Hi everyone, I'm Julie, mom to Nick, who is 16.

When Nick was 12 or so, he started having these episodes of losing his vision.  He'd all of a sudden go completely blind, anywhere from 10 seconds to 2 minutes.  He's had insulin-dependent diabetes since he was 9.  

We started in opthamology.  Clean bill of health, eyes are perfect.  Got referred to neuro.  Went through the whole thing, EEG, CT scan, MRI w/ MRA, buckets of bloodwork.  At that time we found out that the right middle cerebral artery was "narrow" but being the first time anyone had ever looked at it, we didn't know if that was a change for him or what.  We also had an idea that his blood was prone to clot, although his bloodwork was never consistent, it always was on the slightly clotty side, so we put him on baby aspirin.  He was referred to a stroke specialist around that time, and started on verapamil.  All the doctors at that point felt that the next step would be a cerebral arteriogram, but he stabilized really well on the verapamil/aspirin combo and then no one wanted to do something invasive if it wasn't necessary...so they decided to repeat the MRI/MRA in six months to see if there were changes and then make a decision on the other test.

Mind you, all that took a couple of years.  It was complicated by his diabetes.  We kept a detailed diary for the longest time, and could never connect the vision episodes to anything - food, exercise, blood sugars, blood pressure, etc.  It seemed to happen slightly more with activity, but it was nothing close to definitive.

So, in the 6-month waiting period to repeat the MRI, he turned into a cranky teen and ended up going to live with his father out of state.  I sent him with detailed medical records, things that needed to be done and followed up on...every time I spoke with my ex they were on their way to or from a doctor's appt.  I thought all was well.  Not so.  They got into a brawl and Nick ended up back home with me...the things he wasn't telling me while he was gone!  Anyhow, the point being, he didn't see a neuro during the two years he was away, nor was he able to continue on his meds.  He came home on his insulins and Paxil.

That was 6 months ago.  At the very beginning, I talked to him about going back on the aspirin, but he was resistant to that idea.  He said he was only going blind once a month or so, for just a few seconds, and he didn't think it was necessary.  Since I had a very angry and hurting teenage boy on my hands I decided that time would likely take care of that for me and I wouldn't push it.

Fast forward to two weeks ago.  He called from school, having gone blind several times.  I went to get him.  Gave him baby aspirin.  Called the pediatrician who refused to see us and sent us to opthamology.  Naturally, his eyes remain perfect, so they send us to the ER for a neuro consult.  By this time, he's gone blind 13 times, but only once since he had the aspirin.  Now he's just got a killer headache.  We ended up with a lousy neuro resident who booted me out to talk to him, then never spoke to me; it was decided that we could just follow up with neuro and didn't need any sort of imaging at that time.  I really believe that Nick came across to her as a noncompliant kid (he was pretty irritable at that point).

We went home and continued with the baby aspirin.  Our neuro appt was a month out.  He did very well, with only a few scattered episodes in the last two weeks.  Then he started having them two to three times a day for three days in a row, and then the headache came back.  We've managed to bump up the neuro appt to this Friday, but ended up in the ER again last night.  He did get an MRI/MRA.  I didn't get to see it, but the doc said that he did indeed have small arteries on the right, he specifically mentioned the Circle of Willis area, and said that the arteries on the left were too large, in compensation for the smaller ones.  

No clots, no bleeds.  Thank God.

Our appt is Friday, at Children's in Boston.  It's not with Dr. Scott, but it should be easy enough to switch doctors if the need arises.  I'm expecting at this time we will need to do the arteriogram.  What else should I expect?  Ask them?  He's been in bed with this headache since Monday afternoon, Motrin at home won't touch it and the Fioricet (sp?) they gave him in the ER didn't work either.

Thanks for muddling your way through, I'm sorry it was so long!

edited to add: My maternal grandmother had an arterio-venous malformation and died from a massive brain hemmorhage.  I'm doubting that's what's wrong with Nick, since they should have seen that from the first MRI/MRA (right?).  She and my paternal grandfather both had epilepsy.  That's our only significant neuro history.

Julie,
Sorry about your son's health problems. The arteriogram I believe is the same as an angiogram in which case it is considered necessary for a definitive diagnosis of Moya Moya Disease.  You may want to ask if the results show collateral vessals which appear as a "puff of smoke" on the films. This is a distinct feature of Moya Moya. You may want to use this site's resources to find a Moya Moya specialist and send all your results to that person.  I don't know Dr. Scott but he has been highly recommended on this site. You may also ask if there is bi-lateral narrowing which would mean both sides of the brain may be affected.
I wish you and your son all the best.

Russ

Hi Julie,
I thought of something else you may want to inquire about which is blood flow testing. These tests indicate if there is a reduction of blood flow to the brain which is thought to be responsible for the hemmoraging and TIAs associated with Moya Moya. This sounds counter-intuitive that less blood  causes bleeding but the brain compensates by forming collateral vessels which are weak and prone to bleeding.  I had Xenon CT scans but some reports say this is not a commonly accepted method. If you search this site some other terms related to blow flow studies are CT perfusion, CT scan with contrast, MRI/MRA with contrast. Hope all goes well for you and your son.

Russ

Hi Julie -

Well, I had similar symptoms with my vision - but never going completely blind.  What I was told was optic migranes - usually accompanied by blurred/fuzzy vision and headache - I think of more as visual TIA's...that's how I felt anyway!  (My disclaimer - I'm not a doctor....!).

Anyway, I was officially diagnosed with MM in 2003...had my surgeries with Dr. Scott at Boston Childrens.  I believe that MM is very well known at this hospital and Dr. Scott will most likely be consulted at some point in Nick's care there.  I believe they will also give Nick an angio...

Anyway, after my surgeries, I told my eye doc all about my "visual TIA's" and that I had 3 strokes and brain surgery.  For a while, he would give me a field-of-vision test.  I believe that would test peripheral vision and any nerve damage.  Turns out I had slight nerve damage from my strokes.  

Just my experiences....

Good luck!

-Shari