Many chages since previous post in March

[soccerdad]

I apologize in advance for the length of this, but I thought the more detail I provided the better the input I can receive. I have tried my best to break it into reasonable length paragraphs to make it more manageable for those who choose to read it. My first post back in March is closed. Since then I have been through a battery of tests in the whole process of elimination procedure, and even my neurologist said I may be running out of options if the Kennedy’s test results mentioned below came back negative. I just found out today that test was indeed negative.

Things have changed quite a bit since last I posted here back in March. I am now with my third neurologist who was highly recommended by the previous two I saw. He is part of the highly respected Loyola Hospital here in the Chicago area and specializes in Motor Neuron Diseases or Disorders. The tests and blood work I have gone through in the whole process of elimination include brain and spinal MRI’s, tests for Lyme, Myasthenia Gravis, Cancer and most recently Kennedy’s Disease and sensorimotor polynueropathy. Those tests have all been normal. I’m trying to stay positive that it’s something else.

I am not looking for a diagnosis here, rather possibly some input for my appointment with my neurologist on 12/9/2014. I read a post here saying, “In general we don't interpret EMG reports as that is up to your neurologist but if you got the report and wanted to post it maybe it would put things into context a bit and we could help you formulate questions. “ Maybe something here will spark the possibility of something other than ALS.

My neurologist did a full EMG and Nerve Conduction study on 10/22/2014. His Clinical Correlations from that were: “There is acute denervation in several muscles, innervated by multiple cervical or lumboscral roots, in both upper and the left lower limb, with abundant fasciculations. Although this can typically be found in motor neuron disease, the sensory nerve abnormalities and nerve conduction slowing, suggestive of demyelinating component, either a separate process or a sensorimotor polynueropathy. Bulbar weakness, however, would be unusual to occur in the latter. Blood tests for Kennedy’s disease, as well as antibody to GM-1 ganglioside and MAG (myelin associated glycoprotein) are suggested for further review.” He also mentioned he could not get a sensory nerve reading in my left foot.

His recap of my physical exam refers to “At least 6 months of weakness, atrophy and fasciculations in the distal upper limb, relatively painless, with subsequent nasal. Slurred speech and trouble swallowing. The right hand and left foot have gotten weaker also.”

My neurologist also said to date he has not found evidence of Upper Motor Neuron issues. I’m grasping to some hope there as based on everything I’ve read I understand ALS requires both Upper and Lower Motor Neuron issues as part of the diagnosis… is that correct?

I’m also a bit confused in that I’ve read among the many symptoms of damage to the UMN include cramps, laughing or crying too easily, nasal slow speech… and I have exhibited all of these… though I’ve had cramps for many years and I’m not sure if the crying part is just part of the emotions I’m going through with all of this…. I have always been an emotional guy. I have not shown signs of muscle spasticity, brisk reflexes or a Babinski sign to date. Can these develop later?

Like so many I have spent time with Dr. Google, but trying to educate myself to the possibilities of my symptoms. I know…that can be a dangerous thing but there is valid information on the web.

If any of you choose to do so, input based on your experience that may lead to some things I should discuss with my neurologist would be appreciated. Whatever you decide thanks for reading, and God Bless all of you.

 

[Clearwater AL]

Did your Neuro mention PMA (Progressive Muscular Atrophy) ?

Maybe some of our more knowledgeable members can better address your concerns but... most of what you have posted above you should point blank ask your Neuro or the next one you see.

You wrote, ("I have always been an emotional guy.") Work on that, there's a coined phrase around here...."It is... what it is." Nothing changes the day after you get a diagnosis.

Rule #1. Life isn't fair. Rule #2. No one can change Rule #1. Improvise, adapt and accept.

PS. On second thought, I wrote "Work on that". Being more direct if I may... "Get over that."

 

[Atsugi]

You're right that ALS requires destruction of both the UMN and LMN. There are also many things that cause perfectly normal people to have cramps, crying, and nasally speech, so you don't have to consider them UMN symptoms. They could be just cramps, crying and nasally speech without any underlying fatal pathology. Maybe you've just got cramps, crying and nasally speech.

Is that hope? Looks like your neuro is heading in a non-ALS direction. That's good, kind of. There can be other things, nonfatal, that you're developing.

It's true that other symptoms can develop later. But that doesn't mean you're developing ALS. I wish I were a world-class neurologist, so I could give more help, but I can't.

Good luck.

 

[soccerdad]

Thanks for your feedback Clearwater Al and Atsugi, I appreciate it.

I certainly plan to share this information with my neurologist on 12/9, but if feels up to providing any feedback that might have some value in that appointment feel free to share.

My legs have gotten significantly weaker over the past month or so, it seems more so in the thighs. I can still do squats holding on to the back of a chair, and with effort get up from a low couch using my legs only, though sometimes I need my arms to assist. At this point I can still walk on my toes but it’s very shaky and I feel that weakness more in the thighs than the feet and calves. My balance is suffering more and more, and it leads to some dizziness when I Iose that balance.

I did read the sticky where it says “Clinical Weakness—ALS is about failing, not feeling”… but there has to be a progression from “feeling” to “failing”… correct?

As mentioned above fasciculations first noticed in my left arm back in March have spread to my right arm, both thighs and buttocks, and sometimes the stomach area. On occasion I get some facial twitching. Another thing I noticed in the facial area is that when I yawn the facial muscles around my nose and cheeks tend to lock up for a few moments. I feel I have less control of those facial muscles as well. I can no longer whistle.

I have lost a lot of strength / dexterity in my left hand and wrist. My left thumb and forefinger barely work as a unit, but I can still type, push buttons on microwave etc. with those fingers… though more challenging. I can still use those fingers to do things like grip the tie handle of a garbage bag. My right hand has loss some strength and dexterity, but can still function decently. My left shoulder has become very weak, and it does hurt when I try to put it in certain positions like across my body or when trying to take a coat or shirt off (yes, I did read the sticky pertaining to pain… so I’m hoping it’s a positive) These areas showed sensory nerve problems on the EMG.

As far as bulbar issues, my speech has become way more nasal, hard to enunciate and string certain words / sounds together… Family and friends have noticed it. They say they can understand me but notice how much slower I need to talk and they can hear the nasal quality at times. The level of nasality varies throughout the day, but it’s gotten a lot worse in the past few months. . I get winded when I talk at times. I read the sticky saying “Sore throat, scratchy voice, food getting stuck are not bulbar symptoms”, but I do have those issues in addition to slurred / nasal / weak speech.

I do choke / kick back thin liquids, especially water, coffee and apple juice. I noticed it’s actually better to take larger continuous gulps of water / coffee.

The ENT and neurologist have not noticed significant fasiculations in my tongue so far. I do find myself with less control of my tongue and seem to bite it on my right side a few times a day. I can still stick my tongue out, move it side to side and push against the inside of my left cheek, harder to do on my right …. but all of these with less force than I used to have.

The sticky reads “Saliva issues occur late in bulbar disease and noting excess saliva is NOT a sign of bulbar onset.” I battle with my saliva quite often and it can lead to some serious choking issues to the point where I’m gasping for air. But based on the sticky it would seem to be too early in progression if it were ALS… correct?

I have found my mouth getting weaker in general. Food doesn’t stay in there as easily and if I take large bites it is way more difficult to chew it down to a manageable swallow than it used to be.

One last thing. I have become extremely sensitive to scents and other things picked up by my nose. Things like my body wash, a friend’s noticeable scent and things like shower steam make it hard for me to breath.

God bless all of you… and if you made it this far thank you for taking the time and energy to read this.

 

[affected]

I think we get the picture. Most PALS really aren't going to get through all that.

You just need to relax now, print out all you've posted here today and take it with you.

The stickies were developed by us and we all agree with them, if you want to wriggle around them, that's fine.

I agree you have something very real going on, we can't answer to anything, that's why you have this appointment in only a week. Try to relax and wait the week out. It took 9 months before my husband was diagnosed, and we just wrote everything down and went on with the business of living til we got through each appointment. He had progressed far more significantly in that time than you have.

Try to do this now, stop fixating and constantly testing yourself.

All the best with your appointment, I truly hope the neurologist gives you some clear directions to anything but ALS. Of course, let us know the outcome.

 

[Dusty7]

ClearwaterAl mentioned PMA above; PMA in simplest terms is ALS without UMN signs. In more complete terms, this is a post of mine from back in October
-------------------------------------------------------------
The ALS, PLS, PMA Name Debate

Gowers, a founder of British neurology, stated in 1899 that he had never encountered a single case of PMA in which the pyramidal tracts (UMN) were unaffected. He did not think the introduction of the term “ALS” by Charcot (founder of Neurology) to be very helpful because it implied that the primary lesion was degeneration of the pyramidal tracts and that atrophy of anterior horn cells was secondary. He felt that Charcot's distinction in effect gave a new name to an old disease. He concluded that PMA, PBP, and ALS were "essentially one disease."

Brain in 1933 introduced the term "motor neuron disease" so that apparently different conditions could be brought together in a single general category and used the terms “motor neuron disease” and “ALS” interchangeably.

A series of studies have supported the opinion that PMA, PLS, and PBP were subsets of ALS.

Swank and Putnam in 1943 analyzed 197 patients. They considered PLS and PMA to be ALS that had yet to develop fully.

Lawyer and Netsky in 1953 did an analysis of 53 patients with ALS and concluded that motor neuron diseases form a group that includes PMA, PLS, ALS, and PBP.

Mackay in 1963 reviewed 126 patients with ALS and concluded that regardless of the onset, the spastic forms nearly always become atrophic, the atrophic also spastic, while the spinal forms nearly always become bulbar, and the bulbar forms, if the patients live long enough, become also spinal. The entire group is, therefore, best regarded as a single degenerative disease, ALS, which constitutes a spectrum of atrophic process at one end, spastic at the other, and both in the center. With time, cases at each end of the spectrum move towards the center.

This is where most neurologists appear to be today—with PLS (UMN signs only) on one end of the spectrum, PMA (LMN signs only) on the opposite end of the spectrum and ALS (UMN and LMN signs) in the middle, to which both ends are drawn.

Billing Problems
1) MND, since Brain introduced the term, has been used interchangeably with ALS which makes the term confusing at best.
2) The billing codes do not agree with current neurologic thinking. Instead
Amyotrophic lateral sclerosis is 335.20
Progressive muscular atrophy is 335.21
Primary lateral sclerosis is 335.24
And Motor Neuron disease is coded 335.2, which is different from ALS’s code, though the man who coined the term didn’t differentiate between the two.

The Real Problem
The real problem is that, while all the above diseases should be called ALS, the bureaucrats don’t understand that. ALS carries major benefits, while sometimes the terms, PLS, PMA and even MND may not. BUT THEY SHOULD. It is up to you to make sure your billing is coded correctly. Talk to your neuro about the coding problem and if he won’t agree with most other neuros that PLS, PMA and ALS are all the same thing (ALS) then keep fighting with him or find another neuro.
-------------------------------------------------------------

So it's not really true that no UN means no ALS--that would depend on your neurologist's definition of ALS. If there are no UMN signs yet, it may indicate that you have a LMN predominant form of ALS (PMA) or it may be that you do not have ALS at all. If you have a choice, pick PMA overr ALS--it seems to be much more slowly progressive. UMN signs are hard to diagnose, so I wouldn't try to outguess my neurologist.

Since he's still working on a diagnosis, that's a good thing.

And remember ALS is a disease of widespread, acute and chronic denervation. I would ask is your denervation is widespread, acute and chronic.

Good luck, let us know...

 

[Atsugi]

I can still do squats >>> Muscles don't get slowly "weaker" in ALS. The individual muscle is served by a nerve. Once the nerve is killed, the muscle simply can't contract anymore.

I think you're leaning into ALS too much, too soon. While no one can accurately diagnose you over the internet (certainly not me) I do think you're worried about something you don't have, and that's leading you to worry more about something you don't have.

sensitive to scents >>> you're not listening. You must know that this has nothing to do with ALS. That makes me think you're so worried about ALS, you can't think straight. And that's normal. Most people are absolutely stunned by the possibility of ALS. I hope you can see past the panic and realize that, although your diagnosis is yet to come, it really probably isn't fatal.

 

[soccerdad]

Thank you for the kind words Tillie… I have been doing just what you suggested and writing things down to bring with me to the neurologist next week.

Dusty7, thank you for the insight into UMN issues and PMA, I will be adding that to the notes I’ve put together as just mentioned to Tillie.

And thank you Atsugi for your second positive response. You mention that “Muscles don't get slowly "weaker" in ALS. The individual muscle is served by a nerve. Once the nerve is killed, the muscle simply can't contract anymore.” I’ve seen similar posts here, but this is where I get confused. I know it can be dangerous searching Dr. Google, but one of those searches I saved is from WebMD and they refer to the weakness progressing over time saying, “The first sign of ALS is often weakness in one leg, one hand, the face, or the tongue. The weakness slowly spreads to both arms and both legs. This happens because as the motor neurons slowly die, they stop sending signals to the muscles. So the muscles don't have anything telling them to move. Over time, with no signals from the motor neurons telling the muscles to move, the muscles get weaker and smaller.”

I’m not disputing you by any means Dusty7, in fact I have more faith in the people here like you who have gone through all of this. It’s just that this has been a point of confusion for me in trying to understand this aspect of it. Thanks again for your input.

 

[soccerdad]

At appointment with my neurologist this past Tuesday, 12/9, after another physical exam and reviewing all tests done to date, he said ALS has moved to the top of the list. I researched diseases that mimic ALS and CIDP (chronic inflammatory demyelinating polyneuropathy) was one that was not looked into during the process of elimination steps one needs to go through to arrive at a diagnosis of ALS. I have been a type 1 diabetic for 47 years, and neuropathy is a complication of that disease. With my sensory nerve issues my neurologist thought it would be worth investigating the possibility of me having it. He is working with my insurance company to see if they will they will approve the IVIG procedure to see if that helps my symptoms. Has anyone here heard of someone facing an ALS diagnosis and it turning out to be CIDP. I know it’s a faint hope… but a faint one is better than none at all.

Thank you.

 

[azgirl]

About muscles "slowly" getting weaker. It is getting slowly harder for me every day to pick up a cup of coffee with my left hand/wrist/arm. I think this is because so MANY different muscles are required to do so. As the motor neurons die, more muscle function is lost. Eventually, there won't be enough muscle left to lift anything.

 

[Atsugi]

SOCCERDAD:

Concerning this entry:

“The first sign of ALS is often weakness in one leg, one hand, the face, or the tongue. The weakness slowly spreads to both arms and both legs. This happens because as the motor neurons slowly die, they stop sending signals to the muscles. So the muscles don't have anything telling them to move. Over time, with no signals from the motor neurons telling the muscles to move, the muscles get weaker and smaller.”

I think that's probably a quote from WebMD. And I think it gets misunderstood. I like Arizona Girl's explanation.

About muscles "slowly" getting weaker. It is getting slowly harder for me every day to pick up a cup of coffee with my left hand/wrist/arm. I think this is because so MANY different muscles are required to do so. As the motor neurons die, more muscle function is lost. Eventually, there won't be enough muscle left to lift anything.

Yup. There are something like 50 muscles in each hand (and foot) so when one muscle becomes paralyzed, I suppose a patient might say they are experiencing "progressive weakness" as other muscles attempt to compensate.

Still hoping for a nonfatal diagnosis for you.

 

[laurel]

My husband has CIDP-MADSAM Variant (mostly motor symptoms) and has been treated with 120 Grams of IVIG every 3 weeks for the past 7 years. Hubby did have a query of ALS back in 2007, but a second opinion revealed CIDP. He has progressive minor atrophy that continues i.e. little use of right hand, atrophy of thenar muscles and forearm muscles, foot drop of rt. foot. The IVIG holds him at a plateau without major further deterioration. There is always hope. Best of luck. Fingers crossed for CIDP or maybe MMN.

 

[soccerdad]

azgirl and atsugi, thank your for the additional insight into progressive weakness.

laurel, thank you for the information on the CIDP aspect. My concern is the combination of the progressive weakness, fasiculations, swallowing and speech / voice issues. Did your husband experience nasal and slurred speech in the course of his CIPD or knows of anyone who has?

Thanks.
soccerdad

 

[laurel]

You have to be careful and look for a horse and not a zebra. I am guilty of zebra hunting when my husband was being diagnosed. He had a weak thready voice and seemed to choke a bit on saliva. Of course I immediately though bulbar systems. Turns out he has bad acid reflux and if he takes meds for this his voice is normal, but I can tell you without the medication for acid reflux the car navigation system cannot recognize his commands as his voice is so weak sounding and the choking he did was due to build up of acid in his throat. However, I would not describe his voice ever as having been nasal, but slurred from saliva and acid build up yes it was. Hubby is a retired airline pilot and his first noticeable symptom was inability to firmly move toggle switches. He was misdiagnosed for at least two years with carpal tunnel, and he had surgery to release impinged nerves that were not impinged. He used to think that his riding horse was being unruly and was snatching the reins out his hands. This was his first big wake up call that all wasn't normal as the horse was fine and his hand was just so weak he couldn't hold the reins. However he got no proper diagnosis or treatment until he could not hold eating utensils, turn a key, or hold a coffee cup. We should have gone for a second opinion before he experienced atrophy that is permanent. At least the IVIG prevents further major atrophy happening.