Progressive bulbar is when there is definite lower motor neuron involvement ~ the actual cranial nerves that are housed in the bulbar section of the brain stem. (and would show up on EMG testing) are affected.
Pseudo bulbar, is upper motor neuron. There is weakness from this, and brisk/hyper reflexes as well. Often times the pseudo-bulbar predominant people are the ones with lots of saliva. I'm not completely sure I understand the connection. Reduced saliva and/or tears can be a feature of the bulbar palsies as well.
Bulbar onset of MND or ALS means that the disease first presents there, and then continues to spread.
I am not aware of anyone currently active on the forum that has PBP and only that, with no other body involvement, but I started out that way, as did BethU.... I'm not so sure about Brenda.
Because palsy means "weakness" it can refer to anyone that has weakness in the bulbar areas, the type that is MND is progressive.
There are 12 pair of cranial nerves in the brain stem (one for each side of the body) and 12 corresponding pair in the motor cortex (UMN). the UMN communicate directly with the LMN, and in turn the LMN communicate with the actual muscles.
If you are the retired flight attendant, I bet you were a great one.
I bet you were the flight attendant who firmly took no guff from any annoying passenger but did it nicely. (Best line from a flight attendant I remember was on a People's Airlines (dates me) flight NYC-DC I think, and the guy next to me who kept "accidentally" grazing the flight attendant's uniform asked when we'd land in DC. She looked at him in horror and said "OMG, you think we're going to DC? This is the Philly flight, sir." Worth just that fraction of a second on his face. I got her name and wrote a letter commending her.
Rose, you mentioned that you did not know of anyone else in the forum with only bulbar symptoms. I am one who has only bulbar, at least to this point. I have no weakness anywhere else and only mild problems with swallowing. My pulmonary function is good. My only real problem is with speech. I am slurring and having problems with some sounds. The Dr. diagnosed me with Bulbar palsy/probably als. Actually he has used two terms, probable and the other day at our meeting he said possible als. Is this normal? I do have some saliva, not much, and also some emotional lability, again, not much. I started out with symptom of feeling like something was stuck in my throat. Went to an ent and he said it was most likely GERD and put me on aciphex, although I never had heartburn. I took that and everything seemed to go to hell. I felt awful. After 4 months or so I went to general paractioner, who was clueless. I had also had larongospasms. Very scary episodes. After 6 months on the Aciphex, my daughter started investigating the drug and told me to get off it. Stopped taking it and things really settled down. Went to a nuero and he did tests and said he did not think I had ALS. Went to Mayo for second opinion and got the diagnosed above. I am going into the trial Avanir in two weeks. Dr. is putting me on Rilutek and then possibly Lithium with it and because he wants me to get on rilutek, he is going to give me the diagnosed of ALS.
Still I only have the real problem of speech. Sometimes it is worse than other times, depending on stress level, or how tired I am. He said it appears to be slow progression and we will see what happens over the next 3-6 months. Is it possible that this is all it will ever be? I get so confused over all this. I think they are going to send me all the results. It will be interesting to see them and investigate the results. Or will it?
I don't know if it will get worse or if it will not get worse. I have decided to be in touch with anything going on in my body, but I am going to continue to live.
All your posts are so informative. Any thoughts you have on this book I just wrote or on what you have lived through will be greatly appreciated.
I'm sorry you had to join us but am glad to have your input and hope we can help.
I was wondering if you found any connection between slurred speech and what you've eaten or drunk? I find the "meals-in-a-can" such as Boost and Ensure definitely create lots of saliva and mucous and make speech more difficult.
It sounds as though you've had a run around medically and you don't deserve that.
I encourage you to keep a hard copy of all your reports to date, for your own information, and in case you decide to ask any other medical questions of different practitioners.
Yes, I was a flight attendant. I enjoyed your story. Peoples Express actually merged with Continental in 86, and almost always, if I flew with anyone, pilot or flight attendant that was "old P.E." I could expect that they would be fun loving as well as hard working. There were very few exceptions .... I do miss flying.
I'm anxious to hear what you learn on Thursday.
I am sorry for your diagnosis. It is my understanding that there is specific wording used for diagnosing any form of MND. When our doctors talk to us, it sounds like they're describing their feelings, so to speak, but in actuality there is defined language for making a diagnosis. There is suspected, possible, probable, and definite. But, a definite diagnosis for bulbar palsy, could also be possible or probable MND depending on what a doctor finds on exam, and it would not move from that classification over to bulbar onset of MND, until there is clinically supported evidence of it in 2 (?) other regions. Don't remember precisely how many regions are required. ~ am I making sense?
I hope you continue to progress very slowly. I do not know what percentage of people with PBP never experience further body involvement. It sounds like you've got a good attitude, and that is bound to help you greatly. I've been told they believe the earlier a person starts on the Rilutek the more effect that person will gain from the medicine. Mayo is an excellent facility, and you should get very good advice and care from them there.
To answer your question about my progression, I had mostly only bulbar symptoms for about 18 months or so. It was my voice rather than speech to be affected first, and as amazing as it sounds, I didn't really catch how weak my swallow was until it was pointed out to me by my doctor and speech pathologists. I think it was so gradual, I just got used to it as it happened.
Good luck with your appointment that is coming up, and I look forward to seeing you on the forum.
When Rick was given his diagnosis there were about 12 specialists with us in a quiet room, all talking together.... sometimes debating... RIGHT IN FRONT OF US. .. and the consensus was ALS.
He doesn't have Bulbar. He has respiratory onset with leg involvement and now a tiny bit into his hands.
He doesn't have excessive saliva, but in the morning he has to blow his nose with a half a box of Kleenex and coughs the congestion. There is no indication of infection (excuse me) the excretions are clear like water. We are told this is the reaction his lungs give to the deficiency of breathing during sleep (his immune system) He has a Bipap machine.
He delays having breakfast until he's done blowing.... and the rest of the day he is clear.
Hi, When I (or more correctly, my wife) first noticed that something was wrong with me it was slurred speech after I'd had a couple of beer. No problem, I thought, until my speech started to always be slurred. That was in the fall of 2007 I was diagnosed with Bulbar ALS in Jan of 2008. Until recently my only symptoms were deteriorating speech, lots of saliva, and increasing difficulty in swallowing. In the last month I have noticed that my right hand is starting to not work very well. So far it's not all the time but it is a worrying development. I've found that Boost and Ensure do cause thick, mucousy (is that a real word?) saliva which makes it more difficult to speak. I now have a renewed appreciation for my toothbrush.
I hope that you never have any other developments or at least it takes a very long time. I think that stress is a big factor in the disease progression and so my new motto is "relax, be happy".
Al, because Rick doesn't have humidity added to his Bipap, and because he had the morning congestion for years before he even got a Cpap, I think the congestion is part of the ALS.
Because the lungs are straining, the body sends serum to the area. It'a like getting a rug burn or a cut. Have you ever had a rug burn where sticky orange colored "stuff" keeps appearing on the skin or on the bandage? That is serum, and it has antibodies in it to help prevent infections. It is only a guess, but I think the body reacts to the lungs overnight by sending secretions in excess.
I think you made a good point in that the Boost (or any meal-in-a-can) which you need for nutrition and calories, also increases the mucous. In fact, most everything you are eating more easily contributes to mucous and saliva which then makes it harder to eat! (and speak).
Have you found that an over-the-counter product such as Musinex helps at all? My husband was given the suggestion of a suction machine, I suppose like the one at the dentist, but he got angry and said you'd need it 24/7, i.e. literally constantly, which mooted the helpfulness of it.
I haven't tried Musinex or anything else for the mucous but thanks for the idea, I'll keep that in mind. Something else that caused me a lot of problems were milk and other dairy products, I would get such thick, ropey mucous that it would almost cause me to gag. I had radiation treatment to my saliva glands (parotid) last fall and while I still drool occasionally my saliva and mucous is reduced to the point where it is not really a big problem anymore. The radiation treatment was an option that was offered to me by my neurologist and was done at the cancer clinic in Edmonton. It is painful at the time and for a month afterward but it was sure worth it.
As soon as I got the diagnosis from the ALS clinic they gave me a hand operated suction pump and arranged for delivery of a somewhat clunky AC powered suction pump. The only time I've really used either of them was when I came down with a bad head and chest cold last fall and I was on the AC suction a lot. At that time I couldn't imagine not having one. The hand operated pump was given to me and I think I paid about $60 for the AC unit which now sits on the floor beside my bed in case I need it.
I also bought a Waterpik Dental Waterjet (WP100) cleaner which I use at least four times a day. It has a water reservoir and a hose that you can put different ends on. Pretty much the only one I use is the toothbrush end which I use to brush my teeth, gums, tongue and the roof of my mouth and I find that if I brush and flush with the thing after everything that I eat my mouth and everything in it works better. You can probably find it at any large pharmacy but here is the link to the website so you know what I'm talking about: http://www.waterpik.com/oral-health-products/countertop-dental-water-jet/
I've always been a "gadget guy" which drives my wife CRAZY but in this case I figure that if something helps even a little bit it's worth a few bucks. I hope this info helps (or at least makes sense).
We have a 20 year old son who is away at university in Victoria right now. We worry about how all of this stuff with me is affecting him but so far he's been a great help when he's home, going with me to clinics, interpreting my garbled speech for others, etc. How's your daughter taking all of this? I am glad that my son is the age that he is and can understand (as if any of us can really) and help out and maybe even push me around if it comes to that.