Okay, so I have read up on the  moya moya disease and it says it is progressive and I dont understand in what way it is progressive because it doesn't really explain it anywhere? When I was 18 I was diagnosed with moya moya and I later saw a surgeon who said that my brain had found a way around it, so that I shouldnt even feel any symptoms anymore. Does that mean I will never have to worry about it anymore? My neurologist told me to see him every 6 months, which I haven't done, but it just all confuses me because there isn't much information on it anywhere. I just found this site. It's been 2 years and I have been having some weird neurological symptoms (numbness in my mouth and arms and some other stuff), so I am just wondering if it is normal, stress, or could be the moya moya. SO overall my question is in what way is it progressive?

Moyamoya IS a progressive disease. 

To the extent that your brain "found a way around it" (an appallingly ignorant statement coming from a physician, I might add), it may be that you have been able to compensate for it.  My husband presented at age 58 with almost no symptoms.  His neuro said that other arteries (external??) were compensating somewhat for the occusion and atrophy of both his internal carotids and that sometimes people who are of higher intellegence can compensate -- for a while.

I do not mean to be harsh, but it is also foolish that you have not seen your neurologist as directed.  You need to have your disease monitored regularly, even if you are taking a "wait and see" approach (which is madness, as far as I'm concerned).  The numbness you report are symptoms of TIAs (transient ischemic attacks) -- mini-strokes.  Do not ignore these, because they can precede a major stroke, which can result in severe disability. 

My husband came downstairs one day last fall and said "I seem to be having trouble pronouncing some words".  My immediate thought was mini-stroke, but he refused to see a doctor.  It went away and he thought no more about it until he collapsed in the bathroom on July 20 after a chemotherapy session (he also had bladder cancer) and hit his head on the wall.  A CT scan revealed at least one "old infarct" (stroke). 

He had EDAS bypass in August and was doing very well -- good response to his chemo and looking forward to his second EDAS and ending treatment and moving on with his life.  But he did not follow instructions on hydration and aspirin and guzzled gallons of salt-riddled tomato juice.  On September 21 he began slurring his words and his hands shook.  He refused to call his neurosurgeon and refused to go to the ER.  The next morning I woke up and found he had had a stroke.  He was still conscious, but began having seizures even before the EMTs arrived.  He went into status epilepticus and even though I had him transferred from our local hospital to the hospital in NYC where his neuro team was, it was too late.  The local hospital had let him remain in status for too long and he never regained consciousness. 

I struggle between grief and being furious with him for not being willing to follow simple instructions and seek attention when it was needed.  Now, you are young and you do not have the complication of cancer to deal with.  And not everyone with SE dies.  Most of the time they can knock down the seizures with sedation.  He was just unlucky and had bad care until I was able to get him to the experts.   But you are ignoring instructions just the way he did...and you do so at your peril.

Please do not screw around with this any longer.  Please make an appointment with your neuro.  You need CT scan/MRI/MRA and perhaps a carotid angiogram.  Please do not wait until you have a stroke to find out that moyamoya is progressive.  Please promise me that.  And either post an update here or send me a PM.  I am not kidding.  For the rest of my life I am going to be a tireless fighter against both bladder cancer AND moyamoya.  I do not want anyone else to wake up and find their loved one sitting in their own feces, dazed and confused and unable to speak coherently.  :'(

Onestonetwobirds, your post brought tears to my eyes. I know of one other gentleman that lost his battle with moyamoya. Thing is if you find it in time and get the "correct" treatment you can live a long happy life.  I hope your advice is followed.  My daughter had MMon the right side and had surgery. She now has it on the left and we know there is going to be a surgery in her future. She goes for her angiogram this December.

@hrsridermom:  Thanks for your kind words.  The one thing I do wish, though I'm not sure it would have made any difference, is that my husband's surgeon (and I think he is excellent) had stressed that there is still significant risk of stroke for 6-12 months after surgery.  My husband's dilemma was that he didn't want to put off radiotherapy for his cancer, but I'm not sure that 2 EDAS in rapid succession would have made much difference.  He had very severe MM on the right and pretty severe on the left, and the neuros at three different hospitals were utterly flummoxed as to how someone with bilateral MM this severe was walking around just fine and perfectly coherent.  As I said, he had been drinking TONS of tomato juice in the three days prior to his stroke, thinking that was "hydrating" when in fact the salt was DE-hydrating him...and when he landed in hospital they did not do any IV fluids for 24 hours "because no one ordered it."  I think he had probably been having TIAs for years and just ignored them.

Anyway, it sounds like you are on top of your daughter's MM and knowledgeable about the disease so both you and she are fortunate.  I just hope the OP takes my words to heart.  As I have found, MM is nothing to mess around with.

onestonetwobirds, That is such a profound and very sad story about your husband. The one good thing is that you're sharing it and it may just save someone else from the same fate. I'd like to add that we came very close to losing my daughter at just 5 years old. She was diagnosed in Jan 2002 with moyamoya, she was not considered to be at an immediate risk so surgery was scheduled for June. By April she was having very severe strokes that could not be stopped. She needed surgery quickly so we had to go through with surgery even though she was unstable. It's definitely preferable to have surgery when your not having strokes. After her first surgery she had a severe stroke that has left her with lifelong issues. The point of my story is that no one knows how quickly this disease will progress to the point of strokes. One thing you can be sure of is it WILL progress especially if your having TIA's already. The most important thing with this disease is to consult with a specialist. Ilyy, if you look at the list of specialists on this site you can find one that you can send records to and they will advise you as to the proper treatment. Treatment can allow for a long healthy life.
Mary Grace

I was wondering if the OP was asking what "progressive" means here. If so, it means that the conditions get worse and worse if you don't do anything, and very, very often lead to very serious strokes with lasting effects. That is why everyone needs to figure out what to do and doing nothing at all is not an option. I do have a question about waiting and monitoring vs. immediate surgery which I will post as a new topic. OP if that was not your question, sorry! :)

Thank you to everybody that replied, OneStoneTwoBirdsllow, thank you for sharing your story with me. I am very sorry about your husband,,that's very sad. but you're story was definitely a type of a wake up call. I will try to see a neurologist as soon as I can. I just wasn't sure because the surgeon I saw didn't say anything about having to follow up, he didn't really explain anything the consult was all very fast and confusing (it was about 10 minutes long).  And the neurologist told me to follow up but he kind of said it in a way  that made it seem like it was optional, and think it was mostly because I was also having bad migraines. And yes Riverumma, I was asking what the websites meant by saying moya moya was progressive. like if it could occur in a different part of your brain eventually?

Can you get to one of the two major centers that specialize on moyamoya?  That would be either Stanford or Boston Children's Hospital.  I would feel better if you were dealing with at the very least a stroke neurologist, if not a MMD specialist, who understands this disease.  At the very least you should send your scans to Dr. Steinberg at Stanford.