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

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Meeting the experts in Guildford, UK: What about blepharospasm?

Last weekend, 15 movement disorders clinicians from Europe, Russia, USA, and Canada gathered in Guildford, to share views on Botulinum toxin treatment for focal dystonia., This meeting was sponsored by Merz Pharma.

Dr Elin Forsaa,Dr Svetlana Khatkova, Dr Torsten Grehl, Dr Flavia Coroian, Dr JM Meyer, Dr MH Marion, Dr Dirk Dressler, Dr Sylvain Chouinard,Dr Robert Chen, Dr Sophie Sangla, Dr Said Bensakel, Dr Shyalmal Mehta, Dr Olivier Simon, Dr Richard Evans, Ms Muna Bitar

I was asked to present the key therapeutic challenges in blepharospasm and prof Dressler from Hanover was presenting the therapeutic challenges in cervical dystonia.

What came up in the discussion watching many videocases,  was the clinical diversity of the blepharospasms, which raise a lot of questions.

1-Are the young onset blepharospasm , affecting women before the age of 50, different in their progression and response to treatment?

2-How some patients with primary blepharospasm can be still focal, affecting only the eyes after 10 years of duration and other patients (31% in Defazio study) see their dystonia spread to the lower part of the face, in the first 5 years after the onset?

3-How some patients are mainly “” blinkers” and others “shutters” sic a patient?

In another words is there a group of patients who started with increased blinking, following dry eyes and photophobia, and some who never complains of over blinking, who have no sensory symptoms, but can’t reopened their eyes.?

4-How the dystonic spasm around the eyes can be alleviated initially by speaking or singing, then becoming triggered and worsened by speech when the dystonic spasm has spread to all the face (Meige Syndrome) in a same patient over the years.

5-Are both blepharospasm and migraine which share the same sensitivity to bright light are coincidental when occurring in the same patient or do they share some common pathophysiology of central trigeminal desentizisation?

6- is the Bell’s phenomenon seen in some patients, following an improvement of their spasms after Botulinum toxin injection, is a marker of a persistent underlying dystonic activity as a reflex from the brainstem.

7- Is stimulating, the Muller muscle, which is a smooth muscle which plays a role in lifting up the eyelid, with Aproclonidine eye drop can help patients with so-called apraxia of eyelid opening?

Previous studies , in particular the work of G. Defazio, from Italy, have given some answers. Patients with blepharospasm have twice the risk of spreading than cervical dystonia and most spread events occurs after the age of 50..

. Patients with Blepharospasm blinked more at rest than during conversation, by contrast with heatlthy volunteers who blinked more during conversation. Two cases of speech induced Blepharospasm has been described in patients with cranial dystonia.

 I found this session very inspiring, pushing me to look further in the clinical characteristics of blepharospasms.  We always quote the clinical complexity of writer’s cramp, where so many different muscles can be involved. I am convince that despite a only muscle involved, the orbiculatrs oculi, Blepharospasm is not a model of simplicity…

We then all went to a pub in Shere for diner, admiring on the way the beauty of the Surrey hills.  May be in the next few years we will meet again to share the answers to our questions…

 

The William Bray pub in Shere (Surrey)

 

Dr Marie-Helene Marion (London) and Dr Sophie Sangla, a leading neurologist in botulinum toxin treatment in Paris: a friendship of 30 years….

 

Living with dystonia

Dr Marie-Helene Marion (neurologist), Mrs Linda Baker (secretary of the Kent group) and Ms Angie Brown (Dystonia Society representative)

Dr M-H Marion, neurologist, specialised in the treatment of dystonia, explains how dystonia is a source of disability, distress, anxiety and despair and what can be done to help.

Mr Dave Ward, Dr Marie-Helene Marion and Mrs Linda Baker (Kent dystonia society group)

 

 

Living with dystonia

Last Saturday I attended the Kent Dystonia society group meeting in Maidstone.  As the attendees had different types of focal dystonia, I chose to speak about living with dystonia in general, and how dystonia is a source of disability, distress, anxiety and despair and what can be done to help.

1-Dystonia is source of disability, related to the fact that the dystonic spasms are brought up by action and interfere with daily life activities. As soon as the patient with blepharospasm tries to watch TV or drives, his eyes close; the patient with jaw spasms has major difficulties chewing; the patients with neck dystonia, difficulties when walking, and looking in front of him; the patients with writer’s cramp when writing and the spasmodic dysphonia when speaking.

2-Dystonia is source of distress as the diagnosis of dystonia is often delayed.

The awareness of doctors is low for dystonia as it is a rare condition, but it is not the only reason to explain the difficulty for the diagnosis. The presentation is often disconcerting, with the presence of a “geste antagoniste” (for example, touching with one finger the cheek can control severe neck spasms) or a sensory trick (a gum kept in the mouth alleviates jaw spasms), the occurrence on specific tasks, the diversity of the symptoms depending of the site of the dystonia, and the existence of a stressful event prior to the onset of the symptoms. The natural history also is often disconcerting, by its capricious fluctuations with remission and worsening without clear reason.

3- Dystonia is source of anxiety as the patient try to understand what’s going to happen in the future. The patient has difficulties to understand the natural history of the diseasethe spontaneous remissionsthe dystonic stormsthe risk of spread and to know what are the right expectations from the treatment.Doesthe treatment influence the course of the disease? What I should do in my daily life to get better?

4- Dystonia is source of despair as getting the right treatment is often an obstacle course.  In particular it can be challenging to find a specialist in dystonia and a Botulinum toxin service, which perform EMG guided injections into deep muscles, like the jaw muscles, the vocal cords or the forearm muscles. Do I need to see a neurologist, an ENT specialist, an ophthalmologist for the Botox injections??

The diversity of the response to treatment is also frustrating and the Botox injections have a trial and error process before finding the right dose for each individual. The patient needs to understand the therapeutic strategy, such as the role of the drugs, of the Botox injections, of the surgery and of the self-healing process (relaxation, physical exercises).

 Can we help dystonic patients to face all these challenges? 

One of the main answer for me is that the patient needs to become actor of his treatment.

Clear information has to be available for the patient: Every patient with focal dystonia in a way is unique, in the type of spasms and in the response to treatment. The patient needs to be well informed.  A better knowledge of the expected benefit and of the possible side effects from the patient will help the doctor to adjust the dosages of the anticholinergic drugs and of the Botulinum toxin.

The level of anxiety will decrease, as the patient will be able to anticipate and influence the progression of the dystonia by physical exercises, and by adjusting daily life activities to his ability.

 

What is available now in UK?

 1-The Dystonia Society UK www.dystonia.org.uk  has used the modern technology to reach a large population. Google advertising, new website, forum discussion, page on Facebook (Dystonia society UK), Twitter @dystonia society.. very soon there will be no more excuses to not know the word dystonia!!

2-Myself, as a neurologist specialised in dystonia, have developed this blog, www.infodystonia.com, to share my experience in treating dystonic patients over the last 25 years. For the healthcare profession, I have founded the British Neurotoxin Network (BNN) www.neurotoxinnetwork.org to gather all the clinicians involved in the Botox treatment of dystonia in UK for sharing information, techniques and new developments. The British Neurotoxin Network  (BNN) website, also hosts UK service maps www.neurotoxinnetwork.org/botox-treatment-centres.php to localise easily by the patients or the doctors where is the most appropriate centre for treatment.

I am also organising workshops and master class to share knowledge and skills with my colleagues who want to be involved in the care of dystonia.

3-The patients also are working hard to go forward in the best care for dystonia in organizing local support groups, like the Kent group under the umbrella of the Dystonia Society. The list of all these groups in UK are available on the Dystonia society website.

Thank you to Linda Baker and Dave Ward to organize this very well attended and stimulating meeting at Maidstone.

When meeting dystonic patients, it come to my mind  that the British Motto: “Be calm and carry on” is what most of the patients have courageously chosen when living with dystonia.

How much sweat marks can alienate your life?

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.

Sweating in Winter: a serious problem

 

Sweating in summer on a hot day or after a long Sunday jog may feel good, or at least normal. But sweating in winter when the weather is cold or you just come out of the shower can be a nightmare for individuals suffering from a condition called Hyperhydrosis (also spelled Hyperhidrosis).

 

Primary Hyperhydrosis or excessive sweating is in third of the cases a familial condition, which can have various manifestations, depending of the site of the excessive sweating; “primary” means that no obvious cause such as drugs, diabetes, infection etc.. is associated with the excessive sweating. Excessive sweating, in particular starting in adulthood and without family history should be investigated by the GP as it could be secondary sweating.

Armpits sweating:

 In case of Primary Hyperhydrosis affecting  the armpits, it’s often  young adults complaining of sweat marks on their clothes under their arms since their teens. They avoid colourful and white clothes and prefer dark clothes to not show the marks; they feel ashamed and loose their confidence as they are wrongly convinced that the excessive sweating under the arms is associated with excessive body odours. They bring extra clothes at school or at work to change during the day.

Hand sweating:

It can also affect the hands, usually noted when the child learned to write at the age of 5; the pen slipped out the hand; they made wet mark on the paper when writing. then these children don’t dare holding hands of their friends at primary school and later on avoid social contacts; it can also affect  professional manual skills.

Feet Sweating:

The excessive sweating of the feet is usually well tolerated in winter where the socks in the shoes absorb the sweat and I have met patients who were convinced that to have wet socks were normal at the end the day. The worst is in summer when ladies try to wear open sandals, in particular with some heels; the sweaty wet feet slip out and make walking hazardous. I have heard alos young men distress about having wet feet in bed.

Facial sweating:

Facial sweating is also very distressing. The sweat can drip from the scalp, or the forehead along the face, the cheeks and the nose and in back of the neck leaving the hair wet.

Excessive sweating people can benefit from treatment and is not related to excessive shyness or anxiety but it’s a true condition.

Please don’t hesitate to contact support group such as the Hyperhidrosis Support Group at www.hyperhidrosisuk.org

Dr MH Marion is running specialized clinics for all types of primary hyperhidrosis both at St George’s Hospital, London (UK) and at the  London BTX centre.

“With Africa, for Africa”: The World Congress of Neurology

At the XXth World congress of Neurology in Marrakesh, yesterday a group of French neurologists (Dr Christophe Vial from Lyon, Pr Pierre Krystkowiak from Amiens,) with Pr Ouafae Messouak from Fes (Morocco) and I run a workshop of Botulinum toxin injection techniques in Neurology. It was a great success with more than 45 people attending and working with us all day. We had colleagues from Morocco, Tunisia, Ghana, Kenya, Nigeria, Sudan, Syria, Lebanon,  the Emirates, India, Thailand, Belgium, Norway and USA. Mr. Olivier Seguin from the drug company Allergan kindly provided mannequins (called Elvis and Elvira !) which help us to do some “hands on” practices.

It was a very interactive teaching where we met our colleagues in particular from Africa. The BTX services in Africa already exist, in particular in Maghreb where there are very active (Algeria, Tunisia, Morocco). In the other part of Africa, and the Middle East the development of the services is based on individual’s initiative of neurologists, with an interest in movement disorders.

Talking with our African colleagues, we felt the need for setting up an African and Middle East Neurotoxin Network  (AMENN), following the model of the British Neurotoxin Network (the BNN). It will help the neurologists to be less isolated in their practices, and to allow African patients to access services locally without travelling at great cost to Europe for treatment many times a year.

“With Africa, for Africa” was the motto of this world congress.

Picture of the workshop faculty

Dr Marie-Helene Marion  (London BTX centre), Dr Christophe Vial (Lyon, France), Pr Ouafae Messouak (Fes, Marocco), Pr Pierre Krystowiak (Amiens, France)

 

H.Oppenheim, 100 years ago was the first to use the word “ Dystonia”…

H.Oppenheim, 100 years ago was the first to use the word “ Dystonia”…what does the word Dystonia means? Dr Marion will take you back 100 years ago…

Dystonia is a neurological condition, characterized by involuntary sustained pulling of the muscles in one part of the body (focal dystonia, mainly in adult) or spread in the all body (generalized dystonia, mainly in children) and associated with abnormal postures.

The word “Dystonia” is composed of Dys (meaning abnormal) and Tonia ( meaning the tone ) . The tone represents how flexible or stiff is a part of the body. Oppenheim coined the word “Dystonia” in 1911 to describe an abnormal muscle tone, different from what was observed following stroke (spasticity). Oppenheim described children, from Ashkenazi Jewish descent, affected with generalized dystonia and called this condition “ Dystonia musculorum deformans”. In 1989, Laurie Ozelius established that a gene (called DYT1) on chromosome 9 was responsible of the ‘Oppenheim dystonia”.

 http://en.wikipedia.org/wiki/Hermann_Oppenheim

http://brain.oxfordjournals.org/content/97/1/793.extract

http://www.ncbi.nlm.nih.gov/pubmed/2576373

Welcome

I have been treating patients with dystonia for the last 25 years with Botulinum toxin injections. Patients have many questions left unanswered because dystonia is a rare condition and the image of Botox has been diverted to the cosmetic industry. Patients with hyperhydrisosis also feel very isolated and often don’t dare talking about their condition. The aim of this blog is to help patients to carry on …

Dr MH Marion