Idiopathic Intercranial Hypertension

Grace's note:
Today's blog does not concern many folks, but for the few winners, like my daughter at age 11, Idiopathic Intercranial Hypertension is the happy answer to a severe  headache.  The reason it is a happy answer is because the alternative is the devastating diagnosis of a brain tumor.  Idiopathic Intercranial Hypertension used to be called Pseudo Tumor Cerebri, or false brain tumor. Idiopathic means "I don't know why" and intercranial means "in your head" and hypertension means too much pressure on your brain.  I am fine with "I don't know why" when the reason could have been a brain tumor.

My daughter went to the school nurse in the 6th grade complaining of a pounding headache.  She had never had a headache before.  I picked her up and gave her some ibuprofen. She rested and was fine.  The next day, the school nurse called again.   My daughter complained of a severe pounding headache.  I took her home and made an appointment with her pediatrician.

The pediatrician thought perhaps it was a migraine.  Of course my daughter looked great at the doctor's office.  That night, the head ache returned, now with a whooshing sound in her head.  I could actually hear the whooshing sound if I sat near her.  Bazaar! When she vomited, that was it.  Head ache, whooshing sound, vomiting...off to the emergency room we go for a cat scan.
It was negative and, of course, she looked cute as a bug in her hospital gown. We were sent home.  The ER docs gave us odd looks as if we were off our rocker.
We kept her home from school and watched her.  Poor sweetie could only watch TV. Could my daughter be a malingerer?  But alas, no, she started vomiting with the headache and the whooshing again.
Bazaar, Bazaar, bazaar.
My stress levels were high and I was suffering also.  Just to add to the troubles, I have this little quirk that doesn't go well with the field of nursing.  I have sympathetic vomiting.  That means every time my sweetie would vomit, I would too.  We were both wasting away, but at least I didn't have a headache.
After a few more days, and a few frantic calls to the pediatrician,  she had a MRI.  That is when it hit me.  The other children in for a MRI of the head had no hair and looked terribly ill.  The MDs were considering a brain tumor. I didn't share this little tidbit of information with my hubby or my daughter.  She was a trooper.  Again the results were negative and we were sent home with odd looks and I thought whispers of "what a nutty mom" from the MDs.
Two negative results should have made me happy, but we were home with my daughter, whooshing, complaining and vomiting and me sympathetically vomiting right along with her.  We were both wasting away.  I knew this wasn't right.  She didn't even get up to play with her friends. She just lay there crying off and on about her head.  UGH.
My wonderful pediatrician, gave us the last appointment on his half day.  Finally, my sweetie didn't look good at all.  She just lay on the floor moaned and felt nauseated.  Her hair was ratty and hadn't been washed in days.  She was a mess.
The pediatrician met us across the street at the ER and performed a lumbar puncture (a sample of spinal fluid is taken through a needle in the spine). The levels were elevated and the fluids were clear! My daughter smiled and asked for food.  Sweet relief.
Since this was in the olden days before parents Googled, I had no idea what illness this was.  Perhaps I had heard about pseudo tumor cerebri, but it was always in association with acne medicine not sweet little six grade girls.
My pediatrician had been in contact with the pediatric neurologists.  They explained that very rarely girls at puberty can have increased pressure or fluid build up around the brain due to the change in hormones.  Mostly it is a reaction to medications or to obesity.
Two years of treatment with Diamox, a diuretic (makes you pee) that also decreases inter cranial fluid and she is back to new.  Of course along the way there were episodes of blindness and decreased peripheral vision with optic nerve edema. But that is neither here nor there when the happy diagnosis of Idiopathic Intercranial Hypertension is received.
Read more and review the Diagnosis Algorithm  from the American Child Neurology Journal article by Shannon Standridge, Idiopathic Intercranial Hypertension in Children: A review and Algorithm.
Or sign up for the Registry and learn more at Intercranial Hypertension Research Foundation website.