Author: Dr. Marie-Hélène Marion

Treatment of spasmodic torticollis by a psycho-motor retraining: A method developed 100 years ago by Henry Meige

Dr. Marie-Hélène MarionLeave a comment

I was invited last week to lecture at the University of Liege ( Belgium ) about the understanding of dystonia by Henry Meige, a French neurologist . I went back to his original publications and found that he advocated in 1907 a psycho-motor retraining (“discipline psycho-motrice”) for the treatment of spasmodic torticollis…

The Magic Mirror, Magritte, 1929

 

Treatment of spasmodic torticollis by a psycho-motor retraining:  A method developed 100 years ago by Henry Meige

I was invited last week by Professor Gustave Moonen to lecture at the University of Liege (Belgium) about the understanding of dystonia by Henry Meige, a French neurologist.

I went back to his original publications and found that he advocated in 1907 a psycho-motor retraining (“discipline psycho-motrice”) for the treatment of spasmodic torticollis. It was at the time when there was no routine use of anticholinergics and Botox injection for cervical dystonia was not even a blip on the horizon. I could not resist to give you the translation of this therapeutic approach that I found quite inspiring…

1-The patient becomes actor of his treatment: “I don’t say the patient is cured, but the patient has cured himself”. This treatment is based on regular immobilizations and movements in front of a mirror; the patient has to be supported by his family and his doctor, as these daily exercises require a lot of effort and determination from the patient. “The goal of the treatment is to correct the abnormal postures, the put at rest the hyperactive muscles and to learn the control of the motor acts.”

2-The patient needs to have a regular life, going to bed at regular times. The patient is told that the course of the disease will be capricious, that he will have to perseverate and that the exercises will be eventually beneficial.

3-The patient had to exercise in front of a mirror 3 times a day; the patient is sitting, back non supported and hands flat on a table. The mirror is divided by 1 vertical line going through the middle of the face and 2 horizontal lines, through the alignment of the eyes and through the base of the neck above the shoulders, in order for the patient to be aware of the movement of his head. The patient is asked to focus on the point of crossing of the first 2 lines.

 

4-Two types of exercises:

 1-Immobilization: for 5 seconds 10 times with 15 seconds rest in between each immobilizations, increasing of 5 seconds every day the immobilization time.

2- Movement: slow and smooth movement, without saccade of the head in rotation, lateral flexion, flexion forward and extension; also movements of the shoulders, arms and trunk and exercises of relaxation of the muscles.

Then also in front of the mirror, exercises of writing, reading, breathing and speaking and daily tasks exercises.

5- Cervical dystonia has a good prognosis:

 Henry Meige was convinced that over the years the cervical dystonia always settle down, with the dystonic spasms becoming less severe and less frequent, until they disappear; the patient is left with only neck stiffness. He did not give any figure of proportion of patients who improved, but they were patients followed for more than 5 years.

6-Since the 80’s, physiotherapy for cervical dystonia has been developed by Jean-Pierre Bleton in France, but still remains a French specialty despite individual effort to develop it abroad.

Botox injections had become the first line treatment for cervical dystonia,, leaving the patient in the passive expectation of his injections every 3 months.

The mirror is still a very important tool in the therapeutic approach of neck dystonia as the patient is very inaccurate when assessing the position of his head, thinking that his head is straight when in reality the head posture has a 20 degrees tilt or rotation.

May be it’s the time for patients with cervical dystonia to become actor of their treatment and to look for a therapeutic strategy including Botox injections and “psycho-motor retraining “  without forgetting the role of the mirror, like Magritte, a Belgium painter…

Meige H : Les peripeties d’ un torticolis mental. Histoire clinique et therapeutique.. Nouvelle iconographie de la Salpetriere. 1907, 6:461-480

 

Don’t push your dystonia!

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 Push up and Press up have became part of the life of young adults…

Dr MH Marion explains why weight lifting is not advisable in cervical dystonia patients…

 

Don’t push your dystonia!

 

1-Push up and Press up have became part of the life of young adults. Going to the gym is a healthy and an advisable way of keeping fit in a urban society, which expect us to be sitting all day long in front of a computer and to be in full shape for climbing mountains.

However, it’s more recent that weight lifting to reinforce selectively muscle strength and to modify body shape is part of the routine of ordinary people, who are neither athletes, nor body builders.  Weight lifting can quickly be part of the life style, with addictive personal challenges to lift heavier and heavier weights.

2-Cervical dystonia is a neurological condition, affecting young adults, resulting in an unbalanced activity of the neck muscles. Some muscles are hyperactive, and inhibit their counterpart on the other sides (reciprocal inhibition); for instance a patient with an involuntary rotation of the head to the right (right spasmodic torticollis) will have a large , hyperactive left Sterno-Cleido Mastoid muscle and a thin right Sterno-Cleido-Mastoid muscle. For a better understanding, read the blog “What makes my head turn?”.

The treatment of cervical dystonia is based on Botulinum injections, which correct this disequilibrium by relaxing the hyperactive muscles and on physiotherapy by reinforcing the inhibited muscles.

3- Is weight lifting contra-productive in patients with cervical dystonia?

I had the opportunity to treat few patients with cervical dystonia who were adept of weight lifting practice. These patients require larger doses of Botulinum toxin, even if the small number of cases doesn’t allow any scientific conclusion. This could explain by the fact that the exercised muscles became larger and stronger.

But the question is which exercises have an impact on the neck muscles and are some exercise worst than others, by targeting neck muscles involved in the dystonia?

I had the opportunity to discuss resistance training exercises with a specialized exercise instructor , Mr Rajah James who gave me a reference book “ Strength training anatomy” from Frederic Delavier. Every resistance training exercise is analysed in terms of functional anatomy with detailed illustrations of which muscles are targeted for each exercise. It’s an amazing book full of details and drawing, that I will strongly advise to anybody interested in exercising against resistance.

4-. Exercises to avoid at any cost:

The neck muscles are involved in the erect posture of the neck, in another words keeping the neck straight; any weight lifting will tense the neck muscles to stabilize the neck during the effort.

More specifically, shoulders muscles such as Trapezius and Levator scapulae elevate the shoulder but also are involved in the rotation, lateral flexion and extension of the neck.

Lateral Arm Raises and Shoulder Shrugs (Machine and Dumbbells shrugs), have to be avoided at any cost; they both reinforce the Trapezius in his upper and anterior part and in addition the shrugs reinforce the Levator Scapulae.

Back Press, Front Press, and Dumbbell Press are reinforcing the Trapezius muscles in its upper part.

 Therefore I advise strongly against any weight lifting exercise in case of cervical dystonia;it can worsen the dystonia by reinforcing the dystonic muscles and increase the muscle unbalamnce and also it can partially compromise the effect of the Botox injections.

5- Physiotherapy for cervical dystonia

The retraining of the cervical muscles, which are becoming less active because of the dystonia, and the stretching of the overactive muscles are a very important part of the treatment. Jean-Pierre Bleton in Paris has written extensively about his original approach of physiotherapy with dystonic patients.

Frederic Delavier: Strength Training Anatomy

http://ebooksfreedownload.org/2011/04/strength-training-

Jean Pierre Bleton : Role of the physiotherapist in the treatment of dystonia

http://books.google.co.uk/books?id=haKD-PjEJ3MC&pg=PA223&lpg=PA223&dq=jean+pierre+bleton&source=bl&ots=yHGkDzRaN3&sig=VhFnlNAlPGqNlnuo7ieIMKR8yjY&hl=en&sa=X&ei=X9FXT42IDsSg8QOc2Mj-Dg&sqi=2&ved=0CFoQ6AEwBg#v=onepage&q=jean%20pierre%20bleton&f=false anatomy.htm

 

Meige syndrome

Dr. Marie-Hélène MarionLeave a comment

Henry Meige (1866-1940)

A grimacing face … not an expression of pain or disgust but a neurological condition:

A Meige syndrome

 Forceful dystonic spasms of the face, in particular when trying to speak or to eat have been called Meige syndrome.

 Henry Meige was a French neurologist who published as a junior neurologist, about facial tics with his friend Feindel in 1894 and 2 years later with his maitre Brissaud about neck dystonia, which he is called Torticollis mental (1896). From then, he studied patients with facial movements disorders, not only the tics but what he called “les convulsions de la face”. He also kept a fascination for the spasmodic torticollis that he recognized publically as an organic disease in 1929 after seeing patients suffering from Encephalitis Lethargica and reading the work of Oppenheim (1911) on Dystonia Musculorum Deformans.

In 1910 he described a Bi-blepharospasm (what we call now Blepharospasm) to emphasize that both eyes were affected, with sometimes a positive family history and which could spread to the laryngeal, mouth floor, jaw  and even tongue muscles.

Meige syndrome is now an eponym to describe a blepharospasm associated with a dystonia of the muscles of the lower part of the face and the larynx as described by Meige in 1910. Patients with Meige syndrome are patients over the age of 60, who complains of involuntary eye closure when trying to speak or to chew. The speech or the chewing is affected and the tongue is involuntary pulled out. It usually starts with a blepharospasm, which spreads down in 35 % of the cases to the mouth and the neck. The pattern of activation of the eye closure changes from an isolated blepharospasm which is better when the patient is speaking and worst when the patient is silent to a spasm of eye closure when the mouth is activated.

Meige syndrome has to be treated actively with anticholinergic drugs if tolerated, clonazepam and Botulinum toxin injections into all the dystonic muscles of the face (eyes, jaw, tongue, larynx, neck) ; the facial grimaces can settle and patients with Meige syndrome should keep hope for a better future.

More study is needed to understand the long term prognosis of this condition.

For further reading, I advice you 2 papers from 1976 and 1982 of Professor CD Marsden

Spastic dysphonia,Meige disease and torsion dystonia. CD Marsden, MP Sheehy.1982

http://www.neurology.org/content/32/10/1202.extract

Blepharospasm-oromandibular dystonia syndrome: a form of adult –onset torsion dystonia, CD Marsden, 1976

http://ukpmc.ac.uk/articles/PMC492566/pdf/jnnpsyc00174-0060.pdf

The Bali’s dancers head posture in patients with neck dystonia.

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Sometimes the analysis of the posture of a patient with cervical dystonia can be tricky; For instance the horizontal translation of the head is a movement that the dancers from Bali can do side to side so graciously. For us European it’s far from natural to dissociate the movement of the head from the neck in a side to side shift. Dystonic patients sometimes have their head shift to one side ….

The Bali’s dancers head posture in patients with neck dystonia.

The horizontal shift of the head.

Sometimes the analysis of the posture of a patient with cervical dystonia can be tricky; For instance the horizontal translation of the head is a movement that the dancers from Bali can do side to side so graciously. For us European it’s far from natural to dissociate the movement of the head from the neck in a side to side shift. Dystonic patients sometimes have their head shift to one side without a lateral tilt and complained of limitation of their active head and neck movements in daily life. How to analyse this dystonic posture is very important when treating the patient with Botulinum toxin injections.

Professor Reichel from Germany kindly sent me 2 weeks ago the latest English version of his book: Therapy guide spasticity-dystonia, which is a very comprehensive and practical guide of the use of Botuinum toxin in these spasticity and dystonia and reflects his vast clinical experience in these fields.

He illustrates in details the horizontal shift of the head with the underlying principle that the role of the muscles in head and neck posture depends of their insertion either on the cervical spine ( Levator scapulae, Scalenius) or on the head itself , mastoid, linae nuchae ( Sterno-cleido-mastoid, Trapezius, Splenius capitis).

The horizontal shift of the head to the right for instance will occur if at the same time the head is tilt to the left and the neck to the right. Prof Reichel uses the terminology of left laterocaput and right laterocollis.

The right levator scapulae and the right scalenius are responsible of the right laterocollis and the left Sterno-cleido-mastoid muscle, the left cervical portion of the Trapezius, and the left Splenius are responsible of the left laterocaput.

 

It seems complicated to follow but it’s quite obvious when examining the dystonic patient with the Bali’s dancer head posture.

But is this posture a primary dystonic posture or the results of an adaptation of the patient ‘s neck posture with a left laterocaput in order to keep the eye line straight when looking in front? There is a reflex loop between eye movement and neck muscles (the cervico-ocular reflex- COR), which with other reflexes prevents visual slip during head and body motion. But this reflex had been found weak or absent in cervical dystonia.

There is still a lot of unexplained observation in dystonia. Fortunately it does not stop neurologists to treat their patients with cervical dystonia with Botulinum toxin injections, based on a careful analysis of the posture.

Dr Marie-Helene Marion

Therapy Guide Spasticity: Dystonia (Uni-Med Science) G. Reichel

http://www.amazon.co.uk/Therapy-Guide-Spasticity-Dystonia-Uni-Med/dp/389599779X

Cervico-ocular function in patients with spasmodic torticollis

R Stel, M Gresty,T Metcalfe, AM Bronstein.

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1014296/pdf/jnnpsyc00499-0049.pdf

 

How much sweat marks can alienate your life?

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How much sweat marks can alienate your life?

You may found the word alienated as “excessive” as is the sweating of these patients …I was myself very surprised when one my patient wrote to me after being successful treated, that she felt FREE .. when the excessive sweating stopped.

Patients who presents with excessive sweating suffered from a lot of stigmas.

Daily life problems of a young person with excessive sweating under the arms.

The sweat mark on a colourful T shirt or a blouse or even going through a sweat shirt make the patient ashamed; the patient is scared of being smelly ( which is not the case), and considered as having a poor hygiene; so starts the search for the strongest deodorant which is usually based with aluminiun which make the underarms red and itchy.

The teens avoid the changing room at school and they keep their school blazer on, even in summer to hide the sweat marks.; the mother buys a new shirt uniform every month as the material becomes like a cardboard under the arms.

The adults also in their profession struggle like the teacher who has to write on a blackboard or a sale assistant reaching items from shelves., avoiding at any cost  to lift up their arms

So they have a shower twice a day, bring a change of clothes at work, wear only dark colors and avoid public transports.

Body odour?

It’s important to explain that excessive sweating doesn’t affect the body odour. There is 2 types of sweat glands, called the eccrine nad the apocrine glands.

The eccrine glands secrete an odourless, clear fluid which help to control the body temperature when exercising or in summer by allowing heat loss by evaporation. They are most numerous on the palms, soles of the feet, face, axillae and to a lesser extent the back and the chest. They are active from birth.

It’s very different from the apocrine glands which start to work from puberty and produce a thick fluid and are responsible for the body odour, and which are not involved in primary hyperhydrosis.

Treatment with Botulinum toxin.

Botulinum toxin injections under the arms can stop the excessive sweating for 6 to 9 months with a very good tolerance of the treatment. I draw a grid with a skin pen (or a eyeliner pen!), based on 15 small squares, equally distributed in the armpit. I will inject Botulinum toxin in each square, so 15 sites of injection in each armpit. The injections are not painful as performed with a small needle and very superficial to reach the sweat glands. There is no bleeding or hematoma and the patient can go back to a normal life after the treatment. The dryness will be complete 7 to 10 days after the injections and will last 6 to 9 months, depending of the severity of the condition.

Emergency Botox clinic for dystonic patients, provided by Dr Marie-Helene Marion, neurologist.

Dr. Marie-Hélène MarionLeave a comment

Emergency Botox clinic, for dystonic patients, provided by Dr Marie-Helene Marion, neurologist.

Severe cervical dystonia (torticollis), head tremor,  blepharospasm  and jaw spasms are so incapacitating that patients  often can’t wait 3 months or even 3 weeks to be treated. Dr M-H Marion is available to see patients at short notice and to proceed the same day with Botulinum toxin injections.

Patients who have been recently diagnosed, and have severe dystonia or dystonic tremor, sometimes find it difficult to cope any longer with the spasms in their face or neck; they may have been referred to a specialist center but have to wait for a few weeks  (or longer) to be seen and injected. Patients also who are regularly treated, require sometimes to be injected outside their normal therapeutic schedule, because the benefit is wearing off earlier, than usual or because unpredictable life events disrupt their time table or because their dystonia  has recently flared up due to stress. These patients can also benefit from Botox emergency clinics.

They will be seen in the London BTX centre, at the Wilbraham Place practice, located in Sloane square (SW1), have the choice between 2 brands of Botulinum toxin type A, and will benefit from electromyographic guided injections if required.  The exact protocol of injections with dosages and sites of injections, documented by sketches of the body part injected, will be given to the patient before leaving the practice. The GP and the regular neurologist will be kept informed.

I hope that this Emergency Botox clinic will help patients with severe dystonic spasm or tremor to get through difficult times of their life and carry on.

 

Management of arm spasticity in Liverpool, 9th December 2011

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Management of arm spasticity in Liverpool

9tH December 2011

 Last Friday,  I went to a Masterclass of  Management of arm spasticity organized by Professor Paul Mc Arthur hand plastic surgeon and Ms Jane McPhail, consultant maxillo-facial prosthetist.

It amazed me how much I always learn from colleagues from other specialties.. We all learn the same functional anatomy of a given muscle, so what’s make the   view so different  between a neurologist, treating a writer’s cramp and a hand surgeon treating a spastic arm?

 Spasticity and dystonia have a different origin. Spasticity in adults commonly followed a stroke with lesion of the cortex (the skin of the orange!). Writer’s cramp is a focal dystonia, which is due to a circuit problem between the deep nuclei (Basal ganglia-the seeds of the orange!) of the brain. Oppenheim, 100 years ago defined the word dystonia as an increased tone different from what was seen after stroke.

 The difficulties encountered by these patients are very different.

Dystonic patients such as Writer’s Cramp have problems when writing, but otherwise are fine for other tasks and at rest. Patient with spastic arms have a permanent disability at rest with pain, discomfort and abnormal posture, interfering with every action requesting skills and strength.

 The abnormal posture has nothing in common…the spastic arm present with rigid flexion of the elbow, arm in pronation (palm of the hand twisted and looking to the floor), and flexion of the wrist and fingers . In case of a dystonic arm, the abnormal posture is not fixed and will be present only when writing. Sometimes  the dystonia is more severe and the arm has a mobile, variable abnormal dystonic posture when arm outstretched and when walking; the whole arm is  twisted in pronation but without a fixed flexion of the elbow, the wrist in flexion and the fingers can be flexed or extended, animated with slow movements.

But despite all these differences, they both benefit from Botulinum toxin injections…

So the approach of the treatment has to be adjusted to the condition

 

Spastic arm: I learned from professor McArthur the concept of function unit to understand at best the spastic arm, with the biceps, brachialis and brachioradialis for the arm flexion, the pronator teres and the flexor carpi radialis for the arm flexion and pronation, and the synergy between the lumbricals and the flexor digitorum  communis . Also I had the demonstration that ultra sound can be crucial to target the spastic muscles precisely as the arm is permanently in an abnormal posture, modifying normal anatomical reference.  The electromyogram is not helpful, except the stimulation, as the voluntary command of the arm is poor.

 Dystonic arm: My experience of watching and treating dystonic patients taught me, on the other hand, that dystonic arm requires to be assessed during the task which triggered the dystonia, that 1 or 2 muscles in spasms can lead the all arm in a dystonic spasms, and that electromyogram is extremely helpful to document the dystonic bursts in the arm; the selection of the muscles is complex as the posturing is variable , but targeting the muscle itself under e;ectromypogram is not as difficult.

 Confronting experience and developing concepts between clinicians from different specialties, are invaluable, and the Liverpool course confirmed that looking from another perspective brings new ideas.

 Also spending a night in Liverpool at the Hard Days Night hotel, having breakfast surrounded by the Beatle’s portraits make you feel very far from London.

Marie-Helene Marion

London BTX centre

Professor Paul McArthur training course:

http://www.prostheticinnovations.com/surgicalworkshops.php

It’s not only emotion that leaves you speechless!

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It’s not only emotion that leaves you speechless! There are many reasons for losing our speech during life. Dr Marion tells you how spasmodic dysphonia is a disconcerting condition…

 Please go to the spasmodic dysphonia page to read  more or

Click on  http://drmarion-londonbtxcentre.tumblr.com/spasmodic%20dysphonia

Botulinum toxin has transformed the treatment of focal dystonia.

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Botulinum toxin (Botox) has transformed the treatment of focal dystonia over the last 25 years. Dr Marion lectured at the SENA meeting about the contribution of Botox to neurology…

South of England Neurology Association (SENA)

The 2nd December, St George’s Hospital

Botulinum toxin (BTX/ Botox) has transformed the treatment of focal dystonia.

 

Dr Jeff Kimber, neurologist organised The South of England Neurology Association (SENA) meeting, hosted this time at St George’s Hospital, London. In the morning session, several talk were on movement disorders. Dr Salah Omer gave a lecture on Progressive Myoclonic Epilepsy, and Dr Bridget Mcdonald adressed the questions of the long term prognosis of cerebral palsy. I gave a lecture on the contribution of Botulinum toxin to Neurology over the last 25 years. 

In 1985, I remembered as a research fellow running a clinic dedicated to patients with cervical dystonia for which the only treatment was anticholinergic drugs (triheyphenidryl, procyclidine), physiotherapy and peripheral denervation surgery (cutting the nerves of the neck muscles). Patients with focal dystonia have always a major functional disability as the dystonic spasms are triggered by action. Oromandibular dystonia is the source of chewing or speaking difficulties. Blepharospasm can lead to functional blindness. Writer’s cramp stops the patient writing. Cervical dystonia interferes with walking, writing, working in front of a screen. Spasmodic dysphonia  makes talking on the phone an impossible task…

Botox treatment has been a revolution for these patients, giving them a relief and the possibility to carry on their daily activities.

 It’s important to inform the public and the funding body in healthcare profession that Botulinum toxin is not a beauty cream but a major therapeutic tool and  that every department of neurology should be given the resources to offer this treatment to their patients.

Marie-Helene Marion

London Btx Centre