I wish to all of you and in particular to all the patients affected with dystonia a happy New Year. I hope 2012 will be a year of good response to treatment and very mild difficulties due to the condition.
Dr Marie-Helene Marion
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.
Management of arm spasticity in Liverpool
9tH December 2011
Last Friday, I went to a Masterclass of Management of arm spasticity organized by Professor Paul Mc Arthur hand plastic surgeon and Ms Jane McPhail, consultant maxillo-facial prosthetist.
It amazed me how much I always learn from colleagues from other specialties.. We all learn the same functional anatomy of a given muscle, so what’s make the view so different between a neurologist, treating a writer’s cramp and a hand surgeon treating a spastic arm?
Spasticity and dystonia have a different origin. Spasticity in adults commonly followed a stroke with lesion of the cortex (the skin of the orange!). Writer’s cramp is a focal dystonia, which is due to a circuit problem between the deep nuclei (Basal ganglia-the seeds of the orange!) of the brain. Oppenheim, 100 years ago defined the word dystonia as an increased tone different from what was seen after stroke.
The difficulties encountered by these patients are very different.
Dystonic patients such as Writer’s Cramp have problems when writing, but otherwise are fine for other tasks and at rest. Patient with spastic arms have a permanent disability at rest with pain, discomfort and abnormal posture, interfering with every action requesting skills and strength.
The abnormal posture has nothing in common…the spastic arm present with rigid flexion of the elbow, arm in pronation (palm of the hand twisted and looking to the floor), and flexion of the wrist and fingers . In case of a dystonic arm, the abnormal posture is not fixed and will be present only when writing. Sometimes the dystonia is more severe and the arm has a mobile, variable abnormal dystonic posture when arm outstretched and when walking; the whole arm is twisted in pronation but without a fixed flexion of the elbow, the wrist in flexion and the fingers can be flexed or extended, animated with slow movements.
But despite all these differences, they both benefit from Botulinum toxin injections…
So the approach of the treatment has to be adjusted to the condition
Spastic arm: I learned from professor McArthur the concept of function unit to understand at best the spastic arm, with the biceps, brachialis and brachioradialis for the arm flexion, the pronator teres and the flexor carpi radialis for the arm flexion and pronation, and the synergy between the lumbricals and the flexor digitorum communis . Also I had the demonstration that ultra sound can be crucial to target the spastic muscles precisely as the arm is permanently in an abnormal posture, modifying normal anatomical reference. The electromyogram is not helpful, except the stimulation, as the voluntary command of the arm is poor.
Dystonic arm: My experience of watching and treating dystonic patients taught me, on the other hand, that dystonic arm requires to be assessed during the task which triggered the dystonia, that 1 or 2 muscles in spasms can lead the all arm in a dystonic spasms, and that electromyogram is extremely helpful to document the dystonic bursts in the arm; the selection of the muscles is complex as the posturing is variable , but targeting the muscle itself under e;ectromypogram is not as difficult.
Confronting experience and developing concepts between clinicians from different specialties, are invaluable, and the Liverpool course confirmed that looking from another perspective brings new ideas.
Also spending a night in Liverpool at the Hard Days Night hotel, having breakfast surrounded by the Beatle’s portraits make you feel very far from London.
Marie-Helene Marion
London BTX centre
Professor Paul McArthur training course:
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 (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
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.
Infodystonia 