The Dystonia Society

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Treatment

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Treatment for Adults

Botulinum Toxin injections

In most cases of focal dystonia, the usual treatment is regular botulinum toxin injections into the affected muscles, usually around every 12 weeks. Botulinum toxin affects the nerves at their junction with the muscle. It prevents the release of acetylcholine from the nerve endings and thereby prevents the involuntary muscle contractions.

The frequency can vary for some forms of dystonia at the consultant’s clinical discretion. It should not be used more often than every 8 weeks as there is an increased risk of antibody development.
•    Botulinum Toxin (BoNT) A (or type B if there is resistance to type A) can be regarded as first-line treatment for primary cervical dystonia and blepharospasm.
•    BoNT-A can be effective for writer’s cramp and is probably effective in other types of upper limb dystonia, but often EMG guided injections are required to pinpoint the overactive muscles.
•    BoNT-A is usually effective for adductor-type and abductor-type laryngeal dystonia.  However in mixed or atypical abductor laryngeal dystonia it does not work well or consistently

Notes:
•    BoNT injections are relatively safe and efficacious when repeated treatments of recommended doses are performed over many years but excessive doses result in increased risk of side effects at each session. Cumulative doses can result in antibody formation. Doctors should refer to Summaries of Product Characteristics for information on indications and dosing etc.
•    BoNT injections are usually performed by direct clinical assessment; EMG or ultrasound-assisted targeting may improve clinical outcome.
•    BoNT should not be used in patients affected by neuromuscular junction abnormalities or if there is local infection at the injection site. The recommended dosage should not be exceeded.
•    BoNT is not licensed for treatment of dystonia in children (see section 3.4).

Drug treatments

Levodopa
A diagnostic levodopa trial is warranted in every patient with early-onset dystonia in case they have dopa-responsive dystonia.  Following a positive diagnostic trial with levodopa, chronic treatment with levodopa should be initiated and adjusted according to the clinical response.

Anticholinergic agents
The absolute and comparative efficacy and tolerability of anticholinergic agents in dystonia is poorly documented in children and there is no proof of efficacy in adults. Therefore no recommendation can be made to guide prescribing. (See Chapter 5 for additional notes on medication for childhood onset dystonia).

Tetrabenazine/benzodiazepines
Frequently used in the treatment of dystonia but documentation of benefit in well-designed studies is lacking

There is a lack of evidence to give recommendations for the use of antiepileptics.

Surgery

Pallidal Deep Brain Stimulation (DBS). Considered a good option, particularly for primary generalized or segmental dystonia after medication or botulinum toxin injections have failed to provide adequate improvement. In this procedure, two fine electrodes are inserted into the brain powered by a battery implanted in the chest. The electrodes send a pulse that blocks the signals from the brain that cause the involuntary muscle spasms.

In general, DBS is less effective in secondary dystonia with the exception of tardive dystonia.  DBS can have side effects and involves a life-long commitment by the patient (and family) as on-going follow-up is required and may necessitate travel to a DBS centre. DBS can be also considered for cervical dystonia if other treatments have not worked.

Assessment requires a specialised multi-disciplinary team. Some patients may see DBS as a potential ‘cure’ and can be very disappointed if after full assessment they are not thought to be a suitable candidate. Care must be therefore taken to manage patient expectations and the conveying of such a decision must be handled sensitively.

Selective peripheral denervation An alternative approach to treat medically refractory cervical dystonia. There is a significant risk or recurrence of symptoms. Insufficient evidence exists to use this treatment in primary dystonia but the procedure can be indicated in patients where secondary dystonia is combined with spasticity.

Therapeutic Interventions

A number of studies have reported motor improvement in patients with writer’s cramp and other forms of focal dystonia following physical treatment and sensory and motor retraining. The conduction of new randomised controlled studies on these potentially useful interventions for patients with upper limb dystonia may provide more evidence.

Coping strategies

There are a number of techniques called ‘geste antagoniste’ that can be adopted to help manage symptoms. These can include touching the chin to stop the head turning or tilting in cervical dystonia; and touching the temple in blepharospasm to stop the eyelids closing. For some people, symptoms can also be mitigated through focusing on another activity such as talking or playing a musical instrument. It should be noted however that these coping strategies have not been scrutinised in any formal studies. In addition, some people do find the symptoms come back more aggressively when these techniques have been used.

A note on the use of Cognitive Behavioural Therapy (CBT) in a dystonia treatment plan
People with dystonia who are referred for CBT may be concerned because they believe it to imply that the doctor thinks their dystonia is ‘all in the mind’. Good communication with the patient is therefore essential so that the patient understands why CBT is being recommended.  There is currently little research evidence about the use of CBT in dystonia, but the principles on which CBT is based appear to support the theory that it could be helpful in the management of some cases of dystonia. It may also help associated symptoms such as depression, anxiety, anger, sleep problems and chronic pain. CBT should be classified as an experimental treatment.

Treatment for Children

Oral medications are often the mainstay for treating dystonia in children and young people. They may respond well and can tolerate higher doses than adults, but the effect may be shortlived or the medication may cause side effects such as somnolence, drooling, poor trunk and neck control and difficulties concentrating in class. Mood and behavioural disturbances may further limit the use of drugs. There is no one drug that is the definitive treatment but often a combination of several drugs and other treatments can enable effective management. When the cause of dystonia is unknown and the brain MRI scan is normal, a trial of levodopa is required to diagnose the rare genetic disorder known as dopa responsive dystonia which can be managed for years on small doses of levodopa once or twice a day.

All dystonia management should be tailored to individual needs of children and goal-directed under the guidance of experienced doctors and therapists. The Multidisciplinary Team (MDT) is a vital to supporting families of children with dystonia and can help create strategies for coping with many essential functions.

Some of the management options that may be offered include:

Medication:

Levodopa
Trihexyphenidyl
Baclofen* (oral and/or intrathecal)
Diazepam*
Lorazepam
Clonazepam
Nitrazepan
Carbamazepine
Tetrabenazine   
Chloral hydrate
Clonidine
Dantrolene Sodium

* Limited license for use to treat muscle spasms in chlidren

Other Treatment

Botulinum toxin A injections These are not licensed for children. However, they are often used for management directed at specific muscle groups that interfere with function of the neck, jaw, hands, elbows, hips, knees, ankles or feet . Great care must be exercised to avoid botulism through overdosage or excessively frequent injections. Very disabled children with little or any voluntary movements are particularly vulnerable to respiratory difficulties after excessive botulinum toxin A injections.

Intrathecal baclofen (ITB)  This approach offers regional and total body dystonia control without some of the risks of somnolence attendant on oral medication. This will sometimes be used to treat secondary dystonia not amenable to Deep Brain Stimulation.

Deep Brain Stimulation (DBS)
This is very effective in primary dystonias. It should be recognised that the longer the duration of dystonia, the greater the risk of skeletal deformities, dependency (through lack of opportunity) and potential lowered efficacy of DBS. DBS should be considered when dystonia is rapidly progressive and disabling and when two or more drugs have failed to bring adequate relief of dystonia or the drugs are poorly tolerated. Loss of a major skill such as walking, manual ability, speech or feeding are signs that functional neurosurgery should be considered.
Unfortunately, many forms of secondary dystonia that are associated with focal or generalised brain injury will preclude the use of DBS.

Other Considerations

Dystonia onset before puberty is particularly disruptive to a child’s growth and development. The stress of dystonia may prevent the onset of puberty. The effect of puberty in a dystonic child may accelerate the appearance of contracture and deformity which further diminishes function and limits opportunity.

Children with primary dystonia may need to be referred to other specialist departments for treatment for conditions that result from dystonia (e.g. musculoskeletal and orthopaedic problems).  Those with secondary dystonia are also very likely to be under the care of different teams at different stages. It is essential that full communication is established and maintained between the neurology/movement disorder team and these other specialist teams so that treatment is optimal and appropriate.

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Last reviewed October 2011

Disclaimer
The Dystonia Society provides the information on this page as general information only. It is not intended to provide instruction and you should not rely on this information to determine diagnosis, prognosis or a course of treatment. It should not be used in place of a professional consultation with a doctor.
The Dystonia Society is not responsible for the consequences of your decisions resulting from the use of this information, including, but not limited to, your choosing to seek or not to seek professional medical care, or from choosing or not choosing specific treatment based on the information. You should not disregard the advice of your physician or other qualified health care provider because of any information you receive from us. If you have any health care questions, please consult the relevant medical practitioner.

 

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