How many here have Lems?
 

I have just been diagnosed with Lems and wondering if there were other members here with it.

Hello, and welcome!! I believe there may be one person on here with LEMS... I have suspected LEMS with myself, however my antibodies are negative. ..

Do you mind sharing your story about your symptoms and your treatment? I get IVIG and it works for whatever I have, however I have wondered if DAP would help me .. IF that's what I have .. hopefully someone else on here can help you!

The jury's still out in my case.
There does seem to be a pretty active Facebook LEMS group. Just be careful, it's an Open Group so no privacy.

You might also find a more active LEMS forum at http://www.mga-charity.org/forum/index?device=xhtml

Over the past several months my symptoms include fatigue and weakness, pain hind eyes, especially when focusing on near objects such as text messages, extreme neck pain and weakness, difficulty swallowing, especially when chewing, first one eyelid drooped mostly when I was tired at the end of a long day, then both eyelids drooped, then they either both drooped or took turns. Deep wrinkles appeared in my forehead from struggling to keep my eyes open. When really tired, my mouth turns down in a frown and my lower lip gapes open, very weak legs, especially upper leg muscles, everything is heavy to try to pick up and I have difficulty opening things or holding things. Difficulty breathing at times and especially when standing still and upright. Low blood pressure that goes extremely low at times, sporadic heart palpitations and fast beats.
All these things started slowly and became worse over time. At first it was just the weakness and droopy eyes and weird heart and breathing things.
In June the LEMS test was negative.
Only in the past couple of months has the swallowing started to get scary.

In January I got five Ivig treatments and sixty mg prednisone and I got much worse. I was not able to swallow at all and had a feeding tube placed. I was tested again for LEMS while in the hospital and this time it came back highly positive.
I was put back in for plasmapheresis for five days along with twenty mg prednisone. Because i do have lems the plasmapheresis was dicey at times. it was discovered that calcium must be running during the entire treatment or my muscles clampmdown including those needed for breathing. The eye symptoms are mostly gone, the neck pain is gone and I am much stronger, although not normal.
I will not go into the bad treatment I received at Emory University in the interim of these two hospital stays. That is an entirely different story.
All in all I am much improved and looking into trying 3,4 dap to see if it will give me more strength.

wow Limpy!! You sound very similar to myself... I have had pain behind my eyes (with movement) since this all started.. droopy eyelids, proximal weakness... well all over weakness, but buttocks/hips have gotten pretty bad at times.. breathing, swallowing, smiling - tachycardia, GI issues etc.. They said my autonomic system is affected with whatever I have..

Very interesting about the LEMS antibodies.. Do you have any muscle atrophy?? I feel like I do just all over a little bit.. I know that can happen with LEMS.. but I doin't know how common it is...

I have heard wonderful things about DAP.. I have wondered if anyone is ever put on it without positive antibodies.. also for people who are seronegative for LEMS (from what I research that is about 40% of people without cancer)... how are they diagnosed?? I had a repetitive stim, but it was 2 weeks after IVIG, so they don't think it was accurate.. but it did go from weak to stronger to weak again.. just not a big enough difference to say it was abnormal...

Does your chest wall feel weak ever?

I also meant to ask - did you get buttock pain?? I get deep buttock muscle pain at times.. it can also hurt to sit for a while as well.. Funny you said that about the standing still and difficulty breathing - when I sit in the car in the same position for too long my chest starts hurting and feeling weak. ... its awful!

Hi Limpy, When you say the muscles that are needed for breathing "clamp down", what does that look like? Is it like a spasm? How would you describe it? This may be unrelated, but for 3-4 years, I've had a muscle spasms/tightness in leg that wouldn’t release or relax no matter what I did. Ist a LEMS symptom?

Can anyone tell me if there's any symptomatic difference between MG & LEMS (other than the muscles become temporarily stronger with repeated stimulation)?

bny806, 40% of people with LEMS who don't have cancer test negative for the VGCC antibody?!! Where did you get that information? I'd like to read more about it, please! (I got stronger on repeat stimulation & tested neg for VGCC & feel my MG/LEMS is autoimmune-related. Does muscle atrophy not happen with MG (mine have wasted & bones are beginning to pop excessively)

I get pain in my arm & thigh and droopy eye on the side I'm sleeping on. The pain in my thigh can cause me to limp for a couple days. Is that more symptomatic of LEMS or is that also a MG symptom?

Thank you!

I can't remember where I read that it was 60% of people without cancer test positive, so 40% were seronegative.. here is a little more info (though the range is huge) http://emedicine.medscape.com/articl...up#aw2aab6b5b2

I thought I had seen someone on the facebook page for LEMS say that as well (I'm not a member of it, but I do peak in and see what people say)

Bny806,
You sound like you may have LEMS , too. The main thing that makes it difficult for me to breathe when standing is that my chest wall and diaphragm feel heavy. It almost feels as ifmi am collapsing in on myself, if that makes sense. This is promptly relieved if I sit in a reclining position or lay down. Sitting in an upright chair is difficult, but not as bad as standing still. Walking is easier, because I think the muscle use is shifted around instead of calling on the same muscles over and over in standing.
Yes, I do have muscle wasting, especially in my left thumb and wrist, but now mostly to some degree all over. The thumb and wrist atrophy is what brought me to my current neurologist for a second opinion because my orthopedist wanted to do four surgeries on my arm and hand to try to correct this.

And yes, I do get deep muscle pain and cramps. When I was on plasmapheresis and the calcium drip ran out, I had buns and abs of steel, couldn't talk and could not move my diaphragm to breathe. I get really weird spasms in my jaws that hurt and the rest of my face gets contorted when I am really weak. Sometimes at my worst if I look in a mirror, I do not recognize myself!
And, yes sitting in a car, especially upright, is very uncomfortable. I have a squishy travel pillow, the kind with the tiny foam beads to help hold up my head and I have to recline the seat if I can.
And,yes pain with just eye movement, too. Although eye pain is very minimal and only occasional since plasmapheresis. Also, with the eye pain, extremely blurred vision. This is better too after plasmapheresis.
Hope you can get diagnosed soon and on your way to feeling better, because this is no way to live.

here is some good info as well http://www.lems.com/what_is_lems


I meant to say - i have struggled with dry eyes, but also a dry mouth since this started.. my tongue bleeds and I do have a metallic taste too (even when I can't visualize any blood).. gross I know.. my whole body has gone wonky!

Limpy - I can't tell you how relieved I am to read your post!!! To hear that someone with LEMS has muscle wasting just makes me so happy I want to cry!! I have said for a while I feel like LEMS would be a fit.. they say maybe seronegative MG.. but then I feel like I have these other symptoms that just dont' fit with that..

I have read that many LEMS patients have small fiber neuropathy too- do you have any of those symptoms?? My biopsies came back very abnormal - showing non length dependent small fiber neuropathy....

It's really strange you say that about sitting up right... I feel like I can't get my chest to move, and like I have someone sitting on it when I am sitting ..but when I lean back on the couch or walk around I too am much better..

I also feel like I have atrophy all over - very symmetric.. my thumbs freak me out the most though!! I used to have good abs and was so in shape - a runner too.. the other day I looked down and asked where my bum went!?!

My jaw has ached in the past... and at times gets so weak I can't shut my mouth! it also feels like many of my muscles just vibrate when over exerted.. do you have this??

So, what is the process with getting DAP? Is it tightly controlled, and if so why is that? I feel like people more freely use mestinon, but not DAP.. curious why that is

Limpy - did you have EMGs?? were they normal?? Mine have been normal - my needle emg and Ncv (I had one small area of demylenation on my knee at first)... I have had four or five.

This is the way I understand it, but I am not a medical professional by any means
With LEMS the antibodies block the calcium channels so the problem is that I can use a muscle like to bite down on something but then the opposing muscle will not work because it runs out of available calcium, so then I can't open my mouth to even spit out the food that I can no longer swallow. My tongue freezes in place when I try to move food around in my mouth. If this happens at the same time that I have already swallowed something, it stops in my throat at that point, and I have to try to relax as the muscles spasm and hurt and try to get the food down. It is very frightening. While this is going on my heart is racing, I am not sure if it from the anxiety or the disease, because it does this when I am not trying to swallow or chew, too.
When my breathing muscles clamp down, I have to concentrate on breathing, and my chest wall just barely moves. After the episode passes the muscles release and I can inhale deeply again.

Limpy - so you have episodes where your weakness and symptoms are worse?? I can have episodes where it is really hard to do anything.. - I feel like mid morning is the worst many times.. and then I feel better..

Do you ever get myotonia like symptoms? When they hit my thumb with their reflex hammer I have myotonia - sometimes more than others.. The neuros dont' think much about it, but it freaked me out!

When they wanted to do the surgery on my arms nd hand, my neck had been killing me for a while and since I had cervical disc disease, I figured the atrophy was from something in my neck, especially since my right hand was beginning to get weaker and was starting down that same path. I decided to further investigate the neck theory and that is how I met my neurologist who is awesome.
I can't speak to small fiber neuropathy as I have never been checked for that, but I do have a sunburnt feeling in the backs of the calves of my legs that drives me crazy, especially in the evening. That, too has improved with plasmapheresis.
I have the vibrating muscles, especially in my face when I am stressed or overly weak.
The reason why dap is not readily available in the US is that it is not FDA approved, but I understand that it can be obtained for compassionate use in the case of LEMS. My neuro is checking on that now.

My EMG s have been normal. The last one I had was at Emory, and the last part on my left shoulder was somewhat abnormal, but it was done under the influence of mestinon, so I think Iftar was a very painful waste of time.

Limpy
 

Hi...I'm very interested in your situation as well. Can I ask you what your first noticeable symptoms were? and also how long it took for this to progress to your weakest point?

I didn't know what that meant until I looked it up, but yes, when I flexed my neck a certain way while I was waiting to get admitted to the hospital, it stuck in place for a minute or two then relaxed. I have not had a reaction to the hammer, but usually just things that I am doing and muscles will freeze up momentarily.

Limpy - I Just can't get over the similarities!!

Crazy, that you mention the sunburnt feeling - I have that exact feeling - on my palms of my hands and forearms.. a little bit on one thigh as well.. The sun or heat greatly exacerbates it... I kept telling my neuro (who honestly I am not very impressed with, but they did start IVIG on me, so I feel a little obligated to stay there, as I worry another neuro could take it away - as I dont have a firm diagnosis). I kept telling my neuro that I felt like I needed to crawl out of my own skin.. but honestly I think it was more the vibrating muscle/weakness sensation than the small fiber neuropathy, that is why they tested me..

I also notice that the more I use my hands the more that numb/sunburn sensation occurs.. very strange.

All my muscles vibrate when I am weak.. including my face.. My lips will just vibrate.. Like when I was a kid and hummed against a ballon.. it's so odd feeling..

I just don't know how they diagnose people when they are seronegative (repeititve stim I suppose, but then you have to stop IVIG to get an accurate test.. and by 2 weeks post ivig I get more like a limp noodle, can't swallow etc... so I don't think I can do that)

you have given me so much hope! Thank you!!!!

Is the 'vibrating muscles' sensation unique to LEMS? Since all these symptoms started happening, I'd assumed all the bodily changes were MG so I don't know how to distinguish between MG symptoms and LEMS symptoms. Do you guys get undue muscle pain when the muscle is pressed on? That just started for me. Is that LEMS or could it just be a symptom of muscle wasting?

When it was at its worst, I felt a tightening (the sensation of a clay mask drying on the skin) roaming across my face distorting my eye, forehead, locking my jaw, causing a strangling sensation across my neck.... The tightening was actually flickering on & off, ebbing & rising and would allow me to talk then making it hard to move again. Is that a LEMS symptom? Or MG? or both?

For two weeks, my throat has been spasming (I assume it's a spasm) that alters my voice to soprano range when I talk. My voice returns to normal range if I have food in my cheek, tilt my head forward or backward, if I drink, eat, cough, burp or sneeze. Then I'm reverted to soprano again within 1-5 seconds. Is that a LEMS symptom? Or MG? or both?

I have had vague weaknesses for a long time but just figured it was old age or deconditioning, but the thing that got my attention was April of last year, when my heart starting jumping around in my chest and pounding and racing, and I felt really weak and my chest felt heavy and had to concentrate on pulling in each breath. My chest wall felt really weak. i had no chest pain. When I got up to walk, I had to hang onto something and was afraid to let go. I had to go to the ER and was worked up for heart and diagnosed with an arythmia, but didn't think anything of it until I started having more symptoms with the eyes drooping more and more and the facial tightness and progressive body weakness.

again, HUGE relief hearing your symptoms, especially since you are a confirmed LEMs case..
At times (not always), I will angle my foot inwards and it will stick that way for a few seconds.. it doesnt' hurt, it's not cramping.. but it's just not relaxing..

As far as looking in the mirror.. I agree! I see pictures of myself and I can hardly recognize myself!! The weeks before I was admitted into the hospital last year (when they started my IVIG).. There was a picture and video of me with my kids.... I saw it recently and couldn't get over my face.. it was as it had melted off my bones.. I was only 30 at the time.. not supposed to look like that just yet!

Limpy - my first trip to the ER (and thankfully only trip- the admit happened through my dr's office).. was due to fast heart rate with palpitations and well I felt weak, but just thought it was a result of the heart.. I was post partum, so figured it was just post partum hyperthyroid or something (i had my thyroid checked a billion times - always normal)..

Anyhow.. I had some crazy palpitations.. but what got me to go into the ER was my resting heart rate was in the 140's... it was awful.. this stayed that way for weeks on and off, mostly on! My resting heart rate is almost always 100 or more since onset of this.. It used to be in the 60's before onset of symptoms..

seshin- yes my muscles are tender to the touch.. when my kids climb on me it's quite painful.. especially my thighs and calves.. It has got to be a part of all of this.. though I'm dont' know what all of this is!

Yep, the lip buzzing is definitely a sign for me of not enough calcium getting where it needs to go. The doctor that was overseeing the plasmapheresis thought that because my calcium levels were high, I don't need calcium. I could have all the calcium in the world, it just can't get where it needs to go.

If you ever get plasmapheresis, make sure you get at least two grams of calcium to run over the entire exchange or you will be in trouble. I am so glad that my experience has helped someone. And one more tip, if you are seronegative, DO NOT go to Emory University in Atlanta!
My first LEMS test was when I was not really very bad, just beginning to have symptoms. This last one was when I was at my worst.

You have truly helped me!!! I wish that I had a neuro that I felt more comfortable with... I will mention it again.. the fact that they are mentioning seronegative MG (after a year of being with them).. at least they are starting to think outside the box a little.. and again.. at least they gave me IVIG in the first place.. I thought I was going to drop dead at any moment before I was hospitalized.. it was awful.. I'll never forget the day I could hold a crayon with my daughter and color with her again! I am doing well, but sure would like to have a diagnosis so I can plan my future a little better and maybe get medicine that could help me more..

Again. ... THANK YOU!

Limpy, if I send you my complete list of symptoms, would you be willing to take a look at it and tell me if any of the symptoms seem to be unique or more common to LEMS vs MG? The doctor said I got stronger on the muscle fatigability test, and I respond to both Mestinon and Tensilon but I tested negative on all antibody tests and even the SFEMG so he wants to wean me off Mestinon.... :confused:

I am waiting for my tongue to bleed one day. I drink throughout the day, but at night I wake up with my tongue stuck to the roof of my mouth and I have to literally peel them apart. I am amazed that there is skin still left there they are stuck just like I put glue in my mouth. I only have one eye that feels dry sometimes. But it only happens once in awhile. My left eye, I will go to move it and it feels like my eyelid is stuck to it and I will have to look in the mirror because it feels like it has torn something. It feels like it should be bleeding. I went to an eye doctor the first time it happened and he said it was fine.

Exactly, the muscle stiffness is just that they are frozen in place. Not like a true spasm with a lot of pain. Well , except for when trying to swallow against it and it feels like you are trying to swallow a basketball. And when my buns were stiff, I think the pain from that is because I couldn't move to shift my weight so they became painful.
I can change my appearance throughout the day depending on how weak I am at any given time.

Sure, I would be more than happy to try to help. Mestinon helps me, but does nothing for the eyes or the swallowing. The only thing that has helped that is the plasmapheresis.

Thanks, I'll be sending it right away. Mestinon helps with some of the general weakness but hasn't touched my throat.

Yes, me too, as far as the appearance goes.. I'll look in the mirror and think, ah.. I look my age yay! and then hours later I look like an older grumpy person... At my worst symptoms I couldn't even smile.. I was so upset several months into this all that I wanted to cry.. and then just got more upset when my face wouldn't make the "crying face"....


I have had times where it feels like the back of my throat is touching the front..I had a few days about 6 months ago where my swallowing was so bad I just avoided eating and drinking much (smart I know).. but I felt like gagging as my throat felt like it was collapsing... At one point I tried to speak and it was like my throat was closed so no voice came out.. that only lasted a few hours.. thank goodness.. and now I'm almost back to normal as far as that aspect goes (i'm 1 week post IVIG)..

Yes, I have swallowed and then almost gotten sick as my throat was stuck- too dry to swallow.. they thought I may have sjogrens at some point. but dont' feel like that really fits

[QUOTE=bny806;956747]Yes, me too, as far as the appearance goes.. I'll look in the mirror and think, ah.. I look my age yay! and then hours later I look like an older grumpy person... At my worst symptoms I couldn't even smile.. I was so upset several months into this all that I wanted to cry.. and then just got more upset when my face wouldn't make the "crying face"....

When I am really weak my face looks like one of those masks that represent the theater. But not the on that is smiling. And you are right, it is very upsetting.

Seishin,
I tried to answer your pm but I don't know if it went through. It told me there was an error. I may have done something wrong.

I didn't receive it so thanks for letting me know!
I forgot to add to the Timeline I had pain in the bottom of my feet for several months when walking, podiatrist couldn't see why.

I think that some pop-up blockers interfere with neurotalk's private message system. If you're having trouble with Chrome, try Internet Explorer.

Abby

If it's a new message, it'll say there's an error if there's not a title on the message, too. That happened to me yesterday.