I know that MM is suppose to be rare, but my mother at age 28 was diagnosed in the mid 80's. In the late 90's two of her sister's were diagnosed as well, one being in her late 30's and the other in her mid 40's. Both of them were tested back in the 80's and came back negative. I have been tested twice with negative results. I am concerned though, seems to be adult onset in my family. I have been unable to get an answer as to whether I should be rechecked every so often. I am 35 now and right in the age zone of my aunts.

I would call a doctor who knows about moyamoya and see what they suggest, I think you should probably if it makes you feel better about knowing whats going on, ask to get a angio or ct angio. every year. thats what I would do, if family had it, and wanted to be sure i did not have it, I am going to get my kids checked, even if they have no signs, because i have it

In my personal experience, I had an untrasound of the carotid (my neck) which of course showed nothing.  That isn't where the blockage is!  I then had 3 MRI's ordered by 3 different doctors at 3 different facilities.  That aslo showed nothing but the stroke.  I was very insistent that I "just knew" there was something the Dr.'s were missing.  My doctors as well as much of my family just acted like I was a hypochondriac, withthe exception of my primary doctor, who never gave up on me!  Finally the MRA was ordered by the nuero.  His attitude was almost like if I wasn't gonna quit complaining then he would keep ordering expensive tests until I did.  When I finally had an MRA, the blockage was VERY evident (black and white to be exact).  On the MRA, one side of my brain was completely black, indicating no blood flow past the carotid.  The other side is not quite as bad, with some blood flow evident.  After INSISTING their must be a blockage on the other side too, because the TIA's are affecting both sides, the nuero ordered another MRA (I think he just didn't want to admit that he missed the blockage shown on the first MRA) and diagnosed billateral moyamoya.  Every time I went back to my nuero he had a new group of students with him.  He would ask them the same question evertime (with a smirk), "Have you ever seen moyamoya?" Then he would pull up the pictures and watch their faces with a grin.  The students would always respond the same way..."Where is it?" as they gazed at the blackness that should be my brain.  If you have been around medical professionals enough, you learn that when they say something is "quite remarkable", that is NOT a good thing.  They are saying, you have this bad! Of course I left every appointment in tears because this is the same nuero that doesn't believe in the surgery, and refuses to order an angio to fully see what is going on.  The last time I saw him, I asked him about the surgery and the angio, and he jut explained away all my concerns and desire for surgery.  He acted like I was crazy for wanting brain surgery.  All of this happened in front of a group of students.  Unfortunately, that is the next generation of nueros that will probably treat their patients the same way.  On a side note, I am actively "nuero shopping!"  LOL 
I know Stanford is conducting some genetic research, and you can probably participate, but you don't get any results from the genetic testing.  It is only for their research purposes.  I don't know if any genetic markers for moyamoya have been positively identified, or if you could get tested.  I do know the MRI's which didn't show anything were about $5,000 a piece and the MRA's were about $7,000.  For most, that is a pretty expensive "precautionary" test.  I saw somewhere on here that an angio was $45,000.  That is just insane, and anyone with "half a brain" can see that....LOL  (Unless it was a typo!)    
How unfortunate that we have all this technology for medical purposes and then deny people the benefits of it because of finances.

Hi,
I'm so very sorry to hear that your young niece is having troubles. The fact that her numbness is occurring on only one side of her body has probably clued her doctor's into the possibility of her having TIAs or strokes. Unfortunately the CAT scan typically will show bleeding in a brain artery (a hemorrhagic stroke), but doesn't pick up on an ischemic stroke caused by a temporary blockage in a brain artery. The MRI/MRA does a better job of showing that.

Young children with Moyamoya Disease are most likely to experience ischemic stroke as opposed to hemorrhagic stroke.

When I dashed to the local hospital after experiencing temporary periods of numbness on my left side, a CAT scan was performed which showed nothing, though an MRI/MRA, a week later, showed I'd had a couple of small strokes.

Hospitals tend to do the CAT scan first as it is quick, less expensive and doesn't require as specialized a technician to administer. The MRA also requires an injection of dye to which a small number of people have an allergic reaction. Because the CAT scan means exposure to radiation and is a less effective diagnostic tool, I personally insist on an MRI/MRA instead of a CAT scan.

I'm sure there must be many causes other than Moyamoya for what your niece is experiencing. But, if her doctors haven't yet suggested Moyamoya as a possible cause, than I think it would be a good idea to ask them to consider it.

Most doctor's have absolutely no knowledge of Moyamoya disease, so you might suggest that your brother send her MRI/MRA and any other images to Dr. Steinberg at Stanford Medical Center and Moyamoya Clinc. He'll review them and give is recommendation at no expense.

Please, tell you brother not to wait! If she does have Moyamoya Disease, than she could be at risk of a stroke, possibly a devastating one.

I pray that your niece will be fine and return to health quickly.

Kim

    You sound very similar to my wife.  She was having similar symptoms, headaches tingling and numbness.  She never had a stroke or tia's to out knowledge.  She was diagnosed in sept 08. We were told to go on plavix and monitor it.  We read more about mm and how it is progressive and the progression rates vary from person to person.  We chose to get a second opinion from Dr. Scott in Boston in oct.  He said that the only thing to do was surgery. She was scheduled for surgery the end of dec.  She had her second surgery a week ago and is doing great.  We chose to see someone that was a mm specialist because we found that it was such a rare disease that not many neurosurgeons wanted to touch it.  In fact we are from philly and none here was performing surgeries.  Dr. Scott operated on his first mm patient in 1982.  He has a ton of experience.  I would recomend you see someone with that kind of experience.

Terry

Suni,  where do you live?  We are in Florida and I think Dr. Lewis at Shands will also look at your films and offer an opinion.  Destin had her surgery the same day you got your diagnosis!  She also did not have a stroke and we chose not to wait for one.  She only had the right side done as the left is in pretty good shape...we will just have to watch and see if the MM progresses on that side.  I would definitely consider the surgery...meds alone don't prevent the strokes.

I can't say for sure...I think he will.  I know when I called his office to set up an appointment they got right back to me.  I got an appointment right away since we live so close so I can't remember if I sent him Destin's films or just took them with me.  Here is the number to his office.  Shana is his office assistant and she's very helpful.  I would just call and ask her.    1-352-273-9000

Thank you everyone for your response and information.  It really does help to know that I'm not alone in this.  Time is definately not on my side so I want to get moving.  I spoke to Dr. Steinberg's Office yesterday and will be sending my films for his review and opinion.  I spoke to Jill in his Office and she is an angel.  Dr. Steinberg will review and give his opinion at no cost in case any one was wondering.  I do have an appt. with my neuro on the 23rd and I have a list of questions and concerns, even more so now that you've all been so helpful.  I can't tell if the tension from my head is from the stress or the MM.  Does anyone else hear a whooshing sound in there ears ?  I'm wondering if that's a MM thing?

Thanks again,

Suni
Long Beach, CA

Hi Suni,

I’m so glad to hear you’re getting an expert opinion. You’ll find Stanford to be the best there is, IMO, and you’ll confidently know the best path to take for your particular case. It’s so very wise to get a second opinion. It saves lives.

esyou wrote on Jan 13^th, 2009 at 7:11pm:

In regard to your question about the swooshing noise, it’s been discussed here many times over the years. Here are some of the threads and posts referring to it:

Swooshing soundsMay 26th, 2005, 1:45pm
http://www.moyamoya.com/cgi-bin/yabb2/YaBB.pl?num=1117128826

Heart racing in my head !!!
Mar 2nd, 2006, 1:59pm
http://www.moyamoya.com/cgi-bin/yabb2/YaBB.pl?num=1141325957

continuation"THUMP NOISES"Feb 3rd, 2006, 3:21pm
http://www.moyamoya.com/cgi-bin/yabb2/YaBB.pl?num=1138998083

Please keep us posted…

Keeping you in my thoughts and prayers.

Mar

Hi Suni,
Me too with the whooshing. I heard it a lot at night before falling to sleep. Then, after surgery it went away. Now I hear it sometimes at night, but seldom and not nearly as loudly. When I saw Dr. Steinberg during my six-month checkup, he wondered if I was able to hear the blood whooshing through my grafts. As when he put the doppler up to them you could hear the pulse of the blood flow all the way across the room. So, I don't know, maybe the whooshing isn't neccesarily be a bad thing.
Kim

Hi Suni,
Best of luck with your nero visit on Friday.
Kim

Hello everyone,  My sister (juls 38) was told she has MoyaMoya Arthropathy and this is a rare type of MayoMayo.  Are there different questions for the type of MayoMayo?  My uderstanding is if you have this disease that surgery is the only option.  I gave my sister all the questions to ask but the doctors are telling her this is a special kind of MayoMayo.  They have her on steriods to "bring down the swelling" but no surgery talk yet.  And the doctors said its not a question of if she will have another stroke but when.  So I guess what I am asking is there something I'm missing?

Thanks so much

I have never heard of this before, so a quick google search resulted in this definition.
Arthropathy is a blanket term which is used to refer to diseases of the joints. There are a number of different forms of arthropathy with a variety of causes which require different approaches to treatment. Arthropathy can be quite painful, especially in some cases, and it usually requires treatment to inhibit further degeneration of the joint. Staying active and eating a balanced diet can help to reduce the risk of arthropathy, along with other illnesses.

The symptoms of arthropathy vary, depending on the root cause. In many cases, patients feel pain around their joints, and the joints may also feel somewhat stiff. In the case of neuropathic arthropathy, however, these symptoms are reversed, with patients experiencing a loss of sensation around the joint due to nerve damage. People with certain illnesses and over a certain age are at an increased risk of diseased joints, and their doctors will usually keep an eye on their joint health for this very reason, checking for signs of arthropathy regularly.

Another common form of arthropathy is crystal arthropathy, which involves the deposition of crystals into the joint. This form is associated with gout, a painful condition in which crystals of uric acid accumulate in certain joints of the patient. Patients with diabetes are also at risk of associated arthropathy, as are patients with irritable bowel disease.

In addition to being caused by an ongoing medical problem, arthropathy can also be the result of an infection or inflammation of the joint, or latent infection somewhere in the body. This is known as reactive arthropathy, and like all infections, it requires treatment to ensure that it does not spread. Reactive arthropathy can be caused by a malfunction of the immune system as it responds to an infection elsewhere in the body, and an arthropathy with no known cause may be a signal of an infection which requires attention.

In another form of arthropathy, facet arthropathy, back pain is caused as the facet joints of the back experience degeneration and inflammation. This condition can be extremely uncomfortable, often requiring a period of rest and the use of several medications to try to bring the inflammation and associated pain down. In extreme cases, this form of arthropathy may require surgical treatment.