PLS Dominant ALS

[art1959]

In September 2016 I had issues the 1st week speaking so my primary VA Doctor prescribed a week long medication for laryngitis and after taking it, nothing changed.

By the end of the 1st week of October 2016 I was unable to speak, yet ENT Doctors at VA said my voice box was okay from test and muscles around them but believe its motor neurons causing issue with speaking. The first VA neurologist I saw said she believed I have ALS so she sent me to the VA ALS clinic for evaluation. The Neurologist who runs it did a EMG but only on my left side and said since I don't have carpal tunnel she didn't need to do right side.

Well after the EMG she said she believed I had PLS because I had fasciculation and no fibrillations. She then said, since I lacked fibrillation on EMG it was a better diagnoses of PLS than ALS and that it can convert to ALS at anytime!

This is the summary of EMG test as follows---- Nerve conduction studies test of the left arm and leg demonstrated borderline low tibial motor and peroneal motor amplitudes with borderline low tibial conduction velocity. Left sural sensory amplitude was markedly low. Needle examination of the left arm,leg and thoracic paraspinal muscles demomstrated fasciculation potentials in the FDI, triceps,deltoid and gastrocnemius. There were fibrillation potentials in the gastrocnemius and t6 paraspinal muscles.

Now since the report in September, in December I did a sleep study test and spirometry test which showed sleep apnea and my inhale exhale muscles have weakened and now I have a cpap for sleeping and a lung cough assist machine and one for excess saliva.

my next EMG is on the 25th of January this year but my pulmonary Doctor, one other VA neurologist and even my VA podiatrist said in my medical records my issues are from ALS. When PVA (paralyzed veterans of America) tried to get my treating neurologist and other Doctors to sign a DBQ for disability thru VA for my condition they all refused. I am confused as to what is going on and now they are talking g-tube before it gets worse.

I now use a TTY phone and waiting on machine that will help me communicate. I'm tired of fighting the VA over this and now my treating neurologist said it takes 3-4 years before diagnoses can be done and she even increased my Bacoflen meds. I don't know what else to do!

I have read from John Hopkins school of medicine neurology that they believe that PLS is a variant of ALS and that it the rearest form of ALS. they also said that MND and ALS have different names but same disease. I have also read where they may have a new name called PLS dominate ALS. Has anyone heard of this?

 

[KimT]

I'm a little confused. Did you see a neurologist who specializes in motor neuron diseases? If not then that is what you need to do. Yes, PLS can and does evolve into ALS but you are not diagnosed with both. Usually they diagnose PLS and then, down the road, the ALS diagnosis comes. Not the other way around. You need to see a specialist who can confirm your diagnosis.

I'm not a vet but I believe n December 2012 the Board of Veteran's Appeals granted service connection benefits to a veteran with PLS on the grounds that PLS is a form of ALS. I also think it was upheld it court, but one of the vets will be able to comment on PLS.

Thank you for your service to our country. There will be others, who know more about the VA, who will respond to your post.

 

[art1959]

thank you Kim for your response and I understand why your confused because so am I. The VA Neurologist that I see, runs the ALS clinic at James Haley Veterans hospital and when I confronted her about original comments she changed everything around and said I had PLS vs ALS yet she never did a complete whole body test, just the left side . When I see her later this month she is only doing the left side again so she can compare reports. I have found that most Doctors outside the VA won't even consider doing disability form when it comes to the VA and also that they just agree with other Doctors notes. I'm confused as to what to do next. I asked he after she did the left side EMG ifshe was going to do the right side and she said ( since you don't have carpal tunnel on the left I don't need to do the right side.). She then said because she saw my muscles in my legs and arms twitching a lot is why she did the EMG.

 

[lgelb]

The EMG report can't be complete because the size/shape of the motor units and characterization of recruitment are not there. Those are critical to the diagnostic process. Do you have that part?

I don't pretend to understand how you can have an ALS dx and not have one, but, as Kim says, PLS is recognized by the VA. It is a different disease from ALS but often someone who is thought to have PLS "converts" later to ALS. However, the severe sensory deficit in at least one nerve doesn't fit, though it could be an error or some other process. Do you have diabetes or pre-existing spinal issues?

I guess in sum, knowing there are multiple VAs in Florida and you can seek private care as well, I'd be looking for a new team. You look about equidistant from Tampa and Orlando. Maybe go to the one you haven't been. We would advise a second opinion anyway, and I'm not sure what your first one is. Also, if you really have a CPAP, you need to get a BiPAP instead.

Best,
Laurie

 

[art1959]

Thank you Laurie for your response and as far as the other stuff you mentioned , all I have is one paper that has all these numbers for motor nerve conduction, F-waves and sensory and mixed nerve conduction. This page has all these numbers that I don't understand what they mean. The other page is needle EMG Examination: it has name of muscle, insertion activity, SPA- Fibs and Fasc, recruitment which the Tricep Brachi L and Gastrocnemius L showed decrease+ . it also has Volitional MUAPs . What all this means I don't know cause the Neurologist only went over needle exam and said that I needed all Fibrillation potentials to show I have ALS. Then she said that PLS was a better prognosis then ALS and that it can convert to ALS at anytime. If I have just PLS I can live with it but when your treating Neurlogist said it will take 3-4 years to make diagnoses and I feel my body changing in just a short period of time, why would any Doctor want to wait that long if the test shows it. I have gone to a outside Neurologist and all he said was he wanted to do a sleep study since he has his own sleep clinic and that he agreed with the VA Neurologist. The machine they gave me is a mask I wear at night to sleep and I fill the water tank when needed but as far as the other one you mentioned I have no clue what that is. The VA called it a cpap or whatever that they issue for sleep apnea. I am still working on finding another Neurologist that is not influenced by the VA and that is willing to tell me the truth and not just go along with what VA Doctors say.

 

[KimT]

Art,
Did they do a pulmonary function study on you? That would be a better indication, especially in combination with your sleep study, on where your breathing stands and the correct treatment. A BIPAP is like a CPAP but it is designed so it can help people with restrictive breathing (found in motor neuron diseases). CPAPS work great for obstructive diseases like asthma and COPD. A comprehensive pulmonary function study will reveal more about how your lungs operate and also how the muscles controlling your breathing work.

I would check with your doctor to see if you have a CPAP or BiPAP.

I don't know what part of Florida you're in but we have some good ALS centers of Excellence in the State. Mayo Clinic can offer you a second opinion and they will do their own EMG. University of South Florida also has a good clinic as does University of Florida (ALS Clinic in Jacksonville) and another good one in Miami.

 

[art1959]

Laurie they did a pulmonary on me and found my inhale and exhale muscles have got weaker from exam done in 2014. That's why they issued the lung cough assist machine and suction machine for excess saliva. I have contacted a Neurologist in Clearwater FL who will see me in April this year.

 

[lgelb]

Our point was that you need a BiPAP, not a CPAP. If you tell us the name of your machine on the front, a name or number, we can tell you which one you have.

 

[art1959]

Kim and Laurie the machine the VA gave me was Philips Respironics system one. Its used for obstructive sleep apnea. The other machine is Philips respironics coughAssit t70 series circuit, It was given to me because of the results from the spirometry breathing test done at the VA in December 2016 which showed my breathing muscles have weakened from test done in 2014 which showed normal. The 3rd machine is called Devilbiss suction unit and it was given cause excess saliva runs out the side of my mouth at times. Oh Kim I live in Citrus county, Lecanto Florida. I want you two to know I am thankful there are people like you who are willing to help others even if they don't have motor neuron disease and just want advice. May the Lord bless you both and all who are on this site. The other thing is, when O got my medical record from my last visit to the VA Neurologist, I looked at the active problems list and I was surprised to see Amyotrophic Lateral Sclerosis as one of my active medical problems. Yet the Neurologist still refuses to sign disability form for it even though its automatic presumed from service regardless when you got out of the military. I was doing some research and found: ( A new amyotrophic lateral sclerosis (ALS) category named "UMN-dominant ALS" and defined as due predominantly to UMN signs but with minor electromyogram (EMG) denervation or LMN signs on examination has been proposed. Have either of you heard about this. I know a lot of Neurologist believe that PLD is a variant of ALS and That John Hopkins school of medicine has stated on there site that PLS is the rearest form of ALS as well as that motor neuron disease and ALS are two different names but same disease.

 

[lgelb]

System One could be on either a BiPAP or CPAP -- it's just a Respironics brand. You need a BiPAP. Any doc, including primary care, can write one -- does not have to be the VA. Then your equipment company can swap it out.

If you have a dx of ALS, not sure what further EMGs are supposed to show. Is the PVA still trying to help you? Again, I would go to a different center or complain to a higher VA level. If you search the forums (button up top), you will find some posts that reference someone you can email or call, or I am sure some of our P/CALS with VA experience will chime in.

 

[BK2011]

Art,

I wouldn't go to a "neurologist in Clearwater", I'd recommend going to an ALS Clinic for a second opinion.

University of South Florida has an ALS Clinic:

Neurology Scheduling Line
(813) 396-9478

Natalie Tucker
ALS Clinic Coordinator EMG/NCV Technician
813-974-9273 Phone

There is an ALS Clinic at University of Miami:

University of Miami Kessenich MDA ALS Center
Professional Arts Center Building
Medical Campus
1150 NW 14th Street, Suite 609
Miami, FL 33136
Patients Only: 1-800-690-ALS1 (2571)
Local Calls: 305-243-7400
Email: [email protected]

Mayo Clinic in Jacksonville has an ALS CENTER:

Mayo Clinic Florida
904-953-0853
8 a.m. to 5 p.m. Eastern time, Monday through Friday

These folks deal with ALS daily and I think would be best suited for giving you a second opinion. I don't think your average neurologist is as up to speed on ALS and other MNDs.

GOOD LUCK

BILL

 

[art1959]

Thank you for all your advice and I have scheduled an appointment with a outside Neurologist in Clearwater Florida. I also contacted the ALS clinic at the Florida state campus but they said there is a year or longer wait to get in. On the 25th of January 2017 I have a appointment with the VA Neurologist again and I know she is going to do another EMG just the left side to compare with the one she did last year. I find this Neuro to be not as knowledgeable as a lot of other Neurologist considering this year will be her 10th year in practice since receiving her doctors degree in Neurology and Psychiatrist in after graduating from University Central del Caribe School of medicine in 2007. I am not saying she is a bad Doctor but she has interns doing EMG test and it seems if she disagrees with there findings she comes in and changes it. She told me that you have to have all fibrillations on a EMG to show you have ALS and from my research, I have found that not to be the case. I have no clonus on exam which is a sign of UMN. Well either way I hope the outside Neurologist will give me real answers and if I'm lucky will do a Disability Benefits Questionair (DBQ) for VA benefits since all of the VA Doctors including VA Pulminary Doctor refused to do so.

 

[art1959]

I'm wondering since I don't have pay pal how I can subscribe monthly for $19.99 even though a year is cheaper, I would rather help this site by paying a little more.

 

[KimT]

Art,

You can open up a Pay Pal account. It's secure and easy. They will draft the payment out of your account automatically each month so you don't have to worry about anything. You can also have them charge it to your credit card, if that is a better option for you.

I really hate to see you going to another neurologist who isn't an ALS expert. You live so close to University of South Florida and they have a good ALS Clinic. It only took me three weeks to get an appointment for a third opinion. They will look over your EMG and really explain it to you in detail. Then they will do one of their own.

If you can get to Jacksonville, Dr. Pulley does all his own EMGs and that is a great advantage. He doesn't give them to interns or residents or even other attending doctors. He does them all. He is at University of Florida ALS Specialty clinic in Jacksonville.

You shouldn't have to wait to receive benefits if you have PLS or ALS diagnosis. I would try to see a neuromuscular specialist.

 

[art1959]

thank you Kim for your response but dealing with the VA is not easy. I have noticed that with the VA they can get very negative towards you if you complain about anything they do. I cant understand if it is common knowledge in the Neurology world that under motor neuron disease has 4 known conditions that are considered variants of MND or ALS yet no one states that in the field. I don't understand how the VA allows someone who has less experience than others to run a ALS clinic but I guess she answered my idea about that when she told me PLS is not service connectable like ALS and refused to sign DBQ for disability. This type of actions by doctors at the VA make the VA happy so they don't have to give benefits. I have contacted the ALS clinic Florida state in Tampa and they said it would be over a year before I could get in. The other problem is finding a Neurologist who is willing to fill out a DBQ for disability with the VA cause I can tell ya the VA will do whatever they can to deny it. the 2 most common reasons they use are 1) service medical records do not show treatment for injury or disease while in service. 2) comp and pen examiner stated it would be mere speculation to determine if it from service. this is used when it shows you were treated for it while in service. It took me 6 years to get what I have now and I still have pending appeals. I feel that the VA has such a strangle hold on the Doctors outside the VA that they control everything so veterans cant get claims approved. I thank you and everyone else for there comments.