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

Meditation and dystonia: an insight from a lady with blepharospasm

Meditation refers to a family of self -regulation practices that focus on training attention and awareness in order to bring mental processes under greater voluntary control and thereby foster general well-being and development and/or specific capacities such as calm, clarity and concentration.…( Walsh and Shapiro, 2006, quoted in Wikipedia on meditation http://en.wikipedia.org/wiki/Meditation

Several recent studies have shown the influence of mindfulness meditation on brain morphology. In particular, Britta et al (Soc Cogn Affect Neurosci. 2010; 5: 11–17) has shown the influence of an 8-week mindfulness-based stress reduction intervention on the changes in amygdaloid gray matter density, which is a part of the brain involved in response to stress.

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2840837/?tool=pubmed

Patients with dystonia have all experienced the worsening of their dystonic symptoms with stress and Henry Meige at the beginning of the 20th century adviced his dystonic patients to have a regular, calm life to avoid fluctuations in the severity of the cervical dystonia.

The dystonia itself is source of great frustration and stress for the patients, who are limited in their daily tasks (walking, reading, eating, speaking, writing) by the dystonics spasms. In addition, professional and family life can bring a lot of joy , but can be also emotionally challenging.

Can meditation help patients with dystonia? There is no scientific study on that topic but I want to share with you the testimony of Anne, a lady with blepharospasm who has been involved in teaching meditation to groups and practices mediation regularly for her well being.

 

Testimony of Anne 

“I have had Blephoraspasm for 7 years, for me the most difficult things to come to terms with were

Ø Loss of independence

Ø Loss of career

Ø Not being able to drive when I wanted

Ø Learning to use Public Transport alone

Ø Fear of travelling alone

Ø Decrease in social activities

I went through a period of becoming almost house bound only going out with my son and friends.

Having worked as a senior Nurse in Mental Health for over 40 years I was very aware of the symptoms of stress and anxiety but had not fully appreciated just how much these symptoms were affecting my Dystonia, I had spent years as a CPN teaching patients and carers Anxiety Management but had not recognised the tell-tale signs in myself

First I had to accept that I had this disabling disorder and that I had to learn to manage the symptoms, I soon became more and more aware that any sort of negative stress or anxiety made the symptoms a great deal worse. It could be something really trivial and my eyes would start closing.

To assist me with relaxation I tried many different complementary therapies some helped some didn’t, we are all individuals, what will help one person won’t another. I also used anxiety management techniques to help, particularly when I went out i.e. shopping.

I also tried to develop new activities/hobbies that I could do rather than dwell on those that I had lost [not easy]

I had been interested in Meditation for many years and although I had practised this, it had not been in a very disciplined way, now I aim to sit for 30 minutes once or twice daily.

There are many different types of meditation and many definitions.

Meditation for me is about stilling the mind and looking within, facilitating a sense of peace and calmness

                                  Simple meditation exercise.

 The following is a simple method that I use with the aim of reducing stress, identifying tension in various parts of the body and assisting me in controlling negative thoughts and generally aiding relaxation

 Preparation for meditation

Ø Wear comfortable clothing

Ø Use a space for this purpose, a spare room or a corner of a bedroom whatever works for you importantly the space needs to be conducive to sitting quietly with an even temperature            

Ø Attempt to sit at the same time each day, this will help to establish a routine that will be easier to adhere to.

5 to 10minutes is fine to begin with. Don’t beat yourself up if you cannot achieve this.

 Sit comfortably on a chair with your back supported, feet flat on the floor [use a cushion for your feet if they don’t reach the floor] hands on your lap.

Close your eyes if this is comfortable.

Focus on your breath both the inhalation and exhalation [it is important to breathe normally not too deeply] Some people think of the word Relax as they exhale.

You will find that to begin with there are many distracting thoughts going round and round in your mind, this is normal, don’t worry about them let them come and go, just gently bring your attention back to your breath, this may be difficult to begin with but with practise it becomes easier.

It is impossible to think about 2 things simultaneously, as you focus on the breath the distracting thought will disappear at least for that moment.

You may feel fidgety at first, your body and mind need time to adjust to not worrying and rushing to do all the normal daily tasks, practise will help.

As you become more expert in the exercise you will observe which muscles are tenser than others. Gradually you will be able to sit for a longer period leaving you feeling more relaxed.  If it is preferable, quiet gentle background music could be played. Most types of meditations start with this type of exercise how long you sit and how deeply you go within yourself is a matter of personal choice

There are many articles and books re.Meditation for further research”

Anne

It will be interesting to hear the voices of dystonic patients from India for instance, where meditation is part of a long cultural tradition to know if they find it helpful.

Of course, I am not suggesting that meditation is a treatment of dystonia, or that meditation is good for everybody, but it may be a coping strategy for some dystonic patients when the stress in their life has a negative impact on the severity of their muscle spasms.

Swallowing difficulties after Botox injections.What to do and how to avoid them.

Swallowing difficulties can be a side effects of Botox injections, when treating neck dystonia, tongue dystonia, jaw dystonia and voice dystonia. It can occur in the most expert pair of hands as the response to Botulinum toxin varies among patients…

What is important to know? 

Some patients experience swallowing difficulties after Botox injections, due to the spread of the Botulinum toxin to adjacent muscles. Dysphagia (swallowing difficulty, choking on food and/or fluid) is the most serious local side effect, following Botox injections. It can occur in the most expert pair of hands as the response to Botulinum toxin varies among patients. A given dose of Botulinum toxin will be not enough for some patients, but for others it will too much, responsible for spreading to muscles next to the injection site.

Side effects occur most often 10 days after the injection, but may occur earlier, 2 or 3 days after injection. All the side effects are transient, lasting on average 4 to 6 weeks after the injection.

 You can be reassured that all the side effects disappear completely with time.

How it happens?

 

Green arrows represent the Sterno-cleido-mastoid muscle (SCM)

Red arrows represent the strap muscles

 Blue dot represents the Hyoid bone

 

1- Dysphagia following neck muscle injection for cervical dystonia

            a- Dysphagia can follow injections into the front neck muscles, the sterno-cleido-mastoid (SCM) muscles. When these big muscles in the front of the neck are injected, some of the Botox can leak into the surrounding muscles, called the strap muscles.  These strap muscles act on the hyoid bone, which is attached to the bottom of the tongue.  Normally the strap muscles keep the hyoid still and allow the tongue to push the food back into the throat to trigger the swallow.  If the muscles are weak, it does not happen properly and patients can get a feeling of choking, worse with solid food. These swallowing difficulties after SCM injections are more frequent if both sides (right and left SCM ) are injected, if large doses are injected, if the neck is very slim.

          b- The injections of the Longus colli muscles for severe antecollis (dystonic flexion of the neck). These muscles are found deep in the neck and are very close to the muscles of the gullet, responsible for pushing the food down towards the stomach.  If the injection is put in too high up, the ability to push the food down can be reduced; this is usually a problem with swallowing solids.  We usually avoid this happening by testing that we are below the swallow muscles during the injection by performing the injection under EMG (recording of the muscle activity), to check when the patient is drinking, just before the injection, that the needle is out of the pharyngeal constrictors.

2- Dysphagia following tongue muscle injection for tongue dystonia or following mouth floor muscles (supra-hyoid muscles) injection for opening jaw spasms is frequent. The reason some people struggle with swallowing after this injection is basically the same as after SCM injections and the weakening of the strap muscles. The mouth floor muscles and the bottom of the tongue muscles are attached on the hyoid bone. The power of the back of the tongue is reduced and the ability to push the food backwards, towards the top of the gullet,  and trigger the swallow reflex is impaired.

3-Dysphagia , following laryngeal (voice box) injections, is mainly when drinking  fluid and are usually mild. Apart from making a sound for speaking, the main function of the vocal cords is actually to protect the lungs from things falling into them.  When Botox is injected into the vocal cords from the front, the closing action is weakened.  In most patients this does not cause a problem but some people find that they have an increased tendency to cough when drinking fluids.  Very rarely it can also affect eating solids.

Drawing from website on swallowing difficulties after stroke, which explains how aspiration pneumonia can happen.

http://www.strokerehabunit.ie/en/AboutStroke/FeedingandSwallowing/

What to do if you have a problem with swallowing?

As with all Botox side effects, the problem will get better with time.  The average length of side effects for most people is a couple of weeks but some will have less and some more.  There are many ways to help yourself during this time.

1-Take small mouthfuls and chew carefully before trying to swallow.

2- If food feels like it is slow to go down, keep a glass of water nearby to help wash down the food.

3-Consider eating softer consistency food (thick soup, yoghurt, mashed vegetables) for a short while, avoid crusts, large pieces of meat and anything very hard. Eating in front of somebody and not alone is recommended.

4-If swallowing fluid is making you cough, make sure you are sitting up when drinking.  Sip slowly through a straw as this allows you to keep your chin tucked down and this makes it more difficult for the liquid to spill into the voice box. Sucking ice/ice lollies can get you quite a lot of fluid but as it is delivered in very small amounts does not cause any coughing.

5- If food/drink enters the wind pipe and goes into the lungs frequently, a serious infection may result, called aspiration pneumoniaAlso you may not be able to eat or drink enough, so please make sure you get in touch with your GP or the doctor who injected you,  as very occasionally patients will need to be admitted to hospital to be fed through a tube for a few days.. This is very rare and in our experience has happened to less that 1 patient per year

 

What to do to avoid the side effects at the next session of injections?

 

1-Swallowing difficulties, which can exist before the injections, have to be reported to your doctor, before the injection.

–       It has been shown that patients with already swallowing difficulties before injection, due to their dystonia, are more at risk of severe dysphagia with Botulinum toxin treatment.

–       Swallowing difficulties are frequent before any treatment in cervical dystonia, (from 36% clinically to 72% when investigated), in spasmodic dysphonia and in oro-mandibular dystonia.

–       The Speech and Language Therapist may x-ray the mouth and throat area to see what the precise swallowing difficulties are. This x-ray is called a videofluoroscopy. This x-ray will help to determine what types of food and drink are safe to swallow and what dysphagia therapy might be appropriate.

–       Studies showed that Cervical dystonia patient with dysphagia can have difficulty to drink only 1-10ml in one attempt, in contrast with non-dyphagic patients with cervical dystonia who can drink 20 ml in one attempt.

 2 -The injections have to be customised:

a.     If already dysphagia, the doses into the front muscles should be limited.

b.     At a session of injection, following severe dysphagia after the initial injections, the injection sites and dosages should be adjusted to avoid the reoccurrence of any side effect.

c.     It may also require delaying the injection more than usual, 14 weeks instead of 12 weeks for instance, to avoid any cumulative effect.

3-The dysphagia has to be documented:

It’s very important that the patient documents precisely the nature of the side effect, (for instance, choking on fluid or on food, need to drink to wash down the food after eating), in particular if the patient is not seeing his doctor in between 2 sessions. A dairy of the events will be very helpful for the doctor when adjusting the next dose.

Conclusion

 Dysphagia is frequent before injections in patients with dystonia, but can also be a local side effect of the Botox injections. Being cautious when drinking and eating, and talking to your doctor are the best advices. It can be a scary time, but it will all go back to normal after 3 to 6 weeks time .

Also it can be avoided the next time, so it should not discourage you for having further injection.

I wanted to thank my collaborator, Ms Lucy Hicklin, ENT surgeon, a specialist of Botox injection into the vocal cords for spasmodic dysphonia and into the Longus colli  for antecoliis for her contribution to this post.

 References:

Oropharyngeal swallowing in craniocervical dystonia. Ertekin C, Aydogdu I, and al, J Neurol Neurosurg Psychiatry. 2002;73:406-411

The swallowing side effects of botulinum toxin type A injection in spasmodic dysphonia. Holzer SE, Ludlow CL. Laryngoscope.1996; 106: 86-92

0
false

18 pt
18 pt
0
0

false
false
false

/* Style Definitions */
table.MsoNormalTable
{mso-style-name:”Table Normal”;
mso-tstyle-rowband-size:0;
mso-tstyle-colband-size:0;
mso-style-noshow:yes;
mso-style-parent:””;
mso-padding-alt:0cm 5.4pt 0cm 5.4pt;
mso-para-margin:0cm;
mso-para-margin-bottom:.0001pt;
mso-pagination:widow-orphan;
font-size:12.0pt;
font-family:”Times New Roman”;
mso-ascii-font-family:Cambria;
mso-ascii-theme-font:minor-latin;
mso-hansi-font-family:Cambria;
mso-hansi-theme-font:minor-latin;
mso-ansi-language:EN-US;}

Droopy eyelid, double vision..after Botox injection, what to do?

Double vision, droopy eyelid, tearful eye can occur in the most expert pair of hands as the response to Botulinum toxin varies among patients…Dr Marie-Helene Marion tells you  the facts behind these side effects and what to do when they occur . Dr Marie-helene Marion will also share her tips  to avoid them…

 Botulinum toxin injections can cause side effects due to the spread of the Botulinum toxin to adjacent muscles.

They can occur in the most expert pair of hands as the response to Botulinum toxin varies among patients. A given dose of Botulinum toxin will be not enough for some patients, but for others it will too much, responsible for side effects.

Side effects occur most often 10 days after the injection, but may occur earlier, 2 or 3 days after injection. All the side effects are transient, lasting on average 4 to 6 weeks after the injection. You can be reassured that all the side effects disappear completely with time.

1-Droopy eyelid

   -Droopy eyelid (ptosis) is due to a spread of the neurotoxin to the middle part of the superior eyelid, into the Levator palpebrae muscle. This muscle is responsible for holding up the eyelid. The ptosis, can be mild, covering the top of the pupil only, or more pronounced covering half the pupil; very rarely the eye is completely closed. It happens after injection of Botulinum toxin around the eyes (eg for Blepharospasm or Hemifacial spasm).

– What to do?

 Iopidine 0.5% (Apraclonidineeye drop can be used to decrease the ptosis, inducing a contraction of a small eyelid muscle (the Muller muscle), which lift up the eyelid 1-3 mm. Two drops of Iopidine , 3 to 4 times a day for the 2 to 3 weeks is the usual dose; the effect can be seen 30 minutes after administration of the Iopidine drops and lasts about 2 to 3 hours. It’s used for the treatment of Glaucoma (high intra ocular pressure). It can be responsible for redness of the conjunctiva after 1 week, and for dry eye and dry mouth. It should not be used as a long term treatment, more than 1 month. It’s contra-indicated in case of kidney failure, recent heart attack, heart failure, angina, stroke and high blood pressure

The ptosis is different from a droopy eyebrow, which can occur after injection into the forehead for cosmetic injection or facial sweating, and which closes the eye by making the eyelid looking “ heavy”, increasing the skinfold of the upper eyelid. Iopidine eye drop is not useful in this case.

 2 Double vision and blurred vision

-Double vision (called Diplopia) is due to the spread of the Botulinum toxin into the muscles which move the eyeball; if one of these oculo-motor muscles become weak on one side, the patient will see 2 images instead of one, when looking with the 2 eyes; the double vision will disappear when looking with only one eye; the movement of the 2 eyes are no longer synchronous. B lurred vision is often from the same mechanism but less marked, so the image seen by the 2 eyes is blurred.

– 2 types of double vision:

-If  double vision comes from spread of the Botulinum toxin injected into the upper lid, the 2 images will be one above the other, (vertical diplopia) and the weakened muscle will be the Superior Rectus in the middle part of the upper eyelid.

-If double vision comes from spread of the Botulinum toxin injected into the lower lid, the 2 images will be one above the other (either vertical or oblique diplopia). The weakened muscle will be the Inferior Oblique, in the medial part of the lower lid.

What to do?

Double vision can very disturbing, a source of headache and falls as the edge of the pavement, for instance, will be difficult to appreciate. Therefore the patient with double vision has to be careful when walking, and can either cover one eye, or ask his optician for a prism to attach on one side of his glasses to correct the oculo-motor imbalance. It will also disappear spontaneously in 4 to 6 weeks.

3- Tearful eyes.

 Tearful eyes are due to dysfunction of the evacuation of the normal tear production by the tear duct, situated on the edge of the lower lid in the corner near the nose. If the lower eyelid is too saggy, the tear duct is no longer facing the inside of the eye and cannot drain the  tears. It can be source of blurred vision.

   4- Exposure keratitis

 – Exposure keratitis is due to difficulty to close completely the eye (lagophtalmos). If the pretarsal injection of Botulinum toxin into the upper lid is too strong for the patient, the eye can be wide open with decreased blinking and difficulty to close completely the eye at night. The eye becomes dry, sore, red, irritated by any dust or other foreign bodies, which are usually cleared from the surface of the eye (cornea) by blinking.

–  What to do?

 Artificial tears during the day, ointment at night, protection of the eye from the wind by wearing large glasses, and closing the eye at night with an eye pad are the protective measures to avoid keratitis.

5- What to do to avoid the side effects at the next session of injections?

–  Customize the injections:

a.    At the next session of injection, the injection sites and dosages should be adjusted to avoid the reoccurrence of any side effect.

b.    In case of ptosis, the injection can be distanced from the middle part of the upper lid.

c.    In case of diplopia or tearful eye, the injection of the medial (near the nose) lower lid site will be avoided.

d.    It may also require delaying the injection more than usual, 14 weeks instead of 12 weeks for instance, instead of decreasing the dose too much. A low dose could have no side effect but also have no effect on the dystonic spasm.

-Document the side effect:

Therefore, it’s very important that the patient documents precisely the nature of the side effect, in particular if the patient is not seeing his doctor in between 2 sessions.  Pictures of the face for the droopy eyelid, description of the double vision at the time of the event, will be very helpful for the doctor when adjusting the next dose.

 Again, every patient responds differently to the injections, and it may take sometimes 2 or 3 sessions, to customize the injections in order to achieve an optimal benefit without side effect.

Don’t lose hope! The side effects never last and the injections will eventually be tailored to your own condition, fitting you like a dress from a haute couture salon!

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.

 

More than a wink!

A little twitch under the eye (Benign Eyelid Myokimia)

It’s not rare to have a jumpy lower eyelid when tired. It comes and goes, it’s upsetting when trying to read or when talking to somebody; it’s worst when drinking too much coffee, lacking of sleep or being under pressure. It’s called Benign Myokimia and it usually disappears without any treatment after rest and relaxation. Don’t hesitate to talk to your GP if it persists.

 

A spasm of one side of the face (Hemifacial spasm)

Less commonly the twitch in the eyelid continues and spreads to the upper lid with a tendency to close the eye. It also spreads very gradually to the cheek, and to the corner of the mouth, which starts twitching at the same time. The person looks like winking to somebody with the sudden eye closure and the cheek pulled up. Of course it’s very embarrassing in public and can lead to some misunderstanding!

 

Life with Hemifacial spasm.

It interferes with daily life activities like reading. Patients find very tiring to cope with the facial spasm every day. It is worst when going to bed and falling asleep. Every muscle on one side of the face can be jerky from the forehead (Frontalis) muscle to the muscle of the skin of the neck ( Platysma). Patients often are embarrassed when meeting people for the first time. It’s more than a wink!

 

The diagnosis of hemifacial spasm is not always easy!

The spasm is intermittent and often not present during the clinic, like the toothache disappears when sitting in the dentist ‘s chair! The trick is to stretch the muscles of the face to trigger the facial spasm; I ask the patient to do a forceful smile or to close very tight the eyes for few seconds; following these stretches, the facial muscles start to jump on one side of the face. The diagnosis is then much easier for the doctor.

 

Why one side of my face is jerky?

The facial muscles on one side become too active, due to an irritation of the facial nerve on the same side. The irritation is most often due to an artery, which forms a loop and rubs against the facial nerve.

 

What is the treatment?

Botulinum toxin injections are indicated at the first line treatment for hemifacial spasm, as the side effects are very limited and always transient. The results are very good in 80% of the cases. The limitation of the injections is the necessity to repeat them every 4 months and the difficulty to control severe spasm around the mouth without changing the smile.

The surgeon can put a pad of Teflon between the vessel and the nerve to separate them; this surgical treatment calls vascular decompression and is performed by neurosurgeon, trained in this type of surgery. It is usually discussed after having tried Botulinum toxin treatment.