Why Spasmodic dysphonia is often mistaken for a functional disorder?

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Spasmodic dysphonia, also called laryngeal dystonia, is a neurological disorder, responsible of a strangulated, strained voice and rarely of a whispering voice, which has a major negative influence at work and in personal life.

Patients with spasmodic dysphonia require to be distinguishing from patients with functional dysphonia and from patients with “muscle tension dysphonia” (MTD) which is also considered a functional disorder rather than a neurological disorder.

Many reasons can explain why spasmodic dysphonia , which is a dystonia of the larynx, is not well recognized by doctors.

1- Laryngeal dystonia is a rare condition (1/100 000), and like other focal dystonia, the diagnosis is clinical; if you have never heard somebody with spasmodic dysphonia speaking, it will be very difficult to recognize it.

2- It mainly occurs in women, who carry the stigma of being more vulnerable for functional disorders.

3- The onset can be sudden in 45% of the cases (Childs, 2011).  Sudden onset is also a characteristic of functional disorders.

4- When the onset is sudden, the majority of patients recognized triggering factors such as stress (42%), upper respiratory infection (33%), and pregnancy and parturition (10%).(Childs, 2011). Stress should be seen as a non-specific triggering factor making patients vulnerable to develop any medical conditions, and not specifically functional disorders.

5-A phobic component occurs very quickly after onset, as the patient avoid answering the phone, and speaking in public as it’s required such an effort to speak in these circumstances.

6-It’s a task specific condition as every focal dystonia,; spasmodic dysphonia occurs when speaking, and voice can be normal for singing or shouting. This task specific characteristic can be disconcerting for both the patient and the doctor.

At the difference, functional dysphonia is responsible of a permanent speech disturbance, with a whispering voice or a hoarse voice occurring when speaking, and impossibility of shouting or singing.

7- “Muscle tension dysphonia” (MDT) patients tend to exert too much effort on their vocal cords when speaking and can present with a strained voice, as spasmodic dysphonia.

But when examining the speech in details, three tasks: sustained vowel /a/, oral reading of a standard passage, and connected speech describing a standard picture have to be performed, as the spasmodic dysphonia could be more apparent in the connected speech situation, compared to MDT.

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It’s very important to diagnose spasmodic dysphonia, as Botox injections into the vocal cords can alleviate the symptoms and restore confidence for effortless speaking.

At the London BTX centre, in Sloane Square, Dr. Marie-Helene Marion offers multidisciplinary clinics with voice specialists for the diagnosis and  treatment of spasmodic dysphonia with Botox injections.

 

 

 

 

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

               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.

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

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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