>What about any learning or behavioral delays?
>Any craniofacial deformities anywhere?
All of the above for my daughter. But they're far more related to her primary diagnosis of partial trisomy 6p, then to the Moya Moya. Philina, it seems, is totally missing the main vessel that should transport the blood through the center of her brain, and she has stenosis of the supply lines through her neck too. She was diagnosed with Moya Moya at 19 yrs of age, during a 3 1/2 month hospital ordeal of multiple complications, post aortic valve transplant surgery.
Prior to that time, she had had 2, maybe even 3 small strokes. But the temporary paralysis had spontaneously corrected itself so fast (within only hours in fact), that I couldn't get any doctor to acknowledge that they really were even strokes. Initial diagnosis, as an infant, was "probable Herb's Palsy", and I was instructed to just watch her for seizure activity. But this girl has never had a seizure in her life!
Next known episode was at age 8, and on the opposite side of her body. But at least this time the intake nurse in the ER got to see the paralysis, which had of course again disappeared before the doctor ever got around to seeing her. Referred to a neuro this time, but EEG was fine, and MRI showed only some mild damage in her balance center. Sort of a none event finding though since she was already roller skating.
Next suspected episode around age 12. No one actually saw this one, but she got off the school bus with a dark ring around her right eye. Though there also had been a mini riot on the special ed bus that day, so we just naturally assumed someone had hit her in the melee. But 10 yrs later, she's still got most of the discoloration around that eye, and some decreased muscle tone on that side of her face too.
And her last "episode", after the heart surgery, that resulted in her diagnosis, I'm not even really sure was Moya Moya related. Here's the deal. She had so many complications, and bad drug reactions after that surgery, that she ended up in an intentional drug induced coma for 7 weeks, on a heavy duty narcotic pain killer. So when they finally woke her up, she had to be detoxed with Methadone too. Poor kid was shaking pretty bad with DT's. But neuro consult put her on massive doses of Phenobarbital "for seizure activity", on top of everything else. Took me weeks to find a doctor who would even listen to me about her not needing the Phenobarb.
Meanwhile the doctors were trying to figure out why she was unable to even smile or lift her head off the pillow. First a repeat on the MRI, which showed the balance center problem was still there, but that was about it. Followed up by an MRA, and a diagnosis finally that made some sense, and a feeling of real vidication for mom too.
Open minded dr mentioned above, turned out to be the director the hospital's EEG lab. So after about 15 minutes of looking through her old EEG reports, Dr Nespeca wrote up the order to start weaning her off of it. She walked out of that hospital 3 weeks later, but still on fairly large doses of it, and a bit unsteady on her feet. Once weaned all the way off though, she made a total recovery. So who could really even tell if she'd had another Moya Moya event?
Unfortunately, she's too old to be seen by him as an out patient, and the only neuro in his office who will see her is virtually worthless. She doesn't even acknowledge the diagnosis as a fact, just as suspected. Of course when I asked her for the date of the MRA, to order the report, she gave me the date for the MRI instead. But I'd still rather keep Philina there, where I know there's at least someone in the office I trust, if we ever run into a real problem again.
Besides, her ENT not only got me the date for the MRA, he just went ahead and made me a photocopy of both reports. Current neuro also has no objection to my giving Philina an aspirin daily, and neither does the cardiologist, so that's what we've been doing. (Actually, that was recommended by a neuro at Hope Children's in Chicago during the '02 Support Organization for Families of Trisomy Conference's medical clinics...but whatever works.) Since her episodes have always been so few and far between though, it's really hard to tell if it's been any help or not.
Fawna
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