Flail Arm Syndrome ALS

[artist09]

After seeing 3 neurologist that diagnosed my father with ALS. The third at the VA hospital thinks he has a variant called Flail Arm Syndrome due to clinical diagnosis and the absence of some typical ALS signs. We have 2 months before we see an actual ALS specialist at Vanderbilt. (4 month wait for an appointment.) Do any of you know much about Flail Arm Syndrome.

By the way, we have seen many doctors and the care at the VA is by far the best received so far. They actually seem to care about each and every patient. I hope more of you have the VA as a resource.

 

[tdodson]

I don't have much to add but Mom, Dad, and I were at ALS clinic on Friday at Vanderbilt and meet someone with Flail Arm Syndrome. He had been diagnosed with ALS in 2002 and only within the last 2 years was that updated to FAS ALS. He switched Dr's at Vandy. I don't recall the name of the doctor that did diagnose him with FAS. It wasn't Lee or Donofrio at Vandy. Other than that I just know what I read doing very quick searches when we got home. The guy wanted us to look that up for Mom since her ALS started in her arms.

I did not notice the FAS person have any trouble other than his arms. He could get one arm up to his face with help from the other, hands didn't move much nor shoulders. He was much happier with a FAS ALS diagnosis than ALS. Everything else seemed normal with him. Legs, speech, breathing, just arms and hands didn't work, or barely worked.

Sorry I couldn't offer anything else. I just found it odd that after meeting someone at Vandy on Friday with this there was a question on here about it. Wish you all luck at Vanderbilt. I think Mom waited for 3-4 months for her first appointment. After that it seems like it was easier to get in.
Todd

 

[trfogey]

Flail Arm Syndrome, also called brachial amyotrophic diplegia or the "man in the barrel syndrome", is a lower motor neuron disorder that predominantly affects the arms and hands. It can spread but usually does so much more slowly than true ALS -- more along the lines of Kennedy's disease or progressive muscle atrophy (PMA). It is a common differential diagnosis for patients whose progression is slow and stays confined to the upper body for an extended time.

 

[artist09]

Thank you about the info from the guy at Vandy. This sounds exactly like my Dad...using one arm to help raise the other one. He has been dealing with this for 9 months and it is still confined completely to his arms. The first neuro in Jackson said he had only lower neuron findings, but this "resident" at the VA said she saw clinical signs of upper motor neuron. One being brisk reflexes in those areas. (This is not proof enough for me.) His legs are as strong as ever and his pulmonary test was completely normal. He has no trouble speaking or eating. They suggested that since the atrophy was confined only to his arms during the past 9 months that it must be Flail Arm Syndrome. The atrophy is so significant in his arms, that everyone who we have seen has also said that he should be showing signs somewhere else by now. So, I was hoping that if it was truly ALSm that it was a variant. We are seeing Dr. Lee at Vandy. The french lady...who they say is the best....was impossible to get in to see. Did you happen to get contact info for this guy you talked to? I know the FAS ALS is rare and I'm trying to find someone else to discuss it with. We are anxious for our appointment at Vandy and will accept their diagnosis if they say it is FAS ALS too....but until then....more questions than answers.

In the meantime, I've convinced him to go on an Alaskan cruise to fulfill a dream to see Alaska!

Thank you Trofigy for the info.

 

[rcharlton]

It has been suggested that I have FAS ALS although I don't fit the criteria exactly. I'm 44 and I was diagnosed when I was 38.

Six years in and my legs are fine - or at least at 90%. I am still riding a modified bike/trike. Walking and stairs are no problem.

My neck has gotten gradually weaker although I can still hold my head up.

My lung function is pretty low right now - FVC about 30%. I had a sharp decline in breathing function 2 years ago and I started using a bipap - but for 3-4 years post diagnosis my FVC was above 90%.

My speech is becoming unintelligible - that has been a pretty constant linear decline. I am still eating most foods - very slowly. Choking on liquids was always a symptom but I find that I can control it.

For sure your Dad should travel as much as possible while he still can. I travelled a ton during the first 2 years following diagnosis and I am still travelling 1-2 times a year now.

Good luck

 

[rcharlton]

oh yeah

My arms and hands are pretty much useless.

As of a year ago (5 years in) I could no longer feed myself.

I can just manage to go potty by myself at home provided that I am only wearing my boxers. Asides from my underwear, I've needed help getting dressed for a couple of years.

I don't find labelling very helpful for PALS. To my mind, every case of ALS is different and it is seldom helpful trying to gauge your progression by comparing yourself to other PALS.

A PALS first year is the best gauge as to the rate of progression, not a comparison to other PALS.

I would not stress about whether or not it is FAS. A label won't help with the management - and, unfortunately, regardless of what it is called, there will always be uncertainty and questions.

Take care

 

[artist09]

Thank you rcharlton. I just saw your post. Dad's progression has been pretty fast this year. It started at the first of the year by having trouble raising his arms above his head. Now, he can barely raise them above waist level. He lives alone and manages. He still drives and feeds himself, but feeding and dressing are not easy for him. So far, there are no signs in his legs or other areas. These areas remain the same. But, his arms are very weak. I think it has progressed fast, but I don't know if this is fast or not. He FINALLY sees a specialist this week. We have been waiting since his ALS diagnosis in June. The VA is calling it Flail Arm Syndrome. It does appear to be this for now. Yours seemed to progress more slowly than his. Am I right? How are you able to type? Do you have a computer aide for this? I'm wondering what we need to prepare for. Thanks.

 

[rcharlton]

Sorry I just saw your post.

I type using an onscreen keyboard called Wivik and a regular $20 wireless mouse that I operate with my foot.