dr Marion specialist in dystonia

Speaking up about silent swallowing difficulties in dystonia

Dr. Marie-Hélène MarionLeave a comment

Ms Lucy Hicklin ENT, Mr Paul King chief executive Dystonia society UK, Dr Marie-Helene Marion neurologist and Carol Harris speech therapist. London living with dystonia event, on swallowing and speech problems on the 8th november 2014, London.

Every day we swallow solid food like a piece of bread, water and our own saliva. This vital function occurs most of the time without us being aware of it, as a reflex. But in fact swallowing is a very fine and precise mechanism, which can be disrupted in a number of neurological conditions, the most common and dramatic one occurring after a stroke, but also in dystonia.

A few definitions:

1-    Doctors talks about swallowing difficulties as dysphagia, which is the difficulty moving food from mouth to stomach.

2-    Swallowing difficulties can be severe leading to a misdirection of the food to the nose, or to the larynx (voice box) and the lungs, resulting in infection of the lungs called aspiration pneumonia (as the food has been sucked int the airways).

3-    Swallowing difficulties are silent when there is no manifestation which will make the person aware of the swallowing problem, such as cough, choking, needed to drink to push down the food.

4-    Swallowing difficulties are subclinical when the signs are so mild that they are overlooked such as cutting food into small pieces and taking a sip of fluid with each swallow.

 What are the essentials for good swallowing?

1-    Of course the machinery such as  the tongue, the mouth, the throat (the pharynx) the voice box (the larynx), the oesophagus (gullet) have to be healthy. Issues apart from neurological problems, such as a tumour, could obstruct the swallowing process.

2-    The nervous system has to be able to conduct in an orderly fashion the different sequences of the swallowing, like a conductor with his musicians; only one “ fausse note” can disturb the all process. It’s called neurogenic dysphagia.

What are the sequences of the swallowing?

image

1-    The oral phase: the food is collected in the mouth as a soft mass of chewed food, called a bolus.

2-    The pharyngeal phase: the swallowing reflex is triggered by the bolus touching the back of the throat, which give a signal to the brain ( cortex and brainstem) to start the safe guard process: the airway needs to be protected by elevating and closing the larynx and by lifting the soft palate to close the back of the nose. The door to the oesophagus (the crico-pharyngeal sphincter) opens to let in the bolus which has been propelled downwards by the back of the tongue and the pharyngeal muscles.

3-    The oesophageal phase. The bolus then passes though the gullet and into the stomach. This is under the control of the autonomic nervous system

How can swallowing difficulties be assessed?

1-    -During a consultation, a speech therapist will examine the tongue strength, gag reflex, voluntary cough, and speech and perform a trial of swallowing (water, puree) and/or a 3 ounces (90 ml) water test.

2-     FESS (fiberoptic endoscopic evaluation of swallowing).

A fine “spaghetti “( fibre-optic) tube, with a camera at the end, connected to a screen, is introduced by the nose and passed gently down to the throat. With the camera in place the patient is asked to swallow a food of different consistencies.   The food is normally dyed blue or green to make it easy to see against the pink of the gullet.  The speech therapist performing the test can see where it is going, particularly if it is getting stuck or going down into the airway (aspiration) .

3-    Videofluoroscopy is video-radiography of the mouth, throat and oesophagus when the patient swallows a range of foods (biscuit, puree, yoghurt) and liquids that are coated in barium.  The barium shows up on the x-rays (radio-opaque). On the video the radiographer can follow the trajectory of what has been swallowed.

4-  Recording the swallowing process with electrodes in the muscles under the chin and movement sensors on the Adam’s apple can give a good indication of the timing of the different swallowing phases.  It’s called an electrophysiological assessment.

image

Posterior aspect of the base of tongue and larynx , after removing the pharynx http://commons.wikimedia.org/wiki/File:Slide10vvv.JPG#mediaviewer/File:Slide10vvv.JPG

What about swallowing difficulties in dystonia, in untreated patients?

Very few studies have addressed this topic. Most have concentrated on the swallowing difficulties in dystonic patients following treatement with  Botulinum toxin injections.

I will present 5 studies of swallowing difficulties in patients with cervical and craniocervical dystonia, before treatment.

1-Riski et al (1990) studied 43 patients with cervical dystonia with videofluoroscopy and found that 51% of the patients had swallowing difficulties; in 16% the difficulties were subclinical (not known by the patient). The patients had mainly a delay in triggering the swallowing reflex and some residue in the vallecula (a groove, behind the base of tongue, usually cleared by the posterior movement of the base of tongue).

2–Comella et al (1992) studied 18 patients with cervical dystonia with videofluoroscopy before and after Botox injections; 11% patients had clinical symptoms of swallowing difficulties and 22% had radiologic signs of peristaltic abnormality.. after Botox (see below), the signs and symptoms of dysphagia in these patients did not change, but an additional 33% developed new dysphagic symptoms and 50% of the patients developed new peristaltic abnormalities by radiologic studies.

3- Munchau et al (2000) studied 12 patients with cervical dystonia undergoing selective peripheral denervation with videofluoroscopy. 90% patients had swallowing abnormalities with 58% subclinical. The patients presented as in the Riski’s study, with delayed initiation of the swallowing reflex and abnormal tongue base posterior movement, in particular in patients with saggital shift of the head.

The subclinical dysphagia was mainly cutting food into small pieces and taking a sip of fluid with each swallow.

4–Ertekin et al (2002) studied 25 patients with cranio-cervical dystonia with electrophysiological assessment. . 36% had clinical dysphagia and 72 % had abnormal swallowing on electrophysiological recording with again delayed swallowing reflex ; also the sphincter ( the crico-pharyngeal muscle) which opens the oesophagus was hyperactive, instead of being relaxed during swallowing to let the food going through.

Ertekin developed the concept of the dysphagia limit. In this simple test patients are asked to swallow volumes of water ranging from 1ml to 20mls.  In patients with clinical swallowing problems only 1-10 mls can be swallowed in one go. In subclinical patients 10-15mls can be managed.  All normal subjects could swallow 20mls in 1 gulp.

5-Cersosimo et al (2005) studied 20 pts with Meige syndrome with videofluoroscopy. 90% patients had abnormal swallowing compared to 40% in a healthy population of same age. They found also residue in the vallecula as in Riski’s study

What about swallowing difficulties in dystonia in Botox dystonic patients?

Botulinum toxin injections, by weakening adjacent muscles, have been responsible of transient dysphagia in dystonic patients.

The different types of dysphagia depending of the sites of the Botox injections have been detailed in a previous post.

Conclusion

Swallowing difficulties are often silent or subclinical in dystonic patients. Patients and doctors need to speak about them as these swallowing difficulties can be worsened by Botulinum toxin treatment administered for the treatment of the dystonia.

Maybe every injector needs to offer a drink to their dystonic patients before treating them! It may be well received…..

image

 

 References: 

Swallowing function in patients with spasmodic torticollis.; Riski JE1, Horner J, Nashold BS Jr; Neurology. 1990 Sep;40(9):1443-5

Dysphagia after botulinum toxin injections for spasmodic torticollis: clinical and radiologic findings.; Comella CL1, Tanner CM, DeFoor-Hill L, Smith C.; Neurology. 1992 Jul;42(7):1307-10

Prospective study of swallowing function in patients with cervical dystonia undergoing selective peripheral denervation; A Munchau, C Good, S McGowan, N Quinn, J Palmer, and K Bhatia; J Neurol Neurosurg Psychiatry. Jul 2001; 71(1): 67–72.

Oropharyngeal swallowing in craniocervical dystonia, C Ertekin*, I Aydogdu**, Y Seçil, N Kiylioglu, S Tarlaci, T Ozdemirkiran J Neurol Neurosurg Psychiatry 2002;73:406–411

Swallowing disorders in patients with blepharospasm.: Cersósimo MG1, Juri S, Suárez de Chandler S, Clerici R, Micheli FE. Medicina (B Aires). 2005;65(2):117-20

http://www.infodystonia.com/post/24333066534/swallowing-difficulties-after-botox-injections-what-to

 

 

 

Strangulated and whispery voice due to laryngeal dystonia. What does it mean for the patient?

Dr. Marie-Hélène MarionLeave a comment

               Our voice, like our handwriting, is an individual signature.

Just answering Hello on the phone is enough for our relatives or closed friends to identify who is speaking.

The daughter or the son of a friend, who speaks just like their parents, sometimes mistakes us. Study on monozygotic twins showed that the voice pitch can be a familial characteristic, which can be useful in the identification of twins.

 Also our voices may express our emotions, such as anger, stress, and happiness like our facial muscles do, mainly through the pitch of our voice. There are studies in the world of forensic science on vocal stress analysis. But none of them have found so far a reliable way of detecting lie based on recording laryngeal micro-tremor.

 Also our accent told people where we come from geographically and socially.

 So, presenting with a voice disorder can be emotionally difficult as not only the oral communication become laborious, but also the way of expressing our emotions is impaired, and part of our identity is lost.

 –Spasmodic dysphonia, is a dystonic spasm of the vocal cords when speaking and there are 2 main types of spasmodic dysphonia.

1-In the adductor type, the most common form the voice is strained, strangled, frequently interrupted by voiceless pauses, because the vocal cords have difficulties to spread apart when speaking, In that case, the dystonic muscles are the thyro-arythenoids muscles or adductor muscles, which are responsible of getting the vocal cords closed to each other’s.

2-In the abductor type, the less common form, the voice is breathy, with prolonged voiceless consonants because of difficulties with voice onset following voiceless sounds such as /h/, /s/, /f/, /p/, /t/, and /k/. The muscles, which are responsible of spreading apart the vocal cords, are the Crico-pharyngeal muscles or abductor muscles.

 

-Emotional factors can influence spasmodic dysphonia

Stress can precede the onset, or worsen the symptoms, but surprisingly patients report that screaming, crying, laughing, and singing can be normal. This variability of the symptoms can be disconcerting for the patient and raise suspicion of psychogenic pathology (due to psychological problems) in the entourage of the patients and even the doctors. The capricious nature of the dystonic symptoms has to be explained for a better acceptation of the condition .

–Botulinum toxin injections into the vocal cords are the most efficient treatment of this condition, which curiously not only restore the voice fluidity in case of adduction dysphonia but also the voice personality with pitch and accent. Repeated injections every 3 to 6 months are required to maintain a good voice.

-References:

Voice similarity in identical twins. Van Gysel WD, Vercammen J, Debruyne F. Acta Otorhinolaryngol Belg. 2001;55(1):49-55.

 Voice Onset Time Production in Older and Younger Female Monozygotic Twins
Jack Ryalls, Heather Shaw, Marni Simon. 
Folia Phoniatr Logop 2004;56:165-169.

 Voice stress evaluators and lie detection. Hollien H, Geison L, Hicks JW Jr. J Forensic Sci. 1987 Mar;32(2):405-18.

 Voice – How humans communicate? Manjul Tiwari and Maneesha Tiwari , J Nat Sci Biol Med June 2012

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3361774/

Free article

 Patient perceptions of factors leading to spasmodic dysphonia: a combined clinical experience of 350 patients. Childs L, Rickert S, Murry T, Blitzer A, Sulica L. Laryngoscope. 2011 Oct;121(10):2195-8.

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

Dr. Marie-Hélène MarionLeave a comment

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 pneumonia. Also 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

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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.