Dusty7
Senior member
- Joined
- Aug 3, 2013
- Messages
- 521
- Reason
- PALS
- Diagnosis
- 08/2013
- Country
- US
- State
- FL
- City
- Orlando
As Nikki says, PMA (U.S. term) is a purely lower motor neuron disease, sometimes referred to as Lower Motor Predominant ALS because it gets better treatment by insurance companies than if it's simply called PMA. There was a study of PMA (I think it was in 2002) and they would not accept anybody who had had PMA for less than 4 years because they thought that anyone with PMA for less than 4 years could still develop UMN and become ALS. Even so, during their study some people did subsequently develop UMN. Plus, in other studies, people with PMA have been autopsied after death and UMN signs were discovered. In other words, they had undiagnosed ALS. So you can understand why many neuros are hesitant to say you “only” have PMA when there is a good likelihood you will slide into ALS. Technically, I agree with your point that it cannot be ALS without UMN signs, but many neuros still believe that PMA is a form of ALS where the UMN signs have just not revealed themselves yet. And when you consider how rare PMA is (about 5-10% as frequent as ALS) the safer bet is to call it ALS.
As everyone with an MND (U.S. term) will tell you, progression varies from individual to individual and no 2 cases are the same, but it appears that bulbar ALS can be very rapidly progressive, then there’s typical ALS which can be as fast but is probably generally slower and then you have PMA which appears to be very slowly progressive, at least until UMN signs appear. So I would take PMA over full-blown ALS any day. The debate over whether PMA is a separate disease from ALS has been going on for over a hundred and fifty years and I expect it won’t be resolved until we fully understand the ALS process.
So your father’s neuro is probably being conservative because PMA is extremely rare and many times it does develop into ALS. He doesn’t want to say it’s not ALS and then have to tell you he was wrong in a few years. But the good news is that you’re right—it sounds like he does not really have full-blown ALS at this point and hopefully his progression will be slow. Weakness in the legs and left hand is certainly not good, but it’s a situation most PALS on this site would probably trade for. And his pulmonary test was normal. To me, it’s not a “sell the house and start giving away your possessions” kind of thing, but more of a “don’t start drawing up the plans for the dream house you want to build in 10 years”. A warning. Live every day to its fullest. He will outlive a lot of his friends who will die of auto accidents, heart attacks, lung cancer, etc.
It is what it is, so live life to the fullest.
As everyone with an MND (U.S. term) will tell you, progression varies from individual to individual and no 2 cases are the same, but it appears that bulbar ALS can be very rapidly progressive, then there’s typical ALS which can be as fast but is probably generally slower and then you have PMA which appears to be very slowly progressive, at least until UMN signs appear. So I would take PMA over full-blown ALS any day. The debate over whether PMA is a separate disease from ALS has been going on for over a hundred and fifty years and I expect it won’t be resolved until we fully understand the ALS process.
So your father’s neuro is probably being conservative because PMA is extremely rare and many times it does develop into ALS. He doesn’t want to say it’s not ALS and then have to tell you he was wrong in a few years. But the good news is that you’re right—it sounds like he does not really have full-blown ALS at this point and hopefully his progression will be slow. Weakness in the legs and left hand is certainly not good, but it’s a situation most PALS on this site would probably trade for. And his pulmonary test was normal. To me, it’s not a “sell the house and start giving away your possessions” kind of thing, but more of a “don’t start drawing up the plans for the dream house you want to build in 10 years”. A warning. Live every day to its fullest. He will outlive a lot of his friends who will die of auto accidents, heart attacks, lung cancer, etc.
It is what it is, so live life to the fullest.
our motto ...
