My five year old daughter Portia has just been diagnosed with moyamoya syndrome.   Her ancestry is half (Han) Chinese (mom's side) and half caucasian.

Her presentation began about 1.5 years ago with frontal headaches at 3-4 am.  The headache would get more intense over several hours and then she would vomit suddenly, once or twice, and seem to recover.  

After visiting a pediatrician and a neurologist, and having CT scans and MRI's done, we were told that she was having "migraines."   The concern had been a brain tumor, but nothing was seen in the studies.  

At the peak, the frequency of attacks was twice a week. The frequency/severity of the "migraines" then decreased over a year, eventually reaching once a month.   About a week ago, she had a really bad headache and displayed pronounced weakness on the right side.   When a cerebral angiogram was performed, she was diagnosed with moyamoya syndrome.  The attack was evidently a stroke.  There is very little apparent impairment -- just a touch of weakness on the right side.

Her surgeon is Mark Souweidane at Weill Cornell, and we expect to operate on Thursday.

That's the story.   Any advice, comments, and suggestions are welcome.

Is there any role for pharmacological agents in moyamoya?    I noticed a promising study with a calcium channel blocker, nicardipine, for refractory childhood moyamoya.   Does anybody have experience with ergoloid mesylates (e.g. Hydergine, Nicergoline) or vinpocetine -- reputed to improve cerebral circulation and provide neuroprotection?   CDP-choline?

Thanks.

I have no advice to you, as we're just about in the same spot in the process as your family. Except that my daughter was daignosed 11 years ago at the age 4 months. She didn't start having TIA's until age 7, and then they double in intensity and frequency about 6 weeks ago. Now we've found she has a narrowing on both sides.

She's having a full-spine MRI tonight, tomorrow we go to Mayo to meet with the Neurosurgeon and Neurologist, then Friday have a SPECT and Angiogram. My daughter also has Down Syndrome, and the doctor at Mayo has never done the procedure on a child with DS before. So, if/when it's determined she needs surgery we'll be headed to Boston or Stanford.

I will be praying for your family on Thursday.  

Thank you.  I hope the tests go okay.  You all will be in my prayers.

dsg2402 wrote on May 8^th, 2007 at 2:53pm:

Is there any role for pharmacological agents in moyamoya?

I haven't had any experience with what you spoke of. In any of my research, I've only found that certain medications play an important role in treatment, but NO medications prevent the arterial narrowing process from progressing or keeps the moyamoya vessels from developing, and that’s why all MM patients are at risk of a stroke without surgery. The MM vessels (collaterals) form to compensate for the lack of blood flow through the carotid arteries, which is good. However, those collaterals are fragile and weak, and simply not equipped to deliver the volume of blood needed over a long period of time. There lies the risk of having a stroke or hemorrhage, with or without pharmacological agents.

Thank God your daughter didn’t have a significant stroke. With the proper surgical approach her outlook is excellent. Please know your daughter will be in my thoughts and prayers and we’ll be right there with her in spirit on Thursday.

Please keep us posted

Mar

My 10 year old was diagnoised with migranes at first as well....4 years later he was diagnoised with moyamoya .......he is now six months post surgery and doing very well................he is on a baby asprin aday, and on "coq-10" for occasional headaches......coq is a over the counter medicine however we hace it made at a pharamcy to ensure that the dose is right ...zero side effects are a huge bennefit fot us as he did not tolerate the traditional headaches and seizure meds well........best of luck too you and if I can help in any way contact me

Mike

It seems clear that surgery is absolutely necessary.
After the surgery is done, I'll be interested in what else, if anything, can be done to help protect Portia going forward.   I sure hope the surgery suffices.

Your reference to Coenzyme Q10 interests me.  (I take a couple of hundred mgs of Q10 myself in the hope of general health/anti-aging benefits. )   Did the neurologist propose Q10?   How helpful has it been with the headaches?

If Q10 is useful in moyamoya, perhaps I will ask about its chemical cousin Idebenone.  

Has anyone used Idebenone here?    (Idebenone is basically Q10 with a shortened carbon side-chain ... it is purported to be a more effective antioxidant and neuroprotective agent.)  

I'm not qualified to judge these matters, but I will ask the neurologists about Q10, idebenone, etc.    

Thanks for your input and your wishes for Portia's recovery.

yes Roberts neuro prescribed and got us the pharmacy that makes it, Also because it is prescribe insurance covers it. As far as the benefits, presurgery it reduced his headache frequency by about half, also it seemed to reduce their severity when he did get them. post surgery we keep him off it for the first 3 months to see how he did with out meds, he is back on it about 2 months and hasnt complained of any headaches other then his normal spring sinus headaches................It works for him but as mar is found of saying I am not a doctor, check with yours.

Update on Portia ... thanks for your concern ...

She had her operation at Cornell on Friday morning, from 830am to 230pm.   It was a bilateral syn-angiosis, done by Mark Souweidane.  Because of the several delays before the operation, I had time to review matters with the pediatric neurosurgery team at NYU ... they concurred with Mark's game plan.  So I ended up staying with Mark.

Following the operation, one sees 5-6 inch vertical incisions on the side of her head, where a thin strip of hair was removed.   There was some swelling, apparently expected.

She has had some problems in post-op recovery:  running a high fever, requiring a transfusion, and having her classic ischemic attack (frontal headache and vomiting).   Otherwise, she seems pretty stable and lucid, when fully awake.

Hi... I read your post earlier, but did not have a chance to write.  I'm glad to hear Portia has gotten through her surgery.  The incisions you describe and swelling are all normal, as you probably were aware.  I am sorry that she is having some problems post operatively.  It is not uncommon for things to not go quite as smoothly as hoped.  I hope things improve for Portia soon.   She is in my thoughts!

Jenny (mom to Daphne www.daphnestory.com)

Sening LOVE to Portia and all of her family~

My support is with you.

I had migraines in the 1950s when I was a child.
Now I suspect that they were related to my mm.

Glad the surgery is over and the road (altho a bit bumpy) to better health can begin.

Love and strength to you,

Linda A (LA)

Thank you for reaching out with words of encouragement and support.

Portia went home from the hospital, and has been recovering nicely.   Aspirin seems to help - presumably through anti-coagulant effects - but it's hard to tell.   She seems to respond better to 162mg than to 81mg.  So far so good.

The surgery will hopefully be having an impact over coming weeks as blood vessels grow in.

I'm happy that Portia seems to be herself again.  Kids, thankfully, are pretty resilient.

Portia sounds like an amazing little girl!!  

Our daughter, Jennifer,  had surgery in Stanford last august.  she is doing so well.  It's such a hard decision, but you made the right one.

Portia and her family are in my prayers.

Michelle (mom to Jennifer www.myjenniferrose.com)