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Brother Rock

Active member
Joined
May 6, 2012
Messages
44
Reason
DX UMND/PLS
Diagnosis
04/2012
Country
US
State
IN
City
Bloomington
A little over 4 years ago (2008) I started with a cold foot. Well at least it "felt" cold but not to the touch. No reason found.
My next problem was my foot felt REALLY cold one day and I took my sock and shoe off and my foot was ghost white and I could not see any blood vessels. It was cold to the touch. This occurs on an off but not too often and a good foot rub usually restores to blood flow.
I start to experience numbness and tingling in my right foot and that is still happening to this day.
I saw a neurologist in 2009 and started having various MRIs done starting with the pelvis and right hip. Nothing found.
2010 had a CT scan of my lower body - nothing found, X-Ray nothing found
2011 Brain and Lumbar still for numbness and tingling in my right foot.- nothing found as usual. EMG - nothing
Sep 2011 started physical therapy for a month and now my left foot is starting to feel like my right foot with numbness and tingling. My right leg was between 26% to 53% weaker than my left leg but improved some strength but soon I lost most of that strength.
2012 My doctor sent me to a Neurologist. Spine MRI and X-Ray possible problem with lower back so I was given a epidural which did nothing for me.
Finally decided to go to Cleveland clinic and after more MRIs, X-Rays and EMG+ they said I had PLS.
I found this site and after seeing most of you have very bad symptoms and I do not have anything but weakness in my right thigh but not the calf. I cannot tap my right foot. I have tingling and numbness during the day which seems to get worse in the evenings especially the tiredness. No pain, no cramping, no spasticity.

Has anyone one of you started like this? I need to know as when I see what all of you are going though I find it very hard to believe I have PLS. Please help me! :-(
 
Hi Brother Rock,
I'm not a PLS'er, but I'm UMN dominant. It took several EMGs to get to ALS. It's my understanding that any diagnosis of PLS is pretty preliminary for quite a few years. I'm not sure 4 years is enough for them to be certain? I think perhaps your best course of action would be to continue to see the same doctor over the next few years to ensure that progression is documented accurately as it occurs and the diagnosis can be more certain as time goes by. Not ideal, I know. Limbo is no fun AT ALL. Hopefully others can provide some better answers than I can.

As for the right foot sensory and cold issues, I'm assuming they did an NCV? It could be some type of impingement, considering the symptoms come and go. That would not show up on an NCV unless you were experiencing certain symptoms at the time of the test. It took FOREVER for doctors to figure out I had a shoulder impingement in college and it would cause symptoms when I played piano for many hours. After stretching and rest, it would improve and feel normal. You just never know. Food for thought!
 
well rocky..u could have two things going on...your lower back giving one set of symptoms and then you could have pls.....giving another set of symptoms...then maybe you have neither.....could be something else like a undiscovered stroke......or loads of things actually....johnny
 
Thank you for your response
I left out a lot of different test that were done for brevity. I have every test they could think of including the NCV. No signs of strokes found in the two brain MRIs veiwed by two experts. The lower back was just a shot in the dark. They though maybe there was something and gave me the epidural to see if it worked. If it worked then it was was they thought it might be and if not they did not know what could be causing my problems. This is what I find so frustrating in that I have been labled with PLS because nothing else has been found.

Thanks again for you input
Brother Rock
 
The Cleveland Clinic is a good clinic and they have a lot of expertise in PLS. I've had PLS since 2003. It started with speech (Bulbar PLS). It is a very rare disease. It progresses slowly. It took 5 years to move from my speech to my hands, arms and legs. I am confined to a powerchair now. I have been following groups like PLS-Friends on yahoo for years. Symptom progression and severity varies a lot. I had problems for a while with my hands feeling cold. Are you startled easily? Do you have uncontrolled laughing/crying?. Since symptoms and progression vary so much I am not convinced that everyone that has been diagnosed with PLS actually has PLS. If a test is ever developed we'll know. Right now be thankful it is diagnosed as PLS and not ALS. Don't freak out. You have what you have. A tentative diagnosis of PLS makes it easier to explain to friends and others.
 
One thing is Ray nauds -- causes the cold extremities-- which come and go.

That sounds more like a circulation issue. And circulation is not gonna show up in an EMG or NCV.

As for the weakness--I'd think the doctor would have found something abnormal on clinical exam-- PLS is UMN in nature--and UMN issues have very specific things a doctor looks for, including reflexes, muscle tone, clonus, etc. People with PLS aren't 'stronger' -- the muscles weaken.

Tapping your foot requires use of muscles in the ankle--not just the thigh. But all your weakness is in the thigh? And just one sided?
 
I second the idea that the Cleveland Clinic is known to be very good. You wouldn't have been given a diagnosis of anything there if the evidence was not sufficient (in my opinion).

I've had 2 neurologists tell me they suspect I have PLS. Time and testing will eventually tell the tale unless an autopsy comes along first. (not counting on an autopsy any time soon...)

A few hallmarks of PLS are slowly progressing spasticity, hyper/pathologic reflexes, emotional lability, muscle spasms/cramps. It usually starts in a limb but may also start in the bulbar region with pseudo-bulbar palsy. Due to its slow progression, the disease could take decades to run its course. These are just some of the things I've read.
 
Thanks again everyone,
They though I had Raynaud’s disease. I was given medication for it but it did not help at all and later it was determined I did not have Raynaud’s. At first my weakness seemed to be only in my right thigh but now my calf seems to be getting weaker. I have requested physical therapy and just started today. However the nice people at the clinic do not know how to treat someone with PLS but they are willing to learn. One correction in my time frame at the beginning is my cold foot problems started in the fall of 2007. I also believe I do have more that possibly PLS wrong with my right foot.

I have had all the follow done to try and determine what my issues is (take from a website of course)
Tests and diagnosis
There is no single test that confirms a diagnosis of primary lateral sclerosis. In fact, because the disease can mimic signs and symptoms of other neurological diseases such as multiple sclerosis and ALS, several tests are done to rule out other diseases.
After taking a careful record of your medical history and performing a complete neurological examination, your doctor may order the following tests:
+ Blood work. Blood tests are done to check for infections or other possible causes of muscle weakness.
+ Magnetic resonance imaging (MRI) of your brain and spine. An MRI or other imaging tests may reveal signs of nerve cell degeneration and look for other causes of your symptoms, such as structural abnormalities, spinal cord compression, multiple sclerosis and spinal cord tumors.
+ Motor and sensory nerve conduction studies. These tests use a low amount of electrical current to test how quickly your nerves carry impulses through your body, and can indicate damage to nerve cells.
+ Electromyogram (EMG). During this test, the doctor inserts a needle electrode through your skin into various muscles. The electrical activity of your muscles is evaluated when they contract and when they're at rest. This test can measure the involvement of lower motor neurons, which can help to differentiate between PLS and ALS.
+ Cerebrospinal fluid analysis. An analysis of your cerebrospinal fluid, which is taken during a lumbar puncture in your lower back, can help to rule out multiple sclerosis and other causes of spasticity.
 
I am not aware of any marker even at autopsy that conclusively says you have PLS. With ALS you have the protein TDP-43 that gets mangled. I'll ask my doctor in August.
 
Here is one example of PLS and an autopsy report:

Abstract
We report the autopsy findings of an 82-year-old woman who exhibited slowly progressive upper motor neuron signs (pseudobulbar palsy, muscle weakness and positive Babinski's sign) in the absence of lower motor neuron signs, which were followed by progressive dementia and frontotemporal atrophy, and who died 7 years and 4 months after onset of the disease. In this patient, the upper motor neuron system, including the precentral cortex and descending pyramidal tract, was severely degenerated, but the lower motor neurons and innervated skeletal muscles were well preserved. A few lower motor neurons were found to contain cytoplasmic inclusion bodies characteristic of amyotrophic lateral sclerosis (i.e., Bunina bodies and ubiquitin-positive skeins). However, fragmentation of the Golgi apparatus was not evident in the anterior horn cells examined. Therefore, it was considered that the lower motor neurons were also involved, but that the rate of degeneration of these neurons was very slow in the disease process. Marked frontotemporal lobar degeneration characterized by microvacuolation, and ubiquitin-positive neuronal inclusions and dystrophic neurites in cortical layer II were also observed, the precentral cortex being the most severely affected area. Similar ubiquitin-positive structures were also observed in the neostriatum. Finally, a survey of the literature based on this patient's clinical and pathological features led us to conclude that the rare clinical syndrome of primary lateral sclerosis is, in general, a rare upper-motor-predominant form of amyotrophic lateral sclerosis that is often accompanied by frontotemporal lobar degeneration with ubiquitinated neuronal inclusions.

Here is another blurb on the subject:

PLS was first identified in France in the 1850s by Dr. Jean-Martin Charcot. During his landmark analyses of ALS patients, Dr. Charcot encountered a woman with severe spasticity and limb contractures but no muscle wasting. After her death in 1856, the autopsy revealed extensive scarring in the lateral columns of her spinal columns, or primary lateral sclerosis

Autopsies are pretty revealing...

The doctor performing the autopsy isn't looking for some genetic marker because there is no need. The damage done to the lateral columns of the spinal cord pretty much tell it all, from what I gather. Kick me if I'm wrong!
 
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Great info on PLS, Thanks all.
 
I agree they are all good indicators of PLS at autopsy but there is no conclusive marker or protein anomaly that says PLS for sure. I'll check in August.
 
I'm not sure you got my point. When an autopsy is performed on a patient that had been diagnosed with PLS, it may be positively confirmed at that time simply by seeing the damage that had been done in the lateral columns of the spinal cord.

I agree that finding a genetic marker of some sort would be of great use while you are alive but of no use when you are dead. I am just trying to let you know that PLS can be confirmed upon autopsy without hunting for some genetic marker.

Horse is now dead.
 
Thanks Zaphoon, I sure had a hard time reading all those fancy medical words. But for most of us it is too late if they confirm the PLS with and autopsy. ;-( I have made an appointment with a doctor in Indianapolis IN who specializes in ALS. If this doctor comes back with PLS also then I will accept my fate and move on with life. If he cannot figure out what it is I am back to square one and I give up and will move on with life. This means I will work for three more years and retire. So to all of you my most sincere appreciation for your help, information and advice. I pray that some kind of significant breakthrough comes about very soon that greatly benefits all of you who suffer so much from the horrible diseases of ALS and PLS. Brother Rock
 
On Monday I got a call from the the Doctor's nurse or secratary and she did not know what PLS was. She said she had to check with the doctor and see if he would still want me to come. Still waiting for a call from them.
 
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