Thanks All,
I know that people with the 'classic' ALS can exceed given life expectancy but functionality tends to be dimished in most cases.
John, I really don't know about Hawking, just the short overview about MND on his site. I have heard PLS from MNDers here in the UK. He does state he could still dress himself till early 1970s, some years following the diagnosis, so I guess it is slow. I always thought he was on a vent...but no, he is breathing independently.
As for upper motor neuron dominant ALS - I do think it is a 'valid' variant, but it would imply less atrophy, hence greater functionality. I have seen a great range where atrophy is concerned amongst PALS, from what I would call a barely noticeable weakness/mild atrophy to a complete lack of muscle (the latter is the case with my partner's arms). I think atrophy impacts on functionality and independent performance of tasks a great deal more than spasticity. One would have to really push a consultant here in the UK to even utter the phrase 'upper dominant', they simply prefer MND - all in one. Some PALs have disovered they have PMA or PLS years after the final diagnosis, and while there maybe a scope for deferring PMA or PLS diagnosis (in case the patient gradually develops additional symptoms), initially the lack of distinction provides very little hope.
Dani