PLS to ALS

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TippiLeigh

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After months and month of bloodwork, imaging, testing, etc. my neurologist diagnosed me with PLS about a month ago. He referred me to an academic hospital for a final diagnosis/second opinion, although he seemed certain it was PLS. I’ve been waiting weeks to get in with the academic center. They finally called me today and explained why there was such along delay. Apparently the patient coordinator accidentally sent it to the MS clinic first. After that neurologist reviewed it, he said, “She doesn’t have MS!” And he sent it back to the patient coordinator who then decided to send it to the General Neurology clinic. Their neurologist reviewed my chart and said, “She should be going to the neuromuscular/ALS clinic”. Umm, yes! That’s what we’ve been trying to do! (Sorry, that was just a side note story of my frustration from earlier today.)

Which brings me to my question while I wait for eternity to actually get an appointment. I’m starting to show some signs that I think are more LMN, making me worried that maybe I have UMND ALS or just straight ALS. My local neurologist didn’t do an EMG because, in his words, “It will only exclude ALS and the academic hospital will want to do their own so I don’t want to put you through that twice.” A couple of my concerning issues are extreme weight loss. I’m usually around 130lbs, but I’ve dropped down to 96 lbs (since about May) and I can’t get any back on no matter how hard I try. My husband, who is also a physician, noticed atrophy in my buttocks. I have two nice-sized craters. (Although, could just be from the weight loss and my need to sit more.) The tendons in my hands and feet are becoming very prominent. I’ve started having fasciculations in my buttocks where the atrophy is and also in my upper outer left arm. My weakness is progressing. It used to just mostly be my left leg but its gone up my left side and now my right arm and leg are now getting weaker And both hands are increasingly losing dexteritY. Just not sure if those things can be part of PLS. Depending on which literature you read, there’s so much controversy over whether or not PLS and ALS are completely different diseases or if they are just the spectrum of one disease. I just want to figure out which one it is so I can at least have a better idea of how my body might continue to progress so I can plan at least a little bit of my future...

And I realize I will have my answer once I finally get in for an EMG, but by the way they handled my paperwork, I’m not too confident it will be anytime soon! LOL!
 

Nikki J

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I am not sure if you were asking or stating but in case you were asking... truly you need to wait for the emg

such extreme weight loss could be the reason you see the muscle changes. People with pls do twitch and umn disease does cause weakness. Whether this explains your issues I don’t know.

can you see a dietitian to help you try to regain some weight?

can you call the neuromuscular center? Appointments may be affected by covid and yes there is usually a wait ( mine was 2 months in normal times) but nicely making yourself known might help. Especially if you can say that you will and can take a last minute cancellation ( if that is true)
 

TippiLeigh

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I think I was mostly venting and talking out loud because I’m frustrated and worried. My area is lacking resources. I asked my neurologist and my PCP if they could get me in with a dietician in our area and they said there isn’t one. I’ve been trying to have them refer me to a physical therapist but that has been slow going also. Other than Baclofen for the spasticity, I haven’t been able to start any other therapies because my local neurologist wants to wait until I’m seen at the academic center. I ordered a cane off of Amazon to help keep me from falling but as things keep progressing I need help with knowing which mobility aides to use. I just feel so alone right now and unsure of what to do and I know once you get dialed into a clinic you have a resources and experts to help answer questions and guide you in the right direction. I’m so isolated right now. I’m also terrible at just waiting. I hate delaying any treatment, although I realize there isn’t a lot out there to treat/delay PLS, but it seems like there maybe other medication for ALS? I want a correct diagnosis so I can start getting to work dealing with this. (After these ramblings it doesn’t seem like I’m handling it well, at all)
 

KimT

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I think a walker would be safer for you.

I'm sorry you got such a runaround. I find, during Covid, lots of that going on.

Meanwhile, I might be able to help you put on some weight. If you don't have one already, but a Vitamix. You can make a wonderful AND healthy shake by using your choice of milk, whole fat Greek Yogurt, a ripe avocado, a couple of tablespoons of raw cocoa, a tablespoon (to start) of coconut oil, and some sweet fruit. If it isn't sweet enough, add a ripe banana and a couple of figs. Gradually add more coconut oil each day. Experiment and add all kinds of things that appeal to you. It'll be like a milkshake, only healthy. Add extra olive oil and butter to your veggies. Eat between meals.

I never weighed over 107 and I managed to gain nearly 40 pounds but I didn't do it in a healthy way.
 

TippiLeigh

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Thank you Kim! I definitely need to try some milkshakes like that. I do think part of my problem is the difficulty in eating in and of itself. Food and medication is starting to get stuck in my throat quite a bit. I get tired while chewing. So sometimes I feel like I just give up even when I’m not quite full because of the effort it takes.
 

Dee Dee 0617

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I am so sorry you're going through this. Try taking your meds with applesauce. It is a little weird at first but it will go down easily. Please rethink the cane, falling exacerbates the disease. Be kind to yourself, be kind to your body. God bless you. We're here.
 

TippiLeigh

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Thank you Dee Dee! You’re very sweet. That means a lot to me :)
 

KimT

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If you're having trouble swallowing, perhaps a visit to an ENT would be in order. They can do a swallow test to make sure the muscles involved in swallowing are working properly. I've had a couple done and they found it to be acid reflux and a damaged TMJ that were causing my perceived swallowing problem. I still have a hard time with stuff like popcorn, only because it hurts my jaw.

Another thing I discovered is that some symptoms are not related to PLS/ALS. Of course, that's always front and center when any new symptom develops, but you must make sure you address general health issues as well.

I would advise anyone with this disease or anything similar to get your dental work done, especially if you need crowns, etc. Get cleanings as long as you can tolerate them. I even got a bone density test and mammogram two years ago, along with a pelvic exam. I also ended up getting my eyes corrected with new lens implants. Better now than later.

I think you will have much more clarity once you see a neuromuscular specialist. My general neurologist did an EMG and it was wildly off. The nerve conduction study matched Mayo, but the EMG didn't even look like the same person.

You can also add nut butters to your shakes. Peanut butter/chocolate make a great taste and it's loaded with calories.

We're here to help.
 

EricInLA

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I know how frustrating the wait is, particularly when your mind is aflutter with all the possibilities. I was in that same situation, and I know I wasn't sleeping at all during that period. Since my diagnosis (not the one I wanted!), I've been able to sleep better, with the help of Trazodone. To my mind, until you get a diagnosis from a neuromuscular specialist, any diagnosis is tentative. This is really a specialty. I also think an EMG done by a non-neuromuscular specialist should be taken with a grain of salt. Just my feeling. On that note, you may want to reach out to multiple neuromuscular clinics to see which one can see you soonest, or to be on the waitlist at multiple places. It looks like you are in California, and there are many in this state (though depending on where you are, they could involve a long car trip)

Unfortunately your experience of the patient coordinator sending your tests to the wrong person is not unusual. It's so important to follow up to make sure everyone in the health care chain is doing what they should be doing. They make mistakes every day. Also, I would recommend that you compile a list of questions, and whenever you think of something jot it down, so you can get all your questions answered, to the extent they can answer. Some doctors are good at giving you lots of information, and some will just give you the basics unless you push for more info.

Kim's shake sounds delicious. I don't have a weight loss problem (if anything it's going the other way during Covid), but I'm thinking of giving it a try anyway!
 
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Shell R

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Hi there,
I see you have had lots of positive feedback already which is great. I found a cane did not help me at all because if there was any movement in front of me then I would overbalance backwards away from the movement! A walker was a perfect for me - my walker has 4 wheels and as soon as I press down on the handles the brakes come on so you don't slide away into oblivion! Ideal for shopping because it has a seat for when you need to sit and rest.
I managed to stop weightloss by having protein shakes with full cream milk and peanut butter once a day. On hot days I have milkshakes with cream and ice cream. I am the envy of my family to be sure! I cannot chew food so everything has to be in liquid form. I can eat mashed and pureed food and I swallow tablets with a few spoonfuls of custard.
My original diagnosis after 3 EMGs in 2017 was PLS because only my voice and lower limbs were affected. Now that my hands and arms are also weak its ALS. That is how it was explained to me. You are right, there is a lot of differing information out there and, to make it even more difficult, each person's body reacts differently.
A good suggestion was made to get all dental and eye work done now while you can and also retain a sense of humour about everything. I now tell my friends that I am loosening my grip on my broomstick! Its time to take life at a slower pace and enjoy the quieter things in life.
 

BlsdMama

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Hugs - the waiting is hard. PLS is generally given as a "probable" diagnosis because they give us four years to convert to ALS before giving it as a permanent diagnosis. Most convert to ALS and a small percentage will stay PLS. It is usually confusing if it is a slow progression, but the upside that is even if it is ALS, the progression often stays slow.

I currently carry the probable PLS diagnosis at Mayo but ALS at the university hospital. The gold standard is the EMG. PLS and ALS both affect the upper AND lower limbs. However, PLS mostly affects the upper motor neurons and ALS affects both the upper and lower motor neurons and they have different jobs. You'll read that PLS patients don't have fasiculations (lower motor neuron sign) but that isn't entirely true. There is just no way to differentiate between these two diseases without an EMG.

Use a walker or a rollator. I've fallen with a cane/forearm crutch. I wish I had moved to a rollator much sooner. I needed the stability.

But, very important, losing weight isn't a good thing for us. The goal is to put on / keep on as much as possible. You really need that EMG because it arms you with information. Equipped with knowledge, you can start caring for yourself in the best way possible -even if it means supplementation or a PEG tube.
 

lgelb

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Shell, a diagnosis would not change from PLS to ALS on the basis of which limbs are affected. It is based on whether upper motor neurons only or both upper and lower (which have nothing to do with which limbs have issues) are damaged. That is based on symptoms and the clinical exam.

It is true that PLS is rare and most tentative PLS dx become upper motor neuron dominant ALS, but I would not want to give false hope that progression of the latter will "stay slow." It is still at this point a fatal disease.

There are also newer EMG protocols that can detect upper motor neuron (UMN) damage, in use at some academic centers.
 

BlsdMama

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My apologies - I think I should have been clear - slow progressors with ALS tend to advance at a steady rate that they present and I've heard this from Mayo in Rochester.
 

lgelb

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If you mean progression in ALS is somewhat linear, yes, slow onset usually means a relatively slower progression than the median. But progression is not linear/steady the whole way through in either PLS or ALS.
 
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