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My 11 y.o. old daughter had intractable headaches for 2 weeks so I got her in with our ophthalmologist. He found that she had grade two papilledema and a small bleed in the back of her right eye. He got her in with a neuro ophthalmologist THAT DAY. In addition to the papilledema she had enlarged blind spots. My daughter is not overweight and prepubescent, which is of course a rare presentation for PTC. The neuro opthal was okay with waiting on an LP and trying a month of Diamox first. I did TRY to explain that she (and I and several of my kids) have EDS and that I also was Dx with PTC at 14 (though I never had an eye exam – the Dx was based on imaging) that was never treated.
[In more recent years I have not had papilledema, but do have drusen in the macula, floaters, double vision in distance even with my glasses, etc. I also was on Diamox for almost 3 years (thanks to the information here Dr. Diana!) and it helped a lot with the head and neck pain. Sadly though, my kidney’s filled up with dozens of stones and after extensive and very painful surgery and recovery, I had to stop taking it. Incidentally, my neck and head pain went through the roof. My neuro ended up having me try phenobarbital which keeps most of the headache pain and migraines away, but my cervical spine got so much worse. I was losing so much function and the pain was unreal. I went to see Dr. Henderson for a consultation and he found not only that I needed C4-6 ACDF but also that my skull is sliding on C1 to the extent of being surgical. I have had the ACDF but am not jumping into doing anymore surgery at this time.]
The neuro opthal put my daughter on Diamox XR (argh) and basically told me it is never necessary to check blood work while on Diamox. ARGH. He started her at 500 mg BID. He also ordered an MRI. MRI showed flattening of the backs of her eyes, but other wise was normal.I do think she has low lying tonsils just like I do on imaging and her sella is half empty, which wasn’t commented on in the report.
A month later when he rechecked nothing had changed. Still had the papilledema, and enlarged blind spots. So he doubled her Diamox dose and wanted an LP to be sure something else wasn’t going on.
The LP was a nightmare leaving her with a horrendous spinal headache for 5 days. Her opening pressure was normal as were all the labs done on the csf.
We were back again for another recheck this week and although her blind spots are now normal in size, the papilledema is not improved. So now the doctor wants an orbital ultrasound done to be sure there are not drusen in the optic nerves. Though he doubts that is what is going on as that would make the optic nerves look “lumpy” and hers are not. But it is another one of those things to check to be sure we aren’t missing something.
I am frustrated with this doctor because he won’t do labs on her and how he dismisses my concerns about the kidney stone issue (also I developer low K about 9 months into taking Diamox which he says has been proven to not be caused by Diamox in a recent study – argh). He is also one of very few neuro ophthalmologist in our area.
I’m thinking about going to our regular pediatrician and ask for the labs and possibly a referral to another neuro ophthalmologist.
Any thoughts Dr. Diana? She is so young and it is breaking my heart to have her in pain. It’s hard enough with my bad health keeping me down much of the time, let alone trying to get her to appts and tests. Her cognitive abilities seem to be effected by the Diamox (or is it the high pressure?) also. Her short term memory is getting worse and worse and her ability to do things she did a few months ago (such as reading and math) are getting very hard for her.
Hi OFH,
Oh the poor sweet thing, she’s really being put through the mill isn’t she, my heart goes out to her and to you. What did the MRI show regarding her cerebellar tonsils ? because low-lying or herniated cerebellar tonsils can cause high intra-cranial pressure to be missed on a Lumbar Puncture (LP) if they are bunging up the hole.I think any level of empty sella, in the absence of a cyst or the like, is indicative of raised intra-cranial pressure, as is the papilledema and the flattened backs of eyes, so why on earth was the lumbar puncture necessary – oh probably to test the CSF for infection or whatever.
I can’t overstress the importance of correct spinal (especially cervical) alignment in these circumstances, try and encourage her to sit straight with head balanced well over shoulders and place cushions for support if she finds this hard, as many with EDS do. A soft horseshoe shaped cushion to support her neck might be beneficial too and it can stop the back of the head from being pushed forwards, all these things help. Has she had an injury doing sports or anything ? What was the alignment of her vertebrae like on the scan, do you have a copy ?
Regards
Barbara.Barbara,
No injury. Yes,I think she has low tonsils, just like I do. The MRI doesn’t show much of her cervical vertebra as they were looking higher up mostly. I’ve attached two of the best side views. Let me know what you think. Thanks!Hi OFH,
I have just had a quick look at the MRI’s see them annotated below:-The second pic is the para para-sagittal view, which usually has a better chance at trapping cerebellar tonsillar herniation and your daughters show that her tonsils are low lying and protrude through the foramen magnum, see the red line.
The first pic is the mid sagittal view which, if we start at the top shows:-
Blue arrow – a possible narrowing/flattening of the corpus callosum
Green arrow – a flattening of the top of the pituitary gland (partially empty sella)
Purple arrow – encroachment of the posterior arch of C1 into the spinal canal
Red arrow – a retroflex odontoid
Yellow lines – poor alignment (loss of lordosis) of the upper neck vertebrae
Orange ‘V’ shape – The medical sign I see is the ‘V’ sign – between the front of C2 and the back of the anterior arch of C1. Usually these 2 anatomical parts are quite parallel. This type of instability, between C1 and C2, is called atlanto-axial instability, it could be a major player, along with the low-lying cerebellar tonsils, in your daughters headaches and raised intracranial pressure issues.If it was my daughter, I’d be getting her into a Philadelphia collar (with linings!) as soon as possible, to see if it helps alleviate any of her symptoms.
Here’s another post on atlanto-axial instability
http://prettyill.com/forums/viewthread/897/#4194
Regards
Barbara.
(UK)Thank you so very much Barbara! These are the things I thought I was seeing also. Which looks a lot like my own MRI’s.
I think I am going to have to start with getting her pedi to order labs to prove to the neuro ophthalmologist if she is too acidic for the diamox to work. Then ask him to change her Rx to the non extended release form. Then recheck her optic nerves after those changes and see if that doesn’t bring the swelling down. After that, go from there, with maybe having to try to find a neuro that works with kids that will actually believe those other things going on in her brain are at least partially to blame for the high pressure.
The neuro ophthalmologist she is seeing only goes by what he can or has read in studies. I wish there was something I could print and give him that would prove to him about the extended release not being as good and that acidity works against diamox working. When I tried to talk to him about how my potassium dropped off, he adamantly denied that this is due to diamox, stating that there was a recently concluded study that followed first time diamox users for 6 months and their potassium never changed. He said a lot of people/doctors have believed that potassium goes down with diamox use and that is why the study was done. So now that this study disproves it, labs don’t need to be done to monitor a patients potassium, etc. I told him my potassium didn’t drop till 9 months after starting taking diamox and I had to take supplements daily because of it. All he would say is “hmm”. A six month study is just not long enough to prove or disprove this. Argh.
No probs.
Hey I agree with the reduced potassium level thing, I’d been on my Diamox over a year before mine went below normal, so 6 months is certainly too short a study. Dr Diana doesn’t recommend the slow release Diamox either.Have you thought about getting her into a Philadelphia collar (with linings), she’ll hate it I know to start with, as it feels so cumbersome and you have to clean it every day (especially in the hot weather) but when she realises the difference it makes to her symptoms, she’ll start to appreciate it. It’ll help train her own muscles and ligaments to hold her neck and head in a better position. Even if she just wore it intermittently to start with, she may see a difference.
Barbara,
Really don’t want to go the way of the collar quite yet. She isn’t very symptomatic, except for headaches, right now. If that were to change, definitely would try it.Today they did an orbital ultrasound to see if there were drusen in her optic nerves. And, of course, there are not. It’s painful to go through all these elimination steps. But with this out of the way, I’m going to call the neuro ophthalmologist office tomorrow and ask for the Rx to be changed to the regular Diamox and to order a metabolic panel. If he won’t do the blood work, I’ll have to make an appt with her pedi and ask to get the labs done that way.
Any thoughts on what dose of regular Diamox would be vs the ER type? I know it should be dosed 3 times a day instead of the twice a day she is on now. She is on such a high dose of the ER (1000mg BID) I can’t imagine needing that much in regular Diamox, based on my own previous use of it (which I think was 125, 125 and 250 – though I had to take bicarb to keep it working). Pretty much for me I could tell it was working if I got at least a slight tingle in my hands or feet each day and if I didn’t I could pretty much figure it was time for more bicarb. And so, I’ve been really wondering about how effective the ER my daughter is taking is because she has only had a slight tingle maybe twice in the two months since she started taking it. I know everyone is different but since we are related and so similar in health issues I would think she would have more tingle from taking it if it was working well.
Ok, I understand – but keep a check that she holds her head up whenever possible, supported with cushions if necessary (watch if she slumps down though because the cushions will push the back of her head forwards and this must be avoided at all costs). Constant correction will help train her muscles, so eventually she won’t need to think about it and hopefully delay the onset of further symptoms and disk problems. Sorry, you probably know all this already but I’m writing it for those reading this post who are just starting down this ‘journey’ and probably don’t know.
Regarding the Diamox, if I remember rightly, Dr B (then from TCI) recommended I take 250mg x 3 times a day but, I have to admit, I found 125mg x 3 times a day, a better dose for me, the higher dose was too much. We all however, as you know, react differently to drugs, many of us not requiring as much as the recommended dose, so you just have to ‘trial and error’ it until you find the best results.
Regards
Barbara
(UK)
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