Care and mobility considerations
Focal dystonias
Blepharospasm
The vast majority of people with blepharospasm will control their condition with regular botulinum toxin injections. No care or mobility needs would be anticipated. Likely difficulties for this group include recurrent symptoms as their botulinum toxin wears off, these are unlikely to be as severe as prior to initial treatment but may cause difficulties driving.
For a small number of people who have not responded to botulinum toxin and in whom other treatments have been ineffective there may be disabling effects related to the effect of the condition on vision. There are likely to be difficulties with self care or mobility around the home. Cooking and travelling outside the home are likely to be difficult if vision is significantly impaired.
Oromandibular dystonia (cranial dystonia)
This condition affects speech and ability to eat by making these activities difficult and socially awkward. A minority of people will control their condition with regular botulinum toxin injections. No care or mobility needs would be anticipated. Likely difficulties for this group include recurrent symptoms as their botulinum toxin wears off; these are unlikely to be as severe as prior to initial treatment. If the condition cannot be controlled by treatment there will be no care or mobility needs. However the nature of the condition may lead to social isolation, depression or other mental health condition. In these cases assessment of needs should be made on the mental health condition.
Spasmodic dysphonia or laryngeal dystonia
There are typically no care or mobility needs associated with this condition, although it may have necessitated a change in employment if severe. Although the voice may be very quiet or sound strangled, it is still possible to communicate. However the nature of the condition may lead to social isolation, depression or other mental health condition. In these cases assessment of needs should be made on the mental health condition.
Spasmodic torticollis or cervical dystonia
The majority of people will control their condition with regular botulinum toxin injections. No care or mobility needs would be anticipated. Likely difficulties for this group include recurrent symptoms as their botulinum toxin wears off; these are unlikely to be as severe as prior to initial treatment. People with more disabling symptoms or those that do not respond to botulinum toxin injections are likely to try drug treatment. If this treatment also fails then surgery including selective peripheral denervation and deep brain stimulation may be tried. The nature of the condition may lead to social isolation, depression or other mental health condition. This is more likely if chronic pain is present. In these cases assessment of needs should be made on the mental health condition.
In a severe case there may be needs related to physical symptoms. Medical evidence of such disability must be provided. Examples include:
- Where posture is very contorted and there are spinal complications such as radiculopathies and accelerated Spondylosis.
- fixed postures (particularly if the head is fixed down onto the chest or fixed in extension (looking up above)
- the condition cannot be controlled by any treatment
Hemifacial spasm
The vast majority of people will control their condition with regular botulinum toxin injections. No care or mobility needs would be anticipated even if the condition is not controlled. Likely difficulties for this group include recurrent symptoms as their botulinum toxin wears off, these are unlikely to be as severe as prior to initial treatment but may cause difficulties driving. Spasms may interfere with speech but not prevent it. Recurrent botulinum toxin injections eventually may give rise to wasting of the muscles of the face making it look asymmetrical. The nature of the condition and its effect on appearance may lead to loss of employment, social isolation and depression – the effects of the condition on mental health may give rise to needs.
Writer’s cramp or hand dystonia
This condition has no effect on mobility. The dystonia may affect activities of self care; for example washing would be unaffected but there may be difficulty doing up buttons and tying laces in a very severe case especially where tremor is also a feature. Similarly the ability to prepare food may be compromised if the condition is severe -e.g. holding a knife to chop vegetables. In the majority of cases these activities not directly related to writing will not be or minimally be affected by the dystonia and there will be no care needs. Writer’s cramp can impinge significantly on occupations where writing is a major part of the work load.
The exception to this, in a mild condition, will be where self care requires a higher level of manual dexterity than is usual because of other medical problems or disabilities. Examples of this will include any type of stoma including a colostomy, urostomy, laryngectomy stoma etc where small dressings may need to be cut and manipulated into position. The type of activities affected are those which require two hands, one used to hold something steady and the other used to perform the activity. Indwelling catheters and attached night drainage bag for example require the use of both hands to screw small connections into place, these actions may be impossible with a dystonia of the forearm. Complex medication needs such as the following – indwelling catheters and lines, injections, the use of home nebuliser and oxygen equipment may be affected in a severe case.
Generalised dystonias
Idiopathic Torsion dystonia
Adults with this condition are likely to have developed generalised dystonia during childhood. The condition commonly affects the legs from the beginning and immediately affects walking ability. Abnormal spasmodic movements of the legs make taking steps very exhausting and difficult and remaining balanced with uncontrolled movements is also difficult. Within a few years walking more than half a mile may be impossible and gradually the distance walked independently reduces as spasms become more frequent and severe. Speed of walking is much reduced from onset. Severe disability is usual within about 10 years. Such people may be able to mobilise short distances and get into a wheelchair but will need a wheelchair when outside the house. Spasms of the upper limb and trunk may make self care very difficult if not impossible. For example lifting the arm to brush the hair may always result in abnormal movements and help may be required with washing and personal grooming for this reason. Help with personal care is less likely to be needed in people who have some response to drug treatment.
DBS surgery may be considered or used. This surgery may considerably improve movements and improve ability to walk and self care to the level where no help with care or mobility is required. DBS takes some time to improve function as adjustments to stimulation to improve function are made over time. Personal adjustment after a period of severe disability also takes time.
Rare dystonias
Myoclonus dystonia
There are typically no care or mobility needs associated with this condition
Tardive dystonia
In the majority of cases stopping the causative drug will reverse the condition and movement return to normal over several months. If the condition has been present for more than a year it is unlikely to dramatically improve. The disabling effects will depend on which part or parts of the body are affected and how effective treatment is at controlling or reducing the movements. For facial tics affecting the eyes and mouth only see blepharospasm and Oromandibular dystonia. In a severe case the trunk neck, arm and leg muscles are affected and the condition is more like a generalised dystonia, it may affect walking, balance or use of the upper limbs for self care or other activities. The condition is highly visible to others, the nature of the condition and its effect on appearance may lead to loss of employment, social isolation and depression – the effects of the condition on mental health may give rise to needs. If there is concurrent mental illnesses please refer to mental health guidance.
Tardive dyskinesia
The most effective treatment is to stop the causative drug as soon as possible. Symptoms will often disappear if this is done quickly enough. Abnormal movements may be present 24 hours a day, with constant jerking twitching and grimacing movements of the face in addition to fidgeting of the hands and upper limbs. The movements may disturb sleep. Communication is affected because the abnormal movements interfere with facial expression. The condition is highly visible to others, the nature of the condition and its effect on appearance may lead to loss of employment, social isolation and depression – the effects of the condition on mental health may give rise to needs. If there is concurrent mental illnesses please refer to mental health guidance.
Amended May 2009
