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….

 

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

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.

 

Botulinum toxin has transformed the treatment of focal dystonia.

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

Few words about me… Dr Marie-Hélène Marion

Few words about me…

Dr Marie-Hélène Marion, specialist in Botox treatment for 25 years.

My name is Marie-Hélène Marion and I am a consultant neurologist, specialized in the use of Botulinum toxin (also called Botox) in Neurology. I started blogging 2 months ago about Botox, Dystonia and Hyperhydrosis and I enjoyed every minute of it. Of course at the beginning I had some hesitations, but quickly I realized that blogging for patients and talking to patients during a clinic, have a lot in common, except that you don’t know and you don’t see the reactions of your readers.

My training

I studied medicine at the University of Clermont-Ferrand in the heart of the beautiful volcanic region of Auvergne. Then went to Paris to study Neurology as an “ Interne des Hopitaux de Paris” in the most prestigious departments of that time and in parallel studied Neuro-pharmacology (how the drugs work in the brain)

Then I started to have itchy feet and went to London to specialize in movement disorder as a research fellow under Professor David Marsden for 2 years. His passion for Parkinson’s disease, Dystonia and the all field of involuntary movement was contagious and never left me since.

My work as a movement disorder specialist on both sides of the channel

I went back to Paris in September 1986 to work as a Chef de Clinique and pioneer in France the use of Botulinum toxin in Neurology. Then in 1998 I crossed again the channel to follow my Australian husband and worked for 12 years at St George’s hospital, in London, running Movement disorders clinics and various Botulinum toxin clinics. At St George’s I still run a clinic specialized in excessive sweating, in particular facial sweating and hands sweating, and a clinic for voice disorders and severe bent neck.

The London BTX Centre

Now my main clinical activity is at the London BTX Centre in Sloane Square that I founded 6 years ago, dedicated to the treatment of focal dystonia, (Blepharospasm, Cervical dystonia, Jaw dystonia, Writer’s cramp, Musician’s cramp), Hemifacial spasm, Facial palsy, Tremor, Bruxism and excessive sweating.

A special mention for patients with voice disorders who are seen in a joint clinic, unique in London, with an ENT-voice specialist (Ms Lucy Hicklin) and a neurologist (MH Marion).

Dr Marie-Helene Marion (neurologist) and MS Lucy Hicklin ( ENT surgeon, Voice specialist)

Academic interests

In parallel, I pursue academic projects in the movement disorders field and published this year on Parkinson’s disease and also on complex cervical dystonia with my junior colleagues at St George’s Hospital. I recently organized the British Neurotoxin Network (BNN)  which gathered all the clinicians over UK who are running Botox services for neurological conditions.

I am a regular invited speaker in international meetings on the field of movement disorders and organized workshops on the use of Botox treatment in neurology, as European expert in the field.

“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)