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BethU

Extremely helpful member
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PALS
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Sorry ... this is going to be a long one.

As I’ve reported here ad nauseum, my bulbar symptoms started with both slurred speech and blurry vision, sometimes double vision. It was the double vision among other things that led to my mis-diagnosis with myasthenia gravis before I was diagnosed with ALS.

In the last couple of weeks, I’ve been experiencing “ptosis,” a drooping eyelid. It is another “signature symptom” (my term) of MG. Mine does not seem to occur the way it does in MG, where the eyelid is too weak to hold itself open. Mine is a twitch that lowers my eyelid almost closed, and sometimes seems to forceably hold it down. Also, in the last couple of weeks my blurred vision and double distance vision have started up again. My vision had been stable for several months, making me think it was safe to get new glasses. Apparently it wasn’t!

There is no doubt about my ALS, but I have these ocular symptoms left over. They don’t fit in with ALS. So of course, knowing that Wright encourages us all to consult Google whenever possible :)), I Googled “ALS ptosis,” and came up with a paper on the subject.

It was a report about “two old women” (one a couple years younger than me :-( ) who had bulbar-onset ALS and also ptosis and diplopia (double vision), which the neurologists who wrote the paper felt were due to MG. In other words, they felt these women had both conditions and that the conditions were somehow related (if I am understanding the use of the word “association” correctly). The difference between the women in the case study and me is that their bulbar onset ALS progressed very quickly, while mine has been slow so far, knock wood, fingers crossed, from my keyboard to God’s ear..
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Here are two interesting quotes from the report.

Finsterer described that 13.6% of patients initially diagnosed as ALS had mitochondrial myopathy [12]. This author suggests that patients with slow disease course and multi-organ involvement should undergo lactate stress testing and muscle biopsy. Our cases, however, were characterized by a rapid [ALS] progression rate, which precluded the performance of muscle biopsy. We believe that both our patients suffered from an association of ALS with ocular MG.

(Some lines are missing from the articles.)

[I]Several reports elsewhere have described the association of autoimmune disorders with ALS [16]. MG is probably underdiagnosed in old people [17]. Both our patients were old women with bulbar-onset ALS and rapid progression, which might represent a particular phenotype prone to this association. A chance association would be very improbable (based on the incidence of the two diseases the estimation for a chance occurrence would be 1/6 ×1018, for 2 patents). Thus, although the association between ALS and MG may be exceptional, we speculate that some cases have been missed. It is a possibility that the neurologist should be aware of.[/I]

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What this does for me is convince me that maybe I’m not entirely crazy (yet), and my ocular symptoms do have some significance ... but I also was struck by the report that ALS is sometimes associated with (other) autoimmune diseases, which I know is often a subject of discussion among PALS. Also, I’m baffled by the assertion that 13% of diagnosed ALS patients had mitochrondial neuopathy. I wonder, is that instead of ALS, in addition to ALS, or what?

This sentence is interesting, too: This author suggests that patients with slow disease course and multi-organ involvement should undergo lactate stress testing and muscle biopsy.

I don’t know what lactate stress testing and muscle biopsy would prove (MG?), and I can’t see myself wandering into the ALS clinic with this report tucked under my arm and saying “looky here, doc, what I found on the Internet.” I still have not been able to communicate to any neuro that in 3 out of 4 trys, Mestinon greatly improved my physical strength. In fact, my UCLA neuro's report of our first interview wrongly states that Mestinon had no effect. I let it slide because these unaccounted-for symptoms were always just shrugged off.

But, for what it’s worth, I thought the article was interesting, and might be pertinent to others on the forum who seem to have cross-over symptoms with MG. Even if they're not the proverbial "old woman.":roll:

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Here is the citation for the article:
Clinical Neurology and Neurosurgery 110 (2008) 168–170
Case report
Amyotrophic lateral sclerosis patients and ocular ptosis
Susana Pintoa, Mamede de Carvalhoa,b,*
a
Neuromuscular Unit, Institute of Molecular Medicine, Faculty of Medicine, University of Lisbon, Portugal
b
Department of Neurology, Hospital de Santa Maria, Lisbon, Portugal
Received 1 June 2007; received in revised form 24 August 2007; accepted 27 August 2007
Abstract
Ptosis is not a feature observed in amyotrophic lateral sclerosis (ALS). We describe two old women with bulbar-onset ALS and rapid progression, in whom ptosis and diplopia were noted. They did not improve on pyridostigmine or steroids. Antibodies against acetylcholine receptor were negative, thymoma was excluded, but neurophysiological showed marked neuromuscular transmission failure in orbicularis oculi. We discuss the association between ALS and Ocular Myasthenia Gravis in these cases.
 
Beth, that's a lot to take in. I need to read it over more.... I had to laugh at the "old woman" remarks though. I guess everything is relative.

I do intend to try to learn more from my neurologist next time I see her, so, even though you may not want to walk in with this under your arm... maybe I will :cool:

Actually. probably not, but I do want a better explanation of several areas (all?) And she seems willing to spend some time doing this, She'll take out a pen and start drawing brains with arrows to other body parts, etc, while many neuro's feel they don't have the time to do for us.

I see her the second week in November.... I think that being an "interesting atypical case" has worked in my advantage as she primarily is in research rather than seeing patients. She only see's patients on Monday mornings, so I'm thinking the other 4.5 work week days, she might be involved with stuff that can help us, or help us understand. :)

BTW, I've gotten those twitches that try to close the eyelid, but not very often. I also get a good bit of tiny twitches near the left eye area up on my cheek. They're so tiny, it almost feels like something brushing up against my skin, but its not really sensory (you can see the twitches) its just that faint.
 
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Beth

There is a blood test for MG and Mestinon a drug that if you have MG will impove right away.
Ask your neuro for the pill Its the easiest way to diagnosed I heard

Pat
 
Beth,

I wanted to answer one of the questions about being baffled, (This part quote my attention because I am undiagnosed.)

Reread this line:
(Finsterer described that 13.6% of patients INTIALLY DIANOSED as ALS had mitochondrial myopathy.)

They didn't have both, there DX CHANGED after the lactate stress test and muscle biopsy.

Thanks for the info,
 
Hi, Pat ... I took Mestinon for several months and it improved my strength quite a bit, although it has unpleasant side effects ... (in my case very annoying twitching all over my body; it's a common side effect).

However, all my MG blood tests came back negative, and the neuros are so sure it's ALS, I stopped taking it. The bigger problem with Mestinon is that it interacts badly with my heart meds, so it was a question of taking either Mestinon or beta blockers, but not both.

And you don't get tongue fasics and atrophy with MG. But it is interesting that there may be some cases where ALS and MG are somehow connected in the same patient.

Crystal ... Thanks for the clarification. 13% seems to me to be a significant number, if that report is reliable.
 
Brenda,

The lactate testing could be to see if one has Mitochondrial Myopathy. Sounds like the article is suggesting that this be checked in slow cases to see if Mito is masquerading as ALS.

Also, if you had significant improvement from Mestinon, wouldn't that argue for MG over ALS, or are you talking of improvement only in the occur symptoms (ptosis, etc.)?

I wonder about the autoimmune connection...do they test for various autoimmune disorders or antibodies in an ALS diagnostic work up? Including things like Celiac or antiphospholipid antibodies? If not, how could researchers know that these things might not cause or mimic ALS?

For how long have you had ALS and the bulbar symptoms?

Searcher
 
Hi Searcher ... I know neuros eliminate many autoimmune diseases before arriving at a diagnosed of ALS, but don't know if the ones you mentioned are among them. If the symptoms don't mimic ALS, then they wouldn't check it.

I would think that Mestinon improving symptoms would warrant further investigation, but those who diagnosed me with ALS shrugged off the Mestinon. (One neuro at UC Irvine told me that everybody gets increased strength from Mestinon, so it meant nothing. But I still wonder, if it gives everybody an boost in muscular strength and energy, what is everbody taking it?) By the time I saw my first neuro at UCLA, the report contained the erroneous statement that I had no response to Mestinon.

The primary effect was increased muscular strength (lifting a bucket of water that I hadn't been able to lift before the Mestinon. Toting armloads of books from one end of the house to the other). The first time I took it, it also improved my speech ... real improvement, such as increased crispness and breath control.

However, the neuros were so sure I had ALS, I decided not to fight it. My tongue fasics and atrophied tongue do not occur in MG. Nor does any other atrophy except for dis-use.

But if it is possible to have both, that would answer some lingering questions.

Thanks for your input!
 
My blood tests came back negative for MG, so I was diagnosed with bulbar ALS, but my new neurologist gave me Mestinon and it has made a huge difference. My right eye does exactly what you describe. I have researched it and have found that somewhere around 15% of MG patients test negative for the antibodies in the blood test, but have MG. I think that it is something that all patients who have been diagnosed with bulbar ALS and have have little or no progression should check into.
 
Shatzie ... thank you ! The Mestinon helped me, too, but interacted with my heart meds so I stopped it.

But I believe you have hit the nail on the head with the percentage of MG patients who have negative antibody blood tests, and are still diagnosed by clinical exam.

Has your new neuro ruled out ALS?
 
From what he told me, time is what will rule that out. I started having speech problems well over a year ago and it has not progressed. And if I take the Mestinon, most days I can barely tell there is anything wrong with my speech, and my energy is better than it has been in years. I go to see my new neuro again next week, the last visit was June 26.
 
Shatzie,

That's great news! Keep us posted about your nuero appointment.
 
That was an interesting read.... All my bloodwork for MG came back normal, that was tested once MS was ruled out..... The only eye symptoms i have had are when my face would spasm real bad on my left side it felt like my eyeball was going to pop out of my head. I always thought it was my eye because my eye would be red. The doc thought it was uvitis due to my contact and that the pain was muscalature in nature.... This was long ago.... I thought how the heck do your jaw and eye muscles cause this? Fast forward i now have a nice taught thin face like i had a face lift, but it was a long painful process! My left eye started twitching the upper lid, a lot... Now it twitches every now and then... Like what would be considered normal. I have droopy eyelids but that runs in my family. When i had my evoked potential test and they tested my eyes, the doc kept saying, wide open, wake up. I could not hold my eye open that long and apparently it kept closing. Not worried about it because there is nothing to be worried about at this point... Just thought i would chime in about my eye symptoms.. sam
 
That is great news! Let us know what happens at the neuro's.

I know in my case that ALS is it ... my tongue atrophy proves it ... but these leftover symptoms keep nagging at me. So the possibility of two processes going on at once is encouraging, as it would a) answer the nagging questions, and b) possibly allow some treatment that would address some of my symptoms.

Good luck at the neuro's!

(Thanks, Sam, too, for the info. :) The more info about these things, the better!)
 
HI Beth

Does your eye stick shut when tired? I am wondering, one of the first things I noted before thinking anything serious was that my eyes burned and felt strained. My vision got worse. Then My left eye, that is my weak side, will stick shut when I am tired at night. When I wake up in the night for baby, my one eye delays in opening? any thoughts or similiar expienence?

thanks!

april
 
Yes I have delayed eye opening sometimes


Pat1
 
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