Limb progression

[karzy81]

Thanks Ted. You clearly have a lot of knowledge and I am thankful for your response. I’m not quite sure what some of the things are that you mentioned but from what I gather, spasticity and clonus refer to fasiculations correct? He definitely has constant fasiculations. His upper body seems to be fine. No breathing or swallowing issues, just walking. He also has severe spinal stenosis so I am sure that is not helping. Does UMD has a better prognosis?

Karen

 

[lgelb]

Re oral edaravone, the Phase 2/3 study has been in "planning" since 2015. I see the company (Treeway) is recruiting for a clin dev head, meaning they might be a step closer to funding it, but clearly approval is well into the future. I just don't want anyone to delay infusions on that account.

 

[KarenNWendyn]

Karzy, I wanted to address some of your questions.

Spasticity, clonus and fasciculations are different things. Spasticity refers to muscle tone and is a tightness that leads to uncoordinated muscle movement. Spasticity can be detected by a trained examiner when they move your limbs back and forth.

Clonus is a rapid, repetitive, uncontrolled movement at various joints. For example, ankle clonus occurs when the ankle is rapidly and forcefully flexed by the examiner, resulting in ankle repetitively contracting on its own (there are youtube videos demonstrating clonus).

Both spasticity and clonus are upper motor neuron signs.

Fasciculation is a lower motor neuron sign and refers to spontaneous contraction of a group of muscle fibers. It has the appearance of worms crawling under the skin. The movement is on a much smaller scale than clonus and usually doesn’t lead to joint movement. I think there are youtube videos on fasciculations.

Pure lower motor neuron disease (progressive muscle atrophy) and pure upper motor neuron disease (progressive lateral sclerosis) both have a longer survival than ALS which is mixed lower and upper motor neuron disease.

Many people initially felt to have PMA or PLS eventually morph into ALS. Technically, they had either LMN- or UMN-dominant ALS, respectively. I would guess their survival prognosis might be somewhere in between PMA and ALS, or in between PLS and ALS, but that’s just a guess. Your mileage may vary.

 

[Miav123]

We were hoping that my father had a slow progressing form, as that's how it started off. He was diagnosed in August 2016 but had symptoms for about 1.5 years or more. At the time of his diagnosis, he only had weakness in his right hand and would get tired faster than normal. Fast forward a few months and he could no longer use his right arm. By the time he started Edaravone in October 2017, his left hand and arm were getting weaker but he was still walking unassisted, eating and breathing normally. After starting Edaravone, his progression was dramatic. By the first week of November he could no longer walk, he seemed disoriented at times..he basically needed help with everything including breathing. He passed away in January...a year and a half after diagnosis. So while the first year and a bit were slow progressing, his last few months were so dramatically fast. I hope that your father continues on the slow progressing path. Enjoy your time with him as best as you can.

 

[karzy81]

Thanks for that. It sounds like a lot of people seemed to go downhill after starting this Edaravone. Since he started he also seems to be a little more wiped out and unstable but still only in his legs. Geez I hope this medication is helping because not only is it time consuming and at times uncomfortable but it is also expensive. I sure hope the upper body has some time before it is affected.

 

[Tedstehr]

Karzy,

As Karen describes, fasiculations are lower motor neuron symptoms. I have them too to varying degrees at different times. But I have extremely minor muscle loss or weakness - hence UMD. But fasiculations means not PLS.

As for Edaravone it is unlikely that is speeding up progression. In my opinion it may just be timing of the disease that causes the rapid decline. Many of us are frustrated that a large scale study has not been done to properly measure its effectiveness. It could also be that the drug works well early in the disease and then does harm later.

 

[Tedstehr]

By the way, "my opinion" is not unlike the guy covered in grease who crawls out from under his car to explain how to solve all the problems in the Middle East. Perhaps convincing but not based on knowledge. (Unless it is Madeline Albright and I doubt she works on her own car.)