Lid watching, a lost art!

 

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The diagnosis of blepharospasm is not always easy, in particular in case of pretarsal blepharospasm, also called Apraxia of eyelid opening, mistaken quite often for ocular myasthenia.

1- what is ocular myasthenia?

Myasthenia is an autoimmune disease, responsible of muscle weakness, affecting the all body and when localized to the muscles around the eyes, called ocular myasthenia.

Ocular myasthenia can present with isolated droopy eyelids (medically called ptosis), due to a weakness of the levator muscles of the eyelids, the Levator Palpebrae, but is often associated with double vision, due to weakness of the oculomotor muscles. The weakness is worst at the end of the day, due to the fatigue of the myasthenic muscles.

The diagnosis of ocular myasthenia is based on clinical findings, the presence of antibodies in the serum (30 to 60% cases) and the abnormal response of the muscles on repetitive electrical stimulation. Clinically the doctor can look for the “peek sign” by asking the patient to perform a sustained gentle eyelid closure; the fatigue of the orbicularis oculi will be responsible of a slight opening of the eye, as the patient peeking.! ( J. Glaser).

2- why so many patients with Blepharospasm are misdiagnosed with ocular myasthenia?

-Blepharospasm patients can have a misleading presentation of ptosis, with intermittent droopy eyelids, due to the spasms of the pretarsal portion of the orbicularis oculi muscles (also described as pretarsal BSP) pulling down the eyelid like the string of a roller blind down to the window.

-The words that patients used to describe their symptoms such as “my eyes are tired”, “my eyelids feel heavy”, “I feel more comfortable eyes closed” can indicate wrongly a muscle weakness of the eyelids, and reflect in fact the loss fight of the patients against the dystonic spasms closing their eyes.

– the variability of the symptoms through the day ; in myasthenia the patient is worst at the end of the day; in BSP the patient is worst walking outdoors, driving, watching TV, looking up and with any bright light and dazzy winter light.

– A levator palpebre detachement can occurs on some patients after years of spasms, and pulling on the muscle insertion

3-the art of lid watching

The famous neuro-ophtalmologist, J Glaser talked about “ Lid watching, a lost art!” Joel Glaser in Handbook of neuro-ophthalmology, 1999

-Lid watching is optimal when patients are keeping silent; BSP patients have an increased rate of blinking when being quiet and vice versa a low blinking rate when speaking. It’s the opposite of what observed in normal subjects. That may explain why patients find easier to keep her eyes opened when speaking and choose to sing when driving.

-In the case of pretarsal BSP careful lid watching along the eyelashes, looking for a pulling down spasms of the eyelids is a good indicator of pretarsal spasms.

– In addition, the lid closing spasms may be associated with Bell’s phenomenon with elevation of the eyeball, so it’s important to look not only at the eyelid but also the eyeball

– In case of levator palpebre detachment, the forceful opening of the eyes on command will be limited;

– Also the patients sometimes can’t reopen their eyes, after a spontaneous blink or after a closing spasm. It can be for a fraction of second or for up to few minutes; the patient will flicker his eyelashes, or his eyelids to kick them opened. Sometimes it will be a forceful pulling of the eyelids with his fingers, stretching the skin around the eyes, even sometimes resulting in bruising.

All these signs found on careful lid watching are good indicators of eyelids spasms, and not eyelid droopiness. They also direct the hands of the injector to the maximum spasms to achieve optimal results.

In the movement disorders field, most of the diagnosis are made by watching the movement; it’s particular true for eyelid dystonia and vocal cords dystonia where immobility does not always equal paralysis but also permanent tension due to the dystonia; one day I will ask my ENT colleagues to tell us about the art of vocal cords watching!!!

Marie-Helene Marion

 

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Meeting the experts in Guildford, UK: What about blepharospasm?

Last weekend, 15 movement disorders clinicians from Europe, Russia, USA, and Canada gathered in Guildford, to share views on Botulinum toxin treatment for focal dystonia., This meeting was sponsored by Merz Pharma.

Dr Elin Forsaa,Dr Svetlana Khatkova, Dr Torsten Grehl, Dr Flavia Coroian, Dr JM Meyer, Dr MH Marion, Dr Dirk Dressler, Dr Sylvain Chouinard,Dr Robert Chen, Dr Sophie Sangla, Dr Said Bensakel, Dr Shyalmal Mehta, Dr Olivier Simon, Dr Richard Evans, Ms Muna Bitar

I was asked to present the key therapeutic challenges in blepharospasm and prof Dressler from Hanover was presenting the therapeutic challenges in cervical dystonia.

What came up in the discussion watching many videocases,  was the clinical diversity of the blepharospasms, which raise a lot of questions.

1-Are the young onset blepharospasm , affecting women before the age of 50, different in their progression and response to treatment?

2-How some patients with primary blepharospasm can be still focal, affecting only the eyes after 10 years of duration and other patients (31% in Defazio study) see their dystonia spread to the lower part of the face, in the first 5 years after the onset?

3-How some patients are mainly “” blinkers” and others “shutters” sic a patient?

In another words is there a group of patients who started with increased blinking, following dry eyes and photophobia, and some who never complains of over blinking, who have no sensory symptoms, but can’t reopened their eyes.?

4-How the dystonic spasm around the eyes can be alleviated initially by speaking or singing, then becoming triggered and worsened by speech when the dystonic spasm has spread to all the face (Meige Syndrome) in a same patient over the years.

5-Are both blepharospasm and migraine which share the same sensitivity to bright light are coincidental when occurring in the same patient or do they share some common pathophysiology of central trigeminal desentizisation?

6- is the Bell’s phenomenon seen in some patients, following an improvement of their spasms after Botulinum toxin injection, is a marker of a persistent underlying dystonic activity as a reflex from the brainstem.

7- Is stimulating, the Muller muscle, which is a smooth muscle which plays a role in lifting up the eyelid, with Aproclonidine eye drop can help patients with so-called apraxia of eyelid opening?

Previous studies , in particular the work of G. Defazio, from Italy, have given some answers. Patients with blepharospasm have twice the risk of spreading than cervical dystonia and most spread events occurs after the age of 50..

. Patients with Blepharospasm blinked more at rest than during conversation, by contrast with heatlthy volunteers who blinked more during conversation. Two cases of speech induced Blepharospasm has been described in patients with cranial dystonia.

 I found this session very inspiring, pushing me to look further in the clinical characteristics of blepharospasms.  We always quote the clinical complexity of writer’s cramp, where so many different muscles can be involved. I am convince that despite a only muscle involved, the orbiculatrs oculi, Blepharospasm is not a model of simplicity…

We then all went to a pub in Shere for diner, admiring on the way the beauty of the Surrey hills.  May be in the next few years we will meet again to share the answers to our questions…

 

The William Bray pub in Shere (Surrey)

 

Dr Marie-Helene Marion (London) and Dr Sophie Sangla, a leading neurologist in botulinum toxin treatment in Paris: a friendship of 30 years….

 

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.

Meige syndrome

Henry Meige (1866-1940)

A grimacing face … not an expression of pain or disgust but a neurological condition:

A Meige syndrome

 Forceful dystonic spasms of the face, in particular when trying to speak or to eat have been called Meige syndrome.

 Henry Meige was a French neurologist who published as a junior neurologist, about facial tics with his friend Feindel in 1894 and 2 years later with his maitre Brissaud about neck dystonia, which he is called Torticollis mental (1896). From then, he studied patients with facial movements disorders, not only the tics but what he called “les convulsions de la face”. He also kept a fascination for the spasmodic torticollis that he recognized publically as an organic disease in 1929 after seeing patients suffering from Encephalitis Lethargica and reading the work of Oppenheim (1911) on Dystonia Musculorum Deformans.

In 1910 he described a Bi-blepharospasm (what we call now Blepharospasm) to emphasize that both eyes were affected, with sometimes a positive family history and which could spread to the laryngeal, mouth floor, jaw  and even tongue muscles.

Meige syndrome is now an eponym to describe a blepharospasm associated with a dystonia of the muscles of the lower part of the face and the larynx as described by Meige in 1910. Patients with Meige syndrome are patients over the age of 60, who complains of involuntary eye closure when trying to speak or to chew. The speech or the chewing is affected and the tongue is involuntary pulled out. It usually starts with a blepharospasm, which spreads down in 35 % of the cases to the mouth and the neck. The pattern of activation of the eye closure changes from an isolated blepharospasm which is better when the patient is speaking and worst when the patient is silent to a spasm of eye closure when the mouth is activated.

Meige syndrome has to be treated actively with anticholinergic drugs if tolerated, clonazepam and Botulinum toxin injections into all the dystonic muscles of the face (eyes, jaw, tongue, larynx, neck) ; the facial grimaces can settle and patients with Meige syndrome should keep hope for a better future.

More study is needed to understand the long term prognosis of this condition.

For further reading, I advice you 2 papers from 1976 and 1982 of Professor CD Marsden

Spastic dysphonia,Meige disease and torsion dystonia. CD Marsden, MP Sheehy.1982

http://www.neurology.org/content/32/10/1202.extract

Blepharospasm-oromandibular dystonia syndrome: a form of adult –onset torsion dystonia, CD Marsden, 1976

http://ukpmc.ac.uk/articles/PMC492566/pdf/jnnpsyc00174-0060.pdf

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

Are all the blepharospasms the same?

Are all Blepharospasms the same?

 BSP can vary from one patient to another and also change overtime after few sessions of Botulinum toxin injections.In daily life, we can close our eyes in different ways; for instance when we are in the shower and we get shampoo into our eyes (by the way I am not sure that shampoo are still stinging), we spontaneously frown, screw and close our eyes very tight. On the other hand when we go to sleep, we pulled down very gently our eyelids, which feel heavy. In case of a patient with BSP, there is the same diversity; the eyes can closed very tight with a forceful spasm of or closed with only the eyelids pulling down, despite sometimes one finger trying to hold it up.

The orbicularis oculi muscle which is the muscle responsible of closing the eyes are organized in 3 circular parts: an inner circle (the pretarsal part responsible of the eyelid going down), a middle circle (the preseptal part) and an external circle (the orbital part); these 3 parts can contract independently.In the situation of the shampoo in the eyes, it’s the orbital part, which is contracting. When sleeping, it‘s only the pretarsal portion of the orbicularis oculi, which is involved, pulling down the eyelid.

When treating the patient with injection of Botulinum toxin, the sites of the injections have to be placed in the right portion of the orbicularis oculi muscle depending of the type of BSP.  The treatment needs to be customized for each individual, not very far from the world of the haute couture!