MG Severity at diagnosis/Treatment success
 

Does anyone know if

there is a correlation between the severity of weakness/Stage of illness at the time of diagnosis and estimated time to get managed/personally adjust?


Is there a correlation between the severity of weakness/Stage of illness at the time of diagnosis and likelihood of remission?


of successful management of symptoms?


Does MG ultimately get worse with age for everyone? I know cant be cured but are their people who do just fine on the same amount of mestinon for the rest of the lives?

As far as I know there is no correlation between the severity of the symptoms at diagnosis and the chance for remission.
It is not unusual for a patient with crisis to fully respond to treatment and go into complete remission.

What does influence the chance for remission is how much time passed since the diagnosis till the beginning of treatment and also the type of MG that you have. MG is not one disease. There is AchR MG and MuSK MG and probably others not yet discovered. (several other antibodies have been found, but it is not clear yet if they can or can not cause MG-such as anti-ryanodine, anti-LRP4 etc. Those are also not tested routinely). AchR and MuSK are very different in many aspects.

MuSK MG is less likely to respond to treatment, AhcR is more likely to respond.

A significant proportion of AchR MG patients do well with a low dose of immunosupressive treatment or can even stop all treatment.

Most MuSK MG patients require higher doses of immunsupressive medications for prolonged periods, have more steroid induced myopathy and a less stable disease with many recurrent exacerbations.

Some patients with relatively mild forms of AchR MG can probably do well with mestinon alone.
Most patients with MuSK do not have a good response to mestinon, and require other medications.

Finally, some patients do not have autoimmune MG but a congenital abnormality of one of the proteins of the NMJ.

For those of us that are seronegative, is there any speculation out there that we may have a different antibody being produced?

Also, what about the congenital protein thing? Would this be present from childhood? Do you have any references?

I suppose that I am obsessive about obtaining new knowledge on this thing that is trying to take over my life. I am sure that we all want to know what our prognosis is.............

Does that imply that if someone begins treatment early on and the disease still spreads that they did not receive correct dosage/the right combination of medications?

Anacrusis

And another question to go with that one? If mestinon is only a short acting, symptomatic treatment, does it count?

My first blood work was Neg. The second said I was positive for binding (6 months later) My bloodwork a couple of months ago showed I was positive for 4 different antibodies. I have taken Imuran, Cellcept, 60mg and 180 time release Mestinon, Prednisone and bunches of IVIG. The only results I got was the 80mg of Prednisone that stopped my double vision.

Different antibody
 

I think MGFA website said yes different as yet unidentified antibodies but I also think it said only about 5% of cases - seems there are more seronegative patients in this group but that could be function of fact that we are seroneg making us more likely to be involved in the group.

For me this thing has already taken over my life (I have not been able to function as normal for at least 2 months now) but I am trying to be somewhat balanced and not be obsessive about reading about it. :-)

Stephanie

Follow-up ques
 

PindPongMan

If you dont mind sharing some personal information, may I ask...

what your stage on the chart/severity of symptons was at the time of the first blood test?

When in relation to the tests did you take the meds?

Was there any notable differences in your general condition when the test later showed positive?

Sorry if my questions seem to be interrogation, I am a litigator and hard for me to turn that off. Thanks - if my questions are too personal, I understand completely.

Stephanie

I personally believe that there is a significant number of seronegative MG patients who are not diagnosed as MG (and therefore are not part of the statistics).

When you assume that a certain combination of tests virtually rules out a certain diagnosis (when in fact the sensitivity of those tests is unknown), there is no way you can know how many patients truly suffer from the given illness.

Possibly you are right that seronegative (as opposed to seropositive) are over-represented in internet forums, but at the same time those who are less proactive and told they are suffering from psychiatric problems, or other " waste-basket diagnoses" (when in fact they have MG) are under-represented.

It is very hard to know what is the true number of neglected MG patients.
I have been trying to find this out over the last few years, without much success.

The patients we are seeing on those forums may the proactive edge of a very large ice-berg. The neruology community is overall very narrow minded and easily dismisses patients with myasthenic symptoms who do not fit the book.
Some of those patients may eventually (years later) be diagnosed as suffering from myasthenia, and probably many are not.

Proposal
 

AliceMD do you ever sleep? :)

You are in better position than I to assess but my experience with neuro resident at UM hospital made me think it may be more of time or productivity demand issue than narrow minded, at least when they are first starting to practice. At intake in the hospital ER, i was quite impressed with how thorough resident was but once the actual neurologist (who I did think was narrow minded, dismissive and condescending) saw me she was not the same.
After the emg test she actually told me I should think it was great news that blood and emg were negative which clearly showed me she didn't get it at all!!
Hearing that I lost all confidence in her desire, ability to help me.

I have been fortunate enough to find a neurologist who is the chair at medical school and was thinking I might suggest i could volunteer to work on thesis/project with
Neuro students to address this type of issue. Any suggestions for study, project, thesis would be greatly appreciated.

Also I was accountant before lawyer and although my typing skills on iPad are not so great, I am great at compiling information into presentable reports. Perhaps we could do some sort of survey of neuro's to compile info the could be useful?

I've been reading this forum for a couple of years, and I've also noticed that we have a seemingly disproportionately high number of seronegative members. I once set up a poll to find out the numbers, just out of curiosity, but I guess people don't really like to respond to polls. It wouldn't have given any real information, anyway, since like Alice says it may be that we seronegatives are over-represented here (since many of us are struggling to get diagnosed) or it may be that we're under-represented (since many are undiagnosed).

I tested negative for AChR antibodies three times, and negative for MuSK and LEMS once each. I think there are a few more antibody tests I could take, but now that I've been on Imuran for a year, it probably wouldn't make sense, and my treatment wouldn't change if we got a positive anyway. But I consider myself most fortunate to have a diagnosis. My second biggest fear was being undiagnosed. My biggest fear was being diagnosed with something psychogenic, like conversion disorder. I know it would make more sense logically to fear the Really Bad Diseases like ALS, but it wasn't a matter of logic.

When I tested negative for MG and didn't have a diagnosis, my first neuro wanted to just keep seeing me every six months, and when I squawked he suggested Prednisone. My second neuro also wanted to just see me again in six months and do nothing. When I came back with my husband and insisted that we do something, he sent me to Boston (an hour and a half away) to an expert, who diagnosed me. I suppose both neuros wouldn't have been so conservative in their treatment if I had had any dangerous symptoms at the time.

Abby

I was actually hoping for my diagnosis to be "conversion disorder". Somehow I think that if my mind has taken over my body, I can find a way to take it back. It is hard for me to accept that this is real.

But when I am late for a pill, I fall over. I had several emergency room visits before the diagnosis. I fell at a music concert in Macon. Went down some stairs. The emergency room people wanted to insist somebody must have pushed me. They were eyeing my husband with suspicion. Then my health insurance wanted to say it was the music venue's fault. It was hard to convince them that I could fall fair and square.

Stephanie
To answer your questions:
1. At the time of my first blood test I was Class III
2. I started Meds when the neuroopto said I had MG even though the first blood test was neg.
3. When the positive test for binding returned my condition was the same as it was when the tests were neg.

That being said my condition has gotten worse. It is hard to tell if it is my MG or the meds. For example the Imuran made me very sick and I lost 35 pounds. On higher doses of Prednisone my legs became so weak I could hardly walk. My neuromuscular Dr said some people can't take prednisone and immediately started tapering me off. So far nothing is working for me. They are being careful with my treatment because of my age (72).

Feel free to ask all the questions you want.
Mike

Survey
 

I think it is sad (even cruel) that there is even one person who would endure years without diagnosis or with thinking these MG problems were in their head (as most of us were told so many times). I am convinced I have had symptons for at least 10 years but none of them individually were disabling to the point I was non-functional until the last year or so so I didnt insist. That said, doesnt mean I was functioning great or even had a great quality of life, just that I adjusted, and in my case, cut out almost all social activities to save strength for work/supporting myself.

I am most thankful my mind is mostly unaffacted (except when I am too weak to focus) because I thrive on thinking up solutions. I think the forum group should brainstorm - clearly this is a highly intelligent group of people.

Off the top of my head, maybe we could setup a thread that stays at top (like link, intros) that "lurkers" could respond to? people who may think they have something wrong. Of course, I realize it may take years before someone finds forum (I only heard Myasthenia Gravis for the first time 7/24/12 and I had been trying to figure it out although I thought it was from the guillain barre syndrome and I totally misread my symptoms because adjusting for my symptoms was so deeply ingrained in me that I no longer even realized I had many of the symptoms).

Anyway, just some things to consider not only for others but for ourselves, since we all will be living with it forever.

Stephanie

very interesting
 

Mike

thank you for your willingness to share...i shall ponder your responses and I am sure I will have some more questions.

Stephanie

Question
 

Did Your doctor have opinion why test was negative then positive later? From my limited understanding about this illness that doesn't seem logical unless test was wrong initially given symptoms were no worse.

Did you have EMG or SFEMG and if so, when in relation to diagnosis and tests, and was that test positive or negative?

Thanks again for your willingness to share.


Ps do u play ping pong and if so, it actually seems like that might be something I could do...always enjoyed it when I play usually on cruises.

My Neuro-opto said that happened sometimes.
I had EMG as one of the first tests about the same time my first bloodwork (neg) was sent in. I didn't get the single fiber because it was 3 hours away. Since then I have learned you can get a neg single fiber and still have MG. That test is very sensitive and difficult for Dr to read.

I played ping-pong the first year but now my condition has gotten so bad I can only play a couple of games and have to go slow because my balance is horrible. I have fallen twice playing and about 5 more times around the house.
Mike

Unfortunately, it is the exact opposite.

neurologists are ready to accept a mild form of MG with negative tests, but not severe and "seemingly" potentially life threatening symptoms.

I know that from my own experience, I have read about it in Chloe Atkin's book and I am sure there are other patients who are not in the position to do much about it. I believe some of them die from " unexplained causes" or from a " psychiatric disease" without anyone ever knowing that they had MG.

This description is taken from the supplementary material of a recent paper.
It is a description of one of the patient's who's anti-MuSK antibodies was used for the study.
She was "fortunate" to have a diagnostic EMG at that point, found to have anti-MuSK antibodies and responded to high-dose steroid treatment. (at least according to this paper which expresses her physician's opinion and not her "subjective" feeling).

In my field of practice such a delay in the diagnosis of a potentially treatable disease would be considered malpractice. In neurology this management of atypical variants of MG is considered the standard of care.

Possibly a small percentage of those patients do have a psychiatric problem, but I am sure that the vast majority of them don't. I think many of them may have a normal emotional response to their illness and may be depressed and anxious due to the loss of their functional ability and the uncertainty of having to live with such symptoms with no help from the medical profession. (and many times the opposite, they are met with disbelief and have numerous humiliating experiences. Their family and friends are encouraged not to trust them as well).

This woman's life and career was destroyed (and we can't know from this short medical description what other devastating effects it had on her life) when she had an illness that could potentially respond to treatment. She could have easily died at home during one of her " anxiety attacks".

Still the DSM-5 has an old-new entity called " functional neurological disorder" which is a psychiatric diagnosis based on the ignorance of neurology as a field. The treatment for this " disorder" is positive thinking and reassurance and possibly antidepressants, anxiolytics etc. I am sure that many undiagnosed MG patients fall under this category. I am sure very few of them " respond" to this very effective management approach.

Neurologists are very forgiving to themselves and their colleagues for missing a serious disease because the patient "presented" with what " appeared" to be a psychiatric illness.

I am not. I think there is no reasonable excuse for missing the diagnosis of potentially treatable illness for 10 years. The patient described above obviously had MG symptoms and was seen by a neurologist (as she was tested for AchR antibodies and possibly had other tests as well).

If they don't have good tests to diagnose this illness, they should work on finding them or at least not dismiss patients who have myasthenic symptoms and normal test results (using tests which have only been verified in patients with classical AchR MG).

This is written in the official MGFA site-clinical overview of MG for health professional.


And yet, most neurologists (including MG experts) have no problem with that. They also have no problem with dismissing patients with clinically apparent MG symptoms and normal test results who have no other reasonable explanation for their symptoms (other than this " waste-basket" psychiatric diagnosis).

It is not the mildest cases in which the diagnosis is delayed for years, but the most severe ones.
Instead of doing everything possible to improve their diagnostic abilities and to find better management approaches for this illness, they are busy convincing themselves (and their patients) that this is the "most well understood disease" and that patients with this illness lead a normal life with very few side-effects of treatment. They are also convincing themselves that patients with a significant delay in diagnosis or those with normal test results could at the most have a very mild illness.
And those that don't have an easy to diagnose and manage illness, have serious side-effect or don't respond to this excellent treatment?-they obviously suffer from psychiatric problems. Such psychiatric problems are an obstacle in diagnosing the illness, assessing its severity, assessing the response to treatment and even an obstacle in the response to treatment, as such patients do not " realize" that they are in fact in remission and keep on insisting that they still suffer from myasthenic symptoms.

Does this sound ridiculous? Does this sound like something I have made up? I am sure it does, but unfortunately it's not.

I am a lurker, I used to be involved a lot on this forum but since I cant get anyone to agree on whether I have MG along with everything else I tend to stay quiet.

I have had numerous tests for MG all negative (Musk, achr, EMG, SFEMG). The only test I am positive for MG is the ice pack test. Place an ice pack on the eye affected by ptosis and magically the ptosis disappears. No one can explain to me why this test works if I don't have MG, as this test proves that something is wrong at the neuro muscular junction. As agreed by Angela Vincent who does a lot of research in the UK.

What I wanted to add is I am on an American forum for Postural Orthostatic Tachycardia syndrome called DINET. It is amazing how many members on this forum have been tested for MG and have positive ACHR tests and are told by their neurologists that they don't have it.

Research has shown that ACHR has a role in dysautonomia ( wacky autonomic nervous system) and many patients are treated with mestinon with varying success. Mestinon is used off label as it increases blood pressure on standing.

Many people (myself included) on the forum also suffer with ptosis both unilateral and bilateral along with double vision.

Some patients with MG go on to develop dysautonomia - symptoms which include low blood pressure, syncope (fainting), pre syncope, breathing problems, temperature control problems (most of us have an increase in our symptoms if we over heat - like MG).

So like Alice I believe science and medicine have only just started to understand MG / ACHR etc and I would suggest seronegative just means they haven't found the antibody that causes your symptoms, like Alice I believe MG is not one disease, I think there are many different sub sets also. I hope I haven't interpreted you incorrectly or put words in your mouth Alice!

Rach

"Lord, grant me the serenity to accept The things I cannot change,The courage to change The things I can, And the wisdom to hideThe bodies of Doctors I shot When they said,"You're perfectly healthy, It's All In Your Head."

Rach, you just made my day!!!

I have been diagnosed by various doctors, none of which were psychiatrists, with:

1. Depression
2. Anxiety
3. Bipolar disorder
4. Dissociative disorder
5. Conversion disorder

And all of this caused my eyelids to droop, not to mention all the other MG symptoms. And not one of these doctors referred me to a psychiatrist. My new neurologist thinks that I never had any of these, and any psychiatric symptoms were caused by the drugs that I took for the problems I didn't have.

Hi,

I believe I had been diagnosed by my neurologist at the time with somatization disorder - not referred to a psychiatrist and didn't bother to tell me thats what he thought. I only believe this because every medical professionals treatment of me changed dramatically in 2009 or 2010 (cant remember which sorry) when my neuro discharged me.

I eventually saw my now consultant (geriatrics / general medicine) in November 2010 in a clinic for M.E. The appt was supposed to last 2 hrs and we were kicked out in 45mins as I do not have ME. I brought up the fact that I had low blood pressure and tachycardia amongst a host of other things and he agreed to do one last test- a tilt table test, if that came back negative the diagnosis would be somatization disorder! And I would have no further testing on the NHS!

Ten minutes into the Tilt Table Test in January 2011 I threw up. The consultant told me there and then I had postural orthostatic tachycardia syndrome and in May the same year I was diagnosed with Ehler danlos syndrome. The Dr that made that diagnosis said she believed I also had MG and asked my old neuro to see me again. He refused and he refused to allow any neuros on his team see me. I live in a rural area and Im too sick to travel.

No one knows why my muscles fatigue so quickly, why I have ptosis to some degree daily. The latest guess is that its due to my low blood pressure - which I know can cause stroke like symptoms.

The strange thing is my ptosis responds to an ice pack, mestinon and a drug called midodrine. No one knows why....

I also develop ptosis as soon as a migraine starts and I get lovely hemiplegic ones. My consultant believes its due to central nervous system involvement.

I am still to a certain extent convinced there is something else going on, on top of all my other medical issues, possibly MG.

Really pleased I made your day with a nicked version of the serenity prayer!

Rach

SSRIs and SNRIs both make my symptoms worse and have landed me in the hospital, so I wouldn't doubt this.

Hey Rach, just to let you know, I also have orthostatic hypotension and bouts of tachycardia. My cardiologist believes the MG is causing it.

I used to have severe tachycardia. I was diagnosed with paroxysmal supraventricular AV nodal tachycardia. I had an ablation and I am much better. That was long ago. I was among the first patients to have that treatment.

As far as blood pressure, I have noticed that my blood pressure and my heart rate are inversely proportional. If my blood pressure goes up, my heart rate goes down. If my blood pressure goes down, my heart rate goes down. I think this is pretty typical; it keeps the cardiac output steady. My point is, that if you have hypotension, it seems logical that it could contribute to the tachycardia.

Hi

Backward Pawn thats very interesting. Its well known (obviously not actually lol) that autoimmune diseases like diabetes, MG, Lupus can cause Postural orthostatic tachycardia syndrome and other autonomic nervous system issues. I expect if you did a poll on here of all the members a significant proportion of them would have symptoms of autonomic nervous system dysfunction and hadn't realised it. It seems to be a subject that a lot of Dr's don't mention to their patients.

South Blues, I pretty much have tachycardia whether my bp is normal (doesn't happen very often these days) but yes its even worse when my BP drops. Ive spent most of the morning in bed unable to get up as my bp was a staggering 77/40. So I am feeling pretty out of it at the moment even though my bp is a normal 94/60. (I never actually feel well until I hit around 115/80). Unfortunately they don't do ablations for POTS as it tends to improve it for a short period of time and then it comes back worse.

Thank you both of you for involving me in this discussion. Its nice to be more active again instead of just lurking on this forum xx

Rach

Your blog is very interesting and impressive. Great job

Thanks Steph xx

Rach, I am sort of under the impression that the "icepack" test is the most highly accurate test out there. I have had 2 ophthalmologists, a neurologist, and a general practitioner that are totally happy to put their reputations on the line by insisting that I have MG in spite of everything else being negative. The only other indication is that I do respond to mestinon.

Celeste,

You are quite right. Extensive research has shown that the only condition that responds to the ice pack test is MG induced ptosis. In studies they tested patients with diabetic ptosis, horners syndrome and a whole other host of ptosis patients, the only people that the ptosis improved on was those with MG.

Unfortunately I have encountered a world leading expert in the UK who doesn't believe this to be the case. Due to him saying its not MG no one else in the UK is willing to say it is. Despite several of the Dr's I have seen saying off the record it is. (even some Dr's who work for this world leading expert have told me I have MG).

So I continue to be left in limbo....

My ptosis 99% of the time is resolved by mestinon but apparently my response to mestinon and an ice pack is psychosomatic. Go figure!

Today I have been having psychosomatic issues (lots of sarcasm!) with my breathing making my oxygen saturation drop to 94% on room air, yet on oxygen (I have a concentrator due to the fact they cant explain why I have breathing issues other than one consultant suggesting I'm holding my breath!) its gone back up to 99-100%.

In many developing countries like Iran, Iraq, India where access to the achr antibody test maybe limited or impossible the ice pack test is used. I came across an Iranian Dr studying in the UK who could not get her head around the fact that I didn't have the diagnosis of MG despite a positive ice pack test.

Its incredibly frustrating!

Rach x

I am thinking that the reason that the ice pack works is that it inhibits local acetylcholinesterase, thereby allowing the acetylcholine that is there to hang around a while. Is this correct?

I´m going to add my bit here. The ice pack also works with limbs as the cold improves neuromuscular transmission as well. I have used ice test on my forearms and watched as my handwriting has gone from an illegible lunatics to a calligrapher´s dream in just minutes :Writting:

Anacrusis