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ltd17

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Hi guys,

I posted a few times already here about my mother who has a possible diagnosis of ALS, but seronegative Myasthenia Gravis is also still being considered, but it cannot explain the hyperreflexia of her legs. So they think ALS and MG or maybe cervical radiculopathy. She has atypical things for both (for ALS: double vision at the start that disappeared with prednison and mestinon, for MG: Atrophy of deltoid muscles). Next Friday she will finally get an MRI of the neck while using her trilogy machine, as this was impossible before.

I was wondering if ALS patients here have gotten their PCF (peak cough flow) measured and if yes, if it varies.

I am wondering this because when my mother was admitted to the hospital in March with a possible Myasthenia crisis (weakness and not being able to breathe), she got 5 days of Immunoglobulin (IVIg). I Was looking at her charts and saw these results:

March 7th PCF: 90, admitted to hospital, Start of IViG 5 day
March 12th: PCF: 180, 1 day after last infusion of IVIg
March 15th: PCF 260
March 19th: PCF: 270

The better she felt, the higher the PCF went. Breathing also went better. The strength in her ams and neck did not improve though.

From what I understand in ALS the PCF would not improve with IVIg in ALS, so it seems to me that the IVIg reversed it? Am I drawing the right conclusion?
 

Atsugi

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IVIG doesn't improve ALS.
Some people are hyperreflexive naturally, without any disease.
If a person knew they had ALS, then it really wouldn't matter to check PCF or anything else. What would you do with the data?
 

ltd17

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Thanks for answering, that's what I thought too. The hyperreflexia has increased compared to last year, that has the neurologist worried as it doesn't match MG.

At the ALS centre here in Europe, which is a well known and renowned centre, the protocol is to measure PCF and FVC of ALS patients every 3 months. The PCF is to check the cough strength and see if patients can still clear secretions. If the PCF declines and reaches 270 or lower, they recommend the patient to start Air Stacking.
 

lgelb

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SNIP and PCF are among the best tests we have for "is it time for BiPAP"? [and/or some kind of assistance with coughing]

In re your mom, was Lambert-Eaton ruled out?
 
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ltd17

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Yes it was ruled out, no antibodies were found!
 

ltd17

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Laurie or anyone else, are there any forum members here that got diagnosed with both ALS and MG? It's very rare but I am wondering. I know Schatzie (?) was diagnosed with ALS and then the diagnosis was changed to MG after her response to mestinon but I am wondering if there is anyone that has both.

Mostly wondering if it's both, if the MG part of the disease would respond to IVIG and the rest not.
Going crazy! We've been trying to get a diagnosis for 1.5 year, I have read hundreds of articles in the meantime.

Today my mother finally got a second cervical MRI. The one in mayo last year showed moderate foraminal narrowing on multiple levels and advanced foraminal narrowing, especially on C4-C5 . I am really hoping the narrowing has progressed, that could explain why the IVIG works partially on the breathing but not on the arms.
 

lgelb

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I think we asked before and found MG + ALS in the literature but not here. But who knows who is lurking? So certainly worth another shot.

MG and ALS cause problems through different mechanisms, so no biological reason why IVIg couldn't work on the "MG" in theory while not on the ALS; this paper describes just that.
 

KarenNWendyn

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Yes, MG + cervical stenosis with radiculopathy +/- myelopathy would be statistically much more likely than MG + ALS, and could explain many of her findings.
 

ltd17

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Thanks for the link Laurie! I have read that article before, I noticed in thew articles about MG and ALS that there was not 1 patient with resp. onset ALS and MG. Most were limb or bulbar onset and often the ALS symptoms started in a different limb or area then the MG.

Mayo said my mother probably had respiratory onset ALS in the beginning stage, but couldn't rule out superimposed MG with cervical Radiculopathy as she did not fulfil the el Escorial criteria, but they couldn't explain diplopia and 28% decrement was probably secondary to ALS.

Also doesn't help that she did not have antibodies for MG, it's all speculation based on clinical presentation and response. So frustrating! In the beginning the response to Mestinon was really noticeable for her arms, and diplopia also stopped after Mestinon. But now IVIG only worked for the breathing.

Could someone have ALS for 2 years and not really lose weight? And have no fasciculations of the body or even the tongue?

I often read in articles that patients lose a lot of weight, the bulbar onset patients I suppose lose weight faster, but limb onset patients? My mother has gone from limited usage of arms last year to barely being able to lift a glass of water now. The deltoids jus't don't work :(
 

ltd17

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Thanks for your reply Karen! The thing I hate about statistics is that the 1% chance of winning the lottery is always against you, but the 1% of having a rare or shitty disease is the one you win lol!

We stopped Riluzole after the diagnosis was changed in August but now that it's unsure again I wonder if we should have stopped
 

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Respiratory onset ALS is rare, so it's not surprising, ltd, that none of the +MG cases fell into that category. But Karen is also correct, of course, that MG + spine problems is a better statistical bet.
 

ltd17

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Oh Karen and Laurie , I sure hope so, C5 innervates the deltoids which could explain the atrophy. Karen as an MD would probably know this better.
And then the narrowing could explain the progression of hyperreflexia. It's also symmetrical and proximal, and flail arm syndrome would of course not begin with respiration.

If it wasn't a diagnosis (even though they said probable) made by Mayo Clinic we would probably be a bit less stressed.
The FVC of only 33% (was 69% last year, thank god for the trilogy) and atrophy/strength loss of upper arms don't help either
 

Clearwater AL

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ltd17, just a thought..

Hyperreflexia is found among people with considerable stress and anxiety.

Maybe with all your mother is going through and facing, it has brought on this
possibility being overlooked.
 
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