[WildIrish]

The acetylocholine receptor antibody test was negative for binding, blocking, and modulating antibodies. I just got the report for the SFEMG and EMG tests done at Duke last week. This is what they said:

SFEMG: Single fiber EMG was performed of the left frontalis
muscle. 20 fiber pairs were examined with a mean MCD of 30 usec
(ref < 30 usec); median MCD was 29 usec (ref < 30 usec). 5 % of
fiber pairs demonstrated increased jitter without blocking and
the remaining 95 % were normal. SFEMG of the left frontalis
muscle was normal. [emphasis added]

I think I read somewhere that if 2% or more of the fiber pairs showed jitter, then you had MG, but I can't remember where I read it.

If anyone else has had SFEMG, could you please comment on these results?

They also did regular EMG testing and there were some abnormalities:

EMG: Needle EMG of selected muscle of the left arm and leg and
paraspinals is remarkable for a clear population of short
duration, polyphasic motor unit potentials with multiple thin
spikes worse in the upper extremity and C8-T1 paraspinal. [emphasis added]

Has anyone else had this type of EMG test result? Any idea what it could mean?

Their conclusion was that it was an abnormal study, but they blamed it on the 20 mg of hydrocortisone I take for adrenal failure. I plan to get a second opinion, because I disagree with their conclusions, but I wanted to see what you guys thought. Any ideas or suggestions are welcome.

Thanks.

Rose

[AnnieB3]

Hi, Rose.

It's when there are 10% or greater pairs in a SFEMG that doctors consider it to be positive for MG.

http://emedicine.medscape.com/article/1832855-overview

Are your doctors thinking you have a steroid myopathy?

The irony of that kind of thinking is that Hydrocortisone IS a steroid. Steroids reduce or erase any signs of MG or other autoimmune diseases. It doesn't matter if you're on 5 mg. or 50 mg., that's what the drug does.

You could call Dr. James Howard at UNC and see if he has any consultation appointments. He's the main MG expert there. He's hard to get into to see as well. They all are.

The fact that Dr. Massey did not order the blood tests strikes me as very odd. You can't really rule out MG in your situation (due to the steroid) without extra testing.

I think that a second opinion is in order. MG is a clinical diagnosis that's backed up with tests. Maybe you don't have MG. Maybe you have an inflammatory disease. But they can't really say for sure one way or another without further investigation.

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2504120/

You can't go off of that drug without serious consequences, as you well know. So the doctors are going to have to doctor outside of the algorithmic box here.

Other things you could do is to see a neuro-ophthalmologist if you have ptosis (drooping eyelids or eyebrows). Do you? Some N-Os will do the Tensilon test.

If you are short of breath, you could see a pulmonologist to assess what the possible cause of that is. MIP and MEP breathing tests would need to be done, for they are more specific to neuromuscular diseases.

Please don't get frustrated. It won't help YOU.

It was Dr. Howard who said at a conference in 2004 that it takes an average of one year for a man to be diagnosed with MG, but that it takes SEVEN for a woman to be! Sexism is alive and well in medicine. And your weight does NOT cause muscle weakness that is FATIGABLE! If you have fatigable muscle weakness, only LEMS, MG, or a CMS causes that! Duh, doctors.

So take a step back, maybe a deep breath, and take an analytical approach to getting some help. You can always scream or cry later. We all have.

Hang in there!

Annie

[WildIrish]

Hi, Annie. Thanks for the clarification on the SFEMG. I am trying not to get frustrated, but I have been trying to get diagnosed for 40 years! Hard to stay hopeful after that many dismissive, uncaring, condescending, snarky "you just need to lose weight and exercise" responses, from so many useless doctors I've lost count! I didn't expect to get that from Dr. Massey, but she just added herself to the list.

You're right, she is blaming it on the hydrocortisone. The problem is that I have had the muscle weakness and fatigue since I was 12 (or younger) and I only was diagnosed with the adrenal failure in 2011. I was not the only one that was surprised she didn't order any blood tests--so was the Endo that referred me to her. My PCP ordered the Ach Receptor Antibody tests, but that didn't help since it's negative.

While Dr. Massey was doing the SFEMG, she asked me if I had ever had botox injections in my forehead. Then when she was doing the EMG, she asked if I took cholesterol medication. When they were doing the nerve conduction study, her Fellow asked if I had diabetes. So clearly, they were seeing something abnormal on the monitor. I just think she didn't want to be bothered.

I decided this evening that I am going to ask my PCP to refer me to Dr. Howard for a consultation, so your suggestion is just confirmation that he's a good option. The Endo at Duke is saying she doesn't know what else she can do for me, and NO WAY is she going to volunteer or agree to a referral to their arch-rival, UNC. Luckily, my PCP believes there is something wrong.

The only ace up my sleeve is a cardiopulmonary stress test that I had done back in 2002, which I flunked, when I was being evaluated for Chronic Fatigue Syndrome. After baseline levels were recorded, I was told to ride the bicycle at maximum effort for as long as I could. I rode the bicycle until I was drenched in sweat and my leg muscles gave out, which was at about the 6 minute mark.

Six minutes! I hit anaerobic threshold before 5 minutes. They had to help me stagger over to the table to take my core body temperature again, because my leg muscles would not support me. My core body temperature only went up half a degree (normal is one to two degrees). I had to lie there for a long time before I could get up and leave the test room and I was completely exhausted for days afterwards. That's how exercise has always affected me.

Their conclusion was that I did not meet the criteria for CFS, that there was a metabolic cause for the results, which they said were all abnormal, and that it was NOT due to deconditioning. At the time, they said it was a 25% whole-person impairment, and that was before I got so bad I couldn't work full-time. It's gotten a lot worse since then. Now I have the data from the EMG tests to add to that evidence. Some day, I will find a doctor that likes puzzles and is willing to figure it out.

The muscle weakness doesn't improve with exercise, so I think we can rule out LEMS. I don't have ptosis and never have, but one eyebrow is always higher than the other, and it's the only eyebrow that goes up when I try to raise my eyebrows. The other one just sort of twitches. Does that count?

I have shortness of breath if I do much of anything, which I never used to have, and now I also run out of breath before I finish a sentence, which never used to happen. You know how when you sing songs, you know where to breathe to get through the words to the end of the phrase? Well, now I have to breathe half-way there--I can take in the same amount of air, but it doesn't last as long. I don't have asthma or bronchitis, and I don't wheeze, but I have a chronic cough that I never used to have.

Worrisome signs that something is going on, but I don't know what. Maybe I need to find a pulmonologist too. I know I don't have the typical symptoms of MG, but I have enough that someone needs to pay attention. There is something wrong.

Anyway, thanks for listening. If you can tell me any other specific blood tests or other tests that I should ask for, that would help, because my PCP wants to help but doesn't know what tests to order--and neither do I.

Thanks again.

Rose

[AnnieB3]

Rose,

If you get in to see Dr. Howard, he can order pulmonolgy function tests. It's best to try to find a pulmonologist closest to where you live, though. Then they can follow you if you become worse.

Try taking photos of your face in the morning and then later in the day or after you do an activity.

I didn't notice until I was 41 that I had ptosis. When I looked back at all of my photos, I realized that it was there since about birth. I was misdiagnosed at age ten with lazy eye. I guess that day, my right eye was more droopy than the left!

So I "get" the having something a long time and not knowing what it is. I always adapted my activities accordingly and always had a stamina problem.

I think it's pretty sad that doctors see each other as "competition" or "opponents." They really need to start having a team mentality. The goal is not a "goal," but helping a patient—no matter what that takes.

Twitching is also a sign of muscle weakness. It's fatigable weakness that is the hallmark of MG, though.

It doesn't hurt to check your TSH, Vitamin D and B12. Thyroid disorders are common, as are those two vitamin deficiencies. If you have a deficiency, they still can't say, "See, that's what's wrong with you," because neither deficiency causes fatigable muscle weakness.

I'm assuming that your heart checked out during that cardiopulmonary test, is that correct?

Sometimes it takes more than one doctor to figure all of this out. I hope you'll be okay in the meantime. If you do get to a point where you can't move or breathe well, or can't swallow, then get to an ER. Dialing 911 is best. If you have MG, it can tank pretty quickly, especially in hot weather!

Annie

[juliejayne]

Annie, maybe you can help. What is fatigability in muscles? Can it only be seen with a SFEMG or EMG?

The MG Expert here in the Netherlands has declared that I do not have a Myasthenia, because the blood tests were negative, and I don't have a clear ptosis. I do have Vit D deficiency, which they are suggesting is the reason for my muscle weakness.

But one of the other physical tests that the neuro did was to push against resistance, which I tried my best to do. But after a few seconds my "effort" failed completely. To my simple mind that would be fatigability, but apparently the reverse is true, according to the neuro.

[WildIrish]

Hi, Annie,

Yes, my heart checked out okay, and at the time, I wasn't having trouble with running out of air. I will take those test results to the pulmonologist, so he has something to compare with any new testing he does.

I take vitamin D3, because I avoid sunlight whenever possible. I have about a 10-minute sun tolerance before I burn, and I've had too many bad sunburns as a kid. Trying to avoid melanoma.

I take Armour Thyroid, because I have pituitary failure (it is useless to check TSH) and the thyroid has stopped working as a result. Same reason why I have adrenal failure. The fatigue issues have been blamed on my endocrine problems, but the Duke Endo says she thinks the hormonal issues are being adequately addressed now, and the fatigue is just as profound as it always was. That, plus the muscle weakness, is what's making me think MG.

I will ask them to check my B-12 levels on the next blood draw. They've checked them before and said they were fine, but it never hurts to check again.

Thanks for the suggestions.

Rose

[WildIrish]

Hi, Juliejayne,

Fatigable muscle weakness is just what you described. You use the muscles, and at first you can do whatever, but the longer you do it, the weaker the muscles get, until you can't do it anymore. I have that too. Walking, vacuuming, sweeping, scrubbing, stirring. Have to stop and rest the muscles after about 2-3 minutes. No strength. Even something like making the bed is hard for me. By the time I get to the fourth corner, I can't lift the mattress to put the fitted sheet over it.

But I also have trouble opening doors if they are heavy, or carrying anything that weighs more than about 15 pounds. So there is muscle weakness as well as fatigability. I suspect some of the doctors think we are faking it, or just not trying very hard.

I hope you can get a diagnosis soon.

Rose

[AnnieB3]

Rose,

When they check your B12, make sure that you get the actual number!!!

The "normal" range can be very deceptive. If you have any symptoms of a B12 deficiency, that should be what guides you when looking at those numbers and deciding if you need B12 (methylcobalamin).

Any further reports?

Annie

[WildIrish]

Hi, Annie,

My primary care doctor has ordered blood tests for B-12, and folic acid levels inside the RBCs. She also referred me to Dr. Howard at UNC-Chapel Hill for a thorough evaluation. I have no idea how long it will take to get in to see him.

I always get copies of all my test results, and I'll let you know what the B-12 levels are.

I also saw my usual neurologist today, whom I've been seeing since 2000, and he agreed that the EMG, etc. from Duke was abnormal, but says there's no way to determine why my muscles are weak. (Really. ) When I asked if they could test different drugs, he said the side effects are really dangerous, and some of them can cause cancer. (Translate that as: I don't want to do any more work than I have to--just live with it.) He wants to see me again in six months. (I don't know why--maybe to see if I'm still alive?)

I'm completely exhausted, and very frustrated. Let's hope Dr. Howard actually is as good as his reputation. I don't know what I'll do if he pats me on the head as well.

I might have to start looking for places to hide bodies!

Rose

[AnnieB3]

Rose, I've seen Dr. Howard. Yes, he's as good as his rep.

I'm not sure he's tall enough to pat you on the head.

Seriously, though, since you already have an abnormal EMG, he might be able to get you in sooner. MG is a clinical diagnosis that is backed up with tests. He's been around long enough to train other MG experts who are highly regarded as well (Dr. Weiss in St. Pete, FL).

I hope you don't have to wait long. School hasn't started yet there (he teaches, too), so maybe he'll have some time now.

Annie