Download Dystonia – Information Sheet

Dystonia – Information Sheet
Written by Dr Helen Hanson, Movement Disorders Unit, King's College Hospital, London and Dr K
Ray Chaudhuri, Movement Disorders Unit, King's College Hospital, London
What is dystonia?
Dystonia is a syndrome of spasms and sustained contractions of the muscles. These
muscle movements are not under voluntary control and they result in repetitive
abnormal movements of parts of the body or persistently abnormal postures.
Dystonia can affect virtually any single part of the body or several different areas at
once.
What are the risks?
It is estimated that there are more than 38,000 people in the UK affected with
dystonia. It can be very difficult to diagnose and many doctors will never have seen
someone with it before. Therefore, the actual number of people with dystonia may be
much higher than estimated.
Dystonia affects both men and women. It can affect all age groups but the most
common age of onset is between 40 and 60.
It can also develop in childhood but then the pattern is generally different from adultonset dystonia.
When dystonia starts in childhood it usually begins in the leg or foot and commonly
spreads to involve the entire body. If dystonia begins in adult life it tends to be more
localised, usually affecting one part of the body, such as the neck or hand.
What are the causes of dystonia?
Dystonia is a movement disorder. Although the causes of dystonia are not fully
known it is currently thought that the condition results from a malfunction in a part of
the brain called the basal ganglia.
The basal ganglia are structures situated deep in the brain. They help to regulate
voluntary and involuntary movement by controlling muscle contractions in the body.
The problem may mainly lie in an area of the basal ganglia called the globus pallidus.
If this area of the brain is not functioning correctly then the control of another
structure in the brain called the thalamus is affected.
The thalamus controls the planning and execution of movement and sends nerves to
muscles via the spinal cord. The end result is that muscle co-ordination is not
regulated properly. The wrong muscles will contract on movement or all muscles will
contract unnecessarily causing abnormal movement and posture.
Muscles positioned around joints usually work in pairs opposite each other, eg the
biceps and triceps muscles of the upper arm bend or straighten the elbow respectively.
Usually if one muscle of a pair is contracted the other is relaxed. However, in
dystonia both muscles in the pair contract at the same time leading to the abnormal
movement or posture.
It is thought that in some cases there may be a chemical imbalance or 'wiring fault' in
the basal ganglia. Chemical transmitters, such as dopamine, convey messages from
one nerve cell to another within the basal ganglia. If this balance is upset then
incorrect signals will be sent out resulting in loss of regulation of co-ordinated
movements.
Supporting this theory is the fact that people with dystonia do not show structural
abnormalities of the brain. The 'wiring fault' theory is therefore more likely because it
works at a much smaller scale.
The fault in the basal ganglia may be caused by an inherited factor or be secondary to
another problem such as drugs or toxins, or a separate neurological disease. Recently
scanning studies using positron emission tomography (PET) in patients with cervical
(neck muscle) dystonia have revealed reduced basal ganglia density of an important
dopamine receptor (the D2 receptor).
How is dystonia classified?
Dystonia can be classified according to the age of onset (childhood, adolescent or
adult) by body distribution (focal, multifocal, segmental, generalised or hemidystonia)
or by the cause (primary, secondary, 'dystonia plus' syndromes or combinations of
hereditary and degenerative causes).
Focal dystonias affect one part of the body such as eyes, neck, arm or vocal cords and
are the most common type.
Multifocal dystonias affect several different unrelated body parts, such as eyes, hands
and vocal cords. Segmental dystonias involve two or more adjacent body parts, such
as the arm and neck.
Hemidystonias affect only one side of the body, and commonly result from a stroke.
Generalised dystonia is more severe and can affect the entire body.
Primary dystonia refers to the situation where dystonia is the only sign and there is no
identifiable cause or structural abnormality in the central nervous system.
Secondary dystonia implies there is a clear cause, such as a change in the structure of
the brain, an environmental cause, as part of an inherited or acquired neurological
disease or due to drugs or toxins.
'Dystonia-plus' syndromes occur when dystonia is combined with other pathological
changes. It includes dopa-responsive dystonia and myoclonic dystonia.
What are the different types of dystonia?
Primary dystonias
Generalised dystonia
This is also known as primary torsion dystonia or dystonia musculorum deformans.
The usual age of onset is between 5 and 16 years. Parents or teachers may notice an
abnormal turning in of the foot, an awkward gait or contractions of many different
muscle groups.
The involuntary dystonic movements may progress quickly to involve all the limbs
and torso, but the rate of progression usually slows after adolescence.
A genetic basis for generalised dystonia has now been confirmed.
Focal dystonias
Spasmodic torticollis
Torticollis, commonly called wry neck, is the condition of spasm affecting the
muscles of the neck, causing the head to assume unnatural postures or turn
uncontrollably.
Spasmodic torticollis, also known as cervical dystonia, is the most common of the
focal dystonias. There are thought to be 10,000 people in the UK suffering from this
condition.. The average age of onset is in the early 40s and more women are affected
than men.
The head may tilt (laterocollis) or twist to one side (rotational torticollis), forward
(anterocollis) or backward (retrocollis). The movements may be sustained or jerky
(myoclonic torticollis). Muscle spasms or pinching nerves in the neck can be very
painful. The neck may eventually be held permanently in one position.
Torticollis usually develops gradually. At first, the patient may notice that the head
turns during everyday activities. In about a quarter of patients the hand may also
develop some tremor, especially if trying to correct the involuntary movement. The
tremor is common but not usually disabling and is referred to as an enhanced
physiologic tremor.
The severity of torticollis can vary and may be worse if the patient is under stress.
Occasionally drinking alcohol can improve the torticollis.
Some sufferers have a history of head or neck injury, but as yet there is no evidence to
support the theory that torticollis is directly related to trauma.
Most patients find the condition deteriorates over the first five years, but their
symptoms then stabilise. One third of patients progress to a segmental dystonia,
usually involving the arm. The symptoms of about 10 per cent may stop
spontaneously, but then later recur.
Patients with torticollis often find that their daily lives are affected. Head turning can
prevent a proper view of the road when driving, it may become difficult to eat, brush
teeth or apply makeup. Many sufferers find embarrassment and anxiety the major
handicap.
Blepharospasm
Blepharospasm means the involuntary contraction of the eyelids, leading to
uncontrollable blinking and closure of the eyelids.
It affects more women than men and in the UK and it is the second most common
focal dystonia with approximately 4000 people affected. In very extreme cases,
sufferers are unable to prevent their eyes from clamping shut so that despite normal
vision they are functionally blind.
Muscles in the face can also become affected causing facial distortions and grimacing
when the patient attempts to open her eyes.
Blepharospasm usually develops gradually. The first sign a sufferer may notice is eye
irritation and discomfort, light sensitivity and increased blinking. They may find that
the condition worsens when they are tired, under stress or reading. Bright flickering
lights, smoke or wind can all irritate the condition making symptoms worse.
Hemifacial spasm
Hemifacial spasm causes muscles on only one side of the face to contract. It affects
both men and women and usually develops in middle age. More than 4000 people in
the UK are thought to be affected.
Hemifacial spasm develops gradually. Initially the muscles surrounding the eye may
be affected by muscle spasms, which continue to spread and affect other muscles on
the same side of the face, especially the jaw and mouth. Some patients may
experience a clicking sound in the ear on the affected side each time a muscle
contracts.
For unknown reasons hemifacial spasm tends to affect the left side of the face more
often than the right.
The cause of the spasm may be related to the irritation of the nerve that controls the
muscles of facial expression called the facial nerve. This may be due to an abnormally
placed blood vessel at the back of the brain, near where the facial nerve arises. So
hemifacial spasm may not be truly a dystonia.
Oromandibular dystonia
In this form of dystonia the jaw muscles, lips and tongue are affected causing the jaw
to be held open, clamped shut or forced to deviate to one side.
The tongue may be pulled forward, upward, backward or downward.
Sufferers experience problems eating swallowing or speaking. Occasionally, this may
be drug induced. Ulceration of the tongue may also occur due to a continuation of dry
mouth and tongue twisting.
Orofacial-buccal dystonia
This dystonia is also known as Meiges or Brueghels syndrome. It is a combination of
blepharospasm and oromandibular dystonia.
Spasmodic dysphonia
Spasmodic dysphonia (difficulty in voice production) is slightly more common in
women than in men and occurs in middle age. The muscles affected are those
controlling the vocal cords. Sufferers find that their voice sounds strained and
strangled, that it takes a lot of effort to speak and that their voice comes out as
tremulous, weak or a breathless whisper.
There are basically two types of spasmodic dysphonia. In the adductor type, speaking
causes involuntary excessive muscle contraction of the muscles that bring the vocal
cords together. This causes a strained, strangled, choked voice quality, often with
abrupt initiation and termination of voicing, resulting in a broken speech pattern. The
patient may sound hoarse, breathless, anxious or groaning.
In the abductor type, there is an overcontraction of the muscles that separate the vocal
cords, resulting in a choppy and breathy whispering voice pattern.
Spasmodic dysphonia may follow an infection of the respiratory tract, injury to the
larynx or a period of excess voice use.
Most patients find that they are able to use their voices normally in some situations.
Patients with the adductor type may be able to laugh, whisper or sing normally.
Improved speech is noted during emotional or physiological states for example joy,
anger or following yawning. Shouting or stress usually makes the condition worse.
Writer's cramp
In this type of dystonia the muscles of the hand and forearm are affected. Contraction
or extension of the hand and finger muscles prevents activity or causes an exaggerated
posture.
The patient complains of tension and discomfort. They might start to grip the pen too
tightly and the script becomes slow and untidy. After a few words the patient is forced
to stop and rest. The contraction disappears on stopping writing.
Occasionally the hand dystonia may also be associated with a tremor known as
dystonic tremor. Sometimes a primary writing tremor may be mistaken as writing
cramp.
Patients often employ trick manoeuvres to overcome the cramp. Some support their
writing hand with their opposite arm, use thick nibbed pens, alter their grip or hold the
pen in a closed fist. Unfortunately the cramp may arise in the other hand. Patients also
find that they begin to have problems with holding other utensils such as forks and
knives. Occasionally, the dystonia may be preceded by trauma to the limb.
There are other focal dystonias that are associated with a particular activity or
occupation. Examples include typist's cramp, pianist's cramp and golfer's cramp.
Adult-onset primary dystonia
This is a rare subtype of focal dystonia. The symptoms remain localised to the trunk
of the body, but may spread to involve the neck muscles. The dystonia does not
spread to the leg. Unlike other forms of focal dystonia it is more common in men than
women.
The twisting trunk movements have been likened to the Leaning Tower of Pisa, and
the term Pisa syndrome is occasionally applied to these dystonias.
'Dystonia-plus' syndromes
Dopamine, (often called 'dopa' which is in fact an intermediate chemical in
dopamine's production) is a chemical messenger widely used in the nervous system in
passing nerve impulses between nerve cells (neurotransmission). Dopa-responsive
dystonia is an important form that can be successfully treated with drugs such as
levodopa (eg Madopar, Sinemet). Typically it begins in childhood or adolescence and
leads to progressive difficulty in walking and in some cases spasticity (limb stiffness).
The symptoms may fluctuate during the day from relative mobility in the morning to
increasingly worse disability in the afternoon, evening and after exercise.
This is an important condition to recognise as treatment can result in dramatic
improvement in symptoms.
Myoclonic dystonia is a rare type combining dystonia and sudden muscular spasms
(myoclonus). The onset is in adolescence or early adult life. It mainly affects the arms
and body. These patients can be very sensitive to treatment with alcohol and a genetic
basis has been suggested.
Secondary dystonias
Secondary dystonias are often accompanied by other neurological problems. They
begin suddenly at rest and are associated with different hereditary and environmental
causes. Environmental causes include head trauma, stroke, a tumour, multiple
sclerosis, infections in the brain, injury to the spinal cord, or after chemotherapy,
drugs or toxins that affect the basal ganglia, thalamus or brain stem.
They may be associated with other hereditary neurological syndromes. Dystonia may
be the first sign in a patient with Huntington's disease, and is secondary to many other
neurological diseases. These include Parkinson's disease, Wilson's disease and Ataxia
telangiectasia. Examples of metabolic disorders causing secondary dystonia are
Lesch-Nehan syndrome, Niemann-Pick disease and Leigh's disease. All of these
causes are rare.
What drugs can cause dystonia?
Certain drugs have been implicated in causing dystonic reactions or dystonia. This
form of dystonia is referred to as secondary or drug induced dystonia. Some drugs
may not cause dystonia but may aggravate the pre-existing disorder. Patients should
avoid these drugs.
The list of drugs causing drug induced dystonic reactions is long but includes:
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antidepressants (amitriptyline, Amoxapine (Asendis), bupropion,
clomipramine (eg Anafranil), doxepin (eg Sinequan), fluoxetine (eg Prozac),
imipramine, nortriptyline (Allegron), trimipramine (Surmontil) and trazodone
(eg Molipaxin)).
anti-anxiety agents (alprazolam (Xanax), buspirone (eg Buspar))
anti-nausea/vomiting agents (metoclopramide (eg Maxolon), prochlorperazine
(eg Stemetil)).
neuroleptics (chlorpromazine (eg Largactil), clozapine (eg Clozaril),
fluphenazine (eg Moditen), haloperidol (eg Haldol), perphenazine (Fentazin),
promazine, trifluoperazine (eg Stelazine)). The dystonia associated with
neuroleptics is often called tardive dystonia.
other drugs include the psychiatric drug lithium (eg Priadel), midazolam used
in anaesthetics, phenytoin (eg Epanutin) an anticonvulsant, promethazine (eg
Phenergan) an anti-allergy drug and verapamil (eg Securon) an
antihypertensive.
In general, alcohol does not have an adverse effect on dystonia but it is rarely seen to
hasten it. Alcohol may also help dystonia, particularly forms of myoclonic dystonia.
People who chronically abuse alcohol can get a series of involuntary movements or
tremors not related to dystonia. Excess alcohol intake is not advised.
Is dystonia hereditary?
It has long been thought that there is a genetic or hereditary link to dystonia, as
relatives of patients suffering from dystonia often also have some kind of tremor or
dystonia and this link has now been identified in some types of dystonia.
Childhood dystonia (early-onset primary torsion dystonia or dystonia musculorum
deformans) is often inherited through one or more affected/mutated genes.
If a parent has this type of dystonia, there is a 50 per cent chance of passing the gene
to their children. The gene is on chromosome 9 and known as DYT1. (This mutation
has been observed mainly in Ashkenazi Jews.) However, even if the child inherits the
gene, they may not necessarily develop dystonia. This is known as reduced
penetrance. In the UK about 40 per cent of people with the affected gene develop
dystonia.
Research has shown that the gene DYT1 codes for a newly recognised protein called
Torsin A. Its function is unknown. However, large amounts are concentrated in an
area of the basal ganglia called the substantia nigra pars compacta, suggesting it has a
role in dopamine neurotransmission.
Late-onset primary torsion dystonia or focal dystonia is inherited in a more complex
manner than the early-onset dystonia. Genes known as DYT6 on chromosome 8 and
DYT7 on chromosome 18 may be involved. These genes also have reduced
penetrance so only about 12 per cent of people with the affected gene develop the
dystonia. DYT6 has been found in people whose neck or head muscles are affected
causing problems with neck, speech or facial muscles. DYT7 has been found in those
mainly affected with myoclonic torticollis.
Dopa-responsive dystonia also has a genetic basis. Many patients have a mutation in a
gene known as GCHI (GTP cyclohydroxylase) on chromosome 14. There is a 50 per
cent chance of parents passing on the gene, although with reduced penetrance.
However, it occurs more in women. Mutations in this gene cause abnormal production
of a chemical called tetrahydrobiopterin, needed to produce the neurotransmitter
dopamine. The drug levodopa is helpful in treating this form of dystonia as it
increases dopamine levels in the brain.
Myoclonic dystonia also has a genetic component. A mutation in a receptor for the
neurotransmitter dopamine has been found on chromosome 11 or 18.
What can my doctor do?
Your GP can refer you to a neurologist who specialises in movement disorders. The
neurologist may carry out further investigations. If you are suffering from
blepharospasm you may be referred to an ophthalmologist (eye specialist), or if you
are suffering from spasmodic dysphonia or oromandibular dystonia you may be
referred to an ear, nose and throat (ENT) specialist.
How is dystonia diagnosed?
There is no definitive test for dystonia. Diagnosis depends on the presence of
characteristic clinical symptoms and signs. The neurologist will perform a full
neurological examination and may also perform blood tests or a brain scan to rule out
an illness or injury that may be causing the dystonia. If no cause can be found the
dystonia is termed 'idiopathic'.
Is there a cure for dystonia?
Currently there is no cure for dystonia. However, there is a wide range of treatments
available. Although these cannot cure the dystonia they will improve the symptoms.
What treatment is available?
Finding the right medication may take some time and patience and trials of several
drugs. Not all people respond to the same drug or dosage. If the effects of one drug
wear off the replacement with another drug may help.
Anticholinergics
These drugs inhibit the action, release or production of the neurotransmitter or
chemical messenger acetylcholine, which plays an important part in the nervous
system. They produce a good response in some forms of focal dystonia. Around 40
per cent of patients with spasmodic torticollis are helped. The most commonly
prescribed drugs are trihexyphenidyl (eg Broflex), orphenadrine (eg Biorphen),
benzatropine (Cogentin), and procyclidine (eg Kemadrin). High doses of the drug
may be required.
Side effects include dry mouth, blurred vision, constipation, and difficulty in
urinating, memory impairment or confusion.
Benzodiazepines
These drugs regulate the neurotransmitter or chemical messenger GABA (gammaaminobutyric acid), which helps the brain maintain muscle control. They have
sedative, anti-anxiety and anticonvulsant properties as well as muscle relaxing
properties. Diazepam, clonazepam (Rivotril), lorazepam and alprazolam (Xanax) are
commonly used. The side effects depend on the dose but they may include
drowsiness, confusion and light-headedness. They are of used in focal dystonias and
help 20 per cent of patients with torticollis.
Baclofen (eg Lioresal) also works by regulating the neurotransmitter GABA. It
relaxes skeletal muscle and is particularly good at relaxing muscles which are in
spasm. It is relatively free of side effects at low doses. It has a good response in 10 per
cent of patients with torticollis.
Dopamine antagonists and agonists
Drugs such as levodopa (eg Madopar, Sinemet), bromocriptine (Parlodel) and
amantadine (eg Symmetrel) increase dopamine. Dopa-responsive dystonia is
remarkably responsive to small doses of levodopa.
Other drugs that reduce dopamine levels have also helped many people with dystonia.
These include phenothiazines, haloperidol (eg Haldol), pimozide (Orap), sulpiride (eg
Sulpitil) and tetrabenazine (Xenazine). However, they can cause side effects including
another type of abnormal movements called tardive dyskinesia, possibly with the
exception of tetrabenzine. Although these drugs have helped some patients others
have reportedly got worse, so they should be used with caution.
Carbamazepine (eg Tegretol) is an anticonvulsant and antiepileptic. It works in a
small number of patients and may be of use if other treatments have failed.
Cocktail therapy
In some patients, mostly with severe generalised dystonia, a combination of drugs
may be used. This may include benzhexol, baclofen and tetrabenazine, with or
without a benzodiazepine.
Occasionally injection of Baclofen by a pump into the fluid surrounding the brain and
spinal cord (cerebrospinal fluid) may be used for severe resistant dystonia or
aggravated dystonia known as 'dystonic storm'.
Botulinum toxin
Botulinum toxin injections have been used in the treatment of dystonia since the early
1980s. Botulinum toxin is produces by the bacterium Clostridium botulinum and is
the cause of botulism - an extremely serious form of food poisoning. It is a very
potent agent that paralyses muscle by blocking the impulses transmitted to them from
nerve endings. However, used under careful medical supervision it can reduce the
muscle contractions and the muscles become weaker. However, the nerve endings
grow back after about eight weeks, so the treatment needs to be repeated every two to
three months.
The treatment aims to improve muscle function and dexterity, alleviate pain and
correct posture. Focal and well-localised dystonias respond better than segmental or
generalised types.
Botulinum neurotoxin type A (Dysport or Botox) is injected into muscles that are
painful, tender or visibly contracting. It is also injected into muscles that contribute to
the abnormal movement. In very large quantities botulinum can cause fatal paralysis
of the respiratory muscles. However, in this treatment it is diluted and injected into
specific muscles in very small quantities. It does not come into contact with vital
organs such as the liver, kidney or heart. The injections are slightly uncomfortable
and painful for several days, but most people find that the symptom relief outweighs
the pain.
There are no permanent or persistent side effects. Injections around the neck may
produce difficulty in swallowing termed dysphagia, weakness of the neck muscles
leading to the head dropping forward and rarely, flu-like illness for a few days.
Treating spasmodic dysphonia with injections into the vocal cord muscles may cause
temporary softness and breathiness of speech or transient difficulty in swallowing.
Injections around the eye may produce drooping of the eyelid or weakness of the eye
muscles causing double vision or eye irritation. The side effects usually disappear in a
week or so and are unpredictable.
This treatment has been shown to significantly improve dystonia in 75 per cent of
patients with spasmodic torticollis. It is also used for the treatment of blepharospasm,
writer's cramp, and spasmodic dysphonia, hemifacial spasm and oromandibular
dystonia. The rate of success in writer's cramp is less that that of the other focal
dystonias with an improvement in about 60 per cent of patients. It is not used in
tongue dystonia, as there is an unacceptable rate of adverse affects including problems
with swallowing and speaking.
Resistance to the toxin after repeated treatment is rare but about 10 per cent of people
will develop antibodies to toxin A (BTA-AB), so treatment becomes ineffective. In
some patients the antibodies drop with time, whereas in some people they persist for
many years. Removal of these antibodies may be attempted by plasmapheresis
(removal of plasma from the blood).
Botulinum toxin type B, C and F are currently under development for those patients
who develop antibodies or fail to respond to Type A.
How successful is surgery?
Surgery is reserved for only the most severe cases that do not respond to medication.
Surgery for dystonia is destructive. The aim is to interrupt the pathways that maintain
the abnormal muscle movements. It may involve cutting nerves and muscles or the
careful placement of a lesion in the basal ganglia of the brain to reduce movement.
The operation to damage the area of the basal ganglia called the globus pallidus is
called a pallidotomy.
Pallidotomy can be unilateral or bilateral. Firstly an MRI scan is performed to study
the anatomy of the brain and basal ganglia. A stereotactic frame, which is a metal jig,
is then secured to the patient's head after giving them a local anaesthetic. The frame
maps out the areas of the brain for surgery. Under local anaesthetic, a small hole is
made in the skull and a probe is inserted. The probe is used to make a lesion in the
globus pallidus by applying heat of about 60°C-80°C. The procedure is considered to
be safe and have minimal adverse affects.
Studies have shown that most patients do experience a marked improvement in their
dystonic movements, although some benefit to a lesser extent.
Another procedure called deep brain stimulation has also been developed to treat
dystonia. It is similar to pallidotomy but reversible as the globus pallidus is stimulated
rather than destroyed. One study found it resulted in a 65 per cent improvement,
which was virtually immediate. The procedure has been shown to be safe with
minimal adverse effects.
Advanced generalised dystonias have been helped, if only temporarily, by surgical
destruction of parts of the thalamus. The thalamus is a structure deep in the brain that
helps to control movement. This operation is called a thalamotomy. It poses a risk of
causing speech disturbance as the thalamus lies near the brain structures that help to
control speech.
Other operations that can be performed are ones in which the nerves directly to the
contracting muscles can be severed. Such procedures are called denervation
operations.
Another operation is called microvascular decompression. The theory behind this
surgery is that arteries may compress selected nerves and consequently cause dystonic
movements. This is a common occurrence in hemifacial spasm where the facial nerve
that supplies all the muscles of the face may be compressed by an artery. In the
microvascular decompression operation a monitoring procedure ensures that all the
blood vessels causing the problem are removed. Abnormal electrical signals from the
facial nerve are recorded during the operation, when the last blood vessel is removed
the abnormal response disappears. This intra-operative monitoring probably explains
why the procedure has a greater than 90 per cent success rate.
Partial sectioning (cutting) and removal (myectomy) of muscles in the neck may also
treat cervical (neck) dystonia. Some patients who did not respond to other treatment
have undergone sectioning of the trapezius muscle, which is attached between the
spine and the shoulder blade. Other patients with congenital muscular torticollis have
undergone surgical release of contractive muscle bands or lengthening of the
sternocleidomastoid muscle in the neck – the broad muscle that connects the skull
behind the ear with the inner end of the collar bone. These operations have been
shown to have good long-term benefits.
Other treatment
Physical therapies such as physiotherapy with ice, heat or ultrasound, speech therapy
for spasmodic dysphonia, acupuncture, osteopathy or chiropractic techniques help
some patients. However, treatments involving manipulation of the neck are not
recommended for spasmodic torticollis. Relaxation therapies such as hypnosis,
behaviour therapy, biofeedback and meditation may also help.
Good advice
A positive attitude is vital. Dystonia is not life-threatening but can be disabling.
Dystonia can have a profound effect both emotionally and functionally. Sufferers
should try to continue to lead as normal a life as possible. Counselling and family and
social support is helpful.
Many patients with focal dystonias such as torticollis can control the abnormal
posture for a short time by using sensory tricks or 'gestes antagonistiques'. The most
common geste is to place a finger or hand against the lower face on the same or
opposite side to the direction of movement. Other gestes include sucking a pen or
necklace or pulling on the end of the nose or an earlobe. Ambient sound has also been
found to relieve dystonia.
What is the likely outcome?
Focal dystonias such as torticollis and blepharospasm may worsen over several years
before stabilising. They may improve or disappear for no apparent reason. The
likelihood of this has been estimated from anything from 1 in 10 to 1 in 20, but there
is no way to predict whether it will happen. In some cases the dystonia will return
after a period of remission, but other patients can remain symptom free for the rest of
their lives.
Focal dystonias are unlikely to spread to become generalised dystonia. However,
some focal dystonias may be associated with each other.
If the dystonia develops in childhood then it usually spreads to other parts of the body
and becomes generalised. This type of dystonia is much more disabling than the adult
focal type.
Support and information
The Dystonia Society, First Floor Camelford House, 89 Albert Embankment, London
SE1 7TP. Helpline: 0845 458 6322, Mon-Fri 10am-4pm. Website:
www.dystonia.org.uk.
The Dystonia Medical Research Foundation (Canada). Website: www.dystoniafoundation.org.
Last updated 01.08.2005