Category Archives: Dystonia

05Jan/14
Basal Ganglia | Dystonia

Dystonia — facing the world

Basal Ganglia | Dystonia

Basal ganglia | Dystonia

Dystonia — the term used to describe uncontrollable and sometimes painful muscle spasms caused by incorrect signals from the brain. It is estimated to affect at least 70,000 people in the UK; Dystonia Society — has varying impact upon the individual. By this I refer to the way in which it makes the person view themselves, their sense of self, and how the world sees them.

Our physical persona, the way we represent ourselves to the world via body language, posturing and movement to an extent defines us and how we feel. This is bi-directional as we will respond physically to how we are feeling and what we are thinking as much as noticing how our body feels to then ascribe an emotion; for example, butterflies in the stomach will typically trigger thoughts that we must be anxious.

Having spoken to many people with dystonia, this significant part of the experience is rarely addressed. It must be. On a practical level, understanding that movement is affected by a number of influences such as where we are, who we are with, how we are feeling, where we are and what we have been doing, helps to cope and deal with the unwanted motion on several different levels. Further, to know that the brain is constantly predicting what actions will be required next and planning how to use the body, provides insight into how certain involuntary movements can be initiated.

In dystonia, the way in which the brain perceives the body is altered as is the way movements are planned and executed. A loss of precision means that there is unwanted and uncontrolled actions in both a sensory and motor context. This is why training must be multisensory and embrace the sensorimotor system as a whole, from a planning stage through to actual movement.

How we face the world and whether we choose to face the world will be dependent upon how we feel. Being able to cope with this will impact upon the extent to which socialisation continues or is voluntarily curtailed. Cultivating skills in dealing with the thought processes and being able to intervene independently has a positive effect, reducing and minimising the impact. This is also a key skill to use in optimising the outcome of the training programme, by changing the way that the brain is functioning in a motor sense, so that the focus and quality is of a high level. Practice is key.

Focused attention training or mindfulness should form part of a comprehensive treatment programme. It can be thought of as setting the scene, creating a brain state of calm and readiness for the actions that are required to re-train precision of movement and body sense. It is a state that can be cultivated that means troubling thoughts do not get played through the body (tension, altered movement, involuntary movement).

There are many healthy, physical and mental benefits of mindful practice, tackling the so significant non-motor factors that are barely mentioned, yet play such a huge role in the individual’s suffering. We are understanding the brain changes associated with this practice as neuroscientists study the effects upon the wiring — a crude analogy of the brain’s function — and how this translates into a positive experience of life.

Stand alone, mindfulness practice can change our interface with the world. Combined with specific sensorimotor training and the creation of new habits, the potency is magnified. On a practical note, mindfulness can be used at all stages of dystonia, acute through chronic. The sensorimotor training often requires a degree of quiescence and hence will usually begin after botox treatment. The design of any treatment and training programme will depend very much on the individual need and circumstance.

As I have argued recently in writing and speaking at several conferences (Dystonia Society DayBritish Neurotoxin Network meeting 2013) , to address dystonia — and chronic pain — we must consider the biology of the condition but also the biology of the influencing factors that are such a significant part of the narrative.

Appointments — please call us on 07932 689081

 

 

05Dec/13
Dystonia Society

Dystonia Society meeting | Talk on sensorimotor training for cervical dystonia

Dystonia SocietyThe ‘Living with Dystonia’ Day, organised by the Dystonia Society, was held in London in November. This was an opportunity to talk to neck dystonia sufferers and their carers about the sensorimotor training programme that I am using in conjunction with botox treatment — see here.

We have an understanding of how the brain plans and then executes movement. This is based upon a blend of prediction, feedback from the body’s sensory systems and what has been learned (i.e. prior experience). Tests in the clinic can identify an altered sense of body, a common finding in both cases of chronic pain and dystonia, that impacts upon the precision with which we would normally move. It is an issue of ‘threat’ when the movement that actually occurs does not match with the expected pattern or blueprint that exists in the brain. Any sense of threat can be enough for protective measures to be taken by the brain, e.g./ pain, guarding. This is the neurobiology of movement, and involves many parts of the brain including the motor centres, the basal ganglia and the cerebellum.

A key point that I made during the talk, and one that I make to patients, is that not only do we need to think about how the neurobiology of movement or the pain that is manifesting, but we also must consider the array of influences upon this biology. Those that we know well include stress, anxiety, prior experience, beliefs, the environment, the context, who we are with and what we are thinking about. All of these affect movement that is an expression of our ‘self’ at any given moment — what we are doing, thinking and feeling.

Our posturing or body language represents an interface between our sense of self and the outside world. When our movement changes, so does our relationship with the World. Certainly in neck dystonia, almost all of those who come to see me will talk about how it makes them feel to experience involuntary movements whilst in public places. Many will avoid going out if possible.

The involuntary movements, the lack of control and body awareness are all manifest and emergent characteristics of ‘re-wiring’ in the brain; the dark side of neuroplasticity. To improve motor control in these cases, we need to start at the level of planning rather than practicing actual movements. However, it is not just about the movement but also the sensory aspects that inform the brain. Hence the training is ‘sensorimotor’ that develops a better body sense to then normalise how it moves. The two are so interlinked in my view, that they are in fact one and the same; an overall mechanism of expression or function.

There are specific training strategies used for improving sensorimotor action including motor imagery and tactile discrimination exercises. Concurrently we look at the individual’s lifestyle influences: stressors, thoughts that cause anxiety and other non-motor factors that impact upon the movements and muscle activity.

Tension in muscles that persists can cause pain as the blood flow changes, acids build up and nerve endings that sample the tissues become sensitised. The messages sent to the brain about the tension and chemicals can result in a pain experience and often does in neck dystonia. Imagine performing bicep curls all day, every day. It would begin to hurt. With increased muscle activity due to involuntary movements and imprecise control, this is exactly what is happening. Reducing muscle tension by developing better control of movement and using techniques that are known to promote restorative activity are effective ways of re-programming how the body is working. Mindfulness or focussed-attention training are the methods of choice.

It is a very exciting time as we better understand movement and pain from a neuroscience perspective. The research must continue as we continually seek to improve the way in which we treat dystonia. The approach, as I described, must be comprehensive in addressing the physical, cognitive and emotional dimensions of the problem. In doing so we are offering a route forward via sensorimotor training, best applied in conjunction with botox treatment that both changes the muscle activity and eases pain.

For further information or to book an appointment, please contact us on 07932 689081

 

 

07Oct/13
Keble coll

Tackling dystonia | British Neurotoxin Network Conference 2013 | Keble College, Oxford

Keble collIt was a pleasure to speak at the British Neurotoxin Network conference this week, a meeting for specialists in dystonia who use botulinum toxin as a form of treatment. Held at Keble College in Oxford, the surroundings were perfect for meeting, discussion and the sharing of ideas.

BNN Conference Programme here

Before dining in the magnificent hall, the audience was entertained by a talk from Dr. Marion on facial expressions, referring to Duchenne, Darwin and the work of Paul Ekman. Much of our communication relies on body language with facial expression revealing much about the emotional state. This is useful to communicate effectively, to demonstrate empathy and to determine threat—i.e. an angry face. Equally, a loss of facial expression due to cosmetic Botox treatment or facial paralysis affects one’s ability to show genuine emotion. A lesser known feature is that we can change our expression to alter our emotion. A simple technique to improve one’s mood is to grip a pencil between your teeth thereby forcing a smile. The feedback from the face to the brain persuades it it sense something good and hence our mood alters.

The focus of my talk was upon the reconceptualisation of pain, looking at whether this process can be considered as a way to progress the rehabilitation of dystonia. The slow move away from a biomedical model to the comprehensive biopsychosocial model has changed both the way we think about and tackle the problem of pain. Understanding that pain is multidimensional (physical, cognitive and emotional) means that there are a number of considerations that are unique to the patient. This makes it key to address the person as much as the condition.

In addition to the tissue based therapies that play role in the treatment of chronic pain, the modern brain based techniques are becoming increasingly recognised as part of a comprehensive programme. Discussing these therapies for pain in the light of what we know about the underlying mechanisms, it has been apparent that they could apply in dystonia and other movement disorders. The cortical reorganisation that we understand in both pain and dystonia is an important focus of a training programme. Graded motor imagery, tactile discrimination training and other brain targeted strategies not only seek to ‘re-organise’ but also to desensitise. Pain is all about a perceived threat by the brain, so any change or learning that reduces the threat can change pain and also movement.

With movement being an expression of who we are, how we are feeling, what we are doing and what we intend to do (we may not realise this fully), when we have difficulty because of pain or a lack of voluntary control, this impacts upon the way we feel. Our movement and posturing interface with the World, so reflect the situation that we are in as much as how we feel about that situation. The bidirectional nature of this interface offers different ways of changing our emotional state and retraining normal movement.

The science based talks focused upon genes (Dr Sean O’Riordan, Consultant Neurologist), cortical reorganisation, the effects of vibration (Dr Richard Grunewald) and deep brain stimulation (Mr Alex Green, Neurosurgeon), all of which are ‘neuroimmune’ lines of thought. Tying this basic science with what we can do therapeutically is a key way in which we can seek to move forward and cultivate new ideas. Clearly we need further research to look at all of these paradigms and develop our knowledge but we are in an excellent position to use some of the existing pain therapies that target the central nervous system to improve body sense and motor control via sensorimotor congruence.

Thanks to Dr Marion and Mondale Events for a great two days.

Here is some information on our treatment, training and coaching for dystonia

RS

29Sep/13
Dystonia

Chronic pain to #dystonia: how physiotherapy can target cortical reorganisation – speaking this week at BNN Conference

DystoniaI am delighted to be speaking at the British Neurotoxin Network conference this coming week, giving an opportunity to talk about how we can target the higher centres with wise action and therapy for dystonia. The more recent literature has identified functional changes in a widespread network (e.g./ cerebral cortex, basal ganglia, cerebellum), cortical reorganisation and the importance of non-motor factors. In essence, a biopsychosocial look at this hugely impacting condition.

With a background in pain neuroscience it is fundamental to consider the physical, cognitive and emotional dimensions of any condition. The integrated nature of these dimensions are often key points and areas to target with education, therapy and strategies that seek to create the conditions for change and development, i.e. the treatment and training.

Dystonia is not about the basal ganglia as much as pain is not about damaged tissue. It is about an individual with a conscious experience that has evolved as a consequence of the combination of genes and the life story so far (epigenetics). To seek to change the experience we must consider the biology of the condition such as changes in the neuronal activity, the way in which the person thinks about their problem and the emotional responses. In comprehensively tackling the individual’s situation we can make headway by tapping into our neuroplastic mechanisms that underpin learning and adaptation but for the better. This equally applies for both chronic pain and dystonia, the similarities of which I will be discussing and drawing upon to provoke thought and advancement of the treatment for the latter.

Dystonia Clinic in London

Next: Interesting matters arising from the BNN Conference

RS

16May/13
2nd International Congress on the Treatment of Dystonia

2nd Report from the 2nd International Congress on Treatment of Dystonia | Musician’s Cramp

Of the many interesting topics covered at the congress, the talk by Laurent Boullet stood out. He is a Belgian concert painist who experienced focal hand dystonia himself. Now Laurent works with muscians who also suffer focal hand dystonia, using a programme to retain normal control of movement. He follows the principles of rehabilitation, gradually building the challenge of movement and precision required for playing the piano.

A graph that Laurent displayed showed how there is extra and early activity in the muscular system, a feature that is most significant with dystonia – too much activity and at the wrong time. The training seeks to change this patterning and restore the more discrete control.

Laurent has noticed that children often display similar movement patterns to those who have focal hand dystonia. I found this point to be highly relevant as I use the analogy that when there is an issue with movement, we lose precision and perform actions much like a young child, grasping rather than the refined movements of an adult with skill (although we do not often think about our movements as ‘skill’, this is indeed what they must be for success). Of course, precision must be learned by children and re-learned by adults with dystonia.

Recognising that there are changes in the brain functioning that underpin dystonia, there was a focus upon targeting these adaptations. Of course, any learning is a brain-based activity, whether it be a language or a musical instrument.

Two further observations by Laurent were the fact that muscle fibre switching may take place (fast & slow twitch fibres) and that the overall patterning of movement changes including the postural muscles. In respect of the latter, he referred to the work of Hodges et al. (click here), ‘Experimental muscle pain changes feedforward postural responses of the trunk muscles’ that shows how the feedforward system changes postural activity, relating that to dystonia.

In summary, although the problem manifests about the hand we are really thinking about a whole body strategy to move the arm to a certain place where the hand can perform intricate and timed activity. The automatic nature of movement changes and becomes increasingly conscious and therefore where our attention lies is important. The classic example is the yips (a later blog on this fascinating area). In focal hand dystonia we must consider this overall patterning but also the cognitive and emotional dimensions, similar to pain, addressing how these aspects of being human affect movement.

For further information about our Dystonia Clinic in London please call us on 07932 689081 | Clinic locations

10May/13
Dystonia congress

Report from the 2nd International Congress on Treatment of Dystonia

The 2nd International Congress on Treatment of Dystonia

The programme has been packed with talks, discussions and workshops looking at the current state of the knowledge about treating dystonia. Some have looked at the condition from a movement perspective and others have highlighted what we know about the brain and how it changes in dystonia. There is good data upon how the brain has adapted including the basal ganglia, the cerebellum and sensorimotor areas. Similar to pain though, there is not any one region of the brain to consider but rather how the cortical networks change structurally and in terms of connectivity. Additionally, it is not clear whether the changes are causative or consequential. But, we do know that they are there is defective sensorimotor integration that manifests as issues with the body schema and control of movement.

Retraining normal movement was a focus, with a range of speakers talking about their methods. They adhere to the principles of rehabilitation by breaking movements down and gradually building up the challenge and function whether it be for writing, controlling posture and head movement, playing a musical instrument or in sport (the yips). All started with simple movements, or ‘actual movements’ as I term them, as opposed to working with the planning stages of movement.

Dystonia | Cervical Dystonia | Writer’s Cramp | Musician’s Cramp

Of course the way in which the brain plans movement affects the outcome and the final quality of the movement that is emergent. Similar to a pain state when sensitivity and protection are implemented at a planning stage, this requires consideration when designing a programme for dystonia. Where do we start? Actual or motor imagery based? The assessment will guide the baseline.

The focus of our treatment programme is retraining normal sensorimotor integration and hence reconstructing a sense of self and body awareness concurrent with motor strategies. This sits alongside strategies that target some of the influences upon dystonia. At the conference there was talk of the ‘non-motor’ factors such as beliefs, anxiety, depression, stress and self-efficacy. My view is that labelling motor and non-motor is acceptable for explanatory purposes but in practical terms are so interlinked that the focus needs to be more about the person and how the condition manifests and is narrated. For example, who we are with and how we are feeling will produce different types of movement, highly relevant to a movement disorder. We have to address these factors as part of a comprehensive treatment and training programme for dystonia. 

The training programmes are often implemented as part of a combined approach with Botulinum toxin. A significant part of the Congress programme is dedicated to the use of the toxin, describing its potency and specificity for dystonia but also for pain. Working out the lowest dose for the greatest effect is the challenge facing the clinician injecting. For more on the use of Botulinum, see the website of Dr Marie-Helene Marion who is a very experienced neurology consultant specialising in movement disorders.

For more details on our treatment programme for dystonia, click here or call us on 07932 689081

 

 

11Jul/12

Cervical Dystonia | What can we do?

I see a number of cases of cervical dystonia (spasmodic torticollis) that features awkward posturing and movement of the head and neck. This can be painful and have consequences for normal activities. We rely upon being able to orientate ourselves to our environment by controlling our head and gaze direction and then responding appropriately.

Primary dystonia has no neurological or metabolic cause whereas secondary dystonia is attributable to outside factors such as physical trauma, exposure to certain medications and other neurological or metabolic diseases.

Here is a fact sheet from the National Institute of Neurological Disorders & Stroke

Common treatment of cervical dystonia includes botulinum toxin injections and physiotherapy.

Modern physiotherapy for cervical dystonia at the Specialist Pain Physio Clinics

In addition to the manual techniques that are used to help ease tension and the soreness associated with spasm and tightness in the muscles, we use strategies that target the motor centres in the brain where the signals are coming from. In other words, as well as treating the symptoms, we are focusing upon the mechanisms and causes of the muscles going into spasm. The Graded Motor Imagery programme provides a way of aiming to retrain movement by targeting the adaptations that have occured in the motor system. Initially this programme was devised for complex regional pain syndrome, but since then the training has been found to help those with a range of painful problems with associated movement issues.

Typically a treatment programme includes themes that aim to develop a deep understanding of the problem(s), nourish and mobilise the body tissues, improve motor control, body sense and awareness, manage posture, increase exercise an activity tolerance and ultimately improve quality of life. We call the approach biobehavioural because it is a comprehensive way of tackling the issues and influencing factors that are unique to the individual, addressing the physical signs and symptoms as much as the underpinning beliefs and lifestyle factors that impact.

Call for appointments: 07518 445493

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Dr Marie-Helene Marion, a consultant neurologist specialising in the treatment of dystonia and movement disorders has a comprehensive blog here

Recent research papers

Behav Neurol. 2012 May 24.

Cervical dystonia: From pathophysiology to pharmacotherapy.

Patel S, Martino D.

Abstract

Background: Dystonia is a chronic disorder characterised by an aberration in the control of movement. Sustained co-contraction of opposing agonist and antagonist muscles can cause repetitive and twisting movements, or abnormal postures. Cervical dystonia (CD), often referred to as spasmodic torticollis, is a type of focal dystonia involving the muscles of the neck and sometimes the shoulders. Methods: This systematic review collates the available evidence regarding the safety and efficacy of a range of treatments for CD, focusing on their effectiveness as shown by double-blinded, randomised controlled trials. Results: Our review suggests that botulinum toxin type A (BTA), botulinum toxin type B (BTB) and trihexyphenidyl are safe and efficacious treatments for CD. Evidence shows that botulinum toxin therapies are more reliable for symptomatic relief and have fewer adverse effects than trihexyphenidyl. When comparing BTA to BTB, both are found to have similar clinical benefits, with BTA possibly having a longer duration of action and a marginally better side effect profile. BTB is also safe and probably just as efficacious a treatment in those patients who are unresponsive or have become resistant to BTA.

Discussion: The current evidence shows that the pharmacological management of CD relies on BTA and BTB, two agents with established efficacy and tolerability profiles.

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Lancet Neurol. 2002 Sep;1(5):316-25.

Classification and genetics of dystonia.

de Carvalho Aguiar PM, Ozelius LJ.

Abstract

Dystonia is a syndrome characterised by sustained muscle contractions, producing twisting, repetitive, and patterned movements, or abnormal postures. The dystonic syndromes include a large group of diseases that have been classified into various aetiological categories, such as primary, dystonia-plus, heredodegenerative, and secondary. The diverse clinical features of these disorders are reflected in the traditional clinical classification based on age at onset, distribution of symptoms, and site of onset. However, with an increased awareness of the molecular and environmental causes, the classification schemes have changed to reflect different genetic forms of dystonia. To date, at least 13 dystonic syndromes have been distinguished on a genetic basis and their loci are referred to as DYT1 to DYT13. This review focuses on the molecular and phenotypic features of the hereditary dystonias, with emphasis on recent advances.

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Mov Disord. 2002;17 Suppl 3:S49-62.

Pathophysiology of dystonia: a neuronal model.

Vitek JL.

Abstract

Dystonia has commonly been thought to represent a disorder of basal ganglia function. Although long considered a hyperkinetic movement disorder, the evidence to support such a classification was based on the presence of excessive involuntary movement, not on physiological data. Only recently, with the return of surgical procedures using microelectrode guidance for the treatment of dystonia, has electrophysiological data demonstrated an alteration in mean discharge rate, somatosensory responsiveness and the pattern of neuronal activity in the basal ganglia thalamocortical motor circuit. Previous models of dystonia suggested that reduced mean discharge rates in the globus pallidus internus (GPi) led to unopposed increases in activity in the thalamocortical circuit that precipitated the development of involuntary movement associated with dystonia. This model has subsequently been modified given the clear improvement in dystonic symptoms following lesions in the GPi, a procedure that is associated with a further reduction in pallidal output. The improvement in dystonia following pallidal lesions is difficult to reconcile with the “rate” hypothesis for hypokinetic and hyperkinetic movement disorders and has led to the development of alternative models that, in addition to rate, incorporate changes in pattern, somatosensory responsiveness and degree of synchronization of neuronal activity. Present models of dystonia, however, must not only take these changes into account but must reconcile these changes with the reported changes in cortical excitability reported with transcranial magnetic stimulation, the changes in metabolic activity in cortical and subcortical structures documented by positron emission tomography (PET), and the alterations in spinal and brainstem reflexes. A model incorporating these changes together with the reported changes in neuronal activity in the basal ganglia and thalamus is presented.