Thanks for all the info Annie. It was great help to his caregiver, who came back with all these info. From what I read, the patient did start with bulbar symptoms, before it spreading to his limbs. However all these deterioration happened within a span of 5mths. I too would think that its too fast for ALS, though the doctors insist that some ALS deteriorate so quickly within one year, and pass away the same year. I don't think anything showed up in his EMG to point to ALS, but his docs are saying its ALS simply because of his progressive weakness and deterioration, and his lack of fast improvement when started on Mestinon. The doctors seem to be giving lots of excuses, but here's what his son said.

How it started
"Around December 2008 he began having difficulty swallowing and speaking. The symptoms were manageable and tended to come and go. Things remained like this for a while and I didn't really think much of it. In April 2009 we went on a trip to Toronto/Montreal and i noticed had almost completely lost the ability to hold his head up, and his speaking had become much worse. He began complaining of seeing double and having other vision problems. He used to be an avid "city walker" and loved walking for many KMs throughout different cities we were visiting. This trip he wasn't able to go more than 2 city blocks without having to get into a taxi and head back to the hotel. I had noticed that these symptoms were improving with rest. Things began to rapidly deteriorate about a week after returning from vacation. To hold his head up he had to have it propped up with both hands. His speech had become extremely difficult to understand... It seemed to be one long slur most of the time. During all of this we were waiting for a neurology appt. which had come with an 8 week waiting period. By mid-may, he was no longer sleeping through the night and could barely eat or speak. One morning, he was almost totally unable to make it up a flight of stairs and became increasingly confused. He had begun hallucinating and was appearing to have problems breathing. We headed to the hospital and he was admitted immediately as he was in the midst of respiratory failure."

Tests done
"My Dad has had every other test for MG... EMG, Tensilon, two or three different blood tests... even one that had to be sent to the UK... Everything has come back negative. All signs point to him not having MG except for his positive response to the mestinon."
*He didn't state if MusK had been testsed, I'll remind him to check again*

Current Situation
"After 4 days off the Mestinon, his condition deteriorated steadily. He could no longer breath on his own during the day for more than a few hrs (previously he had worked up to 16), his speaking ability was totally gone, and he could no longer walk even a meter (10 days ago it was up to about 350mtrs).

Pleading with the Drs had no results and I was begining to think that perhaps they were right and he wasnt actually a myasthenic and the mestinon never was doing any good. I arrived Saturday morning in the middle of rounds and the first thing the Dr said was that they were restarting the drug because of his rapid decline... The dr was very specific and said that the efficacy of the drug peaks about 8-10 hrs after the dose and that by then we would see results if it ever was helping him.

Saturday night and still no improvment, in fact he seemed worse... He asked to be put into bed at around 7pm (usually he stays up until around 10) and he was still needing more and more ventilator support. Things really didnt look good. Sunday morning however he was improving, seeming stronger and could speak a little bit. The Doc was unimpressed and thought that my Dad might simply just be trying harder during his assesment tests and that we may have been experiencing a placebo effect.

Things got interesting at around 5pm on Sunday. His speech has improved about 75 percent, he could speak in sentences once again, and his head and neck could once again hold themselves up without being propped up by his arms. I went and asked the DR what he thought about the change in condition and he said "i am still pretty certain that the mestinon isnt actually doing anything, I am stopping the orders for it"... I asked him to come into my Dads room and actually take a look at him. He was shocked and kept saying "neurology said that if we didnt see any change in 8 hours that we would never see one" ... He finally admitted that he really does not have much backround knowledge about MG or even MND..."

What really amazed me was what the doctors said when asked if his dad could start the Mestinon again.
"most of the hospital staff are still leaning towards ALS or another progressive MND. As if he had totally forgot what had happened last night, the Dr came in this morning and said "yes, voice is better... neck is a little better... I am still not convinced. Next thing might be to switch him out to sugar pills and not tell you guys and see if you keep noticing 'improvements'... This might be all mental here"

As to what Annie had suggested, the patient actually had Dr Toth from the foothills clinic come over to the hospital he is at and evaluate him for a week. HE is the one who gave the firm diagnosis of MG and said that it was a rare subtype which usually required a lengthly ICU stay if a respiratory crisis was reached. He went back to his "home" hospital though and they have since had 2 different neuros and 3 different intensivists.

Plasmapherisis has not been done. His wife brought it up twice and the first time no one really seemed to know what it was, and the second they said it was "risky" and left it at that. The patient has had two courses of IVIG which seemed also to help him in his recovery. Until the MG medication was stopped, he was on an upwards curve of very gradual improvment. I think what has thrown everyone off is how slowly it was going. One of the Drs just decided that it shouldnt take this long and that he had an MND and not MG.
They do blood gases every few days. When he came in, his CO2 was so high that he has having hallucinations from it. He was that far into respiratory failure. Since being in the hospital it has been a little high but consistently at the same level which they think might just be "normal" for him. For a while they stopped doing blood gas testing because he was breathing fine on his own during the day then being rested at night on the ventilator. His son said one was done 2-3 days ago and it was fine.

The steroids were started, in very large does, about a week after arriving at the hospital. He was just on prednisone from what his son thinks... They have slowly been tapering it off.

Questions I have, any comments?
1. The patient started showing marked improvement after about one day from restarting Mestinon. The doctors refuse to take that as the response from Mestinon, as its after the 8-10 hours specified by their neuros. Can it take longer Mestinon to take effect for some Myasthenics?

2. They mentioned that Plasmapherisis is "risky". I understand that it has complications, but is it considered risky since there are medications and others to help prevent the complications? Is Plasmapherisis suitable in his current condition?

The doctors seem to be thinking that the family is in a stage of denial, and so refuse to take what the family is saying seriously. I've asked him to try his best to get Dr Toth to see his dad again, and to at least inform someone from the MDA chapter of their situation, to see if anything can be done.