In most cases of focal dystonia, the usual management 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. The patients who benefit from botulinum toxin injections are treated at intervals, adjusted to the duration of the benefit of the management (usually 2 to 4 months, sometimes longer).
Botulinum Toxin (BoNT) can be regarded as first-line management for idiopathic (or inherited) cervical dystonia and blepharospasm.
BoNT injections are relatively safe and efficacious when repeated management using recommended doses is performed over many years but excessive doses result in increased risk of side effects at each session. Prolonged use of botulinum toxin has been associated with resistance resulting from antibody in some cases.
Nurse and physiotherapist injectors
The majority of BoNT injections are currently provided by consultants and senior doctors – but injections in many cases can be effectively administered by nurses or physiotherapists provided appropriate training, supervision and patient review is provided by the consultant responsible for patient care (although some complex injections such as those to treat laryngeal dystonia are done only by consultants). Dystonia nurses are sometimes also able to adjust the dose of botulinum toxin within agreed parameters, if necessary seeking advice from the consultant according to agreed protocols (Whitaker et al (2001)).
In 2012, nurses and physiotherapists provided around a quarter of all injections and in some clinics the proportion was substantially above 50%.
Where BoNT is not working
BoNT injections provide significant relief for the majority of patients in the categories identified above. However, a substantial minority of patients report receiving limited relief. In these cases
the following steps should be considered:
Botulinum toxin injections can be effectively provided through outreach services based in local community hospitals and health centres. In rural areas, outreach services address an important patient need. 35% of patients currently have a round trip travel time of more than 2 hours to their injection centre and, among this group, 55% report travel as a problem. The problem of travel is exacerbated by the fact that many patients are elderly – the average age of someone receiving botulinum injections is 60 years with 23% of patients over 70 years.
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 management with levodopa should be initiated and adjusted according to the clinical response.
Anticholinergic drugs are the only group that have been consistently considered efficacious (Cullis 1989; Lang 1989) although only small and methodologically poor Randomised Controlled Trials (RCTs) are available. For many patients the side effects are not manageable. It is important to increase very slowly the dosage over 2 or 3 months to minimise side effects. The drug should be avoided in elderly patients (after the age of 70). Alcohol is contra-indicated with this drug.
Sometimes 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.
It is essential to monitor the effects of management using oral medications carefully and, if more than one healthcare professional is involved, to be clear who is responsible for this.
Pallidal Deep Brain Stimulation (DBS). Considered a good option, particularly for idiopathic (or inherited) generalised or cervical dystonia and dystonic tremor, 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 procedure is carried out while the patient is asleep. The electrodes send a pulse that blocks the signals from the brain that cause the involuntary muscle spasms. DBS is funded by the NHS (and in England is approved as a specialised treatment by the National Commissioning Board).
In general, DBS is less effective in acquired 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. More information on DBS can be obtained from the nearest DBS implanting centre.
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.
After the DBS operation, the patient will need regular follow-up as it can take several months to reach full effectiveness. In addition, the patient may require a range of allied healthcare support (see section 4.4 below).
A number of other surgical approaches are also used in some cases. Selective peripheral denervation is an alternative approach to treat medically refractory cervical dystonia. There is a significant risk of recurrence of symptoms. Insufficient evidence exists to use this intervention in idiopathic or inherited dystonia but the procedure can be indicated in patients where acquired dystonia is combined with spasticity.
BoNT, oral medication and surgery are effective treatments and can substantially mitigate symptoms
for patients with dystonia but they are not a complete solution. In a Dystonia Society survey, patients reported that on average they only mitigate around half their symptoms. A number of allied healthcare interventions should therefore also be considered.
For focal dystonias, the use of rehabilitative physiotherapy is well developed and structured. It aims
to give patients as much independence as possible. The objective of physiotherapy is to correct the affected function through specific interventions. This type of therapy is demanding for both the patient and the therapist (Bleton (2007)). Studies have shown that physiotherapy also enhances the effectiveness of BoNT in treating cervical dystonia but, at present, they have not identified which techniques are the most beneficial. In a Dystonia Society survey, among those referred to specialist neurophysiotherapy, 74% of patients with cervical dystonia and 62% of those with focal hand dystonia reported it as being helpful.
Due to the specialised nature of dystonia, this treatment needs to be provided by a neurophysio-therapist familiar with the condition.
Dystonia is not a mental health condition but it can cause depression and anxiety due to the pain, stigma and social isolation of the condition. The overall rate of psychiatric symptoms amongst individuals with dystonia is often higher than that seen in the general population, with onset of the psychiatric symptoms prior to the physical symptoms of dystonia in some cases.
Psychological therapies such as cognitive behavioural therapy (CBT) and counselling can therefore play an important role. In addition, as stress and anxiety can exacerbate the physical symptoms of dystonia, effective management of these can also improve the outcome of managing the physical symptoms.
Mindfulness (autogenic training or meditation) can also be helpful. In a Dystonia Society questionnaire, mindfulness was reported helpful by 65% of those who tried it.
Pain resulting from dystonia can be in the muscles affected by spasms, or in joints where bone surfaces rub together due to twisting of posture. Sometimes, the resulting intractable pain can dominate a patient’s life and may be unresponsive to medication including that used to manage dystonia. Among those with cervical dystonia, a high prevalence of substantial pain is reported. Pain is also reported for other types of adult onset dystonia in some cases. Referral to pain management programmes has been shown to be effective for management of chronic pain caused by conditions such as dystonia.
Speech and language therapy
A number of dystonias can affect speech. Where speech difficulties occur referral should be made to
a speech and language therapist (SALT):
Last reviewed April 2014
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