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

image

Masseters, closing jaw muscles

image

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.

image

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

Figure adapted from Travell and Simons’

image

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.

image

 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.

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.

Meige syndrome

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

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.

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.

 

Management of arm spasticity in Liverpool, 9th December 2011

 

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!

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.

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

“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)

 

The diagnosis of Blepharospasm is always delayed!

     The diagnosis of Blepharospasm is always delayed!

Blepharospasm is a focal adult- onset dystonia, responsible of an involuntary eye closure. It can start with an increased blinking explained by dry eyes, gritty eyes or intolerance to bright lights. Gradually the patient, more often a woman around her 60’s, complains of difficulty to watch TV, to drive at night or just to walk outdoors on a cloudy day

At that stage, surprisingly the diagnosis is not easily done. Why?

1-    The GP refers this lady to the eye clinic; the diagnosis of blockage of the lacrymal ducts, or of blepharitis (with an inflamation of the eyelids) are much more common conditions and often the first to be considered.

2-    The patient does not spontaneously mention that the eyes are involuntary closing. They more often talk about their heavy eyelids, or tired eyes, or intolerance to bright light (also called photophobia)

3-    The patient has often the eyes well opened when speaking and the doctor can’t document any forceful eye spasms during the clinic.

This explains that the diagnosis of dystonia (Blepharospasm, Cervical dystonia) is usually made after 5.4 years on average after onset of symptoms and at least after seeing 3 different consultants (Canadian survey of Dr Jog ).

The patient will benefit to come to the first clinic with a relative or a friend who may describe it more accurately as an external observer. I also ask my patients to stop talking and be silent for few minutes, fixing a visual target in the room; the spasms with forceful eye closure will occur 2 to 3 minutes later. It’s worthwhile waiting as it’s a great opportunity not only to make the diagnosis but also to identify the type of Blepharospasm.

 Hope this blog will contribute to an earlier diagnosis of Blepharospasm!

 Reference: Causes of treatment delays in dystonia and hemifacial spasm: a canadian survey. Jog M et al, Can J Neurol Sci 2011: 38:704-11