Als - pls - hsp

[MISTRO]

At 52 I had some left leg stiffness in October 2012. Noticed occasional stumbles and clumsiness prior to this. Suddenly could no longer run on the tennis court in September. Noticed fasciculations and a bit of stiffness in left arm in October as well. Neurologist diagnosed possible ALS on 5th December 2012. EMG on 24th December shows fasciculations but no chronic changes that would indicate ALS. Neurologist revised diagnosis to HSP or possible PLS on 8th January 2013. ALS still not ruled out and a repeat MRI and EMG will be completed in 5 months. I don't have any major muscle weakness or wasting at present which goes with the lack of lower motor neurone signs on the EMG.

I am now quite stiff in both legs and my left arm has some pain around the shoulder when I try to raise it. It will occasionally collapse following a painful twinge when lifting or moving something.

Has anyone had fasciculations with PLS or HSP?
What are the early signs of lower motor neurone involvement?
I reckon my spasticity has progressed quite quickly.
Does this fit with PLS or HSP?
How common for ALS to show UMN signs with normal EMG in the early stages?

Thanks

 

[vickim]

Have you read the stickies? You should there is alot of questions answered. I don't know what HSP stand fors.

 

[olly]

Hi mistro,welcome to the forum.
Having umn desease i can identify with your onset and symptoms and will try to answer a couple of your questions.

Fasiculations have been noted by many with hsp/pls including myself........however unlike als they are benign and a probably caused by muscle/nerve irritation which can also be the case with ms patients.

The first few years ofton seem to be the worst time for those with umn desease,it does seem that its progressing fast but usually it does slow down.
Intrestingly i was reading an article i found the other week and it said when ever pls starts in an area disability in that area can be greatly effected .......i found this to be true.
my legs which onset started are my weakest area but when it spread to my arms/hands then bulbar later the disability in those areas were quite bad at first.........everywhere is just slow and steady now.

Your neuro will have to wait 3-5yrs to give a definate diagnosis and rule out lmn involvement completely.
For instance those with umn domminant als may present like a pls patient at first.
Hsp can cause slight umn problems other than legs.......a pls diagnosis would depend on the severity of umn symptons other than legs.
Many have been diagnosed with one but to have it changed a few years later for the other.

Clinical weakness and atrophy are lmn symptoms.
Footdrop is a early symptom in als but also can be seen in pls as with me and showed on my first neuro exam as clinical weakness.

Sorry to say it is early days for you and it could be sometime before you get a definate diagnosis.
Please look in on the pls forum as theres lots of past threads with great information and we can best answer any questions you have there.

 

[laurel]

Elaine mentioned the ABC's of A L S on youtube and it is quite informative as well.

 

[MISTRO]

Thanks for the replies.

A special thanks to Olly for his long, thoughtful, thorough and useful description of his experience. I appreciate the time and effort on my behalf. My real reason for joining the forum was to get in touch with people and learn from those who have shared a similar experience.

Limb disability is difficult enough to cope with but it seems the most difficult task ahead for me is to cope with the uncertainty of it all. It seems I may have 3 years to wait for a diagnosis unless I develop LMN signs that change my current diagnosis to ALS. In the meantime, I think the best plan is to follow the advice of the ancient philosophers and assume I have ALS that develops rapidly, and live life accordingly.

 

[olly]

:grin:Your very welcome mistro.....by the way.....i'm female lol:grin:

 

[MISTRO]

Sorry Olly. I associated Olly with Laurel and Hardy! You can call me Stan.

Look forward to learning from you as I tackle the challenges ahead.

Mike

 

[notme]

HI

Don't know that you need to assume you have ALS -- but living each day to its fullest is certainly a good idea.

Like Olly, I have largely UMN issues and have since the beginning other than major atrophy and degeneration in my left arm (where my weakness started)

The neuro was pretty sure it was ALS based on the left arm and the UMN signs everywhere--but almost two years later, I'm still here and other than atrophy and the lost of most of my left arm and some loss in my right, I'm still only having UMN signs of spasticity, clonus, spasms and cramps and the fasciculations I've had since the start in my arms that were noted by a doctor before I ever saw them.

There are medications that can treat symptoms such as spasticity and cramps. Avoid falls. There are assistive devices for walking if you need them such as canes, walkers and AFOs (supports for weak ankles)

All you can really do now is wait to see what, if anything, else happens.

I've chosen to just watch symptoms and go from there. I don't even go to the doctor anymore. If things get worse, I'll go back--but otherwise, I treat the breathing issues with a Bi-pap, the walking issues with a walker or scooter, and compensate for the loss of my hand usage in various ways. Adaptions comes natural when there is no choice.

 

[MISTRO]

Thanks Notme,

Sage advice on avoiding falls when I went a over t on to concrete this morning. I can laugh now as i got up without serious injury except to pride. My GP gave me a referral to a falls man yesterday. Didn't know falls doctors existed! Sounds like we are twins with my left shoulder and arm. Will read your threads on weakness. My left shoulder feels like I have a badly torn muscle - probably from a previous spill so I find it hard to tell whether it is the pain that makes it weak or something Lmn. Certainly spastic.

Look forward to sharing stories going forward.

Mike

 

[notme]

hi Mistro

Haven't been online the last few days. With your arm pain, it is possible you tore something with a fall. Also always remember that even with the uncertain MND if that's the case, it doesn't mean something else can't be going on. Be sure nothing was hurt in the fall.

As for the falls--please be careful. A walker is a simple thing to use to prevent falls especially early on--as long as you have the arm control to use it safely. I sometimes notice shoulder and hand strain from trying to use mine, and use the scooter instead for longer distances.

Falls are just too dangerous. I probably fall at least once a month.

If you're driving, be careful of spasticity. I am dangerous behind the wheel and I know it, so I don't drive anymore at all. I'm lucky--my daughter lives with me and drives me when I need to go out.

If you have questions--ask away. There are always folks here to answer.

 

[paul71]

I'm curious about the shoulder pain. I have simply been diagnosed with an "unspecified neuromuscular disorder", mostly all UMN at this point. I'm still working (and do alot of walking) but my legs are very heavy, tight, etc. You know what I mean.

But I too have terrible shoulder pain in my left shoulder. This has just started since my neurological symptoms started in September 2012. It feels like something is torn / tearing and the pain can be really bad at times. I feel like I need to hold my arm pinned against my body to avoid the pain.

My question is this: If we have something neuromuscular, would / should doctors be less likely to perform an othewise necessary shoulder surgery? Would our neuromuscular issues mean that we'd be less likely to heal properly? I keep hearing that pain isn't associated with ALS, but I know that my joint issues have coincided exactly with my neurological issues and the shoulder can be excruciating.

Sorry if I am hijacking your thread, but I'm wondering what you guys are planning on doing about your shoulder issues. Surgery? Cortisone shots? Or just treat with pain medications as needed?

 

[vickim]

Have you been to the dr to see if there is damage to the shoulder? It could be nerve pain, there are days where my right leg onthe outside of the calf from the knee down to the ankle is being ripped apart. It aches, burns and stings no matter what I do. I am on neurontin 200 mg 3 times a day which helps with the minor pain and the crawly sensations. Motrin and tylenol doesn't touch it.

 

[olly]

Paul,about 7-8yrsago i had a bad episode with spasms in my arms.
It started in my left and by the next day i could not move both arms.........stuck to my side for weeks.
I had pain and stiffness from shoulders to fingers,my shoulders have been in constant pain ever since.

Umn symptoms definatly cause pain........if a pal has more umn involvement then they will have pain,more lmn then maybe not.

As anything neuromuscular is usually progressive then surgery would not be effective.
Its not like arthritis where they can take out the deseased joint.
Theres no actual desease or even problem totally related to the joint.......its secondary.

 

[paul71]

Olly - what do you do for the shoulder pain? My left shoulder is in constant pain, sometimes bordering on exruciating. I have one kidney so the most I take is 1000mg Tylenol / day and never for more than 2 days in a row. Over a 1 month span, I took a grand total of 2 vicodin and 1 percocet. I also use capsacin cream but mostly I just put up with it. The problem is that it's getting worse and radiating into my collarbone and down my arm. My neruologist does not prescribe pain medication. Since surgery is not an option, I suspect that at some point, reasonable use of narcotic pain meds might be necessary. But of course the first reaction of most doctors is that their patients are "drug seeking" if you bring this up. I am wondering if you had advice for how I should proceed... Thanks.

 

[MISTRO]

Hi all,

Been busy at work for a few days so have only just seen your messages. I am booked in for X-ray and ultrasound of shoulder on Thursday. My GP thinks I may have traumatised my shoulder. I can think of two falls when I strained it. It occasionally seems to click and catch when I lift arm sideways which fits with swelling of the tendon over the shoulder. Pain is quite severe if i lift in wrong direction. Will let you know how ultrasound goes. I guess an injury will show. If ultrasound is normal, then it must be a spasm in muscles causing pain?