Does anyone else have muscle weakness plus ptosis?

[kangaSue]

Sjogren's will often test negative in an ENA panel for many years before you strike a positive result. Quest Diagnostics have a test for a different group of antibodies (Early Sjogren's Syndrome Profile) that can show up before the more common SS antibodies occur as positive.
https://www.questdiagnostics.com/testcenter/BUOrderInfo.action?tc=93748&labCode=AMD

Some good information regarding eye problems in SS in a case study in this article;
https://www.sciencebasedhealth.com/...'s_Melissa_Barnett_Adv_Ocular_Care_Oct_16.pdf

 

[rel8ted]

You mentioned this before (and I apologize for my slow reply ) but is your new allergy to all mammal products from a tick bite or something else? Also, do you think it is permanent or possibly reversible?

It is the dreaded Alpha Gal Syndrome. It is from a tick bite, yes. The problem is that until recently, there has been a very small population affected, so very little interest. In the last 10 or so years, there has been an explosion in Dzib due to both better diagnostics & an exploding tick population.

Some docs will say that if your level decreases, the allergy is gone. However, patients are reporting that even if they think they are clear & can eat mammal meats again, one tick bite brings it back & worse than ever. Patients are also reporting that blood levels do not necessarily correlate with reaction intensity. Some people seem oblivious except for an upset stomach, others cannot even use a soap or lotion with mammal products with anaphylaxis. The good docs generally recommend 2 epi pens on your person at all times. Benadryl is also a problem bc it sometimes contains mammal, so the thing you assumed safe can cause a rebound reaction.

The allergy is actually to a sugar in mammal meat &not the protein, so you don’t react right away. It can be 4,8,12 hours after eating. I don’t feel bad about my dx, I just don’t ever want that stuff near my mouth again. There have been some people saying their doc wants to do an in-office food challenge bc their level dropped-no thanks. (Is someone staying with the patient 12 hours?). I have had hives 8 weeks now. The xolair seemed like it was knocking them out, but I am getting very tiny hives again in very tiny patches. It is also an awful pollen year & like my allergist said “you are allergic to nearly everything at some level. It’s nearly impossible to determine the root cause of this reaction.” We camped 1 night outside Glacier National Park one year, not realizing I was allergic to birch, during birch bloom. I had THE worst allergic reaction I’ve ever had. No phones, no 911, no idea what epi was & no medical help close by. I was pretty sure I was a goner. My eyes were swollen 75% closed for several days after!

 

[pogoman]

How are different muscular dystrophies tested for? Is it with a muscle biopsy?

Yes, along with needle conduction, genetic and enzyme tests for certain ones.
There are a lot of MD types.
This MD variant involves ptosis, there may be others.

https://ghr.nlm.nih.gov/condition/oculopharyngeal-muscular-dystrophy

 

[rel8ted]

Yes, along with needle conduction, genetic and enzyme tests for certain ones.
There are a lot of MD types.
This MD variant involves ptosis, there may be others.

https://ghr.nlm.nih.gov/condition/oculopharyngeal-muscular-dystrophy

That is what I was hoping it would not be...

 

[Gingergrrl]

It is the dreaded Alpha Gal Syndrome. It is from a tick bite, yes.

I knew you'd mentioned Alpha Gal but I was not sure if it was from a tick bite (and thought you had said it was a reaction to a medication or inhaler)? But I might be totally confused ?

Also, and I know this is a long shot, but is there any chance that the muscle weakness and ptosis are from the alpha gal syndrome? I know that neuromuscular symptoms are totally different than allergic/anaphyalxis symptoms (since I've had both as part of my illness and they are clearly different). But I've heard of people having "Neuro-Lyme" and was wondering if there could be a Neuro component to Alpha Gal? (I also was not sure of the time sequence and when you got the tick bite)?

Some people seem oblivious except for an upset stomach, others cannot even use a soap or lotion with mammal products with anaphylaxis. The good docs generally recommend 2 epi pens on your person at all times. Benadryl is also a problem bc it sometimes contains mammal, so the thing you assumed safe can cause a rebound reaction.

This is really scary that there could be mammal products in lotions that could cause you anaphylaxis. Would the ingredients be listed in the lotion? My worst anaphylaxis reaction in 2015 was to yellow dye (tartrazine or "yellow #5") but luckily it is listed in food, meds, lotions, etc. I truly do not even know if I am still allergic to it b/c I have not been brave enough to even test a toothpick amount.

The allergy is actually to a sugar in mammal meat &not the protein, so you don’t react right away. It can be 4,8,12 hours after eating.

There have been some people saying their doc wants to do an in-office food challenge bc their level dropped-no thanks. (Is someone staying with the patient 12 hours?).

I would have to pass on this as well and not worth the risk IMO.

I have had hives 8 weeks now. The xolair seemed like it was knocking them out, but I am getting very tiny hives again in very tiny patches.

How annoying and I'm sorry the hives are back

We camped 1 night outside Glacier National Park one year, not realizing I was allergic to birch, during birch bloom. I had THE worst allergic reaction I’ve ever had. No phones, no 911, no idea what epi was & no medical help close by. I was pretty sure I was a goner. My eyes were swollen 75% closed for several days after!

I am sure I have asked you this many times but do you now carry an EpiPen and wear a Medic Alert bracelet?

Yes, along with needle conduction, genetic and enzyme tests for certain ones. There are a lot of MD types. This MD variant involves ptosis, there may be others.

Thanks and I was curious myself and assumed it was a muscle biopsy or EMG/NCT. I didn't even think about genetic or enzyme testing. Your case turned out to be autoimmune right (@pogoman)?

That is what I was hoping it would not be...

We have no idea that you have MD but I guess it is worth asking about. I think I might have told you that I did a consult in 2015 (or early 2016?) with a POTS Cardio who strongly felt that in addition to having severe POTS, that I had some type of MD (this is not the Neuro at Stanford and is someone different and I did many consults in 2015 & 2016)!

That Cardio said that he knew that I did not have MD but he was baffled by my muscle weakness until Stanford found I had the LEMS autoantibody. It was so confusing to sort it all out (and became my full-time job) but once I started having anaphylaxis, that became the only priority to get it under control before I could go back to trying to figure out all the other stuff.

 

[rel8ted]

Also, and I know this is a long shot, but is there any chance that the muscle weakness and ptosis are from the alpha gal syndrome?

Unlikely. I’ve had the ptosis & muscle weakness for a loooong time. They have just been getting a little worse as time passes until they can no longer be ignored.

Would the ingredients be listed in the lotion?

IF you know what to look for, most likely. Lanolin is a given. Gelatin is problematic, but would mostly be in foods. Glycerin can be mammal derived.

In foods, “natural flavors” can be from mammals. It’s just about being super-aware of what you are eating or putting on your skin. I’ve always had a lot of allergies, so I’m pretty much a detective anyway.

We have no idea that you have MD but I guess it is worth asking about

I know, but it’s not a terrific possibility to face. I am really hoping that this neuro sees me through to dx. My friend told me that it was SO wonderful that my antibodies were negative as I was looking at her through one eye today (very bad ptosis day). Then she got a funny look and asked what else it could be. When I said MD, she almost fell off the chair. I actually laughed bc of the look on her face. If it is, it is. Can’t change it. Just ready to know.

 

[Gingergrrl]

Unlikely. I’ve had the ptosis & muscle weakness for a loooong time. They have just been getting a little worse as time passes until they can no longer be ignored.

Makes sense and I agree the two are most likely not related.

IF you know what to look for, most likely. Lanolin is a given. Gelatin is problematic, but would mostly be in foods. Glycerin can be mammal derived.

Wow, that is scary. I still have to verify in every restaurant I go to that the butter does not contain yellow dye. When it does, then I have to confirm that everything I have ordered is cooked in oil vs. butter (and some restaurants are amazing but others act very inconvenienced like my request is crazy).

I know, but it’s not a terrific possibility to face. I am really hoping that this neuro sees me through to dx.

I hope so, too.

My friend told me that it was SO wonderful that my antibodies were negative as I was looking at her through one eye today (very bad ptosis day). Then she got a funny look and asked what else it could be. When I said MD, she almost fell off the chair. I actually laughed bc of the look on her face. If it is, it is. Can’t change it. Just ready to know.

I reached that point in 2016 that I just wanted to know what was wrong with me. For three years (2013 to 2016), I believed it was ME/CFS and then when my main doctor said he no longer thought it was, I didn't know what to think. Did I have one diagnosis that explained everything or 4-5 different diagnoses? It was so confusing and I don't think I will ever know the full answer of what happened to me except that the core is autoimmune.

 

[pogoman]

That is what I was hoping it would not be...

Agreed altho getting a concrete diagnosis would be great I think.
I have a non inflammatory myopathy with muscle necrosis, after 5+ years doctors still not sure what I have and my old neuro said it could be a dystrophy.

Thanks and I was curious myself and assumed it was a muscle biopsy or EMG/NCT. I didn't even think about genetic or enzyme testing. Your case turned out to be autoimmune right (@pogoman)?

They still don't know for sure what I have
The geneticist up in LA said because the rituximab is helping that its not a primary metabolic disease, the mito damage is secondary to something else.
The pain is much better but the muscle contraction/stiffness still happens every night and morning.
I think I am losing more muscle above the knee but its slowed down.
Saw new neuro last month and was underwhelmed, he is going to do another full nerve conduction test next visit.

 

[Gingergrrl]

Agreed altho getting a concrete diagnosis would be great I think.

I agree that getting a concrete diagnosis would be great. I know that was part of what tortured me for years (not knowing) and I don't think I will ever be 100% certain that my core diagnosis is correct (vs. the POTS, MCAS, & Hashimoto's are for sure correct). But the core being an atypical form of LEMS (in my case), is still something that I wonder if it is correct although I have not found anything else that is a better explanation.

I have a non inflammatory myopathy with muscle necrosis, after 5+ years doctors still not sure what I have and my old neuro said it could be a dystrophy.

They still don't know for sure what I have

Wow, I thought that the doctors did know for sure what was wrong with you (the myopathy with muscle necrosis) or is that more of a description of symptoms vs. a diagnosis?

The geneticist up in LA said because the rituximab is helping that its not a primary metabolic disease, the mito damage is secondary to something else.

That is interesting. Do they feel that Rituximab worked through an autoimmune mechanism (keeping B cells at zero to lower autoantibodies) or by some other mechanism in your case?

The pain is much better but the muscle contraction/ stiffness still happens every night and morning. I think I am losing more muscle above the knee but its slowed down.

I am glad that the pain is better although that sucks about the muscle contractions/stiffness and possibly losing more muscle above the knee.

Saw new neuro last month and was underwhelmed, he is going to do another full nerve conduction test next visit.

To say that I was "underwhelmed" by the three Neuros that I saw in 2016 would be putting it very politely I almost asked you again if you recommend a good Neuromuscular specialist but then I remembered that (I think) you have Kaiser?

 

[pogoman]

That is interesting. Do they feel that Rituximab worked through an autoimmune mechanism (keeping B cells at zero to lower autoantibodies) or by some other mechanism in your case?

Wow, I thought that the doctors did know for sure what was wrong with you (the myopathy with muscle necrosis) or is that more of a description of symptoms vs. a diagnosis?


To say that I was "underwhelmed" by the three Neuros that I saw in 2016 would be putting it very politely I almost asked you again if you recommend a good Neuromuscular specialist but then I remembered that (I think) you have Kaiser?

My muscle biopsy in early 14? definitely showed a non-inflammatory myopathy with necrosis, because its not inflammatory that is supposed to rule out autoimmune diseases.
So the official diagnosis has always been an unknown myopathy, the last nerve conduction test last year confirmed a myopathy in my thigh.
The biopsy report called it a "toxic" myopathy, those are usually caused by drugs or chemicals but I haven't been exposed to any.

The joker in the deck is the discovery of necrotizing autoimmune myopathy the past few years, its not inflammatory but is an autoimmune disease.
So since 2015 my old neuro went along with my requests for autoimmune treatment, I did IVIG, methrotrexate and now rituximab.
But those treatments haven't lowered my CPK levels so I think they won't list NAM as an official diagnosis yet.

And yes I have Kaiser.
Pretty good for the mundane stuff but behind the curve for the more obscure diseases

 

[Gingergrrl]

The joker in the deck is the discovery of necrotizing autoimmune myopathy the past few years, its not inflammatory but is an autoimmune disease.

There are so many overlapping but similar things, you really have to be a medical detective.

So since 2015 my old neuro went along with my requests for autoimmune treatment, I did IVIG, methrotrexate and now rituximab.

I know you've posted about this before, but are these treatments still helping you today? Is your plan to continue Rituximab indefinitely as maintenance infusions?

 

[pogoman]

[QUOTE="Gingergrrl, post: 2203474, member: 150210
I know you've posted about this before, but are these treatments are still helping you today? Is your plan to continue Rituximab indefinitely as maintenance infusions?[/QUOTE]

Yes I definitely am feeling better than a year ago and want to continue the treatments.
I did the second ritux infusion in January six months after the first, was starting to feel worse the end of December.
Noticed an improvement trend end of March, right now I am feeling much better (other than the muscle issues) and have more energy to do things.

 

[pattismith]

@rel8ted
did you get any muscle biopsy for your symptoms?

I forgot to add Myotonic Dystrophy as another potential cause to explore.

 

[rel8ted]

@rel8ted
did you get any muscle biopsy for your symptoms?

I forgot to add Myotonic Dystrophy as another potential cause to explore.

No biopsy, it’s the one thing none of the 5 neuros ever mentioned. Not sure I want to go through that. Have EDS, heal slowly & my recent surgery has brought my MCAS back in full force. I was able to quiet it down with SAAT acupuncture for several months. Retreating to see if it works again.

Myotonic Dystrophy didn’t seem to fit my case. We discussed it at some point

 

[georgina699]

I have a worsening ptosis that has already been corrected once .

Hi. How long do results from ptosis surgery last? I'm considering one.