Understanding dystonia and its treatment. Video on questions & answers

A good knowledge of the dystonia is important for a patient to become actor of its treatment. The Dystonia society UK asked me to answer to the most frequent questions coming from their members. Please follow this link if you want to watch this video posted on the Facebook pageof the Dystonia society.

image

The questions were as follow:

What causes dystonia in adults? Can any vaccine, peripheral nerve damage, the menopause or the surgery has any affects? (Time 1.00)

Does dystonia spread? If so, are there any particular parts of the body it spreads to and from? (Time 6.20)

How long do the symptoms carry on getting worse? (Time 7.58)

I understand Botulinum toxin is a type of poison. Is it safe to use? (Time 13.35)

Can Botulinum toxin be used in pregnancy? (Time 13.39)

Is there a way to avoid side effects from Botulinum toxin such as pain at the injection sites, swallowing difficulties, blurred vision? (Time 15.21)

How can I manage pain in dystonia if it is not relieved by Botulinum toxin? Is it OK to take pain medications on long term basis? (Time 18.57)

Why is my Botulinum toxin not working? Might is this because I become immune to the toxin? (Time 20.41)

Is Artane (Trihexyphenidyl) helpful? Is there an age limit for taking it? (Time 31.54)

Can physiotherapy or mind retraining help? (Time 36.50)

Might diet or complementary therapy help to manage dystonia? (Time 39.09)

I hope it will contribute to a better understanding of dystonia by the patients themselves and help them to cope better in their daily life.

Dr MH Marion

Advertisements

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.

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