Dr. says it's likely to be PLS. Will MRI and EMG help?

[opusensemble]

Hi everyone,

It's with extreme sadness about my life, and the circumstances around which I am led to today, that I just had the realization that I must have in fact PLS.

While reviewing medical literature on the differential diagnosis of ALS/PLS, I realized that once the differential process has been performed for the list of other conditions (which was my case), the presence of T2 MRI hyperintensities on the corticospinal tracts, a.k.a. "wine glass sign", is the mark of the beast and means ALS if there are LMN signs and PLS if there are UMN signs only.

The report of the MRI made this month, mentions regarding the corticospinal tracts, after explicit request from the neurologist that the MRI focused specifically on the pyramidal(corticospinal) tracts:

"We do not observe other signal changes, namely on the pyramidal tracts"

But as you can see attached the "V" or hyperintense T2 "wine glass sign" is clearly present in my corticospinal tracts.
And it was there all the time since my other MRI in April. It seems that radiologists clearly missed it, this second time even with precise instructions from the neurologist to focus on the pyramidal tracts, and 12 days to draw a report.

Could I possibly be interpreting something wrong? As you know I've been complaining of insidious progressive stiffening.

My eyes are burned of so much research during 2018 and fighting with family questioning my symptoms as "all exams came back OK", and dealing with stigma, gaslighting everywhere I turn, together - it seems now - with doctors missing such basic things on MRI.

Can't even find courage to get back to my neurologist. I wish I was wrong about all this, and Portuguese radiologists really knew what they were doing, but from everything I have found online, corticopsinal tracts should not show as a "V" sign on MRI T2 sequences, in healthy individuals.

Still have the TMS test to do, but not even sure if it makes sense at this point, as MRI signs can't exactly be refuted.

I feel I have exhausted my 9 lives, down in the depths of the rabit hole.

Thankful for any comments or advice you can give me,

Joao

 

[Nikki J]

If you do not believe the mri report ask your neurologist to review it. They are trained to read them too.

Please. No more speculative posts. Once you have TMS results and your neurologist’s interpretation of all the data please let us know her conclusion ( not yours)

Please do not argue or respond. Just let us know after the testing is complete

 

[KarenNWendyn]

Opus, it’s very clear that you are way smarter and more knowledgeable than everyone on this site who has PLS and/ or ALS. In fact you’re way smarter than any neuromuscular disease specialist and neuroradiologist on the planet.

So why bother us peons with your intellectual musings when you know better?

Wallow in your self-diagnosis of PLS and let the rest of us go back to our ignorant humble lives.

 

[Emanol]

If you are not happy with the services provided by your current doctors, you could always go elsewhere (although I very much doubt that all Portuguese radiologists would not know what they are doing). Consider crossing the border and going to Ruber International in Madrid. That is my go-to place when I have frustrations with the Spanish public system.

 

[opusensemble]

Hi Karen,

Sorry, I didn't mean that my thought process caused such reactions. I'm really struggling symptom-wise and it probably shows in my words and thought process. These messages took me many hours to type as my hands and fingers are incredibly stiff.

But I confess I thought this sub-thread would welcome people objectively discussing diagnostic criteria and exam results. As if there was a relatable truth-seeking aspect to it.

I never expected to be baby-sitted, specially after realizing how ambiguous describing UMN symptoms onset and progression can be.
My posts were very suffered, reflected and researched messages in a botlle, and I had hopes that someone would good willingly correct me if they thought I was interpreting any exam or reference incorrectly.

✓ My 1-minute duration EMG in the 4 muscles tested was clean. I didn't doubt the operator or the procedure. But by then I thought I could use some opinions.

✓ Then now, the 2 MRIs clearly shows T2 hyperintensities in the corticospinal tracts, a sign commonly referred to in literature as being highly specific (hallmark) in ALS/PLS(and Mills Syndrome) when the clinical signs are present, and said to be a "classical finding" in such cases. The radiologist ignored it, despite knowing my clinical context. Asking your opinion on my analysis was an equaly innocent request.

Please don't put me in such a bad light. I just asked for opinions of anyone not minding sparing one.


Update (20181228 ): Messaged my general neuro today about this MRI issue. He said he's not knowledgeable about such MRI detail and he would reply me after vacation.
That was intriguing, considering he was the one ordering the MRI. Besides, on my last appointment with the MND neuro, he talked with me a few minutes and told me he had discussed the MRI results personally with the radiologist and it looked OK. So I assumed he could at least comment on what he requested to be checked on the MRI. Similarly, my MND neuro only read the MRI report briefly in that last appointment day. So I had this feeling something fell between the cracks.

Herrare Umanum est and as you might imagine I am praying that they all know what they're doing. I'm optimistic by nature but pessimistic by experience, hence my post.

Summarizing, I'll gather then all exam results and doctors opinions', before daring updating it here.

Thank you again,
Joao


P.S.1
@Emanol: Thank you for the comment and the Hospital Reference.

P.S.2
(Some) Refs used, in the absence of a PLS sticky I could find:
[1] "Progression in Primary Lateral Sclerosis: a prospective analysis" - Mary Kay Floeter, M.D., Ph.D; and others
[2] "Primary Lateral Sclerosis", Jeffrey M. Statland, MD; and others
[3] "Differentiation Between Primary Lateral Sclerosis and Amyotrophic Lateral Sclerosis", Maria Carmela Tartaglia, MD; and others
[4] "The magnetic resonance imaging "wine glass" sign of amyotrophic lateral sclerosis", Umesh C Parashari; and others
[5] "Corticospinal tract MRI hyperintensity in X-linked Charcot-Marie-Tooth Disease", Jan Kassubek MD; and others
[6] "'Wine glass' appearance : A unique MRI observation in a case of primary lateral sclerosis"; A. Kuruvilla and others
[7] "Two case reports of an unusual association between Klippel-Feil syndrome and amyotrophic lateral sclerosis: Do they share same genetic defect?"; Koneru Lakashmi Umamaheshwar; A. Kuruvilla and others

 

[lgelb]

Opus,

T2-weighted CST hyperintensity is neither 100% sensitive [detecting all the true cases] or specific [detecting only true cases] to ALS or PLS or MND generally. See the attached paper that lays out percentages considerably <100%. Newer modalities like FLAIR are emerging, but case data even from these shows less than complete confidence.

Honestly, if there were a 100% certain imaging test, do you not think it would be included in the diagnostic criteria and reported here as how PALS were diagnosed?

Our objection is not in your reporting clinical results as reported to you. Rather, it is presenting your interpretation, based on some cherry-picked, selectively-parsed references, in place of a clinical diagnosis.

In short, if you have a self-diagnosis, you don't need our perspectives, so it's rather an insult to ask for advice while at the same time announcing that you are certain of the answer.

As for the neuro, he's not a radiologist, so not sure why you would criticize him for less than full understanding of MRI interpretation. It is common for radiologists to consult with other physicians on conclusions and next steps (often, "clinical correlation," amounting to the neuro's continuing to work with you to rule out or further explore possible diagnoses).

At any rate, as Nikki requested, please don't respond further until you hear more from your neuro or add other results. If you do otherwise, your post will again be deleted and the thread may be closed.

Meanwhile, for your own sake, since you are obviously suffering, I would suggest that you stop researching and start living. If the die is cast or if there's nothing much wrong, either way, it is your choice to turn away from "maybe" and do things that others here would give a great deal to be able to do.

 

[ShiftKicker]

Joao-

You seem to have misinterpreted the purpose of the DIHALS section, as you feel it's a place for debate and an opportunity to try to convince people of your self diagnosis. I will refer you back to the post: https://www.alsforums.com/forum/do-...-common-concerns-about-possible-symptoms.html

If you read it all the way to the end, and read it very carefully, you will find in the section "When You're Wrong" and "Finally" that it provides some guidance as to what to do when you have received answers from the people here. This forum's primary purpose is to support those who have been diagnosed with motor neuron disease and the caregivers of people who have been diagnosed with MND. This DIHALS subforum exists to keep those who have a few questions about ALS but are not diagnosed separate from the forum proper. Those who have not been diagnosed are considered guests. The members of this forum are not obliged to continue debating people who have self diagnosed and insist on piling link after link into encyclopaedic posts, nor do they owe people who insist on debating or using the DIHALS subforum as a medical diary continued attention.

Your symptoms are of course important and worrying to you- please use the appropriate medical resources to work out your concerns- those of your own health system and your own medical care providers. Arguing here nets you zero benefit, and in fact creates a burden for all involved. Your assumption that you know better than everyone, including radiologists and neurologists, indicates an inability to listen, to understand and to accept the answers you have been given, or the fact you may have to wait till you have all your results together in order for your doctors to see the whole picture.

If you wish to debate people about your self diagnosis, visit with someone who has your medical records in front of them and the ability to sit with you and explain in a professional capacity. If the anxiety is too much of a burden and you find yourself eager to engage with people on the internet who have asked you to stop, you may need to visit with a therapist who can provide you tools to help reduce your stress. This isn't the place for that.