Data from 1 RCT	Improvement of at least 20% of TWSTRS-total score 8 weeks 40/92 (43%) with botulinum B toxin 5/30 (17%) with placebo	OR 3.13 95% CI 1.34 to 7.34 P = 0.0008	Moderate effect size	botulinum B toxin
Systematic review	122 adults with cervical dystonia for at least 1 year and previously treated with botulinum A toxin, TWSTRS-total score <20 and TWSTRS-severity <10 Data from 1 RCT	Improvement of at least 20% of TWSTRS-total score 12 weeks 23/92 (25%) with botulinum B toxin 3/30 (10%) with placebo	OR 2.43 95% CI 0.89 to 6.61 P = 0.08		Not significant
Systematic review	122 adults with cervical dystonia for at least 1 year and previously treated with botulinum A toxin, TWSTRS-total score >20 and TWSTRS-severity >10 Data from 1 RCT	Improvement of at least 20% of TWSTRS-total score 16 weeks 12/92 (13%) with botulinum B toxin 2/30 (7%) with placebo	OR 1.86 95% CI 0.51 to 6.75 P = 0.3		Not significant
Systematic review	150 adults with cervical dystonia for at least 1 year and previously treated with botulinum A toxin, TWSTRS-total score >20 and TWSTRS-severity >10 2 RCTs in this analysis	Patient global assessment of change in symptoms 0–4 weeks with botulinum B toxin (10,000 U) with placebo Absolute results not reported	WMD 20.04 95% CI 14.22 to 27.45 P <0.00001	Effect size not calculated	botulinum B toxin
Systematic review	72 adults with cervical dystonia for at least 1 year and previously treated with botulinum A toxin, TWSTRS-total score >20 and TWSTRS-severity >10 Data from 1 RCT	Patient global assessment of change in symptoms 0–4 weeks with botulinum B toxin (5000 U) with placebo Absolute results not reported	WMD 17.00 95% CI 6.93 to 27.07 P <0.0009	Effect size not calculated	botulinum B toxin
Systematic review	150 adults with cervical dystonia for at least 1 year and previously treated with botulinum A toxin, TWSTRS-total score >20 and TWSTRS-severity >10 2 RCTs in this analysis	Principal investigator global assessment of change in symptoms 0–4 weeks with botulinum B toxin (10,000 U) with placebo Absolute results not reported	WMD 12.52 95% CI 7.97 to 17.08 P <0.00001	Effect size not calculated	botulinum B toxin
Systematic review	72 adults with cervical dystonia for at least 1 year and previously treated with botulinum A toxin, TWSTRS-total score >20 and TWSTRS-severity >10 Data from 1 RCT	Principal investigator global assessment of change in symptoms 0–4 weeks with botulinum B toxin (5000 U) with placebo Absolute results not reported	WMD 13.30 95% CI 5.50 to 21.50 P = 0.001	Effect size not calculated	botulinum B toxin
Systematic review	150 adults with cervical dystonia for at least 1 year and previously treated with botulinum A toxin, TWSTRS-total score >20 and TWSTRS-severity >10 2 RCTs in this analysis	Patient analogue pain assessment 0–4 weeks with botulinum B toxin (10,000 U) with placebo Absolute results not reported	WMD 19.63 95% CI 11.69 to 27.56 P = 0.001	Effect size not calculated	botulinum B toxin
Systematic review	72 adults with cervical dystonia for at least 1 year and previously treated with botulinum A toxin, TWSTRS-total score >20 and TWSTRS-severity >10 Data from 1 RCT	Patient analogue pain assessment 0–4 weeks with botulinum B toxin (5000 U) with placebo Absolute results not reported	WMD 18.00 95% CI 5.69 to 30.31 P = 0.004	Effect size not calculated	botulinum B toxin

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Quality of life

No data from the following reference on this outcome.

Adverse effects

Ref (type)	Population	Outcome, Interventions	Results and statistical analysis	Effect size	Favours
Adverse effects
Systematic review	307 adults with cervical dystonia 3 RCTs in this analysis	Dry mouth 45/203 (22%) with botulinum B toxin 3/104 (3%) with placebo	OR 5.19 95% CI 2.69 to 10.03 P <0.00001 NNH 6 95% CI 6 to 8	Large effect size	placebo
Systematic review	307 adults with cervical dystonia 3 RCTs in this analysis	Dysphagia 39/204 (19%) with botulinum B toxin (10,000 U) 3/104 (3%) with placebo	OR 4.97 P <0.00003 NNH 8 95% CI 7 to 11	Moderate effect size	placebo

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Botulinum A toxin versus botulinum B toxin in cervical dystonia in adults:

We found one systematic review (search date 2003), which identified no RCTs. We found three subsequent RCTs.

Neurological disability

Botulinum A toxin compared with botulinum B toxin We don't know how effective botulinum A toxin and botulinum B toxins are, compared with each other, at up to 4 weeks at improving Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) disease-activity rating scores (low-quality evidence).

Ref (type)	Population	Outcome, Interventions	Results and statistical analysis	Effect size	Favours
Neurological disability
RCT	139 adults who previously responded to botulinum A toxin	Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS)-total score 0–4 weeks 10.2 with botulinum A toxin 9.3 with botulinum B toxin	P = 0.75		Not significant
RCT	139 adults who previously responded to botulinum A toxin	TWSTRS-severity score 4 weeks 3.7 with botulinum A toxin 3.7 with botulinum B toxin	Reported as not significant RR not reported P = 0.90		Not significant
RCT	139 adults who previously responded to botulinum A toxin	TWSTRS-disability score 0–4 weeks 2.4 with botulinum A toxin 2.5 with botulinum B toxin	Reported as not significant RR not reported P = 0.71		Not significant
RCT	139 adults who previously responded to botulinum A toxin	TWSTRS-pain score 0–4 weeks 3.2 with botulinum A toxin 4.0 with botulinum B toxin	Reported as not significant RR not reported P = 0.24		Not significant
RCT	139 adults who previously responded to botulinum A toxin	Median duration of effect of treatment 13 weeks with botulinum A toxin 11.7 weeks with botulinum B toxin	Reported as not significant RR not reported P = 0.095		Not significant
RCT	20 adults who responded to botulinum A toxin within the previous year	TWSTRS-severity score 2 weeks 14 with botulinum A toxin 15 with botulinum B toxin	Reported as not significant RR not reported		Not significant
RCT	20 adults who responded to botulinum A toxin within the previous year	TWSTRS-pain score 2 weeks 6 with botulinum A toxin 4 with botulinum B toxin	Reported as not significant RR not reported		Not significant
RCT	20 adults who responded to botulinum A toxin within the previous year	TWSTRS-disability score 2 weeks 10 with botulinum A toxin 12 with botulinum B toxin	Reported as not significant RR not reported		Not significant
RCT	111 adults with cervical dystonia, not previously treated with botulinum toxin (toxin naive), 93/111 (84%) included in analysis	Improvement in TWSTRS-total score 4 weeks 8.8 with botulinum A toxin 11.0 with botulinum B toxin	Difference –2.2 95% CI –5.4 to +1.1 Not intention-to-treat analysis		Not significant
RCT	111 adults with cervical dystonia, not previously treated with botulinum toxin (toxin naive), 93/111 (84%) included in analysis	Improvement in TWSTRS-severity score 4 weeks 4.7 with botulinum A toxin 5.4 with botulinum B toxin	Difference –0.7 95% CI –2.2 to +0.8 Not intention-to-treat analysis		Not significant
RCT	111 adults with cervical dystonia, not previously treated with botulinum toxin (toxin naive), 93/111 (84%) included in analysis	Improvement in TWSTRS-disability score 4 weeks 2.5 with botulinum A toxin 2.9 with botulinum B toxin	Difference –0.5 95% CI –2.0 to +1.0 Not intention-to-treat analysis		Not significant
RCT	111 adults with cervical dystonia, not previously treated with botulinum toxin (toxin naive), 93/111 (84%) included in analysis	Improvement in TWSTRS-pain score 4 weeks 1.7 with botulinum A toxin 2.7 with botulinum B toxin	Difference –1.0 95% CI –2.2 to +0.2 Not intention-to-treat analysis		Not significant

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Quality of life

No data from the following reference on this outcome.

Adverse effects

Ref (type)	Population	Outcome, Interventions	Results and statistical analysis	Effect size	Favours
Adverse effects
RCT	139 adults who previously responded to botulinum A toxin	Dysphagia 4 weeks 19% with botulinum A toxin 48% with botulinum B toxin Absolute numbers not reported	P = 0.0005	Effect size not calculated	botulinum A toxin
RCT	139 adults who previously responded to botulinum A toxin	Dry mouth 4 weeks 41% with botulinum A toxin 80% with botulinum B toxin Absolute numbers not reported	P <0.0001	Effect size not calculated	botulinum A toxin
RCT	20 adults who responded to botulinum A toxin within the previous year	Dysphagia 18% with botulinum A toxin 55% with botulinum B toxin Absolute numbers not reported	P = 0.081		Not significant
RCT	20 adults who responded to botulinum A toxin within the previous year	Constipation 0/11 (0%) with botulinum A toxin 3/9 (33%) with botulinum B toxin	P = 0.037	Effect size not calculated	placebo
RCT	111 adults, not previously treated with botulinum toxin (toxin naive)	Dysphagia 8/55 (15%) with botulinum A toxin 9/56 (16%) with botulinum B toxin	P = 1		Not significant
RCT	111 adults with cervical dystonia, not previously treated with botulinum toxin (toxin naive)	Dry mouth 4/55 (7%) with botulinum A toxin 22/56 (39%) with botulinum B toxin	P = 0.0001	Effect size not calculated	botulinum A toxin
RCT	111 adults with cervical dystonia, not previously treated with botulinum toxin (toxin naive)	Injection site pain 3/55 (5%) with botulinum A toxin 0/56 (0%) with botulinum B toxin	P = 0.12		Not significant

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Low-dose (100 U Botox/250 U Dysport) versus high-dose (>200 U Botox/960 U Dysport) botulinum A toxin in cervical dystonia in adults:

We found one systematic review (search date 2003). It found no RCTs directly comparing high- and low-dose botulinum A toxin. We found one additional RCT.

Neurological disability

Low-dose compared with high-dose botulinum A toxin We don't know whether high-dose botulinum A toxin is more effective than low-dose botulinum A toxin at improving Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) disease-activity rating score and increasing patient- and physician-rated improvements in symptoms (very low-quality evidence).

Ref (type)	Population	Outcome, Interventions	Results and statistical analysis	Effect size	Favours
Neurological disability
RCT	31 adults	Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS)-severity score 4 weeks 0.27 with botulinum A toxin (125 U/mL) 1.07 with botulinum A toxin (500 U/mL)	P = 0.19		Not significant
RCT	31 adults	TWSTRS-disability score 4 weeks 2.6 with botulinum A toxin (125 U/mL) 1.2 with botulinum A toxin (500 U/mL)	P = 0.26		Not significant
RCT	31 adults	TWSTRS-total score 4 weeks 5.6 with botulinum A toxin (125 U/mL) 4.4 with botulinum A toxin (500 U/mL)	P = 0.63		Not significant

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Quality of life

No data from the following reference on this outcome.

Adverse effects

No data from the following reference on this outcome.

Low-dose (2500–5000 U) versus high-dose (10,000 U) botulinum B toxin in cervical dystonia in adults:

We found one systematic review (search date 2003).

Neurological disability

Low-dose compared with high-dose botulinum B toxin Low-dose botulinum B toxin may be less effective than high-dose botulinum B toxin at improving pain, as assessed by Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS)-pain subscale, at 4 weeks; however, we don't know how effective low-dose or high-dose botulinum B toxin are, compared with each other, at improving TWSTRS-total scores at 4 to 16 weeks (low-quality evidence).

Ref (type)	Population	Outcome, Interventions	Results and statistical analysis	Effect size	Favours
Neurological disability
Systematic review	92 adults with cervical dystonia for at least 1 year and previously treated with botulinum A toxin, Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS)-total score >20 and TWSTRS-severity >10 Data from 1 RCT	Improvement in TWSTRS-pain subscale 4 weeks 39/62 (63%) with botulinum B toxin (2500–5000 U) 25/30 (83%) with botulinum B toxin (10,000 U)	OR 0.39 95% CI 0.15 to 0.99 P = 0.05	Effect size not calculated	high-dose botulinum B toxin
Systematic review	92 adults with cervical dystonia for at least 1 year and previously treated with botulinum A toxin, TWSTRS-total score >20 and TWSTRS-severity >10 Data from 1 RCT	Improvement of at least 20% in TWSTRS-total score 4 weeks 37/62 (60%) with botulinum B toxin (2500–5000 U) 23/30 (77%) with botulinum B toxin (10,000 U)	OR 0.50 95% CI 0.20 to 1.25		Not significant
Systematic review	92 adults with cervical dystonia for at least 1 year and previously treated with botulinum A toxin, TWSTRS-total score >20 and TWSTRS-severity >10 Data from 1 RCT	Improvement of at least 20% in TWSTRS-total score 8 weeks 24/62 (39%) with botulinum B toxin (2500–5000 U) 16/30 (53%) with botulinum B toxin (10,000 U)	OR 0.56 95% CI 0.23 to 1.33		Not significant
Systematic review	92 adults with cervical dystonia for at least 1 year and previously treated with botulinum A toxin, TWSTRS-total score >20 and TWSTRS-severity >10 Data from 1 RCT	Improvement of at least 20% in TWSTRS-total score 12 weeks 14/62 (23%) with botulinum B toxin (2500–5000 U) 9/30 (30%) with botulinum B toxin (10,000 U)	OR 0.68 95% CI 0.25 to 1.84		Not significant
Systematic review	92 adults with cervical dystonia for at least 1 year and previously treated with botulinum A toxin, TWSTRS-total score >20 and TWSTRS-severity >10 Data from 1 RCT	Improvement of at least 20% in TWSTRS-total score 16 weeks 7/62 (11%) with botulinum B toxin (2500–5000 U) 5/30 (17%) with botulinum B toxin (10,000 U)	OR 0.63 95% CI 0.17 to 2.27		Not significant

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Quality of life

No data from the following reference on this outcome.

Adverse effects

Ref (type)	Population	Outcome, Interventions	Results and statistical analysis	Effect size	Favours
Adverse effects
Systematic review	308 adults with cervical dystonia for at least 1 year and previously treated with botulinum A toxin, Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS)-total score >20 and TWSTRS-severity >10 2 RCTs in this analysis	Dry mouth 9/98 (9%) with botulinum B toxin (2500–5000 U) 27/69 (39%) with botulinum B toxin (10,000 U)	OR 0.19 95% CI 0.09 to 0.40 P = 0.00002	Small effect size	low-dose botulinum B toxin

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Botulinum A toxin versus trihexyphenidyl in cervical dystonia in adults:

We found one systematic review (search date 2003).

Neurological disability

Botulinum A toxin compared with trihexyphenidyl Botulinum A toxin may be more effective at 12 weeks than trihexyphenidyl at improving Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS)-disability scores, Tsui Scale, and General Health Perception subscale (very low-quality evidence).

Ref (type)	Population	Outcome, Interventions	Results and statistical analysis	Effect size	Favours
Neurological disability
Systematic review	66 adults with cervical dystonia; see further information on studies Data from 1 RCT	Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS)-disability score 12 weeks with botulinum A toxin plus placebo tablets with trihexyphenidyl plus placebo injection Absolute results not reported	WMD 2.50 95% CI 0.68 to 4.32 P = 0.0097	Effect size not calculated	botulinum A toxin
Systematic review	66 adults with cervical dystonia; see further information on studies Data from 1 RCT	Tsui Scale score 12 weeks with botulinum A toxin plus placebo tablets with trihexyphenidyl plus placebo injection Absolute results not reported	WMD 4.60 95% CI 2.14 to 7.06 P = 0.0009	Effect size not calculated	botulinum A toxin
Systematic review	66 adults with cervical dystonia; see further information on studies Data from 1 RCT	Mean difference in General Health Perception subscale score with botulinum A toxin plus placebo tablets with trihexyphenidyl plus placebo injection Absolute results not reported	Mean score difference 6 95% CI 4 to 12 P = 0.0023	Effect size not calculated	botulinum A toxin
Systematic review	66 adults with cervical dystonia; see further information on studies Data from 1 RCT	Proportion of people with at least 3-point improvement on Tsui Scale with botulinum A toxin plus placebo tablets with trihexyphenidyl plus placebo injection Absolute results not reported	OR 3.92 95% CI 1.48 to 10.40	Moderate effect size	botulinum A toxin
Systematic review	66 adults with cervical dystonia; see further information on studies Data from 1 RCT	Proportion of people with at least 3-point improvement on TWSTRS scale with botulinum A toxin plus placebo tablets with trihexyphenidyl plus placebo injection Absolute results not reported	OR 3.14 95% CI 1.10 to 8.97 P = 0.059	Moderate effect size	botulinum A toxin
Systematic review	66 adults with cervical dystonia; see further information on studies Data from 1 RCT	Median improvement in TWSTRS-pain score 3 with botulinum A toxin plus placebo tablets 1 with trihexyphenidyl plus placebo injection	Reported this difference did not reach statistical significance, no further details reported		Not significant

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Quality of life

No data from the following reference on this outcome.

Adverse effects

Ref (type)	Population	Outcome, Interventions	Results and statistical analysis	Effect size	Favours
Adverse effects
Systematic review	66 adults Data from 1 RCT	Number of adverse effects in each group 31 in 32 people with botulinum A toxin plus placebo tablets 76 in 32 people with trihexyphenidyl plus placebo	P = 0.0001	Effect size not calculated	botulinum A toxin
Systematic review	66 adults Data from 1 RCT	Dry mouth with botulinum A toxin plus placebo with trihexyphenidyl plus placebo Absolute results not reported	OR 7.22 95% CI 2.72 to 19.12	Large effect size	botulinum A toxin
Systematic review	66 adults Data from 1 RCT	Forgetfulness with botulinum A toxin plus placebo with trihexyphenidyl plus placebo Absolute results not reported	OR 3.51 95% CI 1.25 to 9.89	Moderate effect size	botulinum A toxin
Systematic review	66 adults Data from 1 RCT	Fatigue with botulinum A toxin plus placebo with trihexyphenidyl plus placebo Absolute results not reported	OR 9.12 95% CI 1.92 to 43.40 The extremely wide confidence intervals make the clinical relevance of this result questionable	Moderate effect size	botulinum A toxin

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Botulinum B toxin in botulinum A toxin-resistant adults versus respondent adults:

We found one systematic review (search date 2003).

Neurological disability

Botulinum B toxin in botulinum A-resistant adults compared with botulinum B toxin in botulinum A responders We don't know whether botulinum B toxin in botulinum A-resistant adults is more effective at 4 weeks at improving Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS)-total scores (low-quality evidence).

Ref (type)	Population	Outcome, Interventions	Results and statistical analysis	Effect size	Favours
Neurological disability
Systematic review	92 adults Data from 1 RCT	Improvement of at least 20% in Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS)-total score 4 weeks with botulinum B toxin in people resistant to botulinum A toxin with botulinum B toxin in people responsive to botulinum A toxin Absolute results not reported	P value not reported Reported as not significant		Not significant

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Quality of life

No data from the following reference on this outcome.

Adverse effects

No data from the following reference on this outcome.

Botulinum A toxin versus placebo in people with blepharospasm (eyelid closure):

We found one systematic review (search date 2003), which found no RCTs. We found no additional or subsequent RCTs satisfying Clinical Evidence inclusion criteria.

Botulinum A toxin versus placebo in people with spasmodic dysphonia (laryngeal dystonia):

We found one systematic review (search date 2005). The review identified one RCT that did not meet Clinical Evidence inclusion criteria (RCT included only 13 people), and so the results are not discussed further.

Botulinum A toxin versus placebo in people with writer's cramp:

We found one RCT, which compared botulinum A versus placebo.

Neurological disability

Botulinum A toxin compared with placebo in people with writer's cramp Botulinum A toxin may be more effective at 8 weeks at improving symptom severity scores, writer's cramp rating scales, handwriting, and writing speed; however, we don't know whether it improves overall functional status (low-quality evidence).

Ref (type)	Population	Outcome, Interventions	Results and statistical analysis	Effect size	Favours
Neurological disability
RCT	40 treatment-naive people with symptoms of idiopathic writer's cramp for at least 1 year	Mean improvement in symptom severity scale –3.60 with botulinum A –1.16 with placebo Assessed using symptom severity scale, reduction in score indicates improvement	P = 0.02	Effect size not calculated	botulinum A toxin
RCT	40 treatment-naive people with symptoms of idiopathic writer's cramp for at least 1 year	Mean improvement in writer's cramp scale –2.30 with botulinum A –0.79 with placebo	P <0.01	Effect size not calculated	botulinum A toxin
RCT	40 treatment-naive people with symptoms of idiopathic writer's cramp for at least 1 year	Mean improvement in handwriting 1.85 with botulinum A 0.53 with placebo Assessed using a visual analogue scale from 0 cm to 10 cm	P = 0.01	Effect size not calculated	botulinum A toxin
RCT	40 treatment-naive people with symptoms of idiopathic writer's cramp for at least 1 year	Mean change in writing speed 1.41 with botulinum A 0.27 with placebo Mean change in writing speed = number of lines written in 2 minutes	P = 0.04	Effect size not calculated	botulinum A toxin
RCT	40 treatment-naive people with symptoms of idiopathic writer's cramp for at least 1 year	Mean improvement in functional status +0.65 with botulinum A –1.42 with placebo	P = 0.10		Not significant

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Quality of life

No data from the following reference on this outcome.

Adverse effects

Ref (type)	Population	Outcome, Interventions	Results and statistical analysis	Effect size	Favours
Adverse effects
RCT	40 treatment-naive people with symptoms of idiopathic writer's cramp for at least 1 year	Weakness in the hand 18/20 (90%) with botulinum A 2/19 (11%) with placebo	P value not reported
RCT	40 treatment-naive people with symptoms of idiopathic writer's cramp for at least 1 year	Pain at injection site 1/20 (5%) with botulinum A 3/19 (16%) with placebo	P value not reported

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Further information on studies

The quality of the 13 trials was assessed by Jadad's Scale as good. However, all trials were of short duration, and examined the effects of only one injection cycle.

This was a two-phase trial. In phase 1, all participants were given onabotulinumtoxinA treatment and in the second phase, only those participants that 'responded' to this initial treatment were eligible for randomisation, to either onabotulinumtoxinA or placebo. 'Response' was considered adequate for inclusion in phase 2 if the Cervical Dystonia Severity Scale (CDSS) had improved so that it reached a score of 4 or higher. The results from phase 2 are reported because they meet the inclusion criteria for this Clinical Evidence review.

Of the three RCTs included in the systematic review, one RCT included both people responsive and resistant to botulinum A toxin, one RCT included only people resistant to botulinum A toxin, and one RCT included only people responsive to botulinum A toxin. The quality of the three RCTs was assessed as good by Jadad's Scale.

The systematic review commented on some limitations of the RCT. It found there were significantly fewer people with history of progressive disease in the botulinum A toxin group than in the trihexyphenidyl group (9/33 [27%] with botulinum A toxin v 21/33 [64%] with trihexyphenidyl; P = 0.003); the short duration of the RCT may not favour trihexyphenidyl; and people were injected with botulinum A toxin at baseline and 8 weeks later, which is a shorter interval than the 12- to 16-week interval between injections that would generally be used in clinical practice.

To optimise treatment effect, people were treated in two sessions: initially after baseline assessment and then 1 month later. People expressing satisfaction with their improvement after one treatment did not receive a second injection. However, if people showed no response to treatment after one session, the dose was doubled for the second session. The primary outcome assessed was the person's response to a question on whether they wished to continue with treatment (assessed at 12 weeks). This is not one of our outcomes of interest, and so the results are not discussed further. Clinical rating scales were measured as secondary outcomes.

Comment

Clinical guide:

Botulinum toxin injections are the mainstay of management of cervical dystonia, and have replaced most treatments used in previous decades. They are sometimes used for other focal dystonias, but with caution, because some focal dystonias (e.g., 'functional dystonias') may have a primarily psychological origin. The evidence supporting botulinum toxin in focal dystonias is strong, partly because there is a strong commercial imperative to show effectiveness, but also because treatment can be localised, and because botulinum toxin is effective at reducing neuromuscular transmission. Its main limitation is that the effect wears off after 12 to 16 weeks, but repeated injections are usually equally effective. Invasive local surgical procedures have largely been displaced by local botulinum toxin injections.

Clinical data from several studies indicate that up to 10% of people treated with botulinum toxin are at risk of developing neutralising antibodies. Presence of antibodies may lead to decreased/no response to further treatment and may necessitate stopping treatment. The risk of formation of antibodies seems to be higher when botulinum toxin is given at frequent intervals at high doses; it has been suggested that the potential for developing antibodies may be ameliorated by using the lowest effective dosing regimen and increasing the time interval between doses.

Different formulations of botulinum A toxin versus each other in people with blepharospasm (eyelid closure):

We found one RCT that compared two different formulations of botulinum A toxin with each other — one formulation (Xeomin), a freeze-dried botulinum A toxin, free from complexing proteins, versus another established commercially available formulation of botulinum A toxin (Botox). The RCT found no significant difference between formulations in improvement in blepharospasm symptoms. It found similar rates of adverse effects (ptosis, abnormal vision, back pain, or xerophthalmia) with both treatments.

We found one subsequent RCT involving 65 patients, which compared two different formulations of botulinum A toxin versus each other — one formulation (Xeomin), a freeze-dried botulinum A toxin, free from complexing proteins, versus another established commercially available formulation of botulinum A toxin (Botox). The RCT found no significant difference between formulations in scores on the Blepharospasm Disability Index, Jankovic Rating Scale, or Patient Global Assessment Scale at 4 or 8 weeks.

Dosage:

The three commercially available formulations of botulinum A toxin — Dysport, Botox, and Xeomin — differ in potency, and so are not interchangeable. One crossover RCT suggested a conversion of 3 U of Dysport to 1 U of Botox, although there were differences in both beneficial outcomes and in adverse effects. The adverse effects unequivocally associated with botulinum toxin injection are those expected from its local action, and they are more common with higher doses — as would be expected from local spread from the injected muscle. The differences in beneficial and adverse effects reflect relative differences in dosage, not intrinsic differences between preparations.

Substantive changes

Botulinum toxins for focal dystonia Two RCTs added. Categorisation of botulinum toxins for focal dystonia unchanged (beneficial).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Amantadine for focal dystonia

Summary

We found no direct information from RCTs about amantadine in the treatment of people with focal dystonia.

Benefits and harms

Amantadine:

We found no systematic review or RCTs of amantadine in people with focal dystonia.

Comment

None.

Substantive changes

Amantadine for focal dystonia Since the last update of this review, we now report drugs individually, rather than as groups. Categorisation of amantadine for focal dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Baclofen for focal dystonia

Summary

We found no direct information from RCTs about baclofen in the treatment of people with focal dystonia.

Benefits and harms

Baclofen:

We found no systematic review or RCTs of baclofen in people with focal dystonia.

Comment

None.

Substantive changes

Baclofen for focal dystonia Since the last update of this review, we now report drugs individually, rather than as groups. Categorisation of baclofen for focal dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Benzatropine for focal dystonia

Summary

We don't know whether benzatropine is effective for focal dystonia.

Benefits and harms

Benzatropine:

We found no systematic review or RCTs of benzatropine in people with focal dystonia.

Comment

None.

Substantive changes

Benzatropine for focal dystonia Since the last update of this review, we now report drugs individually, rather than as groups. Categorisation of benzatropine for focal dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Bromocriptine for focal dystonia

Summary

We found no direct information from RCTs about bromocriptine in the treatment of people with focal dystonia.

Benefits and harms

Bromocriptine:

We found no systematic review or RCTs of bromocriptine in people with focal dystonia.

Comment

None.

Substantive changes

Bromocriptine for focal dystonia Since the last update of this review, we now report drugs individually, rather than as groups. Categorisation of bromocriptine for focal dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Carbamazepine for focal dystonia

Summary

We found no direct information from RCTs about carbamazepine in the treatment of people with focal dystonia.

Benefits and harms

Carbamazepine:

We found no systematic review or RCTs of carbamazepine in people with focal dystonia.

Comment

None.

Substantive changes

Carbamazepine for focal dystonia Since the last update of this review, we now report drugs individually, rather than as groups. Categorisation of carbamazepine for focal dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Carbidopa/levodopa for focal dystonia

Summary

We found no direct information from RCTs about carbidopa/levodopa in the treatment of people with focal dystonia.

Benefits and harms

Carbidopa/levodopa:

We found no systematic review or RCTs of carbidopa/levodopa in people with focal dystonia.

Comment

None.

Substantive changes

Carbidopa/levodopa for focal dystonia Since the last update of this review, we now report drugs individually, rather than as groups. Categorisation of carbidopa/levodopa for focal dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Clonazepam for focal dystonia

Summary

We found no direct information from RCTs about clonazepam in the treatment of people with focal dystonia.

Benefits and harms

Clonazepam:

We found no systematic review or RCTs of clonazepam in people with focal dystonia.

Comment

None.

Substantive changes

Clonazepam for focal dystonia Since the last update of this review, we now report drugs individually, rather than as groups. Categorisation of clonazepam for focal dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Clozapine for focal dystonia

Summary

We found no direct information from RCTs about clozapine in the treatment of people with focal dystonia.

Benefits and harms

Clozapine:

We found no systematic review or RCTs of clozapine in people with focal dystonia.

Comment

None.

Substantive changes

Clozapine for focal dystonia Since the last update of this review, we now report drugs individually, rather than as groups. Categorisation of clozapine for focal dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Diazepam for focal dystonia

Summary

We found no direct information from RCTs about diazepam in the treatment of people with focal dystonia.

Benefits and harms

Diazepam:

We found no systematic review or RCTs of diazepam in people with focal dystonia.

Comment

None.

Substantive changes

Diazepam for focal dystonia Since the last update of this review, we now report drugs individually, rather than as groups. Categorisation of diazepam for focal dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Gabapentin for focal dystonia

Summary

We don't know whether gabapentin is effective for focal dystonia.

Benefits and harms

Gabapentin:

We found no systematic review or RCTs of gabapentin in people with focal dystonia.

Comment

None.

Substantive changes

Gabapentin for focal dystonia Since the last update of this review, we now report drugs individually, rather than as groups. Categorisation of gabapentin for focal dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Haloperidol for focal dystonia

Summary

We found no direct information from RCTs about haloperidol in the treatment of people with focal dystonia.

Benefits and harms

Haloperidol:

We found no systematic review or RCTs of haloperidol in people with focal dystonia.

Comment

None.

Substantive changes

Haloperidol for focal dystonia Since the last update of this review, we now report drugs individually, rather than as groups. Categorisation of haloperidol for focal dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Lorazepam for focal dystonia

Summary

We found no direct information from RCTs about lorazepam in the treatment of people with focal dystonia.

Benefits and harms

Lorazepam:

We found no systematic review or RCTs of lorazepam in people with focal dystonia.

Comment

None.

Substantive changes

Lorazepam for focal dystonia Since the last update of this review, we now report drugs individually, rather than as groups. Categorisation of lorazepam for focal dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Ondansetron for focal dystonia

Summary

We found no direct information from RCTs about ondansetron in the treatment of people with focal dystonia.

Benefits and harms

Ondansetron:

We found no systematic review or RCTs of ondansetron in people with focal dystonia.

Comment

None.

Substantive changes

Ondansetron for focal dystonia Since the last update of this review, we now report drugs individually, rather than as groups. Categorisation of ondansetron for focal dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Pregabalin for focal dystonia

Summary

We don't know whether pregabalin is effective for focal dystonia.

Benefits and harms

Pregabalin:

We found no systematic review or RCTs of pregabalin in people with focal dystonia.

Comment

None.

Substantive changes

Pregabalin for focal dystonia Since the last update of this review, we now report drugs individually, rather than as groups. Categorisation of pregabalin for focal dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Procyclidine for focal dystonia

Summary

We don't know whether procyclidine is effective for focal dystonia.

Benefits and harms

Procyclidine:

We found no systematic review or RCTs of procyclidine in people with focal dystonia.

Comment

None.

Substantive changes

Procyclidine for focal dystonia Since the last update of this review, we now report drugs individually, rather than as groups. Categorisation of procyclidine for focal dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Tizanidine for focal dystonia

Summary

We found no direct information from RCTs about tizanidine in the treatment of people with focal dystonia.

Benefits and harms

Tizanidine:

We found no systematic review or RCTs of tizanidine in people with focal dystonia.

Comment

None.

Substantive changes

Tizanidine for focal dystonia Since the last update of this review, we now report drugs individually, rather than as groups. Categorisation of tizanidine for focal dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Trazodone hydrochloride for focal dystonia

Summary

We found no direct information from RCTs about trazodone hydrochloride in the treatment of people with focal dystonia.

Benefits and harms

Trazodone hydrochloride:

We found no systematic review or RCTs of trazodone hydrochloride in people with focal dystonia.

Comment

None.

Substantive changes

Trazodone hydrochloride for focal dystonia Since the last update of this review, we now report drugs individually, rather than as groups. Categorisation of trazodone hydrochloride for focal dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Trihexyphenidyl for focal dystonia

Summary

We don’t know whether trihexyphenidyl is effective for focal dystonia.

Benefits and harms

Trihexyphenidyl versus botulinum A toxin in cervical dystonia:

See botulinum A toxin versus trihexyphenidyl in cervical dystonia.

Comment

Clinical guide:

Trihexyphenidyl is rarely used in cervical dystonia, given the effectiveness of local botulinum toxin injection, and the perception and risk of more general adverse effects from using an oral drug. There are reports of trihexyphenidyl being administered off-licence for other focal dystonias at doses above the recommended maximum, but this is best considered within specialised services with suitable experience.

Substantive changes

Trihexyphenidyl for focal dystonia Since the last update of this review, we now report drugs individually, rather than as groups. Categorisation of trihexyphenidyl for focal dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Deep brain stimulation of thalamus and globus pallidus for focal dystonia

Summary

We found no direct information from RCTs about deep brain stimulation of the thalamus in people with only focal dystonia. Evidence in a mixed population of people with focal or generalised dystonia suggests that it may improve function at 3 months.

Benefits and harms

Deep brain stimulation versus sham treatment:

We found no systematic review or RCT of deep brain stimulation of the thalamus, solely or predominantly in people with focal dystonia. We found one RCT in a mixed population of people with local and generalised dystonia; however, most people (24/40 [60%]) had generalised dystonia. For details see option on Deep brain stimulation in people with generalised dystonia.

Comment

Clinical guide:

RCTs with longer follow-ups are required (see Comment in deep brain stimulation of thalamus and globus pallidus in people with generalised dystonia).

Substantive changes

No new evidence

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Myectomy for focal dystonia

Summary

We found no direct information from RCTs about myectomy in the treatment of people with focal dystonia.

Benefits and harms

Myectomy:

We found no systematic review or RCTs of myectomy in people with focal dystonia.

Comment

Clinical guide:

Destructive procedures that mechanically prevent the dystonic posture (e.g., myectomy, thalamotomy, pallidotomy, and selective peripheral denervation) were once used (without supporting evidence), but their apparent ineffectiveness, coupled with the effectiveness of botulinum toxin, has led to their demise.

Substantive changes

No new evidence

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Selective peripheral denervation for focal dystonia

Summary

We found no direct information from RCTs about selective peripheral denervation in the treatment of people with focal dystonia.

Benefits and harms

Selective peripheral denervation:

We found no systematic review or RCTs of selective peripheral denervation in people with focal dystonia.

Comment

See Comment on myectomy under surgical treatments for focal dystonia.

Substantive changes

No new evidence

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Physiotherapy for focal dystonia

Summary

Most people will see a physiotherapist after diagnosis, but there is no consistent approach to treatment.

Benefits and harms

Physiotherapy in children with developmental or early congenital cervical dystonia:

We found no systematic review or RCTs (see Comment).

Physiotherapy versus drug treatment:

We found no systematic review or RCTs.

Physiotherapy plus biofeedback plus drug treatment versus drug treatment alone:

We found one crossover RCT, which examined the effect of physical therapy (physiotherapy plus biofeedback) plus botulinum A toxin compared with botulinum A toxin alone.

Neurological disability

Physiotherapy plus biofeedback plus drug treatment compared with drug treatment alone Physical therapy plus botulinum A toxin may be more effective at improving pain and activities of daily living scores, but we don't know whether it is more effective at improving cervical dystonia, as assessed by an improvement in Tsui Scale and Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) (very low-quality evidence).

Ref (type)	Population	Outcome, Interventions	Results and statistical analysis	Effect size	Favours
Neurological disability
RCT Crossover design	40 people with idiopathic cervical dystonia for at least 3 years, and who previously responded to at least 2 botulinum A toxin injections	Improvement in Tsui Scale score end of observation period –8.1 with botulinum A toxin alone –7.2 with botulinum A toxin plus physical therapy (physiotherapy plus biofeedback)	Reported as no significant difference		Not significant
RCT Crossover design	40 people with idiopathic cervical dystonia for at least 3 years, and who previously responded to at least 2 botulinum A toxin injections	Improvement in Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) score end of observation period –9.1 with botulinum A toxin alone –10.2 with botulinum A toxin plus physical therapy (physiotherapy plus biofeedback)	Reported as no significant difference		Not significant
RCT Crossover design	40 people with idiopathic cervical dystonia for at least 3 years, and who previously responded to at least 2 botulinum A toxin injections	Improvement in activities of daily living end of observation period –5.3 with botulinum A toxin alone –9.8 with botulinum A toxin plus physical therapy (physiotherapy plus biofeedback)	P <0.05	Effect size not calculated	botulinum toxin A plus physical therapy
RCT Crossover design	40 people with idiopathic cervical dystonia for at least 3 years, and who previously responded to at least 2 botulinum A toxin injections	Total pain score end of observation period –7.1 with botulinum A toxin alone –13.0 with botulinum A toxin plus physical therapy (physiotherapy plus biofeedback)	P <0.001	Effect size not calculated	botulinum toxin A plus physical therapy

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Quality of life

No data from the following reference on this outcome.

Adverse effects

Ref (type)	Population	Outcome, Interventions	Results and statistical analysis	Effect size	Favours
Adverse effects
RCT Crossover design	40 people with idiopathic cervical dystonia for at least 3 years, and who previously responded to at least 2 botulinum A toxin injections	Adverse effects end of observation period with botulinum A toxin alone with botulinum A toxin plus physical therapy (physiotherapy plus biofeedback) Absolute results not reported

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Physiotherapy versus surgery:

We found no systematic review or RCTs.

Physiotherapy plus relaxation versus no physiotherapy plus relaxation:

We found one RCT, which compared an active exercise programme plus relaxation versus relaxation alone. The active exercise programme comprised individually designed programmes that involved neck exercises to activate and strengthen the neck muscles and whole body relaxation. The RCT initially excluded people having botulinum toxin as part of their management. After nine people were enrolled, this criteria was removed, and further recruitment included participants having botulinum toxin. Hence, some participants also had botulinum toxin and some did not.

Neurological disability

Physiotherapy plus relaxation versus no physiotherapy plus relaxation We don't know whether an active exercise programme (individually designed programmes that involved neck exercises to activate and strengthen the neck muscles and whole body relaxation) plus relaxation is more effective than relaxation alone at reducing neurological disability in adults with cervical dystonia (low-quality evidence).

Ref (type)	Population	Outcome, Interventions	Results and statistical analysis	Effect size	Favours
Neurological disability
RCT	20 adults with cervical dystonia	Mean change from baseline in Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) at week 12 –3.9 with exercise programme plus relaxation –1.5 with relaxation only	Mean difference –1.9 95% CI –9.0 to +5.2 Intention-to-treat analysis Difference between groups was adjusted for baseline (week 0) score based on ANCOVA		Not significant
RCT	20 adults with cervical dystonia	Change from baseline in TWSTRS at week 16 –3.0 with exercise programme plus relaxation –0.6 with relaxation only	Mean difference –2.5 95% CI –8.9 to +3.9 Intention-to-treat analysis Difference between groups was adjusted for baseline (week 0) score based on ANCOVA		Not significant

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Quality of life

Physiotherapy plus relaxation versus no physiotherapy plus relaxation We don't know whether an active exercise programme plus relaxation is more effective than relaxation alone in improving quality of life in adults with cervical dystonia (low-quality evidence).

Ref (type)	Population	Outcome, Interventions	Results and statistical analysis	Effect size	Favours
Quality of life
RCT	20 adults with cervical dystonia	Mean change from baseline in Craniocervical Dystonia Questionnaire 24 at week 12 –6.3 with exercise programme plus relaxation –7.0 with relaxation only	Mean difference –0.2 95% CI –11.6 to +11.1 Intention-to-treat analysis Difference between groups was adjusted for baseline (week 0) score based on ANCOVA		Not significant
RCT	20 adults with cervical dystonia	Mean change from baseline in Craniocervical Dystonia Questionnaire 24 at week 16 –2.6 with exercise programme plus relaxation –7.0 with relaxation only	Mean difference +4.4 95% CI –7.3 to +16.1 Intention-to-treat analysis Difference between groups was adjusted for baseline (week 0) score based on ANCOVA		Not significant

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Adverse effects

No data from the following reference on this outcome.

Further information on studies

The RCT also found that when physical therapy plus botulinum A toxin was given as the first treatment, this resulted in longer duration of clinical benefit before people needed to cross over to the alternative treatment, and that a lower dose of botulinum toxin was required at the next injection, compared with when botulinum A toxin alone was used as the first treatment.

Comment

Physiotherapy in children with developmental cervical dystonia:

We found one case series (23 children, mean age 3.8 months [range 3 weeks–10.5 months] diagnosed with developmental cervical dystonia), which examined the effect of passive cervical stretching by positioning and active strengthening of identified weak muscles. The average number of treatments was 3.8 (range 1.0–10.0) provided over mean treatment duration of 2.9 months. At follow-up (mean age at follow-up: 18 months, range 5–49 months), the case series found that a similar number of parents reported 'good' or 'excellent' outcomes in their children (excellent = symmetrical head features, symmetrical facial features, passive cervical rotation of at least 75° bilaterally, passive cervical lateral flexion of at least 40° bilaterally, complete head righting, and lack of resting head tilt; good = 4–5 of these outcomes; fair = 3 of these outcomes; poor = 1–2 of the outcomes: 11/23 [48%] excellent v 11/23 [48%] good v 1/23 [4%] fair v 0/23 [0%] poor).

Physiotherapy in children with early congenital cervical dystonia

We found one case series (126 children with mild to severe congenital cervical dystonia seen over 30 years), which examined the effect of passive stretching exercises (PSE). Subjective physician measurement of PSE showed that PSE for early congenital cervical dystonia (<3 months) produced excellent results in 52/81 (64%) of cases at an average follow-up of 9 months (excellent = full rotation and no asymmetry; good = full rotation and mild asymmetry or mild limitation of rotation and no asymmetry; fair = mild limitation of rotation and mild asymmetry; poor = no improvement: 65% excellent v 27% good v 8% fair v 0% poor).

Case series should be carefully interpreted, because: (1) the intervention will vary over time; (2) a number of outcomes are subjective; and (3) it is hard to determine whether the specified physiotherapy actually took place in the home setting. Also, the outcome was probably assessed by someone who was not blinded to the treatment — even by the treating therapist.

Clinical guide:

Most people will see a physiotherapist at some point after diagnosis, but there is no consistent approach to treatment, and practice can vary from place to place with no consensus on best practice. At present, physiotherapy cannot be recommended positively and clinicians should consider whether raising unrealistic expectations may be more harmful than any potential and unproven benefit.

Substantive changes

Physiotherapy for focal dystonia One RCT added. Categorisation of physiotherapy for focal dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Acupuncture for focal dystonia

Summary

We found no direct information from systematic reviews or RCTs about the effects of acupuncture in the treatment of people with focal dystonia.

Benefits and harms

Acupuncture:

We found no systematic review or RCTs of acupuncture in people with focal dystonia.

Comment

None.

Substantive changes

No new evidence

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Biofeedback for focal dystonia

Summary

We found insufficient information from systematic reviews or RCTs to judge the effects of biofeedback in the treatment of people with focal dystonia.

Benefits and harms

Biofeedback for cervical dystonia:

We found no systematic review or RCTs (see Comment).

Physiotherapy plus biofeedback plus drug treatment versus drug treatment alone:

See option on physiotherapy.

Comment

Biofeedback for cervical dystonia:

We found one case series (80 adults, 69 with spasmodic cervical dystonia and 11 with focal dystonia) examining auditory and visual EMG biofeedback. It found that clinically significant improvement of dystonia was achieved by 45/80 (56%) people at 8 to 12 weeks with biofeedback. The improvements ranged from a sustained response (as measured by EMG activity and degree of functional deficiency) after feedback was withdrawn, to the person being able to maintain control of head movements for extended periods without feedback. Changes were seen in range of motion, control of oscillation, and activities of daily living.

Clinical guide:

The aim of biofeedback is to re-establish a more normal posture and pattern of muscular activity. While it seems a reasonable approach, it needs to be tested using well-designed RCTs. Enthusiasm for recommending this treatment should be balanced by concerns about raising expectations that are unfulfilled.

Substantive changes

No new evidence

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Occupational therapy for focal dystonia

Summary

We found no direct information from systematic reviews or RCTs about occupational therapy in the treatment of people with focal dystonia.

Benefits and harms

Occupational therapy:

We found no systematic review or RCTs of occupational therapy in people with focal dystonia.

Comment

None.

Substantive changes

No new evidence

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Speech therapy for focal dystonia

Summary

Evidence for benefit of voice therapy plus botulinum toxin versus sham voice therapy plus botulinum toxin versus botulinum toxin-only is unclear.

Benefits and harms

Voice therapy plus botulinum A toxin versus sham voice therapy plus botulinum A toxin versus botulinum A toxin-only for laryngeal dystonia (adductor spasmodic dysphonia):

We found one small RCT, which compared voice therapy plus botulinum toxin versus sham voice therapy plus botulinum toxin versus botulinum toxin-only for laryngeal dystonia. Participants were compensated for their participation in the RCT, and excluded if they were unwilling to be allocated to the voice therapy or sham therapy arms. In addition, the botulinum injection was left to the complete discretion of the physician regarding dosage or administration.

Neurological disability

No data from the following reference on this outcome.

Quality of life

Voice therapy plus botulinum A toxin versus sham voice therapy plus botulinum A toxin versus botulinum A toxin-only for laryngeal dystonia We don't know how effective voice therapy plus botulinum A toxin, sham voice therapy plus botulinum A toxin, and botulinum A toxin alone are, compared with each other, in improving quality of life in people with adductor spasmodic dysphonia (very-low-quality evidence).

Ref (type)	Population	Outcome, Interventions	Results and statistical analysis	Effect size	Favours
Quality of life
RCT	31 adults with adductor spasmodic dysphonia	Voice-related quality of life with voice therapy plus botulinum A toxin with sham voice therapy plus botulinum A toxin with botulinum A toxin-only Absolute results not reported	Reported as not significant P value not provided Statistical analysis not clear		Not significant

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Adverse effects

No data from the following reference on this outcome.

Comment

None.

Substantive changes

Speech therapy for focal dystonia One RCT added. Categorisation of speech therapy for focal dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Amantadine for generalised dystonia

Summary

We found no direct information from RCTs about amantadine in the treatment of people with generalised dystonia.

Benefits and harms

Amantadine:

We found no systematic review or RCTs of amantadine in people with generalised dystonia.

Comment

See Comment on botulinum toxins for generalised dystonia.

Substantive changes

Amantadine for generalised dystonia Since the last update of this review, we now report drugs individually, rather than as groups. Categorisation of amantadine for generalised dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Baclofen for generalised dystonia

Summary

We found no direct information from RCTs about baclofen in the treatment of people with generalised dystonia.

Benefits and harms

Baclofen:

We found no systematic review or RCTs of baclofen in people with generalised dystonia.

Comment

See Comment on botulinum toxins for generalised dystonia.

Substantive changes

Baclofen for generalised dystonia Since the last update of this review, we now report drugs individually, rather than as groups. Categorisation of baclofen for generalised dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Benzatropine for generalised dystonia

Summary

We found no direct information from RCTs about benzatropine in the treatment of people with generalised dystonia.

Benefits and harms

Benzatropine:

We found no systematic review or RCTs of benzatropine in people with generalised dystonia.

Comment

See Comment on botulinum toxins for generalised dystonia.

Substantive changes

Benzatropine for generalised dystonia Since the last update of this review, we now report drugs individually, rather than as groups. Categorisation of benzatropine for generalised dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Botulinum toxins for generalised dystonia (e.g., botulinum A toxin, botulinum B toxin)

Summary

We found no direct information from RCTs about botulinum toxins in the treatment of people with generalised dystonia.

Benefits and harms

Botulinum toxins:

We found no systematic review or RCTs of botulinum toxins in people with generalised dystonia.

Comment

Clinical guide:

As with focal dystonia, the evidence supporting treatments for generalised dystonia is limited. Generalised dystonia is a relatively rare condition (small commercial incentive for research) that is variable both within and between people with the condition, and the effect is not easily measured. There are reports of trihexyphenidyl being administered off-licence at doses above the recommended maximum, but this is best considered within specialised services with suitable experience. People may often see a physiotherapist after diagnosis, but no specific therapeutic manoeuvres are known. Beliefs about the usefulness of physiotherapy vary. Surgical treatments are also used, although there is little long-term evidence of either benefits or risks from surgery.

Substantive changes

No new evidence

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Bromocriptine for generalised dystonia

Summary

We found no direct information from RCTs about bromocriptine in the treatment of people with generalised dystonia.

Benefits and harms

Bromocriptine:

We found no systematic review or RCTs of bromocriptine in people with generalised dystonia.

Comment

See Comment on botulinum toxins for generalised dystonia.

Substantive changes

Bromocriptine for generalised dystonia Since the last update of this review, we now report drugs individually, rather than as groups. Categorisation of bromocriptine for generalised dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Carbamazepine for generalised dystonia

Summary

We found no direct information from RCTs about carbamazepine in the treatment of people with generalised dystonia.

Benefits and harms

Carbamazepine:

We found no systematic review or RCTs of carbamazepine in people with generalised dystonia.

Comment

See Comment on botulinum toxins for generalised dystonia.

Substantive changes

Carbamazepine for generalised dystonia Since the last update of this review, we now report drugs individually, rather than as groups. Categorisation of carbamazepine for generalised dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Carbidopa/levodopa for generalised dystonia

Summary

We found no direct information from RCTs about carbidopa/levodopa in the treatment of people with generalised dystonia.

Benefits and harms

Carbidopa/levodopa:

We found no systematic review or RCTs of carbidopa/levodopa in people with generalised dystonia.

Comment

See Comment on botulinum toxins for generalised dystonia.

Substantive changes

Carbidopa/levodopa for generalised dystonia Since the last update of this review, we now report drugs individually, rather than as groups. Categorisation of carbidopa/levodopa for generalised dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Clonazepam for generalised dystonia

Summary

We found no direct information from RCTs about clonazepam in the treatment of people with generalised dystonia.

Benefits and harms

Clonazepam:

We found no systematic review or RCTs of clonazepam in people with generalised dystonia.

Comment

See Comment on botulinum toxins for generalised dystonia.

Substantive changes

Clonazepam for generalised dystonia Since the last update of this review, we now report drugs individually, rather than as groups. Categorisation of clonazepam for generalised dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Clozapine for generalised dystonia

Summary

We found no direct information from RCTs about clozapine in the treatment of people with generalised dystonia.

Benefits and harms

Clozapine:

We found no systematic review or RCTs of clozapine in people with generalised dystonia.

Comment

See Comment on botulinum toxins for generalised dystonia.

Substantive changes

Clozapine for generalised dystonia Since the last update of this review, we now report drugs individually, rather than as groups. Categorisation of clozapine for generalised dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Diazepam for generalised dystonia

Summary

We found no direct information from RCTs about diazepam in the treatment of people with generalised dystonia.

Benefits and harms

Diazepam:

We found no systematic review or RCTs of diazepam in people with generalised dystonia.

Comment

See Comment on botulinum toxins for generalised dystonia.

Substantive changes

Diazepam for generalised dystonia Since the last update of this review, we now report drugs individually, rather than as groups. Categorisation of diazepam for generalised dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Gabapentin for generalised dystonia

Summary

We found no direct information from RCTs about gabapentin in the treatment of people with generalised dystonia.

Benefits and harms

Gabapentin:

We found no systematic review or RCTs of gabapentin in people with generalised dystonia.

Comment

See Comment on botulinum toxins for generalised dystonia.

Substantive changes

Gabapentin for generalised dystonia Since the last update of this review, we now report drugs individually, rather than as groups. Categorisation of gabapentin for generalised dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Haloperidol for generalised dystonia

Summary

We found no direct information from RCTs about haloperidol in the treatment of people with generalised dystonia.

Benefits and harms

Haloperidol:

We found no systematic review or RCTs of haloperidol in people with generalised dystonia.

Comment

See Comment on botulinum toxins for generalised dystonia.

Substantive changes

Haloperidol for generalised dystonia Since the last update of this review, we now report drugs individually, rather than as groups. Categorisation of haloperidol for generalised dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Lorazepam for generalised dystonia

Summary

We found no direct information from RCTs about lorazepam in the treatment of people with generalised dystonia.

Benefits and harms

Lorazepam:

We found no systematic review or RCTs of lorazepam in people with generalised dystonia.

Comment

See Comment on botulinum toxins for generalised dystonia.

Substantive changes

Lorazepam for generalised dystonia Since the last update of this review, we now report drugs individually, rather than as groups. Categorisation of lorazepam for generalised dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Ondansetron for generalised dystonia

Summary

We found no direct information from RCTs about ondansetron in the treatment of people with generalised dystonia.

Benefits and harms

Ondansetron:

We found no systematic review or RCTs of ondansetron in people with generalised dystonia.

Comment

See Comment on botulinum toxins for generalised dystonia.

Substantive changes

Ondansetron for generalised dystonia Since the last update of this review, we now report drugs individually, rather than as groups. Categorisation of ondansetron for generalised dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Pregabalin for generalised dystonia

Summary

We found no direct information from RCTs about pregabalin in the treatment of people with generalised dystonia.

Benefits and harms

Pregabalin:

We found no systematic review or RCTs of pregabalin in people with generalised dystonia.

Comment

See Comment on botulinum toxins for generalised dystonia.

Substantive changes

Pregabalin for generalised dystonia Since the last update of this review, we now report drugs individually, rather than as groups. Categorisation of pregabalin for generalised dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Procyclidine for generalised dystonia

Summary

We found no direct information from RCTs about procyclidine in the treatment of people with generalised dystonia.

Benefits and harms

Procyclidine:

We found no systematic review or RCTs of procyclidine in people with generalised dystonia.

Comment

See Comment on botulinum toxins for generalised dystonia.

Substantive changes

Procyclidine for generalised dystonia Since the last update of this review, we now report drugs individually, rather than as groups. Categorisation of procyclidine for generalised dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Tizanidine for generalised dystonia

Summary

We found no direct information from RCTs about tizanidine in the treatment of people with generalised dystonia.

Benefits and harms

Tizanidine:

We found no systematic review or RCTs of tizanidine in people with generalised dystonia.

Comment

See Comment on botulinum toxins for generalised dystonia.

Substantive changes

Tizanidine for generalised dystonia Since the last update of this review, we now report drugs individually, rather than as groups. Categorisation of tizanidine for generalised dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Trazodone hydrochloride for generalised dystonia

Summary

We found no direct information from RCTs about trazodone hydrochloride in the treatment of people with generalised dystonia.

Benefits and harms

Trazodone hydrochloride:

We found no systematic review or RCTs of trazodone hydrochloride in people with generalised dystonia.

Comment

See Comment on botulinum toxins for generalised dystonia.

Substantive changes

Trazodone hydrochloride for generalised dystonia Since the last update of this review, we now report drugs individually, rather than as groups. Categorisation of trazodone hydrochloride for generalised dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Trihexyphenidyl for generalised dystonia

Summary

We found no direct information from RCTs about trihexyphenidyl in the treatment of people with generalised dystonia.

Benefits and harms

Trihexyphenidyl:

We found no systematic review or RCTs of trihexyphenidyl in people with generalised dystonia.

Comment

See Comment on botulinum toxins for generalised dystonia.

Substantive changes

Trihexyphenidyl for generalised dystonia Since the last update of this review, we now report drugs individually, rather than as groups. Categorisation of trihexyphenidyl for generalised dystonia unchanged (unknown effectiveness).

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Deep brain stimulation of thalamus and globus pallidus for generalised dystonia

Summary

We found no direct information from RCTs about deep brain stimulation of the thalamus in only people with generalised dystonia. Evidence in a mixed population of people with focal or generalised dystonia suggests that it may improve function at 3 months.

Benefits and harms

Deep brain stimulation versus sham treatment:

We found no systematic review or RCTs of deep brain stimulation of the thalamus in people with only generalised dystonia. We found one RCT that compared deep brain stimulation of the internal globus pallidus versus sham stimulation in people with either generalised or focal dystonia.

Neurological disability

Deep brain stimulation compared with sham stimulation Deep brain stimulation of the thalamus and globus pallidus may be more effective at 3 months at improving total movement (with imputation) and disability scores on the Burke-Fahn-Marsden Dystonia (BFMD) Rating Scale in people with primary segmental and generalised dystonia (very low-quality evidence).

Ref (type)	Population	Outcome, Interventions	Results and statistical analysis	Effect size	Favours
Neurological disability
RCT	40 people aged 14–75 years with primary segmental (16 people) or generalised dystonia (24 people) for a minimum of 5 years	Improvement in Burke-Fahn-Marsden Dystonia (BFMD) Rating Scale total movement score 3 months –15.8 with neurostimulation –1.4 with sham stimulation Change in BFMD score for movement from baseline; possible range of scores of 0–120	P <0.001	Effect size not calculated	neurostimulation
RCT	40 people aged 14–75 years with primary segmental (16 people) or generalised dystonia (24 people) for a minimum of 5 years	Improvement in BFMD Rating Scale disability score 3 months –3.9 with neurostimulation –0.8 with sham stimulation Change in BFMD score for disability from baseline; possible range of scores of 0–30	P <0.001	Effect size not calculated	neurostimulation

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Quality of life

Deep brain stimulation compared with sham stimulation Deep brain stimulation of the thalamus and globus pallidus may be more effective at improving the physical component of quality-of-life scores (assessed using short form [SF]-36 questionnaire) and several subscale scores, including bodily pain score. However, we don't know whether it is more effective at improving the mental component of quality-of-life scores or other subscale scores in people with primary segmental and generalised dystonia (very low-quality evidence).

Ref (type)	Population	Outcome, Interventions	Results and statistical analysis	Effect size	Favours
Quality of life
RCT	40 people aged 14–75 years with primary segmental (16 people) or generalised dystonia (24 people) for a minimum of 5 years	Improvement in short form (SF)-36 physical component score 10.1 with neurostimulation 3.8 with sham stimulation Change in SF-36 physical component score; possible range of scores of 0–100	P = 0.02 33/40 (82.5%) assessed for this outcome	Effect size not calculated	neurostimulation
RCT	40 people aged 14–75 years with primary segmental (16 people) or generalised dystonia (24 people) for a minimum of 5 years	Improvement in SF-36 mental component score 5.2 with neurostimulation 0.2 with sham stimulation Change in SF-36 mental component score; possible range of scores of 0–100	P = 0.39		Not significant
RCT	40 people aged 14–75 years with primary segmental (16 people) or generalised dystonia (24 people) for a minimum of 5 years Further report of reference	Improvement in SF-36 physical function score 3 months 27.3 with neurostimulation 3.0 with sham stimulation	P = 0.001 36/40 (90%) assessed for this outcome	Effect size not calculated	neurostimulation
RCT	40 people aged 14–75 years with primary segmental (16 people) or generalised dystonia (24 people) for a minimum of 5 years Further report of reference	Improvement in SF-36 bodily pain score 3 months 22.7 with neurostimulation 9.7 with sham stimulation	P = 0.04 37/40 (93%) assessed for this outcome	Effect size not calculated	neurostimulation
RCT	40 people aged 14–75 years with primary segmental (16 people) or generalised dystonia (24 people) for a minimum of 5 years Further report of reference	Improvement in SF-36 general health score 3 months 17.6 with neurostimulation 2.1 with sham stimulation	P = 0.02 37/40 (93%) assessed for this outcome	Effect size not calculated	neurostimulation
RCT	40 people aged 14–75 years with primary segmental (16 people) or generalised dystonia (24 people) for a minimum of 5 years Further report of reference	Improvement in SF-36 vitality score 3 months 14.7 with neurostimulation 2.0 with sham stimulation	P = 0.047 37/40 (93%) assessed for this outcome	Effect size not calculated	neurostimulation
RCT	40 people aged 14–75 years with primary segmental (16 people) or generalised dystonia (24 people) for a minimum of 5 years Further report of reference	Improvement in SF-36 role physical score 3 months 25.0 with neurostimulation 13.2 with sham stimulation	P = 0.20 35/40 (88%) assessed for this outcome		Not significant
RCT	40 people aged 14–75 years with primary segmental (16 people) or generalised dystonia (24 people) for a minimum of 5 years Further report of reference	Improvement in SF-36 social function score 3 months 21.1 with neurostimulation 0.7 with sham stimulation	P = 0.07 37/40 (93%) assessed for this outcome		Not significant
RCT	40 people aged 14–75 years with primary segmental (16 people) or generalised dystonia (24 people) for a minimum of 5 years Further report of reference	Improvement in SF-36 role emotional score 3 months 24.6 with neurostimulation 13.7 with sham stimulation	P = 0.43 36/40 (90%) assessed for this outcome		Not significant
RCT	40 people aged 14–75 years with primary segmental (16 people) or generalised dystonia (24 people) for a minimum of 5 years Further report of reference	Improvement in SF-36 mental health score 3 months 10.7 with neurostimulation 2.0 with sham stimulation	P = 0.54 37/40 (93%) assessed for this outcome		Not significant
RCT	40 people aged 14–75 years with primary segmental (16 people) or generalised dystonia (24 people) for a minimum of 5 years Further report of reference	Improvement in Brief Psychiatric Rating Scale 3 months –5.9 with neurostimulation –3.0 with sham stimulation	P = 0.09 37/40 (93%) assessed for this outcome		Not significant

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Adverse effects

Ref (type)	Population	Outcome, Interventions	Results and statistical analysis	Effect size	Favours
Adverse effects
RCT	40 people aged 14–75 years with primary segmental (16 people) or generalised dystonia (24 people) for a minimum of 5 years	Infection at stimulator site 1/20 (5%) with neurostimulation 2/20 (10%) with sham treatment	Reported as not significant

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Further information on studies

The RCT analysed data for all people who underwent randomisation (last observation carried forward). The RCT did not carry out a subgroup analysis of people with generalised dystonia, which may affect the generalisability of the results.

Comment

Clinical guide:

RCTs with longer follow-up are required. The beneficial effects of stimulation may wear off over time, and the long-term risks and adverse effects of implantation into the brain and of brain stimulation itself are not known. We suggest that a minimum of 12 months' controlled observation (i.e., without implantation into the control group) may be required to judge effectiveness, and that a minimum of 5 years of natural history follow-up (i.e., after implantation) may be required to judge safety and long-term risk, and to confirm persistence of any beneficial effect. See also Comment on botulinum toxins for generalised dystonia.

Substantive changes

No new evidence

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Acupuncture for generalised dystonia

Summary

We found no direct information from systematic reviews or RCTs about acupuncture in the treatment of people with generalised dystonia.

Benefits and harms

Acupuncture:

We found no systematic review or RCTs of acupuncture in people with generalised dystonia.

Comment

See Comment on botulinum toxins for generalised dystonia.

Substantive changes

No new evidence

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Biofeedback for generalised dystonia

Summary

We found no direct information from systematic reviews or RCTs about biofeedback in the treatment of people with generalised dystonia.

Benefits and harms

Biofeedback:

We found no systematic review or RCTs of biofeedback in people with generalised dystonia.

Comment

See Comment on botulinum toxins for generalised dystonia.

Substantive changes

No new evidence

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Occupational therapy for generalised dystonia

Summary

We found no direct information from systematic reviews or RCTs about occupational therapy in the treatment of people with generalised dystonia.

Benefits and harms

Occupational therapy:

We found no systematic review or RCTs of occupational therapy in people with generalised dystonia.

Comment

See Comment on botulinum toxins for generalised dystonia.

Substantive changes

No new evidence

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Physiotherapy for generalised dystonia

Summary

We found no direct information from systematic reviews or RCTs about physiotherapy in the treatment of people with generalised dystonia.

Benefits and harms

Physiotherapy:

We found no systematic review or RCTs of physiotherapy in people with generalised dystonia.

Comment

See Comment on botulinum toxins for generalised dystonia.

Substantive changes

No new evidence

BMJ Clin Evid. 2014 Feb 28;2014:1211.

Speech therapy for generalised dystonia

Summary

We found no direct information from systematic reviews or RCTs about speech therapy in the treatment of people with generalised dystonia.

Benefits and harms

Speech therapy:

We found no systematic review or RCTs of speech therapy in people with generalised dystonia.

Comment

See Comment on botulinum toxins for generalised dystonia.

Substantive changes

No new evidence

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PERMALINK

Dystonia

Ailsa Snaith

Derick Wade

Roles

Abstract

Introduction

Methods and outcomes

Results

Conclusions

Key Points

About this condition

Definition

Incidence/ Prevalence

Aetiology/ Risk factors

Prognosis

Aims of intervention

Outcomes

Table 1.

Methods

Table.

Glossary

Disclaimer

Contributor Information

References

Botulinum toxins for focal dystonia (e.g., botulinum A toxin, botulinum B toxin)

Summary

Benefits and harms

Botulinum A toxin versus placebo in cervical dystonia in adults:

Neurological disability

Quality of life

Adverse effects

Botulinum B toxin versus placebo in cervical dystonia in adults:

Neurological disability

Quality of life

Adverse effects

Botulinum A toxin versus botulinum B toxin in cervical dystonia in adults:

Neurological disability

Quality of life

Adverse effects

Low-dose (100 U Botox/250 U Dysport) versus high-dose (>200 U Botox/960 U Dysport) botulinum A toxin in cervical dystonia in adults:

Neurological disability

Quality of life

Adverse effects

Low-dose (2500–5000 U) versus high-dose (10,000 U) botulinum B toxin in cervical dystonia in adults:

Neurological disability

Quality of life

Adverse effects

Botulinum A toxin versus trihexyphenidyl in cervical dystonia in adults:

Neurological disability

Quality of life

Adverse effects

Botulinum B toxin in botulinum A toxin-resistant adults versus respondent adults:

Neurological disability

Quality of life

Adverse effects

Botulinum A toxin versus placebo in people with blepharospasm (eyelid closure):

Botulinum A toxin versus placebo in people with spasmodic dysphonia (laryngeal dystonia):

Botulinum A toxin versus placebo in people with writer's cramp:

Neurological disability

Quality of life

Adverse effects

Further information on studies

Comment

Clinical guide:

Different formulations of botulinum A toxin versus each other in people with blepharospasm (eyelid closure):

Dosage:

Substantive changes

Amantadine for focal dystonia

Summary

Benefits and harms

Amantadine:

Comment

Substantive changes

Baclofen for focal dystonia

Summary

Benefits and harms

Baclofen:

Comment

Substantive changes