Myasthenia Gravis

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Myasthenia Gravis

 

http://www.mgauk.org/main/mg.htm

 

Myasthenia Gravis is a disease which appears to the sufferer to weaken the muscles.

 

It is a disorder of the junction between the nerve and the muscle, caused by the body's immune system attacking a vital part of this junction - this impairs the ability of the nerve to cause muscle contraction, and hence produces the apparent weakness.

 

The cause of this auto-immune reaction is not yet known.

 

http://www.mgauk.org/mganews/0104-03.htm

 

Myasthenia Gravis - What Is It? A Brief Overview

Dr Ian Hart

 

Consulting Neurologist at The Walton Centre, Liverpool

 

Introduction

The defining feature of myasthenia gravis is painless muscle weakness that fatigues - in other words the muscles weaken more rapidly on use than is normal. In addition, the symptoms typically worsen towards the end of the day and after exercise. Myasthenia gravis affects about one person in every 10,000 in the UK. The disorder can start at any age from childhood onwards and the chance of developing it increases with age. In the younger age group, women are affected about twice as often as men are. However, in later life it is commoner in men than women. The prognosis of myasthenia gravis is good. Using the array of therapies that are now available, more than 90 percent of patients return to near-normal function.

 

Symptoms

Between patients, the disease varies widely in severity and pattern of progression. In the early stages, the weakness can be intermittent. Often the first sign is drooping of the eyelids or double vision. About 15 percent of patients only ever have eye muscle weakness – this is called ocular myasthenia. The others also have more widespread weakness - generalized myasthenia. These patients can develop weakness of the face, swallowing, and chewing muscles, slurring of speech, and weakness of the limbs and neck. In severe cases, weakness of the breathing muscles can occur. Problems with swallowing or coughing can cause choking. Chronic fatigue without weakness is not a feature of myasthenia.

 

Causes

Myasthenia gravis is an autoimmune disease caused by abnormal antibodies carried in the blood stream. Nerves release a chemical called acetylcholine that activates receptors on muscles to trigger contraction. The myasthenia antibodies interfere with this process by binding to specific sites on the surface of the muscles. The commonest antibodies are directed against the muscle acetylcholine receptor. In 75 per cent of patients, the abnormal antibody production is associated with abnormalities of a gland in the chest called the thymus, which is part of the immune system. About 10 per cent of patients have a tumour of the thymus (a thymoma) that is usually benign. There are very rare genetic abnormalities that cause problems similar to myasthenia gravis. These diseases are called congenital or inherited myasthenias and usually present in infants.

 

Diagnosis

In most patients, blood tests can detect the antibodies that cause myasthenia gravis. Electromyography (EMG) is a very sensitive method to assess the changes in muscle electrical activity caused by myasthenia. Sometimes an injection of the drug edrophonium hydrochloride is given (the Tensilon test), which causes a swift but brief improvement in most patients’ muscle weakness. Many patients have a chest CT scan to assess the size and shape of the thymus gland.

 

Treatment

Acetylcholine is broken down in the body by enzymes called cholinesterases. This action can be blocked by anticholinesterase drugs such as pyridostigmine. These drugs can control myasthenia in some patients but many others need additional treatment. Surgical removal of the thymus gland (thymectomy) is performed in patients with a thymoma and can also help some other patients, especially those who develop myasthenia before the age of about 45. Drugs such as prednisolone (a steroid) and azathioprine that suppress the immune system are often used in patients with disabling weakness, especially those who do not have, or fail to respond to, thymectomy. When rapid improvement is needed, for example severe weakness causing breathing or swallowing problems, patients can be admitted to hospital for plasma exchange, which removes antibodies from the blood. Another option is an infusion of intravenous immunoglobulin. The benefits of these emergency treatments last only for about six weeks.

 

MGA NEWS Winter 2001

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http://www.mgauk.org/mganews/carer.htm

 

Living With Myasthenia Gravis: A Caring Partner's View

Esther Leslie

 

I live with Myasthenia; at first it seemed to rule my life, all our lives. I do not have the illness - my husband Jim is the one who has MG. At first all help, advice and comfort went to allay his fears and get him back on his feet again.

 

What about the fears of the FAMILY, at first getting a name for the illness was a great relief, no more "24 hr/48 hr/72 hr Virus", "it’s all in his mind" etc etc. We would get help now or so I thought. Month after month going to Hospital and seeing a Neurologist while Jim got worse, I could see him going from a person who could climb mountains to a person who could hardly walk around the garden. Why was Jim not responding to the treatment? Life got worse for all of us, where would it all end? Our GP finally arranged for him to go to the Royal Free Hospital in London. He had to go to London on his own by train; thank goodness we have friends in London who would meet him at the station, I was so worried, I could not leave the children and I felt so guilty, putting Jim on the train by himself as he was so ill.

 

Would he come back to us, or if he did what would he be like? My chin had to go up & up. A brick wall was slowly being erected around me, trying to be the strong one in the family for my husband and the children; it had always been Jim who looked after us all.

 

I knew nothing about the illness. Worry is not an easy bed fellow. Thank goodness I have good friends, I could go and have a good cry and get my worries off my chest. It was a very long six weeks, I was now back in the working world after a break of 15 years, I was not feeling very well and our GP thought I had burned myself out with all the worry, I began to lose weight. By the time Jim came home I had lost one stone, I was diagnosed as having Thyrotoxicosis..

 

When Jim came home he was not very well, but better than he had been, but changed. No longer the healthy out-going person; mood swings, black moods all very hard to understand. I was so tired and still working, the children at school not understanding what had happened to their father, a mother who was tired and shouting WHY?, WHY US? The hospital gave Jim a booklet telling us about MG. This helped to explain what was happening to Jim, but at the same time did not lessen my fears - respirators, wheelchairs - where did it all end, what would become of us.

 

Our eldest daughter Karen would not talk about her father’s illness, our son Ewen became very angry, our youngest daughter Clair went off the rails slightly. The worry and not feeling well, the rows, arguments all this did not make happy families, I was at my wits end, trying to keep us together as a family. Friends were very good at listening to me, but they did not understand about MG. One day I found myself in the basement kicking a cardboard box, tears streaming down my face (I have to admit now that it got rid of a lot of frustration). Another day after yet another row with our youngest daughter I went to get the car our of the garage, I sat in the car and turned on the engine thinking how easy it would be to end it all here and now. Realisation set in. This would not be fair to Jim, he needed me more, but life was so bad nothing seemed real. What was going to happen to us. For six months he had been off work, what did the future hold.

 

I sat down the next day and had a good long think, there must be something good in my life, what? Look for one good thing, Jim still loved me, the children were not bad, I had good friends - there were three good things in my life! Yes I would take it from there, one day at a time - look for something good each day. I was now feeling better, I was in balance with the Thyroid, Karen had passed her "A" levels, Ewen his "O" grades, we were all coming to terms with MG. We were pulling together, being there for each other, Karen still not talking about her dad’s illness, Ewen less angry, Clair still a worry at times, yet life went on. We all came to terms with MG each taking a different route. A long year had passed and Jim went back to work, I stopped working full-time and began a part-time job, hopefully my worries were over. I came home one lunch time to find Jim ill again and a very worried Clair. I telephoned our GP and went back to see Jim, I knew at once that something was very wrong, his throat muscles had collapsed, a 120 mile dash to the Southern General Hospital in Glasgow followed. The worries came surging to the surface again, can I cope? Can I be strong again? At least this time I could drive to Glasgow to see Jim.

 

While Jim was in hospital the ward sister asked him if he would like to have a chat with a gentleman from "BAM" (now MGA), the gentleman introduced himself as Alex Jenkins the Scottish Councillor. The upshot of this meeting was Alex came to visit us at Fort William along with the late Christina MacMillian then Care Officer for Scotland. What a wonderful day that was, Christina and Alex put our fears to an end, Jim had a good chat with Christina and I with Alex. He had been in the same position as myself, as his wife had MG, Alex knew exactly what I was talking about. I could see quite clearly now there is life after MG. I repeat there is "LIFE".

 

Jim was asked by Christina and Alex if he would become a representative for the Highland and Islands, we became part of the MG family. After two years Alex stepped down as Scottish Councillor and Jim was voted in. This was a huge step as he still tired easily and could not travel very far, but we could keep in touch with everyone by letter or telephone. By this time Jim had retired from work, Karen and Clair married and Ewen working, all fundraising for MG along with friends, a Sponsored Walk one year, a Fancy Dress Climb of Ben Nevis next year, life was jogging along, fears and worries sliding to the back of our minds. Once again MG reared its head and again Clair was in the house when Jim’s throat muscles collapsed, thankfully he did not go into hospital this time.

 

Clair has been a great help to me, from being such a worry she has turned into such a caring person and will listen when I am worried about her Dad, it has taken Karen a long time but she can now talk about her Dad’s illness and Ewen is always there to help. Jim has had quite a good spell during which we set up the Scottish Forum bringing the branches and representatives together. Then Jim was asked to become a Trustee for the Charity by Geoff Buckley, he accepted (what a long way he had travelled in fifteen years). One draw back was that he could not travel such a long way on his own, so I had to go with him, later I was asked to become a Trustee, what a daunting thought, what could I bring to the Charity? Then I knew "the families" I could talk to them, listen to their fears and worries, let them know you can have life after MG. Take life one day at a time, it may not be the life you hoped for but you can go on holiday, to weddings etc, say yes to all invitations, we will be there if Jim is well, do all you want to do on the good days, lie low on the bad days, change, adapt, adjust your lifestyle, have lunch out if you can’t go out for dinner. Jim can drive again, at first it was only a short distance then he could do a bit more, but we always share the driving even when he is well.

 

I now find life can be good, but it is the simple things in life that gives me most pleasure, having my husband and family (which has grown, we now have eight grandchildren). In fact living with Myasthenia is not as bad as I first thought, we have all pulled through, the family all involved in some way. Clair did the drawing for our RAG DOLL LOGO, Karen, Ewen and Clair along with friends have just done a parachute jump to raise funds for MGA. I know life with MG will have its up and downs but it will never take me down with it again, I have the MG Family behind me, I only have to make a telephone call and I have all the help and support I need to take me through the next crisis. But mostly I have learned to live with MG.

 

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Note from MGA News Editor

 

Esther and Jim are both Trustees of MGA and Members of the MG Council.

The debt Myasthenics owe to herbalists and poisoners

Dr Maria Elena Farrugia, Dr Ian Spreadbury and Professor Nick Willcox

 

The MGA/ MDC Centre, Oxford

 

In a recent article, we saw how researchers have taken advantage of venoms from creepie crawlies to help us in diagnosis and even treatment. Now it is the turn of the plants. As before, poisons have provided vital starting points for investigating how nerve ? muscle triggering works normally, and some have proved even more crucial friends in treating myasthenia.

 

A brief reminder

 

Figure 5: Nerve- Muscle Junction

The ignition system of our ‘voluntary muscles’ (See Fig 5) depends on the chemical transmitter ACh (the ‘ignition keys’) which is released when the nerve endings are switched on by impulses from the brain. The ACh then latches into special receptors (‘AChR’ = ‘ignition locks’) on the muscle surface – and that triggers the muscle to contract. The spare ACh is broken down by ACh esterase (AChE). The muscle AChR is called ‘nicotinic’ because it is targetted by nicotine from the tobacco plant (Nicotiana affinis). In most myasthenics, the numbers of these AChRs are too low – whether because of an immune attack or an inherited fault.

 

Licensed to kill…

How poisoned darts helped to explain the defects in MG

 

Fig1: One of the curare trees - Strychnos toxifera

Many poisons belong to the large family of 'alkaloids'. One key player is ‘curare’ (it rhymes with Harare in Zimbabwe). It has long been used as an arrow poison by South American Indians (eg in Ecuador and Peru); its name means a venomous decoction, and it is boiled down from the barks of various trees. Sir Walter Raleigh and others mentioned it, but its preparation was first described by the versatile Alexander von Humboldt, in 1807. He was one of the last universal scholars in the natural sciences – not only a geographer but also a naturalist, botanist, author, artist and sociologist. Derived from the trees Strychnos toxifera or Strychnos guianensis and Chondrodendron tomentosum . (See Fig 1), curare was used so much by warring tribes and hunters that it was very valuable. Using arrows and silent blowpipes, they could kill birds in only ~ two minutes, small mammals in ~ 10, and larger ones in ~ 20 minutes.

 

The famous French researcher, Claude Bernard, discovered in ~ 1855 that curare paralyses voluntary muscles but not the heart, and kills by stopping breathing. He concluded that it affected nerve ? muscle triggering. We now know that it simply blocks nicotinic AChRs, and so makes muscles floppy – exactly as in MG when the AChR numbers are too low.

 

….. and to save lives…

 

Figure 2: The Colabar Bean

Now we come back to our heroine, Dr Mary Walker. You may remember (from the last issue of MGA News) that she was discussing a new myasthenic (with the Neurologist Dr Denny Brown). They came to the conclusion that myasthenia seemed like poisoning with curare – to which myasthenics are super-sensitive. Desperate for some treatment – because MG was then often fatal – she injected another plant drug, physostigmine, because it had been the best known curare-antidote since ~ 1900 (as shown by the Viennese Dr J Pal). When she tried it, the patient improved dramatically, a breakthrough she published in the Lancet in 1934. That drug is very similar to 'Tensilon', and is a short-acting version of the myasthenics' dear friend Mestinon (properly called pyridostigmine); this relative of physostigmine was first used in 1954. Mary Walker quickly realised that these drugs worked by blocking AChE, so delaying the breakdown of the ACh and boosting its chances of triggering the muscles – rather like using more choke when the car is cold.

 

Figure 3: Physostigma venenosum

Physostigmine comes from the West African Calabar bean (Physostigma venenosum), and was used there for trial by ordeal, rather like ducking suspected witches under water in this country. (See Figure 2 and 3). In Edinburgh in ~ 1855, it had been shown to constrict the pupils (by enhancing the effects of ACh, as we now know). In 1927, an American myasthenic, Harriet Edgeworth, tested another herbal drug on herself called ephedrine; for the next few years, it was the best hope for myasthenics. It soups up the muscle ignition slightly because it mimics the effects of adrenalin – the ‘fight, fright, flight response’ that you all know so well (pounding heart, sweating, hair standing on end….). It also helped Harriet to tolerate heat much better.

 

Ephedrine is derived from various Ephedra species, especially E. sinica. Also known as Ma huang, it has been used in Chinese medicine, probably as far back as 2800 BC, to treat colds and asthma; its cousins are still used as de-congestants for ‘bunged-up’ noses and asthma. Ephedra was used by the Zen monks to encourage calm concentration during meditation and by Gengis Khan’s bodyguards to keep them alert on sentry duty. Other alkaloids have less desirable side-effects; some are dangerous in MG, eg muscle relaxants or pain-killers such as Acetaminophen and Oxycodone, and also some herbal remedies.

 

Understanding the side-efffects of Mestinon...

 

Fig 4:Amanita Muscara

Many ‘automatic functions’ also depend on ACh as transmitter; they include pupil contraction (in the eye), slowing of the heart and stimulating gut and bladder movements. They are not affected by the MG, because these ‘muscarinic’ AChRs are completely different from the nicotinic AChRs in muscle; unlike them, they are stimulated by the toadstool poison, muscarine. (See Figure 4). Its effects include diarrhoea, gut cramps and sweating – rather like a mestinon overdose (see below). It is one of several poisons in a toadstool you know well – the one with the red cap and white spots beloved of garden gnomes – the Fly Agaric (Amanita muscaria). Figure 5 shows the nicotinic and muscurinic Acetylcholine receptors.

 

…. and treating them too…

Two lovely big eyes?

 

Fig 6: Atropa Belladonna

Atropine comes from a plant you probably know, Deadly Nightshade (Figure 6); it was called Atropa belladonna because the wide pupils enhance the beauty of a comely girl. It is now used as a ‘pre-med’ before anaesthetics/surgery because it dries up the juices in the mouth and lungs, and so prevents blockages and infections in the airways. In conclusion, no matter how lethal such poisons may be, they may well have their uses in diagnosis and treatment. What is more, we should all be grateful to all the herbalists, collectors and researchers for investigating them. How many further valuable drugs are now at risk because of extinctions?

 

MGA NEWS Winter 2002

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http://myastheniagravis.org/education.htm

 

Myasthenia Gravis can strike anyone at any age. MG is a highly misdiagnosed autoimmune disease in which communication between nerve and muscle is impaired, causing weakness. Its primary symptoms, alone or in any combination are: droopy eyelid(s) , weak arms, hands, neck, face or legs, difficult chewing, smiling, swallowing, talking, breathing or shallow respiration, blurred or double vision, sense of balance difficulty or undue fatigue. MG can masquerade as overwork, under-rest, anemia, vitamin deficiency, disease of the involved organ(s), or even emotional disturbances. Its cause is unknown; there is no cure. Learn more about MG.

 

Myasthenia Gravis Foundation of America (MGFA) was founded November 10, 1952 by Jane Dewey Ellsworth because of her daughter Patricia who has MG. The MGFA, a non-profit voluntary health organization has as its purpose to serve patient needs, promote public awareness, provide lay and medical education and provide for research. The MGFA is the only organization dedicated exclusively to fighting MG.

An important part of the mission of the MGFI is to education and provide information to the public. If you would like additional information on any of the public education and information items described please click on the link or call the office at the number below.

 

The Myasthenia Gravis Foundation of Illinois, Inc. (MGFI), Chapter of the MGFA, was formed October 29, 1972 by a local group of caring individuals. We are a part of the network of concerned and dedicated volunteers who strive in behalf of the best interests of the MG patient. The MGFI offers the following public educational services:

 

Congressional Support There are often medical issues that can be improved through the actions of our congressmen. One such issue, for example, is IVIG injections in-hospital vs. in-home. If you would write a short letter to your congressmen about your position on this or any MG issue, it would be most appreciated.

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There is much more at these sites.

 

There are so many things we have no idea about, never heard of before even.

 

 

[Mother]

Snowmom, I'm sure you didn't know when you posted this that it would touch very close to home for me. I do not have MG but the letter, from the wife who's family is dealing with it, is a letter that could have been written by almost any memeber of a family of someone who has a chronic disease, be it Lupus, MS, Chronic Fatigue, Fibromyalgia, diabetes, heart disease, lung disease or almost any other. When a family member has a debilitating illness, the whole family lives with it. The illness seems to dictate to every facet of life and she is right, the only way to deal with it is to get on with life the best you can. I don't know how many times I have said or my DH has said we will be glad to be there if I feel up to it. We make plans, believing and hoping that I WILL be up to it.

 

I hope that even those who are not interested in learning more about this particular disease will take time to read that part for it tells more than just about MG, it tells about a family that had to learn to roll with the punches, who had to finally come full circle to using the disease to do good in other people's lives. It is extremely difficult for a person to have a disease of this sort. The pain and misery, both physically and emotionally, is ongoing and pervasive but it seems to validates one's suffering if it can be used for good.

 

Thank you so much for posting this, Snowmom.