Can we discuss reflexes?

[BlsdMama]

I have a working diagnosis of PLS. I’m aware of the statistics. So far two clean EMGs.

Had a Neurology appointment at my research hospital, not Mayo. Mayo has dx’d PLS as well but because a glycine receptor antibody was found a trial of IVIG for Stiff Persons was ordered. I’m about seven weeks into my twelve week trial. No improvement in rigidity or spasticity.

My appointment was disappointing. My right leg is weak but now my left leg has very hyper reflexes as well. Two oddities noted - my Babinski is now gone in the right foot? I have a shallow area that is new in the right foot - inside curve. Clonus is present and stronger in the right foot. It is newly present in left though weak.

For those of you diagnosed - were you initially hyperactive reflexes and later hypoactive?

Doc tells me a new EMG is coming my way after the trial is over. No surprise. We’re all waiting for it to go bad unfortunately. (I also have a flat muscle in my calf.)

But, Mayo called out of the blue the Monday after my exam and they want me back up there in two weeks. I’m guessing that isn’t good news. Initially it was supposed to be the end of February.

Thoughts?

 

[lgelb]

A PLS dx is not going to convert to an ALS dx based on reflexes alone, but if you want to know why Mayo is hunting you down, why not ask? You have the complete right to know before you agree to the trip.

 

[ShiftKicker]

Mod note- moving to PLS forum.

Laurie has a good point- asking why is a good idea! I was called in earlier than my original appointment, and it just ended up that they had a cancellation and were trying to move things around to allow for my doctor to go to a meeting off site.

A few other questions that might help you figure out what the appt is for...

How long do I need to plan for?
Is there anything I need to bring?
Which doctor will I be seeing?
Is this to meet with the clinic care team?

 

[BlsdMama]

Laurie,
My neuro up there gave me a diagnosis of PLS and then the GlyR came back positive. So, I still carry PLS (and my doctor here thinks it is PLS) she wanted me to go through the initial treatment (IVIG) for Stiff Persons. Our original plan was to meet at the end of the 12 week trial. When her nurse called she said that Dr. would like to see me in office on the second week of January and scheduled me for January 10th. I said that I was not done with trial. She said that she was sure she had reviewed my file and still specifically wanted to meet with me. I suspect to stop the IVIG and try plasmapheresis. But I am seeing nothing from IVIG and I have NO pain. (And SPS is a dreadfully painful disease.) I'm not saying I won't try it, of course I will.

But I am very skeptical and I was reassigned neuros here to one that is in the ALS Clinic and they have me attend ALS Clinic every three months as they would any other PLS patient. Obviously we all know that PLS patients have a high rate of transitioning to ALS and I know that I am *very* blessed to have gotten this past year with slow movement of my disease. I'm very, very thankful.

However, plasmapheresis is going to be very difficult for our family. It can be 1-5 times a week, during the day, and we have small children. I cannot imagine driving an hour there, undergoing plasmapheresis, and driving an hour back, if this is not SPS.

How demanding do I be about this?

What I would LIKE is if they are going to cancel the trial and push for the trial plasmapheresis is to have my next EMG BEFORE the trial begins. SPS is essentially the opposite of MND on an EMG. SPS is constantly turned "on" rather than off. And I think it is crazy to go through a difficult trial without the new EMG. But, then, is this a question for my doctor here or for my doctor at Mayo? AND I WANT the EMG done here. I feel my doctor is an expert in this (he is former Mayo Rochester and did his fellowship in Neurophysiology/Electromyography at Mayo. He would do it himself and he did my second EMG. He's very thorough.

Obviously I would be thrilled to change all of this to SPS. But nothing fits and my doctor here says we really don't know enough about the GlyR antibody to do anything more than essentially try this and that that have worked for others and see if they work. In the meantime, he's chosen to keep the PLS diagnosis and keep me in Clinic.

Grandma had FTD and it's just getting to the point that rabbit trails are tiring and I hope that doesn't come off as ungrateful because I am really and truly very grateful and remind myself of it a lot. But I just feel as though these kids are being bounced around as well. Do people just not tell their kids ANYTHING? Right now they know I have PLS or SPS and that it could be serious but that I feel really good and while my walking is bad, I'm doing just fine. But, man, one of them is a worrier and I worry about her.

 

[lgelb]

Iowa, your whole post says that you are guessing based on what the nurse said, and using that as a platform for considering what to do next.

Call or email back and tell her you need to know what the recommendation will be. This is not the Dark Ages. And just because they may want to stop the IVIG trial does not mean they will want to start plasmapheresis. When I look at the indications for plasmapheresis, SPS is Category IV, meaning it is considered generally ineffective at best, harmful at worst.

So that isn't something I would agree to unless there is persuasive evidence of something that is considered a better indication, e.g. CIDP. But non-response to IVIG doesn't magically change your provisional dx, which appears to be PLS, to any indication for plasma exchange that I see. So I don't see any reason why it would be recommended at this point, from what you have presented.

I guess overall I am not seeing any reason for you go to back to Mayo -- you don't have to show up to stop the IVIG -- unless and until they share in advance something that they would be starting or screening for, that there is new evidence for, and that you can't get closer to home. The burden of proof is on them to show that there is an "indication" (sound reason) for you to make the trip at all.

 

[BlsdMama]

No, perhaps I gave too much back story. My question is - for those diagnosed with PLS, we’re did you *initially* have hyperactive reflexes that later became hypoactive?

The truth is that I think we’ll have this answer very soon. I’ll have another EMG in February/March and, not to be a negative Nancy, but I think they’re going to find denervation. Two muscle areas flattening out, confirmed by an ALS award doc, isn’t exactly roses. But my specific question is about reflexes. I apologize for talking too much. It’s my real life problem too.

 

[Gorby]

After seven years with PLS, my reflexes are still hyper.

 

[ShiftKicker]

Mama-

Most of my reflexes have remained the same or become more intense. The one exception is my left ankle, which is non-existent. My Babinski reflex on my left foot is now either mute or downgoing- which is a change. I was informed by the doc that this is perfectly in line with advancing PLS, and to be expected. I was fortunate in my last appointment that she had a resident with her and she explained everything as she went.

I don't think the worry will ever stop about possible LMN symptoms, eh? I completely understand your concern and I wish you well at your next appointment.

 

[Trippy]

My reflexes have also remained the same or become more intense over the past 5+ years and I also have some muscle "flattening" or shrinking in calves, feet, forearms, and hands. I was sure last EMG in May would be bad news, and although it showed some chronic denervation (as opposed to the active denervation with ALS), they said it was still consistent with PLS. However, I get the feeling from posts I read that I have quite a bit more disability than most with PLS. All limbs are affected with serious spasticity, hands lack dexterity, and now speech is affected too. Happened fast over five or so years. Still haven't found a helpful drug. Baclofen pump trial may be on the horizon.

Good luck at your next appointment-hope you find answers and some medications that help. Everyone has a different set of symptoms with these MND's.