re: recently diagnosed with PLS-very afraid.

[Guest guest]

In a message dated 3/20/2006 11:01:25 PM Eastern Standard Time,

jhmarks@... writes:

I was recently diagnosed with PLS. Where can I get more info like this.What

seperates HSP and PLS. They are so similar and I prefer to think I have HSP.

No family history though.

Hi ,

I'll jump in here, too. I can understand your being afraid.

Neither PLS nor HSP in and of themselves shorten life. But it is possible

they lead to something that does, like a bad fall or a car accident caused

by inability to drive well. So, be safe!

HSP and PLS are very similar. Both cause the same type of progressive

spasticity and weakness in the lower body. PLS is thought to cause

spasticity and weakness in muscles of the upper body as well - arms and

voice.

So when the symptoms are just in the legs and there is no family history, it

really is unknown which condition someone has. Then, it is a waiting game

to see what symptoms develop.

Lots of people who probably have HSP do not have a family history. Dr.

Fink, based on his experience and expertise, has estimated that 1/3 of

people with HSP don't show a family history.

This could be because it's a recessive type of HSP. HSP can skip

generations and then appear " out of nowhere " . Many neurologists do not

want to give a diagnosis of HSP when there isn't definitive family history, so

they will give a PLS diagnosis. In fact, it used to always be that way but

that is changing as they understand HSP more and the genes that cause it.

Other neurologists will call the condition (bad legs and no family history and

no other reason to explain it) Spastic Paraplegia or Apparently Sporadic

Spastic Paraplegia, which is probably IMHO the best way to go.

Besides the case of recessive HSP, there are other reasons someone with HSP

may not have an evident family history. It could be an initial mutation

(mutations always start somewhere...). It could be the person was adopted or

they have a different parent than they thought (it happens). It could be that

the parent is deceased and died before symptoms appeared, as sometimes,

HSP can begin late in life. Or, a parent's HSP was so mild that it was

attributed to something else, like a bad back or knees.

So, in cases like yours, it is a waiting game. No matter which condition

you have, the treatment is the same and our community is here to understand

and support you. Thank goodness we have our internet world! If you can, tr

y to get to a Connection of some type, or, hold one yourself, so you can

meet someone in person and talk about this. It helps!!!

In case you are interested, here's a little bit about what is going on in

the body with PLS and HSP:

They both cause degeneration in the " upper motor nerves " . These are nerves

that reside in the brain. They have long appendages called " axons " that

make up the spinal cord, where they connect to " lower motor nerves " that then

carry the impulse to move out to the various muscles and tell them to move.

Degeneration in the upper motor nerves causes the messages to get scrambled

as they relay to the lower motor nerves so the lower motor nerves don't give

the right message to the muscles. The result is spasticity and weakness in

the affected muscles. As the nerves continue to degenerate, there is

increasing spasticity and weakness.

In HSP, it is the ends of the longest axons (that end low in the spinal

cord) that are affected, and so that's why the legs are affected. In PLS,

the nerves in the lower spinal cord are affected but so are the ones higher

in the spinal cord (that relay out to the arms) and brainstem (that relays

out to the voice and swallowing muscles). Usually, PLS begins in the legs (and

hencesforth the confusion over whether it's PLS or HSP) but sometimes, it

begins in the upper body and then affects the lower body.

Hope that helps!!!

best

Kathi

[Guest guest]

, welcome to our group.

Kathi, I found what you wrote below very informative and relates very

much to me. The last 2 neurologists I have seen are having a hard

time deciding whether I have HSP or PLS. so for the moment, I am back

to Spastic paraperisis with startle myoclonus.

Aussie Maureen

>

> Lots of people who probably have HSP do not have a family history.

Dr.

> Fink, based on his experience and expertise, has estimated that 1/3

of

> people with HSP don't show a family history.

>

> This could be because it's a recessive type of HSP. HSP can skip

> generations and then appear " out of nowhere " . Many neurologists do

not

> want to give a diagnosis of HSP when there isn't definitive family

history, so

> they will give a PLS diagnosis. In fact, it used to always be that

way but

> that is changing as they understand HSP more and the genes that

cause it.

> Other neurologists will call the condition (bad legs and no family

history and

> no other reason to explain it) Spastic Paraplegia or Apparently

Sporadic

> Spastic Paraplegia, which is probably IMHO the best way to go.

>

> Besides the case of recessive HSP, there are other reasons someone

with HSP

> may not have an evident family history. It could be an initial

mutation

> (mutations always start somewhere...). It could be the person was

adopted or

> they have a different parent than they thought (it happens). It

could be that

> the parent is deceased and died before symptoms appeared, as

sometimes,

> HSP can begin late in life. Or, a parent's HSP was so mild that it

was

> attributed to something else, like a bad back or knees.

>

> So, in cases like yours, it is a waiting game. No matter which

[Guest guest]

Welcome to our group! You will never find a better , more supportive

and informative group anywhere! I was VERY scared when I was first

diagnosed with PLS...then i found this group and they got me on the

right track. Check in with us every day and read some of the posts.

Don't be afraid to ask any questions!

I have had PLS about 9 years, but was only formally diagnosed 2

years ago. Right now, my progression is quite rapid, but it

could 'plateau' at any time.

We are all different . But generally, PLS is a very slow moving

disease. Some have had it 25 years and have only just started using

a walker. Others of us have had it much shorter length of time and

are in wheelchairs (like me). But this group is like one big family

and we are here to support, give tips and generally 'be there'.

I am 48 and have 2 teenage daughters. i am a family counselor,

although on medical retirement now. Glad to have you aboard .

Keep talking......and questioning. If you want to talk privately,

hit my email symbol at the side.

Take care!

Di.........PLS.........Canada

n PLS-FRIENDS , " Maureen " wrote:

>

> , welcome to our group.

>

> Kathi, I found what you wrote below very informative and relates

very

> much to me. The last 2 neurologists I have seen are having a hard

> time deciding whether I have HSP or PLS. so for the moment, I am

back

> to Spastic paraperisis with startle myoclonus.

>

> Aussie Maureen

> >

> > Lots of people who probably have HSP do not have a family

history.

> Dr.

> > Fink, based on his experience and expertise, has estimated that

1/3

> of

> > people with HSP don't show a family history.

> >

> > This could be because it's a recessive type of HSP. HSP can

skip

> > generations and then appear " out of nowhere " . Many

neurologists do

> not

> > want to give a diagnosis of HSP when there isn't definitive

family

> history, so

> > they will give a PLS diagnosis. In fact, it used to always be

that

> way but

> > that is changing as they understand HSP more and the genes that

> cause it.

> > Other neurologists will call the condition (bad legs and no

family

> history and

> > no other reason to explain it) Spastic Paraplegia or Apparently

> Sporadic

> > Spastic Paraplegia, which is probably IMHO the best way to go.

> >

> > Besides the case of recessive HSP, there are other reasons

someone

> with HSP

> > may not have an evident family history. It could be an initial

> mutation

> > (mutations always start somewhere...). It could be the person

was

> adopted or

> > they have a different parent than they thought (it happens). It

> could be that

> > the parent is deceased and died before symptoms appeared, as

> sometimes,

> > HSP can begin late in life. Or, a parent's HSP was so mild that

it

> was

> > attributed to something else, like a bad back or knees.

> >

> > So, in cases like yours, it is a waiting game. No matter which

>