Maybe PLS?

[wellington]

Hello all

I haven't yet posted much, largely because I'm increasingly doubtful I have ALS. Reasons being, I have some autonomic symptoms, I have pain and recent (quite dramatic) speech and swallowing symptoms have at least for now 70% abated AND while i get weaker and weaker I've now been ill for almost 2 years. Also negative EMG. MS ruled out.

I'm wondering about PLS. Do sufferers experience speech\swallowing symptoms that come and go, or does the universal ALS rule of things just getting worse apply with PLS too? And do PLSers get autonomic disfunction?

My symptoms - right arm almost doesn't work any more, drop foot (left), increasing weakness (I use a cane), sleep disturbance, dramatic, but now intermittent speech & swallowing issues, twitches in affected limbs, weak trunk muscles (left), stiffness, and an out-of-this world painful thigh (left). As well, I am sick every day from about lunch time. I suffer from fatigue and temperature disregulation.

Neuro says I have positive Babinski, tremor, myoclonic jerks, and reduced fine motor skills and hyperreflexia. Multiple System Atrophy is also possible. I have tongue weakness confirmed by speech pathologist.

You guys are amazing and I am a little sheepish about taking your time, but I'd be so grateful for help/advice. I am almost at the point of not being able to care for my 5 year old daughter and have no family support, so a little bit frightened.

Thank you

Lilly

 

[AKmom]

Pls is hard to diagnose. It sometimes takes years. What does the neurologist say? Sometimes they put you on a wait and see approach if they are not sure. However, pls does not come and go. When it starts to effect one area, it will always affect that area. It doesn't improve, at least this has been my experience. Pain can be from constant spasticity or injuries to tendons as they tear or snap from all the stress of the spasticity. Usually, pls starts in one leg or foot, then spreads to the other leg or foot before working its way up to your trunk and arms and hands. Eventually if you have had it long enough it will involve all muscles, I have had it almost 20 years. It's slow moving, I am a part time wheel chair user. I use forarm crutches the rest of the time. It just now within the past year or so, effecting my arms and hands to the point that I am loosing the ability to not only support my weight with the crutches, but I cannot open jars and even a ten pound baby is too heavy for me to hold without someone helping support the baby. Pls is so slow in progression and is very rare. So your pattern of symptoms do not sound like pls. I hope the docs can find what is ailing you.

 

[wellington]

Thank you so much for your thoughts AKmom.

The neurologist has me on wait and see watch. What he says so far is I have a neurodegenerative brain disorder. At the moment he's considering Multiple System Atrophy, a frightening condition with a life expectancy of 6-9 years. As well as the ultimate loss of movement, it apparently involves a range of constitutional symptoms and a high likelihood of mental decline.

Thanks for confirming for me that symptoms don't 'come and go'. When I think about it again the speech and swallowing symptoms probably haven't gone, just got a whole lot better than when they first arrived the pain on eating (neuro says oesophageal spasms) has gone.

My problems started with a mildly weak arm 10 years or so ago. That arm's almost useless now. Everything else started 2 years ago, with new symptoms every week. My symptoms didn't start with a hand or foot. It was my upper arm first, then my upper leg (thigh). Now I have cramps in both feet and drop foot in one. I don't have the arm strength to cut my food without enormous effort.

Horrible new symptom now! The last 2 days I've had trouble holding my torso up - spasms all up and down left side. Feels like I'm in a vice. I'm having to take shallow breaths. Doctor said to take Baclofen for a couple of days ( I took it before, but hated it!). I now take Gabapentin, but this isn't touching it!

AKmom you sound like you have a great attitude. I'd so like to just get on with life, but can't do it without a diagnosis. I'd do anything for a diagnosis - whatever it is....

Thank you so much. I hope you are having a wonderful week. It is 5am on Friday here and dark and rainy outside. The first day of winter and 14 degrees.

 

[notme]

Baclofen is for spasms-- neurontin is for nerve pain. I'll never understand why people take it---I'm biased, I think it's a horrid, horrid drug.

Baclofen is much much easier to become accustomed to. They can see the spasms--surely the doctor would prescribe something? I didn't leave the neuro without an RX for valium and baclofen (valium for cramps) -- and didn't ask for it.

Clean EMG is a good sign! I hope you find answers--your symptoms are very similar to mine - though my EMG isn't normal in my arms--I have nerve damage (mild carpal tunnel) which one doc said is the cause of the mega-atrophy in my dominant hand.

Personally, I haven't had anything get better. Only issue that is better is my breathing--and that's with O2 and a bi-pap at night. Legs spasms and cramps or horrid--and my hands contort to impossible positions I have to have help relieving--as my fingers 'freeze' in that spasm.

See a neuromuscular specialist if you haven't yet.

 

[wellington]

Hi notme

I find the whole drug thing so confusing! I did take Baclofen for a while - and it worked! BUT as I got up into the higher doses (40-50mg) and was waiting for the next dose I felt like I was going cold turkey - elevated heart rate, sweats, shakes etc. So, I stopped taking it. At the same time I stopped driving (had to) and this seemed to reduce the aggravation on my leg, and so some of the spasms.

My other big problem was/is the pain driving me nuts at night and I wasn't able to sleep (leg, arm). It is this problem that the Neurontin has helped. I can now sleep for about 6hours straight, and compared to how it was that is heaven!

I take Valium from time to time, but not much. I'm pretty drug-phobic actually, so I put up with a lot before I start taking anything. But the time has come .....

So ... I think I'll keep taking Neurontin at night. I'll maybe give Baclofen another try.

The neuro wants to try my on Sifrol - a Parkinson's drug. He says it will either work or not. If it works - great! If it doesn't work - well, I clearly don't have a movement disorder. So, it would help further narrow the diagnosis. Multiple System Atrophy is apparently a type of movement disorder, sometimes called Parkinson's Plus I think.

We ARE similar (but I think you suffer a great deal more than I do) - my hands are starting to do weird things too. Just this week my neuro asked me if my hands 'always do that'? I didn't straight away know what he meant, but he pointed out that every time he looked my fist was curled up and tucked under my arm or somewhere else, and I do know I can't hold my right hand open for long before I want to close it. It twitches a bit too, my wrist hurts, as does the area between my thumb and forefinger. I can't open bottles any more either.

Thank you notme. What an incredibly generous woman you are to help people like me.

All the best
Lilly

 

[olly]

hi lilly.
i have had pls 13yrs and like joyce its slow progressing,started in my legs and spread up to involve bulbar symptoms.
yes,i find that swallowing can improve a bit if you have less spasms in the throat...........when throat spasms are worse and it feels tight its harder to get food down,easier when its more relaxed.
like in the limbs in the begining theres just spacticity which varies in degrees ,eventually over time it does cause weakness but not at the same rate as als so its possible for swallowing or speech to be a bit better sometimes.

they thought that i too had ms then msa.

 

[notme]

Just keep a close watch on the side-effects of neurontin. Seriously. It caused major brain fog for me, massive swelling in my legs, and I'm sure it is what ultimately caused the ulcer on my foot--due to the massive swelling.

I wouldn't touch it. I've had no issues at all with Baclofen. But, I only take 30mgs a day. valium 2x a day. Keeps me out of absolute agony most days

 

[wellington]

Notme - I will keep in mind what you said about Neurontin. At this stage I'll probably just try and keep it to just the 300mg night dose. I didn't sleep before I took it and now I do, so - while I'm a bit dull with it, I'm reluctant to interfere with it. I find you can be a whole lot more optimistic if you sleep!

Olly - what a time you've had! 13 years - and you can still find the time to help others by sharing your experiences! Did I read you have a 23 year old son? I have one of those too. And a 5 year old. I feel very lucky, but am worried about my care of my 5 year old daughter.

It's interesting that you too were suspected of having MS and then MSA. Thanks for your comments on swallowing and spasms. My condition broadly gets worse - I'm getting weaker as the months go by. The spasms though do 'come and go' in intensity and the worse they are the more trouble I have. I can walk better with spasms than without. On the odd day I have none, it feels like my legs can't hold me up.

Lilly

 

[wellington]

Olly - given you were considered for a diagnosis of MSA, did you and/or do you have automomic symptoms? Mine include bowel and urinary issues (occasional), vertigo (also occasional) temperature disregulation and overwhelming fatigue every single day from about noon accomapnied by a strong feeling of being extremely unwell.

With the temperature disregulation, It can be a relatively warm (australian) night and i can be utterly freezing with blankets, coats and sicks piled on. Nothing works to stop my teeth chattering except a very hot shower. Olly does any of this sound familiar?

Thank you so much for your help. It may be a while before I find out what the problem is, but I am curious by nature. I like to work things out and I am going nuts.

Cheer
Lilly

 

[olly]

hi lilly.
yes i have hypotention,as it can sometimes happen if i get up too quick .....possible orthostatic.
went for the tilt test but my bp was 90/60 abouts so they would not do it.
also i do find if its hot it gives me extreme fatigue.
had so many mri's and no sign of ms or msa,i think (and it has been seen on autopsy)that sometimes mnd can effect other central nervous systems.
some develop ftd or cognitive issues,some others it has effected eyes ect ect.
shows mnd for each person can be different.

 

[momap53]

Hi Lilly, I have ALS and have been using gabapentin for my muscle spasms for almost a year without any noticeable side effects. Isn't it amazing what a good nights' sleep can do for us? Hope the docs can get your symptoms figured out soon.

 

[wellington]

Thank you Momap53 and Olly.

After a good night's sleep I feel like anything at all is possible, and I don't worry at all about the spasms and the weak limbs. But, by lunch time I'm so so sick and in pain. Anyway ... there are people a lot worse off than me in life, and if reading this site has taught me anything it is that I have a lot to be grateful for AND that kindness, optimism and generosity are endless.

Reading this site is a lesson in how to live a good life. All the best to both of you.

Lilly

 

[notme]

Some will say to avoid pain meds--but I'm a firm believer in treating what hurts. I seriously would have done myself in without them by now. I don't do pain anymore. There are low-impact meds like ultram that can work for many.

300 mgs of that nasty med shouldn't cause any of the effects I had--I was on 3600mgs per day. A huge dose.

 

[John1]

...I'm wondering about PLS. Do sufferers experience speech\swallowing symptoms that come and go, or does the universal ALS rule of things just getting worse apply with PLS too?

Hi Lilly,

I have either PLS or upper motor neuron dominant ALS. My symptoms began with speech problems 18 years ago and progressed over the next few years to swallowing problems. Both symptoms have varied widely in severity since they began although neither has ever disappeared entirely. My speech is very low and sometimes difficult to understand. It still varies over time in severity. Likewise, my swallowing is tricky but I still eat all foods. Crumbs tends to cause the most problems.

This is in contrast to limb weakness and spasticity which have been relentless in progression. I have been unable to walk for several years and my hands have lost a lot of small motor function.

John

 

[wellington]

Thanks John. That's interesting. The neuro told me recently that the chest pain I have is oesophageal spasm. I've worked out (I think) that - for now at least - I only have speech and swallowing difficulties when I have the spasms. No chest pain - almost no difficulties, except my mother keeps telling me I'm mumbling lately, and when I sing to my daughter at bedtime I now sound like a strangled cat!

It was a similar experience for me in the beginnning with my left leg. I was reminded of this with Olly's post above. On the rare occasion there was no spasm, there was no problem. Now as time goes on the leg gets weaker and weaker. Olly says above that spasms CAUSE weakness. I'm starting to make that connection. My walking is not too bad on a flat surface (I don't always use my cane), but any sort of incline or rough ground and it gets very tricky, very fast.

My speech and swallowing problems came on so quickly. It was frightening, and it went on for weeks, always with throat/chest pain. I'm amazed that it has abated somewhat. Hopefully the damage is minimal, and like with you John, it doesn't become a regular feature for me.

Thanks again John

All the best
Lilly