csf myelin question.....

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olly

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sral

i too have more of a waddling gait due to weak thigh muscles and weak hip girdle.
if my spasms are bad and my legs are in a more spastic state my gait is more spastic and my balance is worse.
apparently in spastic paralysis this is the case,after a spastic/stiff episode the muscles become more weak and flaccid and this alternates,this is what my neuro and pt told me.
i have problems going up stairs due to weakness but downstairs due to balance problems,it is confusing.
does your mum have hypereflexia? in mg from what i have read reflexes are normal or absent according to the type,in mg there normal....in lambert-eaton syndrome there reduced.
i also considered muscular dystrophys but reflexes would be reduced or absent.
if reflexes are hypereflexive umn involvement is usually the cause.
hope this helps a bit.
take good care of yourself and your mum.
caroline:-D
 

BethU

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Beth you seem to know quite abit about MG. I guess this is because you were at one point diagnosed with it.

Also, with MG I read that people have difficulty going up stairs and my mom definitely has issues going both up and down stairs (more up than down). People on this forum with PLS say that they really have trouble going down but not up.

Would you know about the MG gait and trouble with stairs?

Hi, Sral ... I don't know much about the gait and stairs issue, but Caroline posted a really informative reply. I read messages on an MG forum for a year, and I do remember people talking about how difficult stairs were.

I was in TOTAL denial about ALS for a year ... from the time a doctor first noticed tongue fasics and said I needed to see a neurologist, which was 9 months after my speech problems began. Before that, I thought my slurred speech was from the entubation after surgery, and that my eyes were just getting worse from old age.

I refused to even consider the possibility of Lou Gehrig's Disease ... it made me furious when anyone suggested it! My primary care physician was the first to suggest MG, and I glommed onto Google, and agreed with her instantly! My insurance case worker, who is a nurse, also thought I had "classic MG." When neuro #3 gave me a prescription for mestinon, I thought all my problems were over.

However, the mestinon only improved my strength and energy (although that was a dramatic improvement for me), and as my symptoms have progressed in the last few months, I can see that ALS is almost certainly the correct diagnosed.

The important thing, I think, is to make sure that ALL other possibilities have been ruled out before you diagnosed ALS. It is a terrible feeling to think that your doctors are overlooking other possibilities, or have made a mistake.

What has helped me accept the ALS diagnosed is the fact that finally, all the tests for MG were done. I had a ct scan looking for a tumor on my thymus gland (a rare cause of MG), two specific blood tests, and two EMGs (although I understand an EMG can't differentiate between ALS and MG -- but I'm a little hazy on that). Most importantly, I had a clear explanation from the last neuro as to WHY he was convinced it is ALS and not MG. (In my case, it was the tongue atrophy.)

It was my PCP who ordered the MG blood tests for me. It sounds like your doctor is pretty understanding. I would talk to him/her again, and explain your concerns. Until you are convinced that every diagnostic procedure that can be done has been done, this will bother you. I think the burden of proof is on neuros to explain fully why they have made this diagnosed.

Good luck. It would be so wonderful if your hunch is right!
BethU
 

crystalkk

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Beth,

Did you have positive MG antibodies? How long were you on the mestinon?
 

BethU

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Beth,

Did you have positive MG antibodies? How long were you on the mestinon?

I was at the very top of the "normal" range on the Achr test. The next point on the scale would put me in the "possible MG" range.

I started mestinon January 22, and had remarkable results across the board for 2 days. My speech improved incredibly ... maybe by 50%. The slurring not only improved, but the speed, too. It was fabulous. Also, my swallowing seemed better, and the energy ... Wow! I was able to empty a large wall-to-wall, floor-to-ceiling bookcase, and carry armloads of books to a new bookcase at the other end of the house. Lots of walking, lifting, carrying heavy loads for 5 hours! The day before, I couldn't lift a bucket of water.

After a couple of days, however, the speech and swallowing improvements went away, and then I got interactions with my beta blockers ... dizziness, low b/p, etc, ... so stopped the mestinon till I could see a cardiologist. The cardi OK'd my stopping the heart meds and I resumed mestinon on March 5. Once again, got much improved strength and energy, but no improvements in the other symptoms.

I took mestinon for two months, till May 4, when I had to stop for the EMG. The neuro diagnosed ALS on May 5, and was extremely emphatic about it. He said there was no doubt whatsoever that it was ALS. He's the one who told me "everyone" gets strength and energy from mestinon, so my reaction was not important clinically.

(But I still wonder, if "everyone" gets strength and energy from mestinon, why the heck isn't everyone with a neurological problem taking it?)

Anyway, I figured I'd be better off in the long run by resuming the heart meds instead of taking mestinon, so I stopped it and have not had that kind of strength/energy since.

But there's still that tiny nagging doubt. I have decided to ask my cardi to switch me to a different type of medicine for heart (calcium channel blockers, instead of beta blockers), and I'm going to try mestinon again. If I get improved strength again, I think the neuro will have to reexamine my history and possibly do the blood tests again. Or else start prescribing mestinon for all ALS patients!

The neuro who diagnosed MG told me I should be upping the dosage ... he said "take as much as you can stand" ... to try to achieve results in my speech and swallowing again ... and I know lots of MG patients were taking a much higher dose than me ... but I didn't want to do that without monitoring, and none of the neuros I've dealt with were willing to do that. Since mestinon does not address the underlying disease ... it's symptomatic relief only .... I thought I could do without the energy. And there are side effects to mestinon.

It's confusing because I have some symptoms that are clearly indicative of MG, and some that are clearly indicative of ALS. Which leads me to believe that I might have both, or just a strange variant of ALS. And I have read (online, natch) that "some" ALS patients do report benefits from mestinon.

Scientists are also currently investigating the possibility that ALS could be a problem at the nerve/muscle junction, which is exactly what mestinon addresses. So there are lots of "ifs."

After 2 1/2 years, it's just a relief to have a name put on this blasted condition. Even if it is a name I dreaded ... ALS.

Sorry this is so long. I just can't seem to shut up!
BethU
 

CindyM

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I, for one, am glad you are sharing so much, Beth. I have been waiting for 2 years for a disease that fits my symptoms but never knew that half of the time a test for the anitbodies will not show MG in the early stages. I had given up on MG as a possibility until I read that on these forums.

Now, MG is my new favorite disease! :-D Much better than MND! :(

Plus, I started with profound weakness that did improve so now all I have is exercise intolerance. Another good sign. My newest symptom is breathing issues - another sign of MG. So I am hopeful.

Your posts really help.
 

awieleba

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Cindy~

hi, and agree-mg is much better! do you have any area's that are thinng or losing muscle?
I have host of problems but exercise intolerence is a biggie for me. I would love to think mg, my neuro has even brought it up as a possibilty. But when I look at the slight atrophy on my ankle, thimmimg calves and thigh, I am sure its not mg.

has anyone on this thread had a muscle biopsy? I am and I am nervous that it wii hurt.

thanks,

april
 

BethU

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IPlus, I started with profound weakness that did improve so now all I have is exercise intolerance. Another good sign. My newest symptom is breathing issues - another sign of MG. So I am hopeful.

Your posts really help.

Hi, Cindy ... it sounds like you have a lot to be hopeful about! But be really careful if you are having breathing issues, and there's any possibility of MG. "Myasthenia Crisis" is the one big danger with MG ... it's a medical emergency. My respiratory failure came on fairly quickly ... I'd been short of breath for 1 1/2 years, then over a period of about 24 hours, my breathing got worse and worse and worse until I panicked late at night and called 911. Which led to 3 weeks in hospital, entubation, (yuk), constant respiratory therapy, etc. But I survivied and recovered a lot of my lung function.

You might want to talk to your PCP or a pulmonologist about your breathing.

It certainly sounds like your symptoms should be re-evaluated! I think neuros just hate to keep saying, "I don't know yet" They want to close the file, and not second guess themselves. So it's up to us to keep an open mind and explore other possibilities.

Fingers crossed for you!
BethU
 

olly

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hi everyone.

my neuro also ruled out mg early on in my illness along with neuropathy and many other things.
my ck levels were elevated but my neuro just said he had seen them higher in some people,whatever that means:confused:
i did have a normal ncv and only realised after joining this forum and finding out about emg's that i have never had one or a muscle biopsy:roll:
so at my next appointment i am going to ask for these 2 tests,as being labled with mnd but no definate diagnosed of pls is "not playing cricket" as us britt's would say:lol:
i too have had respitory problems on/off the past 18mths,about the same time i started with the bulbar symptoms.
cindy....get your breathing checked out to make sure your ok,i'd hate to see you get really ill with that.
take good care everyone:-D
 

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Caroline/Beth thanks for your replies.

Beth,

You said that after 2.5 years you finally got diagnosed because of the tongue atrophy. What did you say your symptoms are after 2.5 years? It sounds like you are fairly mobile from your description of the energy you had back in March when you took Mestinon for the 2nd time. If you do indeed have ALS it's moving very slowly.
 

BethU

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Beth,

You said that after 2.5 years you finally got diagnosed because of the tongue atrophy. What did you say your symptoms are after 2.5 years? It sounds like you are fairly mobile from your description of the energy you had back in March when you took Mestinon for the 2nd time. If you do indeed have ALS it's moving very slowly.


Sral, right now I have weakness in both hands, but worse in left hand. Takes two hands to push the button on the gear shift, or turn a key. Sometimes, it takes two hands to squeeze the toothpaste out of the tube!

I have definite atrophy on both sides of my left hand, I think, and it looks like there's a little in my right hand, too. Have gentle twitching all down my left arm, which I rarely feel. It's not a rolling motion or waves, just ... twitches.

My speech is gawd-awful. It has really degenerated, and I cannot pronounce any consonants clearly.

One of the most annoying problems is the excess saliva ... it has become so thick, it's like having a mouthful of jelly all the time. Hate it! (Don't know if the thickness is from the Scop patch or not.)

The other big problem is no lip seal. With every bite or sip, I have to press a towel over my mouth in order to keep the food or liquid in my mouth. Even with the towel, I have to wash my face after eating, I'm such a mess from drooling, etc., pushing food back in my mouth. There is no way I would eat with anyone watching!

I don't know if it goes along with the lip seal, but my mouth droops open and way down on the right side. This has been like that for 1 1/2 years. The left side of my face is stiff, and I've lost my smile again. (I had it back for a couple months this spring.)

Still have double vision at a reading distance, especially at night. If I'm very tired, the double vision will "swim" ... columns of a magazine float over each other and it takes a real physical effort to hold a focus.

But ... knock wood, I am not aspirating liquids, and am eating solid foods in small bites. Living mostly on fruit smoothies and shakes and some solids. Have managed to gain 6 1/2 pounds since diagnosed, which the neuro said was important to do. (I'd lost 24 pounds since the symptoms started, and still don't have much appetite, but am loading up my smoothies with protein powders and supplements, etc.)

And I am mobile ... still driving, walking, and am moderately active. I have had to cut out cardiac rehab (which was mostly treadmill) as even a slow 20 minutes exhausted me, but I can shop and do light housework. I do Pilates once a week using resistance (light weights) on the leg exercises, and just range-of-motion exercises for my arms.

My legs are still strong, although I think there's a little atrophy on my left foot. My right arm is noticeably stronger than the left.

I have hyper-active reflexes, and bouts of weepiness over stupid things.

I am VERY grateful for bulbar onset and slow progression, as it's giving me the motivation to get all my closets and drawers organized before I lose use of my hands and arms. I would hate for my poor nieces who will have to go through everything when I kick the bucket to have seen my closets in their normal condition! :)
BethU
 

sral

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Wow Beth you really have been progressing slowly. By the way hyper reflexes are a symptom of PLS. Maybe there is a possibiltiy that you have PLS rather than ALS. If it is ALS it is definitely a much slower variant - which is great. My mom's symptoms started roughtly just over 2 and 1/2 years ago and the progression is relatively the same or maybe faster. Instead of affecting her arms after her speech (as in your case) her legs were affected next. Her arms she claims feel weaker but they are totally functional so far.

Wishing you all the best. Let's pray for a cure.
 

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I have lost small muscles in my hands and feet, and although my calf measurements remain the same for the last 24 months (when we first started measuring them) my muscle tone is non-existent. The best way I can describe this is that I have suddenly too much skin- in my legs, buttocks, and shoulders. I can push my fingers way into the bones and the indent will remain for a few minutes.

The ALS clinic noted on my records that I have "muscle wasting" below both wrists and now I see it in my feet but really, with no other changes in the last 2 years, I feel quite optimistic. I’m even getting used to not breathing well. I tell everyone that breathing is highly overrated! :-D
 

sral

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Hi Cindy,

Thanks for sharing. You mentioned a previous post that you've been waiting two years for a diagnosis. Did you symptoms start two years ago?

Based on the neuros notes of muscle wasting was a diagnosis made or are you still waiting? Is the muscle wasting and breathing your only symptoms or do you too have difficulty walking and with stairs?
 

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Yes- they did start a little over 2 years ago- first with pain and cramping in my hands after workouts. They noticed exercise fatigue at physical therapy, where I was sent for the hands. (They thought it was carpel tunnel and I do have some arthritis in my neck.) The PT guy urged me to get a second opinion, and that first neuro noticed muscle wasting but we were thinking MS. (Or at least they were. I was not used to being sick and kept thinking there would be some logical answer.

Then I started having bowel incontinence- another MS sign. I got so weak at work that I could not walk, so they sent me to hospital, where yet another neuro noticed my muscles twitching and sent me off to the ALS clinic.

I still have trouble climbing stairs, and need 2 hands to do a lot of things that used to only take one. And I have lost my ability to hop or jump or do straight leg lifts. I have no strength.

It all sounds very dire, and very clear-cut. But in neurology, things are not always clear-cut. And sometimes clinical evidence does not add up right away. By the time I got to the ALS clinic I was stronger, so while they are sure it is neuromuscular, they are not ready to say what it is. I am much more optimistic after learning about diseases like MG. :-D
 

sral

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Cindy,

It's good to hear that you are doing so well. It seems that you are totally functional and really have had no limb impairment.
 
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