dr MH Marion

Mental imagery and dystonia

Dr. Marie-Hélène MarionLeave a comment

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)

How does my jaw move?

Dr. Marie-Hélène MarionLeave a comment

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 head shakes: is it dystonia?

Dr. Marie-Hélène MarionLeave a comment

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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

Dr. Marie-Hélène MarionLeave a comment

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 disease: the spontaneous remissions, the dystonic storms, the 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.

It’s not only emotion that leaves you speechless!

Dr. Marie-Hélène MarionLeave a comment

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

The diagnosis of Blepharospasm is always delayed!

Dr. Marie-Hélène MarionLeave a comment

     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

So many different types of focal dystonia!

Dr. Marie-Hélène MarionLeave a comment

So many different types of focal dystonia!

 Depending of the body part affected,

 ·      Eye closure is called Blepharospasm (blepharo means eyelid in Greek).

·      Jaw opening or clenching is called Oro-mandibular dystonia (oro means mouth and mandibular means jaw in Latin).

·      Tongue protrusion is called Tongue dystonia

·      Twisting of the head to one side is called Cervical dystonia (cervic means neck in Latin).

·      Writing difficulty is called Writer’s cramp.

·      Back arching, or trunk bending forward is called Axial dystonia (axial means axis as the spine).

·      Walking or running difficulty is called the “ Crampe du marcheur” in France!

·      Difficulty playing a musical instrument is called Musician’s cramp.

·      Speaking difficulty is called Spasmodic dysphonia (phonia means the speech).

 They usually start in adulthood and remain focal, without spreading to adjacent part of the body.