Club membership at hand

[KarenNWendyn]

Benign cramp-fasciculation syndrome is certainly a contender. However, we are really not here to speculate what other possibilities may explain your symptoms. A neuromuscular specialist who can examine you and have access to all your records would be the one to ask.

In fairness to people here diagnosed with ALS or caring for those that are, I’d appreciate it if you could go to your log-in profile and change your interest from “Ive been diagnosed with ALS” to “I’m interested in learning about ALS” — at least until you have a confirmed diagnosis.

Thanks

 

[lgelb]

Sorry, I wasn't equating dysfunction with incontinence, either, only commenting that incontinence if/when it occurs in MND is generally muscular weakness/spasticity that is sometimes wrongly attributed to some kind of sphincter issue.

As for differentials, there is a long list, but in terms of "kicking the can," we always advise a second opinion anyway, for any potentially life-changing diagnosis, not just ALS.

 

[KevinM]

Of course, Karen. I had only indicated such after my neurologist had sat y family down to discuss NMD with the possibility of ALS, and referred me to the ALS clinic for a second opinion. I should have waited until after that evaluation.

 

[ThroughThatValley]

Hi Kevin.
Let's do some basic physiology here.
The eardrum muscle is not a voluntary muscle. You can't use it at will. It will respond to a sound that could impair your audition and close up the earcanal by sheer reflex.
MND affects motoneurons, aka voluntary muscle function.
I really don't get why your neuro would label it an ALS typical feature at it isn't.
There's not much in your report that seems to bear the hallmark of ALS.
The second opinion is a must-go. Keep us posted.

 

[KevinM]

Thanks so much for weighing in. I absolutely will update this thread after my April 25th second opinion.

My local neuro said the eardrum pulsing/auditory issue could very well have a neurological origin, as he has seen that in his practice. My wife's recollection was that he did not say that it was ALS specific. I see in my first post that was what I indicated, which was not accurate. It was wrapped into the broader symptom discussion in which ALS was identified as a possibility.

He has 27-years of experience, so when he told my wife and I he was referring us to the Neurology/ALS clinic, we panicked.

We totally understand--and he made clear--that without clinical weakness/atrophy/progression, there cannot be a definite ALS diagnosis. The main reason for his referral is based on the elimination of other, more common issues over more than two months of tests, the limited limb EMG that showed some abnormalities, and the continuation/progression of muscle-related symptoms.

Everyone here is so very kind in responding. I hope your collective knowledge and positive thoughts prove correct. Kevin

 

[Firefighter58]

Hi Kevin,like all the others I hate to welcome you here but I guess it is the polite thing to do. Any way try not to worry, it won’t do any good, if your planning to travel then travel, what ever your plans were just carry on with them. Take every day as it comes and look forward for tomorrow and just carry on like you don’t have this concern because that in my world that is all it is, a concern up there with a cold or a runny nose,you’re not going to die tomorrow so don’t act like it. Many folks here were diagnosed 10 years ago or more and are still carrying on. Life throws us many curves and that s what ALS is, just a bump in the road. Live each day as best as you can and don’t let this disease control you , it will try but just carry on and and ignore it. Stay strong Kevin and don’t worry about things you can’t control.
Al

 

[KevinM]

Thanks so much Al. I’m in a scary limbo place right now, and your words really help. Kevin

 

[KevinM]

Re: Club membership at hand: UPDATE

I went through a thorough 2 hour exam/EMG at Jacksonville Shands with Dr. Pulley on Thursday with my wife’s support. The bottom line is that I am still in the Clinically Possible category. Now that he has his own baseline, he wants to see me in two months to check for progression before making a call.

His clinical exam was much more thorough than the previous two I had done locally in early February and March, and he found some slight but demonstrable weakness in my right tricep and left thumb (4+ but still apparent), which concerned him in conjunction with my fasciculations and other muscle symptoms. I had not noticed the weakness in everyday activities.

However, when he did the EMG on five or six different muscles (again, much different and more painful than the one done by my local neuro because he left the needle in much longer, plus he was training a newly minted Dr.) he said it did not exhibit classic ALS results. It was interesting that he noted numerous fasciculations, but that was not what he was looking for.

He also said there is no evidence of UMN involvement right now. My reflexes were normal, and there is no sign of bulbar involvement. He said it’s about 50/50, maybe a little less than 50% chance, as to whether or not he thinks it is ALS. It will take a week or more for the full write-up on the EMG findings.

He said don’t make a bucket list just yet, and try to life my life until the next exam. Easier said than done. This is pure torture, because I know my body and know what is happening to me every day.

I am struggling to find the courage, as I’m sure everyone here went through at this diagnosis limbo stage.

Thank you for being here. Kevin

 

[Nikki J]

Thank you for reporting back. Less than 50 percent chance is good. When you get the emg results we would be interested if you would share them.

A word about terminology. Reading your post you were told you do not currently meet the criteria for ALS but he wishes to observe you in case of progression because maybe - just maybe- this is the start of something. This is NOT the same at all as clinically possible ALS. That term refers to people who have upper motor neuron findings and an EMG that demonstrates the abnormalities of ALS in one region of the body ( such as lower extremities, upper extremities or bulbar). Two area equal probable and three definite. All of these people are told they have ALS offered riluzole and radicava usually and even people with possible qualify for many clinical trials. A large percentage of PALS die before reaching the definite stage by these criteria. Confusing as this is it is a really important distinction.

 

[KevinM]

Thanks Nikki. He was the one who said if he had to categorize it right now it would be clinically possible. So apparently there was enough there with the weakness, symptoms, and whatever he saw on the EMG for him to put it in that category. Even without UMN involvement at this point.

I would add, though, that he said he wasn’t at the point of prescribing anything or talking about clinical trials, so I’m not sure what to make of it. Kevin

 

[Nikki J]

If you speak to him again ask if it is clinically possible by El escorial. Or look at your discharge paper for the code. If he didn’t see ALS on the emg Or find upper motor neuron signs I don’t think it can be

 

[KevinM]

Will do. All I got at discharge was a copy of the clinical exam scoring and a paper that described how to establish an online account to view records. It sounds like it will be a week or more before the full summary of the exam will be available.

 

[KarenNWendyn]

Kevin, you state “this is pure torture”.

You don’t have a confirmed diagnosis of ALS at this point. You weren’t even given a diagnosis of “probable” ALS. The odds are in your favor and your description of what the specialist said about the EMG doesn’t sound particularly worrisome. I second Nikki’s comments that “clinically possible” is ambiguous unless he was specifically referring to El Escorial criteria. His EMG report should help clarify this. We look forward to seeing the summary and conclusions when available.

In any event, there’s not a PALS here who wouldn’t love to have a greater than 50% chance of what we have being something other than ALS.

I wish you the best, truly.

 

[KevinM]

Thank you Karen. This lengthy diagnostic process over the past three months has been very difficult, and I apologize if it appears I am overreacting. It is very real and tortuous to me and my family, though.

I absolutely understand that the incredible individuals on this forum would give anything to be told they had less than a 50% chance of having this horrific disease, but I also don’t doubt that many here went through months of uncertainty, often conflicting statements from medical professionals, and inconclusive exams before a final determination was made. My own PALS friend here in Tallahassee went through the exact same situation in 2015 with Shands, and it took a follow up at Mayo for a definitive diagnosis.

Maybe that will be my fate, maybe not, but the process from healthy to most certainly unhealthy has been difficult. Months of testing, abnormal EMGs, the continued progression of my symptoms, and now clinical weakness rightfully has me depressed and anxious.

I will report back after my next exam. Dr. Pulley did say that it was possible that the EMG could be done too early, and he will want to do additional EMG testing this summer. Thinking of everyone here, Kevin

 

[KarenNWendyn]

Someday there may be a biomarker or some other test to detect ALS in people who have a few worrisome symptoms but otherwise lack full diagnostic criteria. For now, the EMG is the best we have.

Some people on this forum waited over a year to be diagnosed. As Nikki says, some people die waiting.

If you do end up being diagnosed, you probably have slow progression and you’d be early enough in your course that drug studies and drugs like Edaravone and Riluzole stand a better chance of doing you some good.

Meanwhile you’re very functional. So that means you can travel and do fun things. In the two month wait before your follow up appointment, I recommend taking the position that you don’t have ALS, and doing as many fun things as you can with your family and friends. It’s time well spent no matter what.