Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

without pain or functional impairment

1st line – observation

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ACUTE
|
without pain or functional impairment
1st line – 

observation

Asymptomatic patients require no treatment.

Plus – physical therapy

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ACUTE
|
without pain or functional impairment
Plus – 

physical therapy

Treatment recommended for ALL patients in selected patient group

Physical therapy and stretching should be considered to maintain range of motion.

with functional impairment or pain or diminished quality of life

1st line – oral medication

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ACUTE
|
with functional impairment or pain or diminished quality of life
1st line – 

oral medication

Multiple oral medications from various classes have been reported to ameliorate dystonia.

The medications listed below are those most commonly used in clinical practice and span a range of different pharmacologic mechanisms of action (anticholinergic, GABAergic and centrally acting).[19]

Some principles are applicable to all agents: efficacy is limited at tolerable dosing, central side effects (i.e., fatigue or confusion) are usually dose limiting, and all agents should be started at low doses and increased to efficacy or until tolerability limits dose.

Primary options

trihexyphenidyl: 1 mg orally once daily at bedtime initially, increase according to response, maximum 12 mg/day given in 3-4 divided doses

OR

clonazepam: 0.25 mg orally twice daily initially, increase according to response, maximum 6 mg/day given in 2-3 divided doses

OR

baclofen: 5 mg orally three times daily initially, increase according to response, maximum 160 mg/day given in 3-4 divided doses

OR

tizanidine: 2 mg orally once daily at bedtime initially, increase according to response, maximum 24 mg/day given in 3-4 divided doses

Plus – physical therapy

Back
ACUTE
|
with functional impairment or pain or diminished quality of life
Plus – 

physical therapy

Treatment recommended for ALL patients in selected patient group

While a robust evidence base for physical therapy in cervical dystonia is lacking, physical therapy can be helpful for some patients and does not carry significant risk of harm.[20][21] It is thus reasonable to consider physical therapy as part of any treatment plan.

2nd line – intramuscular botulinum toxin

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ACUTE
|
with functional impairment or pain or diminished quality of life
2nd line – 

intramuscular botulinum toxin

The intramuscular administration of botulinum toxin provides the best proven efficacy in acquired torticollis. [ Cochrane Clinical Answers logo ] Sustained efficacy in reduction of dystonia severity has been demonstrated after 7 years of repeated injections with onabotulinumtoxinA.[25] Botulinum toxin injections are considered as first-line therapy for acquired torticollis by the European Federation of Neurological Societies and are recommended by the American Academy of Neurology.[18][26]

There are four different types of botulinum toxin available. The unit doses are not equivalent/interchangeable. OnabotulinumtoxinA, rimabotulinumtoxinB, abobotulinumtoxinA, and incobotulinumtoxinA have all been shown to provide significant benefit in placebo-controlled trials.[33][54] Botulinum toxin has also been found to be significantly more beneficial than oral trihexyphenidyl.[26][27][28][29][30][31] No significant differences in efficacy of onabotulinum toxinA and rimabotulinumtoxinB have been demonstrated.[24][32][33] Dry mouth was a more frequent adverse event with rimabotulinumtoxinB.[33]

The American Academy of Neurology states that abobotulinumtoxinA and rimabotulinumtoxinB are established effective treatments for cervical dystonia and and should be offered, and onabotulinumtoxinA and incobotulinumtoxinA are probably effective and should be considered.[26]

Technical considerations for its use include: injection into dystonic muscles which serves to block acetylcholine release into the neuromuscular junction resulting in partial denervation; clinical effect typically lasts 3 to 4 months;[35] injections are typically repeated every 3 to 4 months;[35] some clinicians advocate the use of electromyography to localize muscles for injection; electromyography is particularly useful in injecting deep or small muscles. Limited evidence suggests that using electromyography guidance may improve outcomes.[36] Ultrasound imaging is also sometimes used to guide injections.[37]

Side effects of therapy with intramuscular botulinum toxin are related to induced muscle weakness and can include significant dysphagia (when injected into cervical muscles) or systemic uptake of toxin resulting in mild botulism (of which difficulty swallowing is an early symptom). When compared with placebo, patients treated with rimabotulinumtoxinB were at increased risk of dry mouth and dysphagia.[31] Ultrasound-guided injections into the sternocleidomastoid are being investigated as a way to reduce the risk of dysphagia.[38]

Neutralizing antibody development occurs in 2% of patients and may manifest as loss of efficacy, although some patients with demonstrated antibodies to abobotulinumtoxinA continue to have a clinical response to injection.[40] Risk can be reduced by using the lowest effective dose of botulinum toxin and temporally spacing injections by at least 3 months.[39]

The Food and Drug Administration (FDA) posted an early communication in February 2008 warning of potential botulism with local infection of botulinum toxin.[42] These products now come with a boxed warning highlighting the risk of potential distant spread of toxin effects resulting in systemic symptoms with local injection. Most cases occurred in children treated for spasticity in the setting of cerebral palsy.[42]

Primary options

onabotulinumtoxinA: consult specialist for guidance on dose

OR

rimabotulinumtoxinB: consult specialist for guidance on dose

OR

abobotulinumtoxinA: consult specialist for guidance on dose

OR

incobotulinumtoxinA: consult specialist for guidance on dose

Plus – physical therapy

Back
ACUTE
|
with functional impairment or pain or diminished quality of life
Plus – 

physical therapy

Treatment recommended for ALL patients in selected patient group

While a robust evidence base for physical therapy in cervical dystonia is lacking, physical therapy can be helpful for some patients and does not carry significant risk of harm.[20][21] It is thus reasonable to consider physical therapy as part of any treatment plan. One small randomized study (26 patients with initial suboptimal response to botulinum toxin treatment) found physical therapy plus botulinum toxin treatment to be superior to botulinum toxin treatment alone.[22]

ONGOING

refractory to therapy with botulinum toxin

1st line – deep brain stimulation

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ONGOING
|
refractory to therapy with botulinum toxin
1st line – 

deep brain stimulation

Deep brain stimulation of the globus pallidus pars interna (GPi) is a proven treatment for generalized dystonia and has been shown to provide significant benefit to patients with medically refractory acquired torticollis.[18][43] However, a Cochrane Review rated current clinical evidence for deep brain stimulation in cervical dystonia as low quality.[44] In a randomized sham-controlled trial, GPi deep brain stimulation significantly reduced severity of torticollis compared with sham stimulation at 3 months.[45]

Stimulation of bilateral GPi has been shown to have a durable effect at 3 years, and a positive effect was reported for deep brain stimulation of the bilateral subthalamic nucleus in 9 patients with refractory cervical dystonia.[46][48] Another series showed significant improvement in dystonia severity at a median of 30 months in a blinded evaluation.[47]

Referral for these procedures is best made by a movement disorders specialist.

Plus – physical therapy

Back
ONGOING
|
refractory to therapy with botulinum toxin
Plus – 

physical therapy

Treatment recommended for ALL patients in selected patient group

While a robust evidence base for physical therapy in cervical dystonia is lacking, physical therapy can be helpful for some patients and does not carry significant risk of harm.[20][21] It is thus reasonable to consider physical therapy as part of any treatment plan.

1st line – radiofrequency ablation or selective surgical sectioning of peripheral nerves

Back
ONGOING
|
refractory to therapy with botulinum toxin
1st line – 

radiofrequency ablation or selective surgical sectioning of peripheral nerves

Selective denervation by radiofrequency ablation may be helpful in patients with severe or refractory acquired torticollis. Significant and durable improvement in symptoms has been reported in 70% of patients undergoing radiofrequency ablation and post-procedure physical therapy.[49] Case series of selective surgical denervation have shown significant reduction in disease severity scores.[50]

A single institution series found similar results with selective peripheral denervation compared with deep brain stimulation.[51] There are limited but positive published data regarding combining deep brain stimulation and selective denervation in refractory cases.[52]

Referral for these procedures is best made by a movement disorders specialist.

Plus – physical therapy

Back
ONGOING
|
refractory to therapy with botulinum toxin
Plus – 

physical therapy

Treatment recommended for ALL patients in selected patient group

While a robust evidence base for physical therapy in cervical dystonia is lacking, physical therapy can be helpful for some patients and does not carry significant risk of harm.[20][21] It is thus reasonable to consider physical therapy as part of any treatment plan.

2nd line – injected phenol

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ONGOING
|
refractory to therapy with botulinum toxin
2nd line – 

injected phenol

Injections of 2% phenol have been shown to be effective.[53]

Needle electromyography or motor stimulation is used to localize the region of the motor end plate, and phenol solution is injected.

Adverse effects can include pain, swelling, tissue necrosis, and thrombosis.

Primary options

phenol: (2%) consult specialist for guidance on dose

Plus – physical therapy

Back
ONGOING
|
refractory to therapy with botulinum toxin
Plus – 

physical therapy

Treatment recommended for ALL patients in selected patient group

While a robust evidence base for physical therapy in cervical dystonia is lacking, physical therapy can be helpful for some patients and does not carry significant risk of harm.[20][21] It is thus reasonable to consider physical therapy as part of any treatment plan.

2nd line – baclofen injection via continuous pump infusion

Back
ONGOING
|
refractory to therapy with botulinum toxin
2nd line – 

baclofen injection via continuous pump infusion

Intrathecal baclofen may be helpful in patients with severe or refractory acquired torticollis, and in particular those with concomitant spasticity.

Referral for these procedures is best made by a movement disorders specialist.

Primary options

baclofen intrathecal: consult specialist for guidance on dose

Plus – physical therapy

Back
ONGOING
|
refractory to therapy with botulinum toxin
Plus – 

physical therapy

Treatment recommended for ALL patients in selected patient group

While a robust evidence base for physical therapy in cervical dystonia is lacking, physical therapy can be helpful for some patients and does not carry significant risk of harm.[20][21] It is thus reasonable to consider physical therapy as part of any treatment plan.

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Choose a patient group to see our recommendations

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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