KevinM
Senior member
- Joined
- Mar 30, 2019
- Messages
- 559
- Reason
- PALS
- Diagnosis
- 06/2019
- Country
- US
- State
- FL
- City
- Tallahassee
Hi all. Rainy Saturday in north Florida, and as usual I have too much tIme on my hands and too many words on the tips of my fingers.
I saw this question posed on the public Facebook page, and it is one I would like to explore with our forum members. The question is “what is meant by slow progression? As the poster pointed out, we all have a good idea of fast progression, but I’ve not seen a medical definition for slow progression I realize there are many variations within each “category,” and it may be that only the passage of time will allow for a clearer idea of whether one is slow or not.
Timeout...we have a tornado warning and one has been spotted on radar a few miles from our house. Be right back...
Okay, back on the porch. The thunderstorm showing radar rotation went right over us, but apparently the twister didn’t touch down in our immediate area. Hoping the rest of the region fares as well.
So back to the question. Have any of your neurologists attempted to quantify slow progression? The easy (and incomplete) answer would be that if the average loss of function is about 1 FRS point per month from diagnosis, then anyone losing function faster than that would probably be considered a faster progressor, and anyone slower than that would be in the slower category, but that seems too simplistic. Plus, as has been pointed out here, there are plateaus, UMN vs. LMN predominance, bulbar vs. limb onset, etc. And progression is rarely linear.
This question also ties into when the clock actually starts ticking, more precisely from symptom onset or from diagnosis. I have read of many PALS that had mild symptoms for a year or longer—sometimes much longer—before seeking a diagnosis, which would seem to point to slow progression if one defines progression from symptom onset. However, by the time they did seek a diagnosis functional failure was starting to occur, and from that point on progression occurred more rapidly since enough motor neurons had died to reach a tipping point. So would they still be seen as having slow progression?
Perhaps there is no hard and fast definition because every case is unique, and only time will tell. One PALS friend of mine lost the use of her right hand and then arm within a year of diagnosis, but it was confined to that limb for almost five years before other failures started to occur. That would definitely seem to fall under the category of slow progression, at least for the first five years. Others have gone for years without losing complete use of any one muscle group (seems more frequent with UMN dominant PALS), instead having a slowly progressing systemic weakening. In a number of cases, FRS scores have remained in the mid 30’s to mid 40’s for several years.
Love to hear some thoughts on this, especially if your neurologist has defined it for you. Happily safe with house intact, Kevin
I saw this question posed on the public Facebook page, and it is one I would like to explore with our forum members. The question is “what is meant by slow progression? As the poster pointed out, we all have a good idea of fast progression, but I’ve not seen a medical definition for slow progression I realize there are many variations within each “category,” and it may be that only the passage of time will allow for a clearer idea of whether one is slow or not.
Timeout...we have a tornado warning and one has been spotted on radar a few miles from our house. Be right back...
Okay, back on the porch. The thunderstorm showing radar rotation went right over us, but apparently the twister didn’t touch down in our immediate area. Hoping the rest of the region fares as well.
So back to the question. Have any of your neurologists attempted to quantify slow progression? The easy (and incomplete) answer would be that if the average loss of function is about 1 FRS point per month from diagnosis, then anyone losing function faster than that would probably be considered a faster progressor, and anyone slower than that would be in the slower category, but that seems too simplistic. Plus, as has been pointed out here, there are plateaus, UMN vs. LMN predominance, bulbar vs. limb onset, etc. And progression is rarely linear.
This question also ties into when the clock actually starts ticking, more precisely from symptom onset or from diagnosis. I have read of many PALS that had mild symptoms for a year or longer—sometimes much longer—before seeking a diagnosis, which would seem to point to slow progression if one defines progression from symptom onset. However, by the time they did seek a diagnosis functional failure was starting to occur, and from that point on progression occurred more rapidly since enough motor neurons had died to reach a tipping point. So would they still be seen as having slow progression?
Perhaps there is no hard and fast definition because every case is unique, and only time will tell. One PALS friend of mine lost the use of her right hand and then arm within a year of diagnosis, but it was confined to that limb for almost five years before other failures started to occur. That would definitely seem to fall under the category of slow progression, at least for the first five years. Others have gone for years without losing complete use of any one muscle group (seems more frequent with UMN dominant PALS), instead having a slowly progressing systemic weakening. In a number of cases, FRS scores have remained in the mid 30’s to mid 40’s for several years.
Love to hear some thoughts on this, especially if your neurologist has defined it for you. Happily safe with house intact, Kevin