[rach73]

Hi,

For those of you who know me I will keep my history short as I have been doing the diagnosis dance since 2007! I have had ptosis right eye and occasionally left eye since 2007. I was diagnosed with MG, but the sfemgs and blood tests came back negative - although mestinon always resolved the ptosis, along with ice pack tests.

In 2009 the MG diagnosis was removed and I wasn't informed but my medical notes said I had somatoform disorder. In 2010 I worked out I had autonomic nervous system issues and Ehlers Danlos Syndrome. All the doctors I have met outside neurology have said I have MG. I am so sick and tired of it I never bring it up anymore. ANS problems can originate from an autoimmune disease and its really weird but my sister has also developed ptosis now and no one is giving her an explanation either.

However today I have found new vigour as whilst going through some old paperwork I found a letter thats states "Certainly as you point out, tests for MG were negative and although the icepack test was dramatically positive, unfortunately this is not entirely specific for this condition"

All my other paper work ignores the ice pack test - this idiot then wrote another letter to the neurologist suggesting I had CBT for my ptosis.

So my first question is do any of you know any other diseases where the icepack test would be positive? I have spent the morning on Google scholar and I am yet to find anything.

I have pulled off medical papers which suggest the ice pack test is 80-100% for ocular myasthenia, but it doesn't state what other diseases it would be positive for. Also states blood tests are negative for 46% patients with ocular MG and SFEMG is negative in around 50% of patients. I have always believed I have MG, it was just a case of proving it.

Are there any good medical articles you know of and could provide links to regarding the ice pack test and also any on ocular MG that state the negative blood tests and sfemgs in these patients.

I would like to be armed with irrefutable proof of ocular MG ( I actually think I have quite mild generalised MG but thats a story / battle for another day) before I broach this verboten subject with my doctors.

Thanks in advance

Rach

[rach73]

Im guessing after 94 views no one knows of anything?

[AnnieB3]

Well, there is no simple answer to your question, Rach!

The ice pack test does show improvement in MGers, but it's not a reliable "definitive" test for MG. That's why they suggest further testing.

My digging revealed some conflicting evidence, and makes me realize that this heat/cold effect on MG is not so simple.

http://www.ncbi.nlm.nih.gov/pubmed/3614664

http://pmj.bmj.com/content/76/893/162.full

http://www.scielo.br/scielo.php?pid=...pt=sci_arttext

In this article it states, "The physiological theory behind the test is very simple; it is thought that by cooling the tissues, and more specifically the skeletal muscle fibres, the activity of the acetylcholinesterases are inhibited (laboratory data suggest below 28°C)."

http://shr.sagepub.com/content/1/1/14.full

And it goes on to say, "This simplistic theory may not be the only factor involved in the resolution of the ptosis; some studies have concluded that it may not be simply cold that can produce a positive ice pack test and that heat can produce similar results, almost certainly by the same physiological effect."

And this brings up the issue of heat and cold making MG better or worse. The say that cold inhibits acetylcholinesterase and, therefore, creates more localized acetylcholine.

Other articles have stated that heat and cold increase acetylcholinesterase in the body, thereby reducing acetylcholine and making MG worse.

http://www.ncbi.nlm.nih.gov/pubmed/1959652

And, curiously, what happens in corn to AChE and ACh.

http://www.ncbi.nlm.nih.gov/pubmed/21757255

And a more scientific article on heat stress mechanisms.

http://jap.physiology.org/content/93/6/1947

That article is an eye opener. The body uses acetylcholine in the mechanisms to modulate cutaneous vasodilation when the body becomes heated up. Maybe. Why aren't there more accurate and definitive studies on this?

So what does heat do to MG? Does it use up more ACh because ACh is busy doing other "tasks" in the body other than keeping our muscles strong?

And do people with more nitric oxide or better nitric oxide use fair better in the heat and cold (i.e., Abby)? I'm also wondering how MGers who take NSAIDS do out in the cold/heat. Do prostaglandins also play a role?

And what does cold do to the body? Does acetylcholine rush in to the cold area due to vasoconstriction?

Does acetylcholine regulate vasodilation and vasoconstriction in the body?

It's all mediated by nitric oxide (NO) and prostaglandins (PG).

http://jap.physiology.org/content/98/2/629

Stay with me here. This is not a simple topic, Rach!

Could POTS be caused by a NO or PG regulation error in vasodilation/vasoconstriction?!!!

And then I found this.

http://www.ncbi.nlm.nih.gov/pubmed/21642500

http://jaha.ahajournals.org/content/3/1/e000755.full

I'd like to do more digging, but I'm worn out. There's a definite connection here between acetylcholine and POTS.

Is the ice pack test definitive for MG? No. But it is a strong sign of MG.

The neurologists in the UK apparently don't want to do the testing necessary to figure out if you have MG or CMS. I find that to be reprehensible and unethical.

Does any of this help or do you now have a mammoth sized headache?


Annie

[rach73]

Thanks Annie for all your hard work.

Some of those papers I have already discovered but others I hadn't. What I felt as I was reading the papers about the ice pack test was that some doctors embrace it whole heartedly stating 80-100% accuracy rate yet other doctors seem to disregard it. I know in the developing world the test is used to diagnose MG as its cheaper than a blood test.

I dont understand the doctors when they try and say rest is what improves the ptosis not the ice itself. Any rest test I have seen has said the patient should rest for 30 mins in a dark room. The ice pack test is a maximum application of 5 mins - wheres the prolonged rest period?

I sometimes use heat on my forehead to relieve the pain of the muscle spasm that happens when I have prolonged ptosis - its agony and feel like my forehead is being torn apart. Heat never resolves my ptosis, only cooling.

I have discussed the ice pack test with Angela Vincent in Oxford through emails. She said that the ice pack test showed there was an issue at the neuromuscular junction - but wouldn't say it was MG. Purely as it was the hospital and people she worked with that had dismissed it. So that was a fun discussion.

Placebo effect? when I first did an ice pack test on myself I didnt know why my mum was asking me to do it. It worked but I had no idea what that meant.

I have also had one positive tensilon test and one negative tensilon test. Just to make things even more confusing.

Mestinon is used in POTS to raise standing blood pressure. Another red herring thrown into my diagnostic dance.

In the UK the temperature has suddenly shot up and I am doing badly today. Very light headed and weak, this reply has taken me over 40 minutes to type. I know you arent doing so well yourself so I appreciate the time and effort you have put into this. I am still currently wading through the medical papers you have provided! I may have to re read them as my brain isnt working too well. I am having to decide if I need to get my husband home from work. So you know how bad I am!

Thank you hun so much. There does seem to be a link with ACHR and POTS as many people on DINET have reported positive tests but are told they dont have MG. Now either they have MG and arent being treated or this ACHR chappy is responsible for a whole lot more than MG and medical science isnt joining up the dots yet.

Having chatted at length with another Dr she believes it is something to do with the ganglionic (may have really badly spelt that) receptors in the brain. Another MG type autoimmune disease and there is starting to be evidence that POTS is an autoimmune disease where the body is attacking the barro receptors (again bad spelling) - the ones responsible for vasoconstriction and vasodilation. So again Annie as always you are on the ball with your thinking.

Unfortunately when we start getting into that more complicated area of thinking I cant keep up, my mind cant understand that level of thinking! I can only just understand the autonomic nervous system start throwing in the parasympathetic nervous system or central nervous system and its way over my head.

I just want to say a massive thank you for the time you have spent on this and sorting stuff out for me. THANK YOU xx

Rach x

[rach73]

No headache as yet my lovely! Just a feeling of being completely and utterly spaced out. Downing gatorade like its going out of fashion and have taken fludrocortisone to try and sort myself out!

Hope with some rest you feel better

Rach

[rach73]

Sorry I should have read everything before I started replying. I have just read the paper that talks about splanic issues - sorry my brain isnt switched on fully.

Did you know that with splanic pooling - post prandial hypoyension one of the first lines of attack ( for the want of a better word) is taking ibuprofen (a nsaid) 30 mins before a meal to see if that counter acts the bodies desire to send all the extra blood to the abdominal cavity? It didnt work with me nor did strong coffee ( another line of attack) mine is so bad that any food group will cause a black out, so I am now taking octreotide injections 40 minutes before eating which work but as usual I can only tolerate one a day!

There does seem to be some overlap between MG / dysautonomia / autoimmune diseases. I wonder if many MGers had a tilt table test if they would show symptoms of POTS or Orthostatic Intolerance.

Maybe we are just ahead of the curve?

Many thanks for your thought provoking post and I am sorry my replies are so lame due to being a space cadet today!

Rach x