Mental imagery and dystonia

Mental imagery is already used in high
level sports competition, and athletes are trained to rehearse their race in
their mind, to increase their performance.

Could
dystonic patients benefit from mental imagery focusing on their motor
difficulties
? There are scientific facts, and anecdotal
reports from dystonic patients to suggest that it may be very helpful on the
long term.

Real and mentally stimulated movements rely
upon largely overlapping networks. In another words, writing or imagining
writing involved common networks in our brain. Studies have shown that the
mental imagery of a movement in patients with dystonia follows the same
difficulties than the execution of the dystonic movement.

A study by Fioro, 2006 has looked at the mental rotation of body
part (hands, feet) in which the subjects imagine moving their body part, from
their actual posture into that seen on a picture to recognise which side is
belonged in patients with writer’s cramp. Writer’s cramp patients are slower than controls in mentally rotating
hands but not feet; it suggests that the mental rotation of body parts reflects
the anatomical constraints of real hand movements.

Could
the mind reshape the brain?

Neuroplasticity is a normal process by which the brain
develops new connections at different levels, following interaction with environment,
emotions, behavior. Dystonia is understood to be a condition characterized by
an excessive neuroplasticity of the brain leading to abnormal learning of motor
program. To be able to influence this brain plasticity It’s important to create an environment for
positive learning and recovery.  Mindfulness can be helpful in patients with focal
dystonia to minimise stress and increase well being before embarking for
retraining.

Retrain your brain, not your muscles!

The visual and mental
imagery training will encourage you to imagine successful, normal execution of tasks,
which the dystonia prevents you to do..

Remember the time when
you could perform the movement normally, and how easy it was to do it and the
pleasure, which come with it.

It will be best
performed after the Botox treatment has already controlled the dystonic spasm
in the affected body part and together with sensory retraining.

Visualisation exercises are not always easy
and you may benefit of the advice of professionals such as hypnotherapist or specialized physiotherapist

Try daily to visualize yourself free of the
dystonia, walking eye opened, turning your head freely looking around you,
writing a page with our favorite pen or chewing a delicious meal.

Also music and melody can help you to IMAGINE…

You may say I’m a dreamer, But I’m not the only one, I hope someday
you’ll join us, And the world will live as one  (John Lennon)

Speaking up about silent swallowing difficulties in dystonia

                     

Ms Lucy Hicklin ENT, Mr Paul King chief executive Dystonia society UK, Dr Marie-Helene Marion neurologist and Carol Harris speech therapist. London living with dystonia event, on swallowing and speech problems on the 8th november 2014, London.

Every day we swallow solid food like a piece of bread, water and our own saliva. This vital function occurs most of the time without us being aware of it, as a reflex. But in fact swallowing is a very fine and precise mechanism, which can be disrupted in a number of neurological conditions, the most common and dramatic one occurring after a stroke, but also in dystonia.

A few definitions:

1-    Doctors talks about swallowing difficulties as dysphagia, which is the difficulty moving food from mouth to stomach. 

2-    Swallowing difficulties can be severe leading to a misdirection of the food to the nose, or to the larynx (voice box) and the lungs, resulting in infection of the lungs called aspiration pneumonia (as the food has been sucked int the airways).

3-    Swallowing difficulties are silent when there is no manifestation which will make the person aware of the swallowing problem, such as cough, choking, needed to drink to push down the food.

4-    Swallowing difficulties are subclinical when the signs are so mild that they are overlooked such as cutting food into small pieces and taking a sip of fluid with each swallow.

 What are the essentials for good swallowing?

1-    Of course the machinery such as  the tongue, the mouth, the throat (the pharynx) the voice box (the larynx), the oesophagus (gullet) have to be healthy. Issues apart from neurological problems, such as a tumour, could obstruct the swallowing process.

2-    The nervous system has to be able to conduct in an orderly fashion the different sequences of the swallowing, like a conductor with his musicians; only one “ fausse note” can disturb the all process. It’s called neurogenic dysphagia.

What are the sequences of the swallowing?

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1-    The oral phase: the food is collected in the mouth as a soft mass of chewed food, called a bolus.

2-    The pharyngeal phase: the swallowing reflex is triggered by the bolus touching the back of the throat, which give a signal to the brain ( cortex and brainstem) to start the safe guard process: the airway needs to be protected by elevating and closing the larynx and by lifting the soft palate to close the back of the nose. The door to the oesophagus (the crico-pharyngeal sphincter) opens to let in the bolus which has been propelled downwards by the back of the tongue and the pharyngeal muscles. 

3-    The oesophageal phase. The bolus then passes though the gullet and into the stomach. This is under the control of the autonomic nervous system

How can swallowing difficulties be assessed?

1-    -During a consultation, a speech therapist will examine the tongue strength, gag reflex, voluntary cough, and speech and perform a trial of swallowing (water, puree) and/or a 3 ounces (90 ml) water test.

2-     FESS (fiberoptic endoscopic evaluation of swallowing). 

A fine “spaghetti “( fibre-optic) tube, with a camera at the end, connected to a screen, is introduced by the nose and passed gently down to the throat. With the camera in place the patient is asked to swallow a food of different consistencies.   The food is normally dyed blue or green to make it easy to see against the pink of the gullet.  The speech therapist performing the test can see where it is going, particularly if it is getting stuck or going down into the airway (aspiration) .

3-    Videofluoroscopy is video-radiography of the mouth, throat and oesophagus when the patient swallows a range of foods (biscuit, puree, yoghurt) and liquids that are coated in barium.  The barium shows up on the x-rays (radio-opaque). On the video the radiographer can follow the trajectory of what has been swallowed.

4-  Recording the swallowing process with electrodes in the muscles under the chin and movement sensors on the Adam’s apple can give a good indication of the timing of the different swallowing phases.  It’s called an electrophysiological assessment.

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Posterior aspect of the base of tongue and larynx , after removing the pharynx http://commons.wikimedia.org/wiki/File:Slide10vvv.JPG#mediaviewer/File:Slide10vvv.JPG

What about swallowing difficulties in dystonia, in untreated patients?

Very few studies have addressed this topic. Most have concentrated on the swallowing difficulties in dystonic patients following treatement with  Botulinum toxin injections.

I will present 5 studies of swallowing difficulties in patients with cervical and craniocervical dystonia, before treatment.

1-Riski et al (1990) studied 43 patients with cervical dystonia with videofluoroscopy and found that 51% of the patients had swallowing difficulties; in 16% the difficulties were subclinical (not known by the patient). The patients had mainly a delay in triggering the swallowing reflex and some residue in the vallecula (a groove, behind the base of tongue, usually cleared by the posterior movement of the base of tongue). 

2Comella et al (1992) studied 18 patients with cervical dystonia with videofluoroscopy before and after Botox injections; 11% patients had clinical symptoms of swallowing difficulties and 22% had radiologic signs of peristaltic abnormality.. after Botox (see below), the signs and symptoms of dysphagia in these patients did not change, but an additional 33% developed new dysphagic symptoms and 50% of the patients developed new peristaltic abnormalities by radiologic studies. 

3- Munchau et al (2000) studied 12 patients with cervical dystonia undergoing selective peripheral denervation with videofluoroscopy. 90% patients had swallowing abnormalities with 58% subclinical. The patients presented as in the Riski’s study, with delayed initiation of the swallowing reflex and abnormal tongue base posterior movement, in particular in patients with saggital shift of the head.

The subclinical dysphagia was mainly cutting food into small pieces and taking a sip of fluid with each swallow.  

4Ertekin et al (2002) studied 25 patients with cranio-cervical dystonia with electrophysiological assessment. . 36% had clinical dysphagia and 72 % had abnormal swallowing on electrophysiological recording with again delayed swallowing reflex ; also the sphincter ( the crico-pharyngeal muscle) which opens the oesophagus was hyperactive, instead of being relaxed during swallowing to let the food going through.

Ertekin developed the concept of the dysphagia limit. In this simple test patients are asked to swallow volumes of water ranging from 1ml to 20mls.  In patients with clinical swallowing problems only 1-10 mls can be swallowed in one go. In subclinical patients 10-15mls can be managed.  All normal subjects could swallow 20mls in 1 gulp.

5-Cersosimo et al (2005) studied 20 pts with Meige syndrome with videofluoroscopy. 90% patients had abnormal swallowing compared to 40% in a healthy population of same age. They found also residue in the vallecula as in Riski’s study

What about swallowing difficulties in dystonia in Botox dystonic patients?

 Botulinum toxin injections, by weakening adjacent muscles, have been responsible of transient dysphagia in dystonic patients.

The different types of dysphagia depending of the sites of the Botox injections have been detailed in a previous post.

Conclusion

Swallowing difficulties are often silent or subclinical in dystonic patients. Patients and doctors need to speak about them as these swallowing difficulties can be worsened by Botulinum toxin treatment administered for the treatment of the dystonia.

Maybe every injector needs to offer a drink to their dystonic patients before treating them! It may be well received…..

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 References: 

Swallowing function in patients with spasmodic torticollis.; Riski JE1, Horner JNashold BS JrNeurology. 1990 Sep;40(9):1443-5

Dysphagia after botulinum toxin injections for spasmodic torticollis: clinical and radiologic findings.; Comella CL1, Tanner CMDeFoor-Hill LSmith C.; Neurology. 1992 Jul;42(7):1307-10

Prospective study of swallowing function in patients with cervical dystonia undergoing selective peripheral denervation; A MunchauC GoodS McGowanN QuinnJ Palmer, and K Bhatia; J Neurol Neurosurg Psychiatry. Jul 2001; 71(1): 67–72.

Oropharyngeal swallowing in craniocervical dystonia, C Ertekin*I Aydogdu**Y SeçilN KiyliogluS TarlaciT Ozdemirkiran J Neurol Neurosurg Psychiatry 2002;73:406–411

Swallowing disorders in patients with blepharospasm.: Cersósimo MG1, Juri SSuárez de Chandler SClerici RMicheli FE. Medicina (B Aires). 2005;65(2):117-20

http://www.infodystonia.com/post/24333066534/swallowing-difficulties-after-botox-injections-what-to

 

 

 

Understanding dystonia and its treatment. Video on questions & answers

A good knowledge of the dystonia is important for a patient to become actor of its treatment. The Dystonia society UK asked me to answer to the most frequent questions coming from their members. Please follow this link if you want to watch this video posted on the Facebook pageof the Dystonia society.

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The questions were as follow:

What causes dystonia in adults? Can any vaccine, peripheral nerve damage, the menopause or the surgery has any affects? (Time 1.00)

Does dystonia spread? If so, are there any particular parts of the body it spreads to and from? (Time 6.20)

How long do the symptoms carry on getting worse? (Time 7.58)

I understand Botulinum toxin is a type of poison. Is it safe to use? (Time 13.35)

Can Botulinum toxin be used in pregnancy? (Time 13.39)

Is there a way to avoid side effects from Botulinum toxin such as pain at the injection sites, swallowing difficulties, blurred vision? (Time 15.21)

How can I manage pain in dystonia if it is not relieved by Botulinum toxin? Is it OK to take pain medications on long term basis? (Time 18.57)

Why is my Botulinum toxin not working? Might is this because I become immune to the toxin? (Time 20.41)

Is Artane (Trihexyphenidyl) helpful? Is there an age limit for taking it? (Time 31.54)

Can physiotherapy or mind retraining help? (Time 36.50)

Might diet or complementary therapy help to manage dystonia? (Time 39.09)

I hope it will contribute to a better understanding of dystonia by the patients themselves and help them to cope better in their daily life.

Dr MH Marion

Why Spasmodic dysphonia is often mistaken for a functional disorder?


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Spasmodic dysphonia, also called laryngeal dystonia, is a neurological disorder, responsible of a strangulated, strained voice and rarely of a whispering voice, which has a major negative influence at work and in personal life.

Patients with spasmodic dysphonia require to be distinguishing from patients with functional dysphonia and from patients with “muscle tension dysphonia” (MTD) which is also considered a functional disorder rather than a neurological disorder.

 

Many reasons can explain why spasmodic dysphonia , which is a dystonia of the larynx, is not well recognized by doctors.

 

1-Laryngeal dystonia is a rare condition (1/100 000), and like other focal dystonia, the diagnosis is clinical; if you have never heard somebody with spasmodic dysphonia speaking, it will be very difficult to recognize it.

 

2- It mainly occurs in women, who carry the stigma of being more vulnerable for functional disorders.

 

3- The onset can be sudden in 45% of the cases (Childs, 2011).  Sudden onset is also a characteristic of functional disorders.

 

4- When the onset is sudden, the majority of patients recognized triggering factors such as stress (42%), upper respiratory infection (33%), and pregnancy and parturition (10%).(Childs, 2011). Stress should be seen as a non-specific triggering factor making patients vulnerable to develop any medical conditions, and not specifically functional disorders.

 

5-A phobic component occurs very quickly after onset, as the patient avoid answering the phone, and speaking in public as it’s required such an effort to speak in these circumstances.

 

6-It’s a task specific condition as every focal dystonia,; spasmodic dysphonia occurs when speaking, and voice can be normal for singing or shouting. This task specific characteristic can be disconcerting for both the patient and the doctor.

At the difference, functional dysphonia is responsible of a permanent speech disturbance, with a whispering voice or a hoarse voice occurring when speaking, and impossibility of shouting or singing.

 

7- “Muscle tension dysphonia” (MDT) patients tend to exert too much effort on their vocal cords when speaking and can present with a strained voice, as spasmodic dysphonia.

But when examining the speech in details, three tasks: sustained vowel /a/, oral reading of a standard passage, and connected speech describing a standard picture have to be performed, as the spasmodic dysphonia could be more apparent in the connected speech situation, compared to MDT.

 

                                          image 

It’s very important to diagnose spasmodic dysphonia, as Botox injections into the vocal cords can alleviate the symptoms and restore confidence for effortless speaking.

At the London BTX centre, in Sloane Square, Dr Marie-Helene Marion offers multidisciplinary clinics with voice specialists for the diagnosis and  treatment of spasmodic dysphonia with Botox injections.

 

 

 

 

How does my jaw move?

The functional anatomy of the jaw is essential to understand dystonic movements of the jaw when a patient is eating or speaking. The chewing movements are extremely complex and I will detail only the main posture of the jaw, following dystonic spasms.

The jaw can move in a vertical plan, with opening or closing the mouth

The jaw can move in a saggital plan, the jaw going forward ( protrusion) or backward ( retrusion)

The jaw can move in a lateral plan, the jaw going side to side or going down and to one side, called deviation of the jaw.

The masticatory muscles, responsible of these movements are 4 pairs of muscles ( masseters, temporalis, median pterygoid and lateral pterygoid muscles) and the mouth floor muscles ( supra-hyoid muscles).

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A combination of these movements is usually involved in the dystonic spams of the jaw. The video recording of a patient when chewing can be very helpful for analysing in details the dystonic spasms.

Dr MH Marion at the London BTX centre, is specialised in the treatment of dystonia, and injects under electromyogram guidance the masticatory muscles involved in oro-mandibular dystonia.

My jaw is out of control when speaking or chewing: what does it mean?


                                     image

                                                    the Iron jaw in circus 


Eating is a simple pleasure of life and a necessity. Biting into a biscuit could require a tremendous effort when the movements of the jaw become out of control. The automatic movements of the jaw and the tongue, which allow us to eat or speak, can be disrupted by involuntary jaw spasms.


What are the causes of jaw spasms?

1-Idiopathic jaw dystonia is the most common cause of jaw spasms. The onset is between the age of 50 to 60 and it is more frequent in women. Dental works can trigger it. It’s called idiopathic dystonia as no underlying disease can be found. A genetic mutation has been identified in familial form of jaw dystonia ( DYT6).

2-Tardive jaw dystonia can follow a treatment with drugs used for the treatment of psychosis, called neuroleptics.

3- Hereditary disease affecting the brain is often the cause of jaw spasms occuring in young people, under the age of 20.

4- Hemi-masticatory spasms is usually a consequence of radiotherapy of the jaw area for cancer of the ENT sphere. In that case, the spasm is painful and affecting one side of the jaw with sudden, unexpected painful clenching of the jaw.

 

Are there different types of jaw dystonia?

1-The jaw spasms can be closing spasms with sudden clenching, responsible of tongue biting, teeth breaking and limitation to open the mouth wide

2-The jaw spasms can be opening spasms, responsible of difficulties to keep the mouth closed and to keep the food into the mouth. Often the tongue is involved and has a tendency to poke out the mouth.

3- The jaw spasms can also move involuntary the jaw side to side, or on one side only, or forward (protrusion) or backward (retrusion)

 

The movement involved in eating and speaking are incredibly complex and the dystonic spasms can be a combination of opening, deviation to one side and going backward or forward.

 

What are the characteristics of jaw dystonia?

 

The jaw spasm occurs in any attempts of eating and/or speaking, therefore the diagnosis requires looking at the patient performing these tasks. The doctors should have a box of biscuit available to examine their patients with dystonia!

The spasm are relieved by some tricks like keeping a sweet in the mouth or a chewing gum, sucking a matches or the temples of their spectacles.

 

 

How to treat jaw dystonia?

 

The most efficient treatment is the Botox injections of the masticatory muscles.

            -The muscles, which close the jaw, are the masseters, the temporalis and the median pterygoid muscles

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Masseters, closing jaw muscles                                


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Temporalis, closing jaw muscles

Figure adapted from Travell and Simons’               



-The muscles, which open the mouth, are the lateral pterygoid and the mouth floor muscles.

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Mouth floor muscles ( opening jaw muscles), also called supra-hyoid muscles

Figure adapted from Travell and Simons’

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Median ( closing jaw muscles) and lateral pterygoid (opening jaw muscles)

Figure adapted from Travell and Simons’

‘Some of these muscles are superficial and easy to inject ( masseters, temporalis,mouth floor muscles), some are deeper (median an lateral pterygoid muscles) and requires to be injected with electromyography guidance.

The difficult part is to analyse the abnormal movements and understand which muscles are involved in the dystonia. It can take many injections sessions, to get the spasms under control. The 2 limiting factors are the swallowing difficulties, due to the spread of the Botox , in particular when the tongue  muscles have to be injected.  Starting with small doses and increasing gradually the dosages is advisable.

The human masticatory muscles are very strong, in particular the muscles which closed the mouth; just think of the acrobats in a circus who get suspended by biting a mouthpiece. But at least injecting jaw muscles in human is possible if we compared them to the jaw clamping muscles of the crocodiles, which are extremely strong, and as hard as bone.

 

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 Strong closing jaw muscles of the crocodiles

Dr Marie-Helene Marion is a specialist in Botox treatment for jaw spasms, and in particular for jaw and tongue dystonia.

British Neurotoxin Network 2013 meeting, Keble college, Oxford

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 The 3rd British Neurotoxin Network annual meeting was hold in Oxford last week; 74 Botulinum toxin injectors gathered for all over UK in the superb location of Keble college. The program covered both practical and scientific aspects of botulinum toxin and dystonia.

 I gave a pre dinner talk on facial expression of emotions, looking on how the concepts have evolved from the 19th century with Charles Bells ‘s anatomical dissection of the facial muscles, to the 21st century with the influence of facial Botox injections on our expression of emotions.The collaboration of Duchenne de Boulogne with his neurophysiology study and Charles Darwin on expression of emotions in man and animals contributes to the present understanding of the universality of expressions and the recognition of basic emotions on a face, which was confirmed by Paul Ekman, an American psychologist.The Botox has been used as a research tool in the 21-st century to understand the relation between emotion and facial expression. Repressing the expression of emotions by too much cosmetic Botox or by neurological conditions such as facial palsy could disturb in return our ability to perceive emotions.

image      Duchenne de Boulogne, 1862

 imageCharles Darwin, 1872                        

             

The following morning was dedicated on reviewing the techniques and indications of the treatment of drooling by Ms Helen Witherow, maxillo-facial surgeon in London, the treatment of jaw dystonia by myself and the comparison of 2 techniques of injection: either electromyographic guided or ultra sound guided injection by Dr Sabine Klepsch , neurophysiologist in Bristol. Ms Catharina Pearce, medical student from Cardiff presented the results of a national survey on the use of Botulinum toxin in pregnant women.

In the afternoon the lectures were focusing on new concepts in dystonia and in particular the attempt of defining a new endophenotype (heritable clinical markers) for adult onset focal dystonia by Dr Sean O’riordan from Dublin and Dr Richard Grunewald from Sheffield.

 Outside the Botulinum toxin treatment, Mr Alex Green, neurosurgeon in Oxford, explored the effect of Deep Brain Surgery on dystonia and Mr Richmond Stace physiotherapist in London, the basis for retraining of cervical dystonia.

A video session, where colleagues were presenting video of dystonic patients with unexpected outcome, was animated and the opportunity again to share our individual approach to treatment.

The meeting was also a great opportunity for the secretary Mr Bells to update the UK maps of the service using botulinum toxin for treating neurological conditions. it’s accessible to anybody who is looking for a specialist centre to be treated for dystonia in particular.

 Dr Marie-Helene Marion, chair of the British Neurotoxin Network

 

My head shakes: is it dystonia?

 

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Keith Haring

Millions of people all over the world, uses her head and neck to carry heavy goods. The control of her head posture must be phenomenal and does not allow any slight involuntary movement of her head. Unfortunately, for some people the head starts shaking and even without relying on their head as a way of carrying, they found this condition very disabling.

 Head tremor described a movement of oscillation of the head, making the head going side to side, called no-no tremor or up and down, called yes-yes tremor. The tremor can be regular like a pendulum or irregular, with the head going further away from time to time.

Head tremor can be due to a condition called essential tremor (essential head tremor: EHT) or due to dystonia and in that case the tremor is labeled dystonic tremor (dystonic head tremor: DHT).

The clinical diagnosis of the different types of head tremor can be a dilemma when the head tremor is isolated, as neurologists don’t agree between themselves on the definition of dystonic tremor. Nevertheless, the distinction needs to be done, as the treatment and the prognosis may differ.

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Table1: Differential diagnosis between essential head tremor and dystonic head tremor. Dr Marie-Helene Marion, 2013, www.infodystonia.com

ESSENTIAL HEAD TREMOR

  1. Essential tremor (ET) is a clinical description of a familial regular tremor, which affects 50% of the siblings when one parent is affected.
  2. ET can affect hands, head, chin, tongue and voice.
  3. Women with ET have more chance to develop a head tremor.
  4. ET occurs on posture, so the essential head tremor (EHT) is present when sitting, walking and disappears when lying down.
  5. ET, mainly when the tremor is affecting the hands, has the particularity of being improved by drinking alcohol and responding to beta-blockers drugs such as Propanolol.
  6. EHT is not controlled by sensory trick such as touching the cheek or the chin with the hand.
  7. EHT is always associated with other tremor in the arms or tongue or voice.  A collaborative study between Turkey and USA looking at head tremor due ET (583 ET cases) has not found one isolated case of head tremor; all were associated with at least a mild hand tremor. 

DYSTONIC HEAD TREMOR, WITH FOCAL DYSTONIA.

  1. · Dystonic head tremor (DHT) is an irregular head tremor, which is easy to diagnose when it occurs in a patient with a obvious cervical dystonia (with abnormal head posture and muscle spasms), and considers as a tremulous cervical dystonia. DHT is one of the first symptoms of cervical dystonia in 30% of the cases and is reported in 30% to 60% of the established CD cases.
  2. · DHT can also be present in absence of neck dystonia, but associated with other focal dystonia, such as blepharospasm, oro-mandibular dystonia, voice dystonia or even a writer’s cramp. Therefore patients with head tremor need to be examine thoroughly when speaking, eating, writing…

 ISOLATED DYSTONIC HEAD TREMOR

DHT can be present but without any associated dystonia on examination; the diagnosis in that case is more difficult.

·      The family history of tremor or dystonia can be absent as the penetrance of the gene for focal dystonia is low (it means that the genetic abnormality can stay silent and is not always associated with dystonic symptoms and therefore can “skip a generation”).

·      The diagnosis of dystonic head tremor will be made clinically on the association of others signs.

1.    The head tremor is irregular on observation

2.    The head tremor is relieved by a sensory trick (similar to what describes for cervical dystonia) with fingers on the cheek or the chin.

3.    The head tremor persists when patient lying down.

4.    The head tremor is worst in certain position of the head (position dependant).

5.    The head tremor is associated with an irregular tremor of one hand, suggestive of a dystonic arm tremor as irregular and existing only in one hand. (at the difference of ET tremor which affects both hands).

6.    The head tremor is isolated, with no tremor in the hands or in the face or voice. (at the difference of EHT which is always associated with tremor in other part of the body).

CONCLUSION

Despite the fact that the patients are not always aware of their head tremor, in particular in case of EHT, head tremor can be very disabling socially.

Head tremor can benefit from therapeutic options such as drug treatment and Botox injections, which have to be offered depending of the type of tremor.

Further research is needed to characterize more precisely this movement disorder. May be it will come from unexpected study such as the episodic head tremor observed in Doberman Pinsher dog …

 Dr Marie-Helene Marion is a London neurologist, specialist in dystonia and its treatment with Botox.

Doberman Pinsher dog

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REFERENCES:

1-Can J Neurol Sci. 2000 May;27(2):137-42.

Head tremor in cervical dystonia. Pal PK, Samii A, Schulzer M, Mak E, Tsui JK

2-Arq Neuropsiquiatr. 2008 Dec;66(4):805-8.

Head tremor in patients with cervical dystonia: different outcome? Godeiro-Junior C, Felicio AC, Aguiar PC, Borges V, Silva SM, Ferraz HB

3-J Neurol Neurosurg Psychiatry. 2012 Feb;83(2):179-81. doi: 10.1136/jnnp-2011-300823. Epub 2011 Nov 5.

Supine head tremor: a clinical comparison of essential tremor and spasmodic torticollis patients.Agnew A, Frucht SJ, Louis ED

4-Mov Disord. 2000 Sep;15(5):960-4.

Quantification of sensory trick impact on tremor amplitude and frequency in 60 patients with head tremor.Masuhr F, Wissel J, Müller J, Scholz U, Poewe W

5-Mov Disord. 2009 Nov 15;24(15):2281-5. doi: 10.1002/mds.22777.

Isolated head tremor: part of the clinical spectrum of essential tremor? Data from population-based and clinic-based case samples.. Louis ED, Dogu O

 6-Mov Disord. 2011 Nov;26(13):2381-6. doi: 10.1002/mds.23936. Epub 2011 Sep 13.

An inherited episodic head tremor syndrome in Doberman pinscher dogs.Wolf M, Bruehschwein A, Sauter-Louis C, Sewell AC, Fischer A.

 

 

 

 

 

 

 

 

 

 

Living with dystonia: disability, distress and despair

Recently an English lady suffering with longstanding cervical dystonia decided to end her life, with the help of Dignitas in Switzerland.. This tragic event reminds us as doctors how difficult it can be to live with dystonia. I gave a lecture few months ago for the Dystonia Society in London about disability, distress and despair when living with dystonia. The patients afterwards told me how important is it to feel understood and recognized in their fight against this condition..

 –Disability can be overwhelming, as dystonia is brought up by action and interferes with daily life activities. Some patients are blind as they can’t open their eyes, some can’t write, others can’t speak, or can’t walk without fighting with their painful twisty neck.

-Distress at the time of the diagnosis can be profound. The diagnosis is always delayed; the presenting symptoms are often unusual and intermittent at the beginning, making it difficult to be recognized by non-specialists.

-Fall into despair is precipitated by the patient’s lack of knowledge about his condition.  They do not understand what’s happened to them and what to expect from the treatment. They need help to begin to accept that their life may be scheduled into 3 months interval of repeated Botox injection. The chronicity of the symptoms and the associated pain can also lead to a vicious circle of depression, chronic pain and worsening of dystonia. The depression needs to be treated to break this vicious circle.

 

I feel strongly that we can fight each of these dramatic consequences and improve the life of the dystonic patients.

-Education of the patients about dystonia ( Dystonia society,  Patient’s support groups, this blog) allows them to become the actor of their treatment and to work with neurologists  for a positive outcome.

-The therapeutic strategies (drugs, botox injection, physiotherapy, brain surgery) are available to alleviate the disability.

 –A multi disciplinary approach working with physiotherapists specialized in pain and dystonia, and a holistic approach of the patient  focusing on both motor and non-motor symptoms, can help patients to live with their dystonia.

The specialists in dystonia and its treatment are joining their efforts at every level: national ( British Neurotoxin network), European ( European Dystonia Federation) and world wide ( Dystonia Coalition). Research on dystonia is very active all over the world.

Both patients and doctors have to be convinced that there is light at the end of the tunnel and keep hoping for a better future.

 

 

Dr MH Marion is a neurologist in London, specialist in Botox treatment for dystonia: blepharospasm, cervical dystonia, jaw spasms, writer’s cramp and spasmodic dysphonia.