I'm a new member to this board and have been diagnosed with Moyamoya for about a year and a half. I am being followed by the UW and Harborview Hospital in Seattle.
About two years ago, I had two TIA episodes.  Right after the TIA's, I was diagnosed with celiac disease, and the TIA's seemed to be associated with my GI distress episodes.  MoyaMoya was actually an incidental find on a routine TCD to evaluate the stroke activity.
Since the initial findings I've had two cranial angiograms, multiple CT, MRI and periodic TCD scans.  All show typical MM collateral formations. One MCA is totally occluded, and as of yesterday the other side is still holding at about 90%blocked.  I am currently without major symptoms aside from some transient tingling/numbness in my hands and feet.  My neurological exams and a very recent EMG have shown nothing unusual.  However I get the feeling like everyone is amazed that I'm still upright and moving around, and they're just waiting for the other shoe to drop.  I guess you could say I'm a bit uncomfortable with that idea, myself.
Since I have just joined this group, I'm curious about the work that is being done at Stanford.  From what I've read here, it looks like just about everyone has opted for surgery.  My doctors seem to be waiting for something to happen in order to "justify" surgical intervention.  Is it typical for other doctors to wait like this?
I also want to mention that my neurologist has been in contact with Stanford regarding my case.  They don't seem to be in any hurry either.
So....I guess I just wanted to put my info out to the group.  See if anything I've had to say raises any red flags.  Any and all comments are welcome.
I'm really glad to have found this group.  I was feeling pretty isolated.
Thanks again for your input, comments and advice.
Sheila

Hi Sheila,  Welcome to our MM family!

After reading your post, I have to urge you to seek treatment with MM experienced surgeon ASAP. (IMMEDIATLY!) Your doctor could NOT be a MM experienced doctor if he is waiting. Waiting for what, a stroke or God forbid worse?? The ONLY time a doctor waits with results like you have, is when they’re totally inexperienced in dealing with this rare disease, IMO. You have to know a stroke is coming Sheila, you just don’t know when it’s coming. In fact, EVERY MM patient is at risk for a stroke, but in your case, with that percentage of blockage,  I have to tell you, it’s a wonder that you haven’t had a stroke. You are VERY fortunate!

The reason you've read that people opt for surgery is because surgical intervention is the ONLY treatment for a MM patient. MM is a progressive disease and only gets worse over time. The collaterals that formed are ABNORMAL MM vessels, and although they are supplying blood and helping for now, they are weak and fragile vessels and they can rupture or bleed at any time, which can cause a stroke/hemorrhage and that’s why every MM patient is at risk for a stroke. Those MM vessels are not equipped to deliver the volume of blood needed over a long period of time. Please don’t wait!! I certainly don’t want to frighten you, but rather educate you to the particular pitfalls we see and hear with MM cases every day. This is a rare disease and many doctors in the medical community are not familiar enough with this disease. Please seek a doctor with MM experience ASAP!

MM is definitely manageable by surgery, and extremely successful IF treated by an experienced MM neurosurgeon. Surgery introduces new blood flow to the brain by either “direct” or “indirect” bypasses, to avoid that stroke waiting to happen.

We always advise anyone whose been told to wait, to please get a second opinion from a MM specialist. One who deals with this disease on a daily basis. Patients simply mail their films to the MM specialist and he calls you back with his expert opinion for free. You then know if you’re heading in the right direction, and IF it's safe to wait.

Unfortunately, you will find that there are only two MM experts in the country that deal with MM on a daily basis. One is in Boston, Dr. Scott, he’s a pediatric MM specialist, and Dr. Steinberg, at Stanford, California, and he’s both a pediatric/adult expert. Both experts have done hundreds of surgeries for MMD and treat this disease daily.

I don’t know if you viewed this Stanford video under Links on the website, but if not, this may be helpful in your research and understanding it all better.

Stanford video – Moyamoya Disease
http://www.moyamoya.com/stanfordvideo/moyamoya2.wmv

I wish to God that my family and I knew what I’m telling you today. Perhaps if I explain briefly what happened in our family, you’ll understand what I mean a little better. My 20 year old niece had a stroke out of nowhere and they sent her from our local hospital here, to the University of Pennsylvania in the city, where she was diagnosed with MMD, and they told us that we had some of the best neurosurgeons in the country there, and that they knew all about MMD, BUT they told us she didn’t need surgery at this time, to WAIT, so naturally we believed them. So, long story short, she ended up having 4 devastating strokes that left her in a coma, on a feeding tube, paralyzed, incontinent, etc and fighting for her life. Thank God I found this website. DJ told us to get a second opinion from a MM expert, Dr. Steinberg, at Stanford. So we overnighted a copy of her films to Dr. Steinberg for his expert opinion, and he called us the very next day. He said she needed surgery immediately, she had a severe blockage on both sides of her brain, just as you do. We had her medically air lifted from Philly to CA and Dr. Steinberg saved her life, BUT those strokes could have been avoided if she didn’t wait, the damage was done. Had we known that surgery was the ONLY option for treatment, her life may not have been changed forever as it is now.

I’m sorry for the length of this response, but I do NOT want that to happen to you. Please get a second opinion ASAP! You can contact Dr. Steinberg yourself, you don't not have to wait on your doctor. The contact info is all here on the website under links

You'll be in my thoughts and prayers.

Mar

Mar:

Thanks very much for your reply.
I really appreciate all of the information that you shared.  
I am calling my doctor today to talk to her about getting my films and records
together and sending them to Dr. Steinberg for a second opinion.

Sheila

Welcome Shelia to our family ;) FIRST OF ALL MY DRS. GAVE ME THE WAIT & SEE THING TOO.  THEN WHEN I HAD 2 STROKES ON CHRISTMAS DAY THEN ON THE 27th THEY FINALLY DECIDED TO DO MY SURGERY ON NEW YEARS EVE. 1998. NOW MY FEELING IS THAT BEACUSE I HAD 2 STROKES AND SURGERY ALL INLESS THAN A WEEK. I WAS IN A COMA FOR A COUPLE WEEKS AND HAD THE BRAIN OF A SEVEN YEAR OLD FOR MONTHS AND THIS WAS THE MOST HORRIBLE EXPERINCE I HAVE EVER HAD [smiley=huh.gif] AND WOULD NOT WANT TO SEE ANYONE GO THRU WHAT I DID, I WOULD NOT DO THE WAIT AND SEE GAME.  NOW WHEN I STARTED HAVING THE NUMBNESS A YEAR LATER I WENT BACK AND ASK IF I SHOULD HAVE THE OTHER SIDE DONE AND THAT DR. NEUROLOGIST SAID LET'S JUST WAIT AND SEE-----NO WAY WAS I GONNA GO THRU THAT AGAIN------I CHANGED DRS. AND HAD THE SURGERY IN LESS THAN A WEEK AFTER MY ANGIOGRAM,,,,,,NOW THAT SURGERY WENT VERY WELL WAS HOME IN 4 DAYS AND MOST OF ALL NO PAIN !!!!!!! FIRST SURGERY I HAD TO GO TO ER SEVERAL TIMES FOR PAIN SHOTS......AGAIN I THINK THE DIFFERENCE BETWEEN MY 2 SURGRIES IS THAT WHEN THE SYSTEMS  STARTED AGAIN----I WASN'T GONNA DO THE WAIT AND SEE THING AGAIN..... :D GOOD LUCK AND GOD BLESS YOU  8)
Elaine Calvert

Hi Shiela,
I had surgeries with Dr. Steinberg at Stanford just 4 and 5 weeks ago. I believe it saved my life. I too was sent to Harborview and after extensive testing, which should have but did NOT include a Brain Spect Scan to show just how well the collateral vessels were actually supplying blood to the brain) was told to take a blood thinner and baby aspirin and wait and see. No way was I waiting for the stroke they expected would come to me within three years. If you want to read my story look at the Going to Stanford... message.
I had been referred to Harborview because it was believed doctors representing all of the different specialties would meet in a weekly conference to discuss my diagnostic tests and propose a plethora of different treatment options. Well, that didn't happen. The week after I returned home from surgery at Stanford, I called the Co-Director of the Harborview Stroke Clinic to find out why I had not been told of the option for revascularization surgery. It turns out no neurosurgeon was ever present when my case was presented to the conference. When I asked why a neurosurgeon had not been consulted, I was told that of course "neurorsurgeons ALWAYS recommend surgery". There is a neurosurgeon of staff at Harborview, Dr. Laligam Sekhar, who has experience with Moyamoya. Perhaps he no longer attends these stroke conferences because his colleagues don't listen to his input - who knows. I was also told that it was thought that there was sufficient collateral flow, yet don't know they could have known for sure without the Brain Spect Scan and nevertheless as Mar said those vessels tend to become fragile and rupture - not a risk I was willing to take.
Given the resistance that I encountered when seeking a surgical opinion, you might want to pickup copies of your tests and overnight them to Dr. Steinberg yourself. He reviews all films received during the week on Monday afternoons and most likely you'll hear his recommendation from his nurse, Teresa Bell-Stephens later that same evening.
I also got a second opinion from Dr. David Newell at Swedish Neuroscience Institute. He has performed about 200 by-pass surgeries for moyamoya. He concurred with Dr. Steinberg's recommendation.
I'm curious to know if you were seeing the same neurologists as me. Send me an email (vanriper@whidbey.com), if you'd like to compare notes.
Best wishes to you - you'll be in my prayers.
Kim

Hi Sheila
If you adopt a wait and see attitude with this disease you will not be around to talk about this!!
Why take a risk and wait when you know that sooner or later there will be a stroke - the only varying factor is if you survive it and how much damage it will do to you.
It defies logic to me to then have to deal with the aftermath of a stroke - maybe learning to walk and talk again - if you can. To me the only option was surgery - I did not consider anything else and now my life has been saved.
In reply to your response - everything that you have voiced raises big red flags to me. MRI's can only tell what damage has been caused by TIA's and strokes that have already happened - there is no test available to my knowledge that will tell you when you will have a stroke. It is a game of russian roulette if you take a chance and they are odds I would not want to take. There have been a couple of members on the board who have died because of this disease and if you have an opportunity to avoid this happening before anything major has happened to you I would say that you would be a bloody idiot for not seizing that opportunity as fast as you humanly can. I tell it like it is - I don't believe in beating around the bush - Surgery is your ONLY option and if you don't follow this path, I will say to you, it's been nice knowing you as you won't be around in your current state for very long. Listen to what Mar has to say - she knows from personal experience what strokes can do to loved ones. The bottom line is specialists arent the ones who have to live with the repercussions of ignoring this disease, they still get paid whatever the outcome so you have to make them work for your cause - the best thing you could do right now is go and phone/page/hammer down your specialists door until you get the right decision to save your life
Moira

Shelia.......

I had 3 MEGA TOP Universities give me opinions as to how I should proceed. Two were exactly the same......One said "let's wait and see"

I then asked the 3rd "can you guarantee I won't have a major event in the interum?" The doc then said...."well....then how about we do one side now ... and see how that goes." We scratched that Hosp/Doc right off the short list that moment.

In the end....I went to Stanford...however Cleveland Clinic would have been a very solid, competent option....however, my comfort level went with Dr Steinburg as he clearly has the utmost experience and I only get 1 shot at this!

Keep us posted....and hang in there!

Hugs....Diane

Thanks to everyone who has posted to my original message.  I have received great ideas and a lot of support from those of you who have walked this path ahead of me.  Truly, you have my most sincere thanks.

My immediate plan is to obtain my medical records and films, and personally mail them to Stanford.  First thing Monday morning I will be calling Dr. Steinberg's office to let them know to expect my info early this coming week.  I'll have to have some patience until they can sift through all the files and films and make sure they have all that they need.

Posting on the internet can become a passionate enterprise.  While realizing that a discussion about medicine and doctors can become an emotional war zone, I personally try to remember that doctors are just well educated people who provide a difficult service to their patients.  Patients are also people that sometimes forget that medicine is not an exact science.  Regular people rely on those with expertise in their fields to do their utmost within that expertise to heal us.  It's taking much too long for me to get from point A to point B, but I choose to believe that there is a reason why things are going the way they are.  After all of the waiting around, NOW is MY time to take the next step.  I intend, with my family's help, to make the phase go as quickly as possible.  It's time to be proactive for my medical needs while respecting what the physicians can do for me.  My grandma said that you catch more flies with honey than with vinegar, so I'm choosing a good, but assertive attitude from here on out.   "Wait and see" just doesn't cut it anymore!
I will keep you informed here.

Thank you again to everyone, and especially to Kim who has shared everything she could, and would share with me anything I could think of to ask.  You are a blessing.

Sheila