My 12 year old daughter was recently diagnosed with moyamoya.  She had been plagued with 'dizzy' spells, was often light-headed and would often complain about seeing flashes of light.  She experienced her first TIA about a month ago.  The neurologist at UNC performed a number of tests including an MRI and MRA which provided her diagnosis. I am not very happy with the approach that her neurologist is taking as he wants to just monitor her and take a 'wait-and-see' approach.  At her follow up appointment yesterday I inquired about when he thought we should consider surgery and his response was 'not until she has a stroke because the surgery is too risky'.  He also said that she may not progress any more than what she already has.  I am very concerned because all of this information goes against everything I have read regarding the disease. Needless to say, my husband and I are seeking a second opinion.

One major question that I have and have been unable to get an answer on is whether or not other children afflicted with this disorder have learning problems in school.  My daughter has always struggled in school, from kindergarten until now.  I have had her tested and we have done different types of therapy and tutoring in the past.  I was told when she was 6 years old that she was ADHD (although she was never 'hyper') and she has been on medication since then.  Now I am worried that perhaps the moyamoya has been the problem all along.  Her comprehension is very poor, she doesn't retain information well and she is very easily confused.  She has trouble keeping up with her homework assignments, multitasking and I have to help her with pretty much all of her work or she is unable to complete it.  I really feel that all of these issues have gotten worse, especially in the past year.  Has anyone else had children with similar issues??

Hi Ashley, Welcome to our MM family!

You are so right to be concerned about the “wait and see” approach, it is definately dangerous and risky, so go with your gut instinct. The more you learn about this rare disease, the more easily the answers will come. The facts will speak for themselves. The fact your neuro wants to wait until after a stroke is barbaric, and automatically tells you he does NOT have enough MM experience with this disease. The surgery is meant to PREVENT that stroke/death, not wait for it. This is a successfully treatable disease in most cases, if you have a MM experienced doctor. The "risk" he spoke about is more about his LACK of his knowledge of MM, rather than actual risk to the patient, and what’s important to know is, surgical management is the ONLY treatment for MMD, so MM experience is everything for success!!

MM symptoms are different for everyone, depending on where the blockage is in the brain, and to what degree. It can even have no symptoms for some, but fatally strike. It certainly can affect her learning without a doubt. She is not getting the proper blood/oxygen, nutrients to the brain, and this is a progressive disease. It only gets worse over time. Having MM with some people is just likely to cause symptoms such as TIA’s (mini strokes) fatigue, fogginess, reactions to extreme heat, headaches, tingling, confusion (Especially when doing many things at a time - easily distracted) etc.. This is all due to the body having such a hard time getting the blood around your brain, due to all the roadwork of vessels. Things move much slower. Her MM vessels grew to try and help with the blood flow, but they are ABNORMAL vessels. They are weak and fragile and can rupture at any time. That is why all MM patients are at risk for a stroke or hemorrhage. Waiting is dangerous!

Please learn all you can about this disease, and you will learn that without surgery, the majority of individuals with Moyamoya disease will experience mental decline and multiple strokes because of the progressive narrowing of arteries. Without treatment, this disease can be fatal as the result of intracerebral hemorrhage.

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 in understanding MMD as well.

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

It’s very wise to get a second opinion with a MM specialist. They deal with this disease on a daily basis and can put you on the best path for your case. A second opinion usually costs nothing but postage. Simply get copies of any films and send them, and they will review them and give you their expert opinion.

I’ll keep you and your daughter in my thoughts and prayers.

Mar

Ashley,

Welcome!!  My daughter Emmily is now 15 years old.  She was diagnosed at the age of 3 and our neurologist at the time also had the recommendation to wait and treat the disease with medication.  She ended up progressing and having to have surgery at the age of 7.  I kick myself everyday for not being more aggressive at pursuing the surgery.  She ended up having a stroke one week after her first surgery.  If we had surgery earlier she would have been stronger and the progression would have not been so bad, I honestly believe she would not be living with the disabilities that she has now.  The stroke took half of her visual field.  She is blind from the middle to the left in both eyes and it will neer return.  She had her second surgery  3 years later and since then she has continued to improve.  Dr. Scott at Children's Boston was her Neurosurgeon and he is fabulous.  he will review your daughter's case and is an excellent communicator.  It has never taken him over 24 hours to respond to an email.  I would HIGHLY recommend a second opinion.

My daugher does have learning disabilities, but she is progressing normally in school with modifications.  We just had a follow up MRI last week everything is stable, so we are optimistic that the worst is behind us. 

Good Luck!!  Please feel free to contact me if you need any more information!!

Roxanne

I think there is some confusion about the classification of Moyamoya.  There is moyamoya disease, and moyamoya syndrome.  Those with the disease are faced with progression of the narrowing and abnormal collateral growth, while those with moyamoya syndrome may only have the abnormal vessels without progression.  Dr. Scott, who cares for my daughter, has also taken the "wait and see" approach and he is considered an "expert" in this disease.  I think that each case should be investigated  thoroughly and decisions made on a case by case basis.  As to the learning disability question.  My daughter has dyslexia and visual processing issues.  There is no way to know for sure, but speculation is that there is a relationship between these problems and low perfusion.

Mar, very well put.... I think it's always a good idea to consult with an expert on moyamoya.Even the experts will acknowledge the fact that no one knows how quickly it will progress.My daughter's progressed to a very severe stroke in a matter of a couple months. She's still recovering from this 7 yrs later.The other thing that is disturbing is to have a Dr. to not jsut say "wait and see" but wait until she has a stroke. If you wait for a stroke then ultimatly you would want to then wait until the patient stabalises which could take several weeks.My daughter's strokes were getting so severe and close together we didn't have the option to allow her to recover from her strokes before surgery.This made it so much more complicated and risky. Just my 2 cents.........
Mary Grace

My heart goes out to everyone who is struggling with learning problems, dyslexia, odd and sundry memory retention, coordination, inability to carry on a task or keep employment and wondering why,  and  all the other things that can go along with mm. Actually knowing I have mm has explained much of my frustration and answered many questions.  So, I relate to much of this.

At an early age (in the 1950's) they thought I might be retarded, or have a brain tumor. I had difficulties with 'left brain' sorts of things. Still do. No wonder, I had no vascular system there.
Now, tenish yrs post stokes and surgery, I'm amazed at the things I have to re-learn. It just goes on and on. Seemingly simple things that I expect (right!) to be able to do.
I'm actually doing quite well if I'm not faced with 'issues' but, since we're on  this very valid  topic I'm remembering, and realizing how much effort things take. Empathizing.

Moyamoya is so far reaching in it's significance to our lives.

I send love to everyone.

Linda (LA)