May be PLS...

[Just J]

After 3 years of continued progression and no answers we finally have some possibilities. Two weeks ago my husband went to Johns Hopkins and met with new Neruo. Possibilities are PPMS, PLS, or HSP. Even though I knew from my own research these were possibilities this is the first doctor that has mentioned them. None of these illnesses seem to be life threatening but I am still concerned about PLS...can that turn into ALS? EMGs for my husband are clean. In his current state he can barely walk and needs a cane for support, sitting is a problem too. He has to lay down for most comfort.

[olly]

i am so sorry to hear your husband is struggling,i know how that feels.
after 3yrs with no lmn signs and clean emgs that is good news.
they do usually say 3-5yrs before a definate dx of pls.
i have had mnd for 9yrs but due to some hypotonia/lmn found did not get a definate dx of pls.
i have been doing alot of research and have found acute umn lesions can cause hypotonia and weakness causes disuse atrophy,i think this explains my mixed symptoms.
i found some fantastic info and it even tells you how the umn problems effects the hips and feet like mine do.

http://books.google.com/books?id=KMW...esult#PPA90,M1

i dont think the link will work as it is too long and i can not shorten it.
in google search type in.........hypotonia and umn symptoms.
it is the second one down........neurological physiotherapy/google books,p89 to 99.

your husband sounds like me,i have great weakness in the hip girdle/torso areas.
i too have great discomfort when sitting too long and do find lying down better.
i find lying propped up in bed with the knees bent,as you would if you were sitting on the floor then relaxing the hips helps a great deal.
it takes the strain away and any discomfort.

i hope any of this has been of some help,i do have tons of info if you ever need any.
by the way what is ppms? not sure i have heard of that.
take good care.
caroline

[sral]

I must say that the waiting period seems like the worst part.

Olly, did your hypotonia (LMN) show up after the 5 year point or did you have this lower motor neuron symptom from the beginning.

Thanks for the link by the way, I'll look into it tomorrow. My mom too has a hip weakness. I don't get this though. With PLS isn't it a lack of signal so the muscles don't work the way they are suppose to? How does this affect the hips?

[Just J]

Thanks Olly for the info. I will definitely look into this book. PPMS is Primary Progressive Multiple Sclerosis. This is a continuously progressive form of MS where patient has continued symptoms of decline with no leveling off or respite from their symptoms.

PS~ I like the photo of your kitty. I am also a cat person and have a 7 yr male Bengal.

[olly]

the link does work,i can be a bit hopeless with these sometimes.
basically its all about body enlinement,this is effected by spasticity,balance and gait problems.
if the enlinement of the body is out of whack it puts stress on the joints.
muscle spasms/spasticity can cause alot of stress to nearby joints.

the hypotonia was found a few years ago in my left lower leg but now suspect it effects other areas like my torso area.
when i tried to go swimming a year ago i could not do one stroke,this was because my lower body would just sink due to weakness.
this is a typical sign of widespread hypotonia.
up untill 2yrs ago they were still ruling ms and other stuff out with serial mri's ect.
so who knows how long the hypotonia has been there,it is usually spotted by a pt.
the neuro who found it was not my usuall one,he scraped the side of my foot i think with my leg at a certain angle.
my lower leg muscles starting quivering like mad,he did it twice and stood in amazement watching with a nurse.
i was like............"hello",what is going on.i,m not a freak show.
anyway, i have started to develop atrophy in the left foot ankle now.
from reading the info from the site above i have finally realised what disuse atrophy really is.
i was thinking how can i have atrophy if i am still using the foot and ankle.
it is because the weaker muscles even if being used are not really able to reach there true potential,so are not being used as in a healthy muscle.
i did not get a dx till late,these symptoms confused things a bit.
pls is rare in the usa,but its even rarer here. very few neuro,s ever see a case of pls.
take good care.
caroline

[sral]

thanks Olly.

And yes the link does work, but I was unable to find the part on how disuse atrophy looks like. I could not do a find on the article.

[olly]

p92 under heading atrophy
it just describes why disuse atrophy occurs.
i still after months of research and tons of downloaded info get confused.
not everyone is a classis case with the exact same symptoms.
my left leg is the weakest with hypotonia, yet some months ago i started with fassics and cramping in my right lower leg.........................this is so crazy,i have decided not even to think about analizing it.
my brain can,t take anymore lol.
take care.
caroline

[sral]

I don't blame you Caroline.... overanalyzing things can drive someone nuts - trust me I know first hand!

[Geo]

Can You imagine , being a very articulate person .Then one day you become a very Clumsey person . And your whole world comes tumbling down . Well some get devistated for a while then something grabs you and says GET UP ,GET GOING Do something .And you get your back up and start .DOING . I have to say ,i spent my first 4 years in devistation SCRAMBLING for answers . And now i geuss ive been given more time to be here . Geo

[olly]

lol geo.
i used to be such a clean feak,everything had to be just so and perfect.
now if just the basics get done i am happy.
when you go from a young fulltime working hyperactive single parent to slow barely moving geriatric before your 40yrs..............it is a bit of a shock to your self asteem.
but we are still here and for that we have to be gratefull.
take good care.
caroline

[miabethr]

Thank you for being so positive- i am almost to the point where you are Geo, but am still somewhat grieving my old life, mostly because of my 12 year old. But my body feels exactly like you describe olly and i persevere until i make it home at the end of the day. we can't change it so we must learn how to best live with it. many days it is easier than others and today was a good day! thank you both for sharing for those of us still struggling mentally with a disease that keeps most of us guessing.

God Bless you all

[sral]

Today my mom went to her neuro appointment and the Dr saw only slight change since he last saw her 9 months ago. He told her she was lucky and then I reminded him that although my mom's progression was slow compared to typical ALS, this pace for her is still very fast because it has changed her life. It is all relative and personal!

[olly]

it is great news to hear your mum has not progressed too much since her last appointment,lets hope it continues that way.
those without a disability can not begin to comprehend what its like,even subtle changes in decline can have a big impact on what we do or how we can do it.
say hi to your mum from me and give her a big hug.
take good care.
caroline

[sral]

Caroline,

Thank you so much for your kind words. Everyone on this forum is so nice.

I too hope and pray that progression continues to be slow or even stops. Although the Dr thought the progression was not significant in the past 9 mths, to me and even more importantly to my mom, it appeared to be significant.

As for the hug, I'll definitely pass it on!

Have a great day

[Geo]

Its not soo much Devistation ,Its life changing . Im of the thaught now that i get to Live another Day . Keeping in mind i was told i would be dead in 2 years Its now Close to 8 So HMMM . I have to be thankful
See, im seeing the Glass as Full ,not empty . Give me Lemons, What do i make ? Geo