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

[opusensemble]

Hi everyone,

This is my second time posting. First time I had not been checked by a neuromuscular illness doctor yet.

Meanwhile I got checked and this doctor identified the following symptoms with 1 year progression since onset:

✓ Generalized Hyperreflexia
✓ Generalized stiffness in 4 limbs
✓ Fasciculations in the limbs (+ electrical buzzing/crawling sensations)

My Dr. said that since everything was ruled out by bloodwork, and brain/spine, MRIs are clean, based on the symptoms above with no apparent weakness he could see, and no muscle wasting, that it does not match ALS, but he believes that it's PLS and scheduled me for a 3 Tesla MRI to find atrophies and for an EMG, both to happen soon.

I was shocked and asked him if it could be BFS but he said no because of the Hyperreflexia and I am not exactly sure what I can expect in terms of odds.

Do you think doing the 3 Tesla MRI will possibly show anything helping on PLS diagnosis just 1 year into the symptoms?

Will the EMG result possibly give any clue towards PLS confirmation or rule it out? I've seen references saying that in PLS usually has normal EMGs,

So do you think my two options are really what they seem and the doctor couldn't deny when asked:

✓ If no MRI atrophy signs and clean EMG, I still need to wait some more years before a PLS diagnosis can be made, assuming no lower motor symptoms occur.

✓ If the MRI has atrophy signs then it warrants a PLS diagnosis

✓ If the EMG comes out dirty, then it increases the chances that ALS is the diagnosis once weakness or atrophy develop.

Do you think my logic is correct or that I am seeing something wrongly?

Thank you/Regards

 

[lgelb]

It depends on what kind of EMG equipment and expertise they have. Some centers can detect signs of PLS (UMN dysfunction) on EMG. I don't know if yours is one such. But for most centers, your logic in the first case is true, in the second case, you would have to rule out all other atrophy causes, and as I say, the third case depends.

There are also some differences in MRI technology and how much 3D modeling they can do on the data.

As with any MND, we would recommend seeking a second opinion after they present you with the findings.

Best,
Laurie

 

[KarenNWendyn]

Hello again Opus.

Here is your previous thread for reference. The moderators may want to merge it with the current thread.

https://www.alsforums.com/forum/do-i-have-als-als/42982-i-guess-real.html

My first reaction to reading your current post was sadness over the diagnosis. Then I went through your previous posts where you wrote a thesis making a case for why you felt you had ALS based on cherrypicking lines out of Wikipedia. All this coming five years after you stated you were bedridden for depression for 4 years.

Sorry, but I’m not buying any of the ALS/ PLS stuff for you. We don’t feel ALS for reasons we have detailed extensively in your las post.

To then jump to PLS seems far fetched to me for the same reasons.

Please forgive me if I’m wrong.

In any case, go through with the workup the current doctor suggests. If there is still the thinking that it’s PLS, please seek a second opinion.

 

[ShiftKicker]

Karen, no merging possible- old thread is closed due to inactivity.

PLS is via a long process of elimination and must be made by a neuromuscular specialist familiar with the disease. It's not just hyperreflexia but a particular spread of pathological reflexes that shows increase and signature pattern spread over time. People with BFS absolutely can have hyperreflexia- and commonly do. The issue is the pattern of reflexes- and how the entire body reacts when a specialist elicits a reflexive action.

Edited to add- PLS is not just about reflexes but also a variety of other upper motor issues. Again, only to be assessed by an experienced clinician. Spasticity is obvious to a clinician who is following you, even if it's mild. PLS is a degenerative disease and shows increase and spread over time in a pretty predictable pattern of spread. There is a difference in "feel" to the diagnostician assessing you, from what I've been told, between spasticity from different causes- stroke, HSP, PLS and so on. Rigidity or tight muscles do not always equal spasticity.

All tests ordered by specialists will provide information to help with diagnosis. You really should be asking them your questions, as they are the ones ordering the tests and will be able to tell you their justification.

 

[opusensemble]

Thank you for your replies. I will proceed with the exams.

Am writing this from the waiting room of the MRI center in Coimbra/Portugal. This time with a 3 Tesla machine upon doctor request of investigation of "pyramidal tracts" and will try to have the EMG next week on Friday.

I would like to add in a single reply covering your comments, that regarding the pattern of the stiffness I have, it progressed from extremities and moved upwards toward my legs and arms, "conquering" more "length" of the limbs gradually over the last 10 months.

Other thing I would like to add is that I had 50 rTMS stimulation sessions in 2016 for depression protocol, and back then the doctor could not ilicit motor potentials for the left thumb, only to the right ones and has mentioned at the time that was very strange.

Coincidentally, my current stiffness/dexterity-loss/mild-weakness symptoms were first noticed this year in that same thumb. And according to the PLS literature I've read, this is a common finding in PLS patients (i.e. the unmeasurable motor threshold potentials on rTMS).

i have recovered from the depression since then, so I wouldn't look at it as a suspicious confounder, after I long overcame that depressive phase and moved on grateful.

My recovery happened due to Magnesium L-Threonate effectively obliterating a 4 year long depression in a matter of days. Go figure why.

(As a side thought, considering both ALS and PLS have in their pathogenesis hypothesis a possible contribution of Glutamate excitotoxity which Magnesium L Threonate influences, I wonder if that could have played a role in "whatever I have". I did stopped it months ago and the symptoms kept progressing so hard to gauge)

Sumarizing this, what brought me to this new premature post, was really about the widespread brisk Hyperreflexia detected yesterday by my neuromuscular Doctor. I was caught by surprise and shocked to see how my limbs reacted to simple taps. I was no idea I was like that. I could kick a standard football at least 2 meters on a soft floor, with such reflexes. Arms and wrists also extremely reactive to my total disbelief.

I was not really thinking about Lou Gehrig's after having time to absorb your comments in my first post. I wanted to believe you taught me a cyberchondria lesson in the other post.

And was ready to long for the BFS hypothesis, if the doctor didn't write everywhere in the exam requests "Primary Lateral Sclerosis?" and told me it was his best guess, asking me to stay positive as (on his words) "PLS will give you more time" (than ALS, implied)

I will try to have the reports of the MRI and the EMG before leaving my case here properly documented, although PLS takes 3-5 years to diagnose anyway

Thank you for your valuable advice.

 

[ShiftKicker]

I will try to have the reports of the MRI and the EMG before leaving my case here properly documented, although PLS takes 3-5 years to diagnose anyway

Yes, please post any documented indication from your doctors that MND is the diagnosis. Until then there is not much the people here can do for you. This is a support group for people with diagnosed Motor Neuron Disease. The recommendations you received in your last thread (closed to prevent continued debate) was to continue working with your doctors and to post ONLY when you had received documented proof of MND. This still stands. This forum is not equipped for lengthy debate and self directed research intended to convince folks here of your health status. Not that the people here are uninterested, but it serves no purpose and does not provide benefit in any way. Doctors are the people you need to be articulating your history with as they are in a position to actually do something for you.

 

[opusensemble]

Hi again everyone,

I have performed the EMG (with Nerve Conduction Test included) and here's the follow up:

I was inside the doctor's office for a total of 5 minutes and 30 seconds (made an audio recording of it) and during that time was asked by the doctor to lie down on a stretcher with my clothes on (Dr. just rolled up my sleeves and pants)

Each extremity took a total of 8-10 seconds to be examined and the exam took exactly 1 minute and 8 seconds from start to finish, after which the doctor declared: "_Pronto! Everything's OK!"

I was in disbelief about how quick the procedure was. I was expecting an EMG to last at least 15 minutes, and it took only 1 (one!) minute.

I didn't see what was done but had just one needle sticked on my thenar muscle on each hand and somewhere around my ankles and one or two electric pulses applied in each extremity from what I can remember. Blazzing fast exam. Fastest 80 EUR I ever paid in a doctor's office.

Could someone comment on this? I didn't even get time to warm the stretcher.

I am attaching pictures of the results:

✓ All the fasciculations I have been having permanently for many months, keeping me awake at night, misteriously calmed down in the day of the EMG, just to make me sound ridiculous if I tell someone that they've been keeping me awake at night all this time.

That also would possibly discredit my claims that since Jan 2018, the progressive Insidious stiffness on the fingers, hands, forehands, toes, feet, lower legs, and now elbows and knees, had me from being an active and highly motivated person working 70+ hrs a week to lying in bed with my hands between my legs in fetal position unable to use them for typing without feeling highly disturbed, together with how psychologically disturbing the stiffness in my feet, legs and knees has become, unresponsive to any holistic lifestyle changes.

So, in summary: No fasciculations picked up in the 4 sites briefly tested.

✓ No fibrillations or PSW picked up in the 4 sites briefly tested, either.

I'd benefit from any more informed insights or collective experience you could give me on this, namely:

✓ Can such an 1 minute duration EMG exam be trusted just with 4 extremities quickly tested, specially considering the widespread and de-facto debilitating nature of my stiffness?

(I'm not even mentioning all the buzzing and electrical popcorn and flickering in my limbs as on that I will assume that you were right all the time in my first post, and fibrillations can't in fact be felt. Still highly suspicious about that one, but okay, giving the benefit of the doubt as the collective wisdom of this forum possibly surpasses the one of any MND clinical center on the planet)

✓ In case I can trust such EMG, what in your considerate experience do you feel this exactly tells me in terms of ruling out PLS? (Remember I was diagnosed with generalized Hyperreflexia)

I have the notion that PLS usually presents with clean EMGs, but since there is no clear cut between ALS and PLS, I was led to believe from literature that eventually some minor LMN signs can show up in EMGs (please correct me if you feel this is not accurate)

Considering the lower odds either for UMN dominant ALS and for PLS, I am fueling zero cyberchondria at this moment, but the insidious stiffness symptomatology progressing like the plague, remember me constantly that something serious is going on and that I can keep denying it like did most of the year, telling myself it couldn't possibly be an MND as that "only happens to others".

I'm sure you have seen all sorts of false claims and exaggerations out of psychosomatic conversion or patients' subjectivity, and like you, I am trying to remain objective.

I'd be so grateful if you could comment on the trustworthyness of these EMG results and based on my clinical presentation, on the odds that any UMN dominant MND is lurking.

I will soon receive the results of the 3 Tesla brain MRI looking for pyramidal tracts atrophy and other signs, which I will post here as soon as I get them.

Thankful in advance for any opinion you can give me.

Joao

 

[KarenNWendyn]

These are the symptoms you report:
“ Generalized Hyperreflexia
Generalized stiffness in 4 limbs
Fasciculations in the limbs (+ electrical buzzing/crawling sensations)”

Even though my Portuguese is a tad rusty, I can see that on the EMG, you had testing of both the right and left 1st dorsal interosseous muscles and both right and left tibialis anterior muscles. This means they tested representative muscles in both hands and feet.

All the results indicate normal or absent (“absent” meaning the absence of abnormal electrical signals).

This means your EMG was normal.

Normal EMG = no ALS

Considering you complained of symptoms in all 4 limbs, this EMG is adequate to clear you of ALS.

 

[lgelb]

As we have said, atrophy is minimal in PLS, much more pronounced, ultimately in ALS, and from the report you did not have an EMG that could detect UMN dysfunction.

But it did not detect LMN dysfunction, so again there is no reason to suspect ALS, Joao.

As for PLS, I will leave comparison with their symptoms to those here who have PLS, but Karen is right that there is more to UMN dysfunction than reflexes or stiffness. Not reading Portuguese, what does the final note below the EMG findings say? And the doctor who is throwing out the possibility, is he a specialist in neuromuscular disease? I believe you said not.

From what you have said, I don't know the basis for his suspecting PLS, but I don't see it.

 

[KarenNWendyn]

Laurie, “This exam reveals no significant lesions of peripheral nerves to suggest neuromuscular disease or other motor neuron degenerative pathology or signs of muscle fiber lesions”.

I can’t read the very last sentence, but suspect it refers to previous comparative studies.

 

[ShiftKicker]

I speak for my experience being diagnosed with what is likely PLS, but definitely UMN onset motor neuron disease. Long winded.

I did not ever have a normal clinical exam.

I was referred to a neurophthalmologist, who found nothing wrong with my eyes, but noted Upper Motor/Pyramidal issues when doing a general neurological exam. Clonus. Abnormal reflex response in some areas. Weakness. Never "stiffness".

A general neurologist conducted my first EMG, which indicated there was chronic denervation and abnormal recruitment patterns. I did not ever have a normal EMG test (I've had 6 over the years). This combined with abnormal reflexes gave him cause to diagnose Motor Neuron disease- NOT bilateral, NOT just "brisk", but whole body pathological response to particular reflex and strength tests.

UMN issues are not just "stiffness" or brisk reflexes, but a distinct pattern of abnormal response to stimuli. There are particular patterns that go along with particular neurological issues- the specialists are trained to sort out the patterns and figure out where in the brain or body the signals are being interfered with based on how you respond to various tests. How you respond to particular stimuli (trembling, bad aim, stop/start, limits, etc, etc) dictates where in the brain there are issues. For example, they could be whacking your knee to elicit the usual patellar reflex, but they are very carefully watching how the rest of your body responds too- because UMN issues can cause reflexive response elsewhere when a neuro tries to elicit just patellar response.

UMN issues have a very distinct pattern and "feel" to a neurologist. Same with Parkinsonism. Same with Dystrophies, ataxia, etc, etc. When a neuro tests muscle movement and response to stimuli, the body responds in a particular way depending what part of the brain is being affected and this guides the neurologist in their course of inquiry.

The neuromuscular specialist I went to- the person who officially diagnosed me- indicated there are signals on an EMG that can help diagnose UMN issues. A particular overall recruitment pattern and distinctive "sound". No idea what it is, but there you go. Not direct, like how Lower Motor issues come up, but part of an overall pattern, along with the abnormal muscular response to particular stimuli.

You'll have to keep talking to your doctors to find out what's going on with you. I'm hoping they know your entire medical history, including the meds (prescribed and otherwise) you've taken over the years- as this can have bearing on movement and muscular issues too. Particularly any heavy duty sedatives or psych meds. Hopefully your MRI will provide further insight.

 

[Clearwater AL]

opusensemble,

You have presented us with another studied, well worded, and very descriptive
near thesis again.

Your Thread is somewhat familiar to Threads we have reviewed before from
others.

But, what makes yours uniquely different is... I believe you are the first to
have timed a Neurological exam.

"I was inside the doctor's office for a total of 5 minutes and 30 seconds (made an audio recording of it) and during that time was asked by the doctor to lie down on a stretcher with my clothes on (Dr. just rolled up my sleeves and pants)"

"Each extremity took a total of 8-10 seconds to be examined and the exam took exactly 1 minute and 8 seconds from start to finish, after which the doctor declared: "_Pronto! Everything's OK!."

"I was in disbelief about how quick the procedure was. I was expecting an EMG to last at least 15 minutes, and it took only 1 (one!) minute."

I think you need to check the battery in your watch.

My opinion, you knew how the exam was going to go before you went in there
and wanted to have another rebuttal available.

Again, I believe you are the first to post here having timed (near stop watch timing) the different procedures of a Neurological exam. Congrats.

 

[KarenNWendyn]

I think we’ve pretty well beaten this one into the ground, but since I know many people are out there reading the DIHALS forum, this is for those people...

Obviously there’s a lot more to diagnosing PLS than brisk reflexes or even brisk reflexes with a little clonus and positive Babinskis. Many people have those findings as normal variants. If everyone with brisk reflexes and a normal EMG had PLS, that would be many, many people.

And in terms of ALS, as we’ve said repeatedly, normal EMG means no ALS.

 

[ShiftKicker]

I am only leaving this thread open for opus to post the result of his MRI when it comes. It is not open for anything else as there is no point in discussing till all information is received by the op.

 

[opusensemble]

Hi again good friends,

(Never said enough times, I would like to deeply thank you for sharing your collective knowledge and individual experiences. This forum is a true reference and world lighthouse for MND information, specially for someone like me in a small country like Portugal where no online resources exist for it.)

Please find below some clarifications and the updates about my case (which I intend to leave documented here the best possible, for eventual benefit of others)


Adding to your comments:

✓ (on lgelb question) Yes, my EMG was requested by a general neurologist suspecting PLS.

✓ The EMG was performed by a MND specialist but only tested 4 muscle groups. (I've been assuming based on Karen's comments it is probably enough, considering it covered the 4 extremities I complain about: my extremely stiff hands and feet, forearms and lower legs)

✓ Sorry for not having provided English translations of the EMG report, and btw kudos for your Portuguese abilities. The EMG report basically said in medical lingo: "Normal results", which I know clears me from ALS (assuming as I am, that it was enough and well performed).

✓ (on ShiftKicker's comments) Absolutely Yes, I told the new MND doctor I will talk about below, about my history of antidepressant meds which I have stopped 2 years ago. She factored that in.

✓ (on ClearWater AL's comments) The EMG timing was not intentional. I simply recorded it thinking I'd like to replay the EMG sounds later. I thought the one setting a world record was the doctor with such quick exam


So, Updates: I've got the 3 Tesla Brain MRI results and was referred to a MND-specialized neurologist. Here's what she had to say:

✓ She looked at the EMG report and questioned me "_What about the other muscles?, You only were tested for these 4?" I didn't know what to answer her, but remembered what Karen said here about such muscles being representative and throwed it at her and she said: "_Well, in principle...".

✓ She then looked at the MRI scan (Portuguese report attached: Basically it mentions "some simple milimetric sized bilateral white matter lesions, non-specific", so not requiring translation). She said that the MRI results are uneventful in the context of the PLS investigation, and that at least it didn't point to any early atrophy related with the PLS hypothesis.

✓ She then performed me a physical examination (first time I got it done by this doctor) and she noticed the generalized hyperreflexia as well as muscle deep tremors in my lower legs, feet and hands. I told her that it didn't bother me much compared with the insidious hand/feet/arm/leg stiffness, but she still prescribed me Inderal for it saying the tremors could affect my balance.

✓ She then summarized the appointment saying that although the antidepressant medication I took in the past could be responsible for some motor issues even later in time, that the insidious and progressive-ascending pattern of my muscle stiffness from the extremities towards my torso, requires further investigation with Transcraneal Magnetic Stimulation and she will do it at another clinical center here in Coimbra/Portugal together with other MND specialist, in about 2-3 weeks from now.

When she mentioned this I told her that when doing rTMS for depression in Valencia/Spain in 2016, the doctor could not determine the motor evoked potentials (MEPs) for my left hand/forearm. The MEPs were unobtainable. He found this very strange at the time and so did I, but since I had no other symptoms back then other than depression, I didn't worry about it, nor did the rTMS doctor. And this 2 year memory is haunting me now, considering this is a sign of UMN problems used for complementary diagnosis of PLS.


So to summarize, factoring in these inputs and your helpful insights I'd summarize it like this until I can get the TMS data:

✓ Clean EMG (although just made in 4 muscles, but on a optimistic perspective), means no ALS.

✓ "Clean MRI" in terms of atrophies means at least no noticeable PLS signs (according to the doctor probably too early in time for being considered as relevant in ruling out PLS, but done anyway)

✓ Generalized hyperreflexia, insidious (ever worsening) hand/feet/forearms/lower legs stiffness, and deep muscle tremors: As per your comments, not going to speculate on what it means or not, (I agree with Igelb, ShiftKicker, and Karen on this)

✓ TMS as a complementary diagnosis test for the PLS hypothesis (still kept on the table by this second MND doctor) was now requested.


Seems it will be a long 2-3 week wait until the TMS exam. I will document my results here, if the thread does not auto-close, if you agree with that.

I believe I didn't forget anything,
Meanwhile if you have any comments, I'd love to hear from You.

Xmas spirit for You all,
Joao