how fast?

[easy]

hey all. havent logged on since march. ive had pls for 4 yrs. cant walk, talk, write, practically a quad. ive seen people w/varying degrees of pls but none seem to progress as fast. fell out of whlchair last wk and shattered 4 front teeth! i know its not fatal but more falls like that will must decrease life expectancy. sound familiar?

 

[Roger Sanders]

I am sorry to hear that you have progressed so much so fast. I was diagnosed 2 years ago. I walk with a cane 100% of the time because of weakness and very bad ballance.
My knees tend buckle a lot so it causes me to fall a lot. Because of this I have torn my ACL, broke my left wrist and 2 months ago fell and broke my right wrist. This is tough for me because I am right handed and can't walk good using my cane in my left hand. It sounds like you are having a rough time right now, my prayers are with you.

 

[Dougly]

Me too!

:?:I was diagnosed with PLS form of ALS back in January 2001. By 2004 I was essentially a quadriplegic and my lung volume and gone down to 47% as well. But since then things have stabilized at virtually haven't changed a bit since then. I am able to work out (physical exercise) in a local handicap accessible pool three times a week. In that way I have been able to maintain my muscles. I dictate this message using that Dragon NaturallySpeaking program.

 

[Zaphoon]

Easy, Roger and Dougly,

I'm sorry to hear you have PLS and am really surprised to hear how quickly the progression has been. You read this stuff on the web that talks about it progressing slowly and isn't diagnosed until your 3 or 4 years into it or longer.

How long did the diagnostic process take for each of you?

Zaphoon

 

[Roger Sanders]

Zaphoon, I was having symptoms 4 years before my diagnosis. So that has been at least 6 years ago. It started out with general fatigue, leg weakness and leg pain. Then it got to clumsiness, falling and tripping. During the 4 years before diagnosis I went to my general doctor a few times complaining about these symptoms and especially the leg cramps but they would always blow it off that it was because I was on my feet all day long on concrete floors or because of stress or something. These symptoms kept getting worse and worse to where I started falling and breaking bones and in constant pain (legs cramping) 24 hours a day, that's when I said something is wrong with me and I want something done now. That's when my GP sent me to a Neurologist and he said that there was something wrong. It was tough, I had worked out with weights for 15 years and in 2002 was bench pressing 400 pounds and all of the sudden I wasn't interested anymore and was getting weeker. My Neuro at first thought I had MS. My great grandfather died in 1946 of ALS and my first cousin was diagnosed with MS in 2006 also. After my spinal tap and MRI's were clear they did lots of other tests including EMG's. They sent me to an ALS specialist at the University of Kentucky (The Kentucky Clinic) were I went through tests and he concurred I have PLS. He told me that he did not think it would turn into ALS years down the road but it doesn't mean that it can't either. This is just a summary and there are some other things going on with my body also. Things have just gotten a lot worse the last 2 years. I hope this answers some of your question. Roger

 

[lydia]

Easy, Dougly, and Roger,

I echo Zaphoon's sentiments...your progress seems so fast. Easy-so sorry to hear about your fall, how did that happen? I lost 4 front teeth in a bizarre collision years ago. I hope you are able to have them repaired.

Roger, I saw that you had 24 hour cramps...I am curious about that if you don't mind elaborating? I have experienced 3 "episodes" where the cramps come and stay for days at a time. The longest stretch was 9 days, the shortest 3 days. I was having as many as 12-15 cramps an hour, they were strong, they seemed impossible to shake off (had to ride them out), they were in odd spots (ribcage, upper back, throat), and if in the feet the foot would contort into positions you wouldn't think possible. During these "episodes" there might be an hour here and there where I might have temporary relief. It was the most horrible pain I have ever experienced beyond the pain of childbirth and neither the GP or neuro gave a hoot.

Take care,

Lydia

 

[Roger Sanders]

Lydia, the only way I know how to explain the cramps is that they never go away. Sometimes they are much more intense than other times. I can always feel it in my calf muscles, hamstrings and behind my knee where the hamstring and calf muscles attach. Even when I am feeling good I can still feel it throbbing very lightly. I am not complaining about it rather just giving details about my symtoms to you and others. I just put it in my mind that it is something that I have to deal with the rest of my life. I take 60mg of bacofen a day along with some other pain medications and this has helped a lot, enough to were I can bear it most of the time. I deal with getting rolled around in a wheel chair by my wife better than I do the pain, it kills me sometimes. I along with my Neuro think it is because I have hyperreflexia so bad. I can't even squat down hardly because my hamstrings cramp and get on fire when I do because they are so tight. I can set in the hot tub and stretch while I am in there and get out and my hamstrings are as hard as a rock and they remain like that all the time. The cramp itself varies from throbbing, to a more like aching cramp and sometimes very tender to touch cramp. Like I said before please don't think I am complaining or whinning to get attention. I am just giving my symtoms. I don't even tell my wife anymore she can just tell when I am worse. Roger

 

[Zaphoon]

Roger,

We share the leg cramping 24/7 thing. I think my problems started in my right arm; at least thats where some atrophy was found in my shoulder. They think the atrophy is due to disuse. There are some PLS'ers on this forum that have gone through a lot of what I'm experiencing. It makes me wonder.

Anyway, your story is quite something! The falling episodes you experienced sound pretty bad. Part of me wants to put blame on your GP for not acting quickly enough, especially given the extended time frame he had to look at you before referring you to a neuro.

Then again, these MND's don't exactly wave a flag and proclaim themselves over a loud speaker either. I appreciate you sharing with us on the forum, Roger.

Zaphoon

 

[lydia]

Roger, thank you so much for sharing your experience with cramps. I don't perceive sharing of symptoms in this setting as whining or looking for attention; I asked for details, right? I know outside of this forum I rarely describe anything I am experiencing for those exact two reasons, so I think the two of us have a very similar mindset. I find it hard to even talk about it with the doctors. But you are experiencing something real and nasty, you should be able to share that experience here and in the real world without feeling whiny or being treated as if you are whiny. Ahhhh, if only we lived in a perfect world!

Your experience does sound pretty brutal with respect to cramps and tightness. Do you get any relief from medication? From a previous thread it sounded like there could be a considerable trade-off for relief; just trade these unpleasant experiences for others (side effects). My number one issue is hamstring tightness, cramps are second (been a while since the last episode; otherwise that would be first) and I was hoping for some medication to come out of my upcoming visit.

Thank you again for taking the time,

Lydia

 

[Dougly]

My story

First of all I would like to explain what I understand of the differences between ALS, PLS, and dementia. From what I understand ALS occurs when the long motor neurons of the spine degenerate thereby associated muscles to die. In PLS, the motor neurons are dying in the back part of the brain. These are the neurons that give the signals to the long motor neurons of the spine. Because in PLS the long motor neurons of the spine are healthy, the muscles attached to them should remain viable and if an impulse can be generated somehow through that dying area of the brain those muscles can be maintained and need not atrophy. Dementia on the other hand occurs in 7% of people with ALS and it is because some neurons are dying in the cranium or high part or cognitive part of the brain. It all depends on where in your nervous system the focus of degeneration is occurring. For me it appears to be at the very base of my brain just before it goes down the spinal cord. As a result I am very close to having bulbar ALS. For me the symptoms could very easily turn into ALS symptoms if the degeneration continues any lower. As a result because the degeneration is so close to my breathing center of the brain, my lung volume has been compromised. But that hasn't changed in four years. When I go to take a breathing test my cranium says to take a deep breath but the impulse has trouble getting through the dead area at the base of my brain to reach the muscles of my rib cage and diaphragm. As a result I am not able to take a deep breath. They get me in the warm pool I am able to generate impulses through those areas and activate those muscles of my arms legs and trunk. It is amazing transformation. Initially I have no strength in my arms and legs but with positive thinking and patience the strength starts coming and I can do more and more. I'm actually able to walk in the pool (whereas I have not walked on land for about four years). I am able to swim with my arms (can't get my legs to work there though) and when my assistant tips my head up to breathe, I take in huge breaths of air. So I know my lung volume is actually very good, I'm just not able to prove it. You see in the pool when my head is a lift it out of the water my body says it desperately needs air. this bypasses my cranium at my body reflectively takes a deep breath without me having to tell it. Same way when I get a tickle in my throat a local reflex takes over at my body heaves a great big breath of air and I have a violent cough. But ask me to cough on my own and it is very poor. I go to the pool three times a week and this is maintaining me very strong and my endurance has definitely improved over the years because I'm not even tired after pool therapy anymore. As to how it started I first noticed my left leg dragging periodically back in the spring of 2000. By the spring of 2001 day diagnosed me as a PLS form of ALS. They have never been able find a day muscle facsiculations on me. I too went through a time when they thought it might be MS. Basically they have to rule out everything else before they come up with this diagnosis. At of course the EMG shows if the muscles are being affected. I progressed fairly rapidly in losing my right leg and left arm and right arm so that by 2004 I was essentially quadriplegic. Everyone has their own uniqueness in this disease because everyone's focus of degeneration is a little bit different. Fortunately scientists now have been able to map out the spinal cord and they feel they will be great strides in treating ALS over the next 10 years because they will be able to identify the exact focus of degeneration in the cord and apply treatment directly at that site. PLS on the other is in the brain.

 

[Dougly]

Additional info

As far as cramps, I find using the muscles in the pool and then aggressively stretching those muscles every day very much alleviate that for me. I am also on baclofen 30 mg qid and Neurontin qid as I have been very spastic in the past. I also have a vibrator in my bed which helps relieve any cramping a my legs that night.

 

[hopingforcure]

I too agree with exactly what Roger explained, and have the same feeling in my legs. I also think it has to do with the hyperflexia, which in turn causes clonus, which is a big cause of this feeling. I get a little feeling of relief when others can understand the strange things that the body experiences with these diseases, it is just someone does get it.. thanks all

 

[hopingforcure]

PLS is Upper motor neuron symptoms, while ALS is upper and lower both. Now that does not have anything to do with the upper or lower part of the body, it is the neuron's themselves. PLS, causes Hyperreflexes, clonus, spasicity, spasms, weakness, and such. While lower causes Atrophy, weakness, fasics. Now you can have some lower motor neuron symptoms with PLS, many do, and if it does not show up as damage on the emg. it is still classified as PLS. It is very confusing, but if I can explain it to anyone just ask. I have done more research on MND than should be humanly allowed.

 

[olly]

hello everyone

easy,doug,roger sorry to hear you are not doing so well.
pls like als progresses differently in others,you can not go by someone elses progression.
roger,your history of diagnosed is like mine. firstly i was sent to a rehumatologist who ruled out autoimmune ect,then serial mri,s and other tests for 7yrs to rule out ms and multi system/cerebeller atrophy.
i have had mnd 9yrs now with slow progression up my body and bulbar symptoms 2yrs.
but i do have more weakness than spasms now(been on baclofen 8yrs) and atrophy/wasting in a number of places.
i do have lmn involvement in my left leg.
my neuro will not give a definate pls diagnosed,rather diagnosed mnd he focuses on progression and says in some cases a diagnosed is only possible on autopsy(they will have to wait alot longer for that:lol
as hoping said there are ones with a diagnosed of pls with slight lmn involvement that is very slow progressing,in clinic trials these would be determined to have umn dominant als .
some neuros would diagnosed pls others als,i think this is confusing as to those who do not fit into either diagnosed criteria(this is me and was said by my neuro and gp)
we just have to stay as positive as we can and do what we can(i know,easier said than done)
spasms tend to cause more prolonged pain as in 24/7 with stiffness,were as cramps are more in bursts.
clonus is caused by sustained contraction when there is spasticity,you can have spasms that may not be as severe enough to cause the clonus/spasticity so these can come and go.
i also get myoclonus in my right side of the body(rarely left)this causes abnormal movements of the limbs and torso.
i have been away the past few weeks as i moved to a disabled ground floor flat/apartment as i was in a two storey house and could not manage the stairs and other things.
hoping..........great to see you posting again my friend,i will send you a pm as soon as i can.
godbless you all:smile:

 

[hopingforcure]

Sweet Caroline, Welcome back, Welcome back, welcome back, welcome back
so many times these last couple of weeks I have thought about your wonderful words to so many and believe me your were surely missed. I hope the move went well, and both feline and human inhabitants are doing super. I think your explanation was so perfect, and will clear up things for many. This darn PLS/ALS question is just mind boggling and different from so many physcians. I am thinking of you my dear dear friend, and am so glad to see you and that sweet OLLY back.
How is that most adorable kitten.....?