or subtitled: Why should a 20 year old still be dealing with this for the last 3+ years???
Information from the website Mayoclinic.com is in italics. My comments are in bold.
Pseudotumor cerebri occurs when the pressure inside your skull (intracranial pressure) increases for no obvious reason. Symptoms mimic those of a brain tumor, but no tumor is present. Pseudotumor cerebri can occur in children and adults, but it’s most common in obese women of childbearing age.
While Abi is of childbearing year (though she better not be bearing any children any time soon) she is definitely NOT obese and has never been in the 3+ years we’ve been dealing with this.
When no underlying cause for the increased intracranial pressure can be discovered, pseudotumor cerebri may also be called idiopathic intracranial hypertension.
That is my favorite thing to call it (I.I.H) because it is big words and people look at you like you really know what you are talking about. In this case, sadly we do.
The increased intracranial pressure associated with pseudotumor cerebri can cause swelling of the optic nerve and result in vision loss. Medications often can reduce this pressure, but in some cases, surgery is necessary.
There has not been any loss of vision for Abi, but the medication is not working anymore….. other options will need to be explored.
Pseudotumor cerebri signs and symptoms may include:
- Moderate to severe headaches that may originate behind your eyes, wake you from sleep and worsen with eye movement
- Ringing in the ears that pulses in time with your heartbeat
- Nausea, vomiting or dizziness
- Blurred or dimmed vision
- Brief episodes of blindness, lasting only a few seconds and affecting one or both eyes (visual obscurations)
- Difficulty seeing to the side
- Double vision (diplopia)
- Seeing light flashes (photopsia)
- Neck, shoulder or back pain
Abi mainly has headaches that worsen through the day and when she is overly tired (it was a really, really bad idea for her to council night camp a couple weeks ago) or stressed. The medicine (diuretic) coupled with No Doze (high doses of caffeine) have usually worked to keep the headaches at bay. Funny that she would take No Doze in order to go to sleep!
The exact cause of pseudotumor cerebri in most individuals is unknown, but it may be linked to an excess amount of cerebrospinal fluid within the bony confines of your skull.
This was confirmed 3 years ago when she had a spinal tap to check the pressure of the fluid in her spine. It was up. They “drained a pint” and the headaches stopped for a time.
Your brain and spinal cord are surrounded by cerebrospinal fluid, which acts like a cushion to protect these vital tissues from injury. This fluid is produced in the brain and eventually is absorbed into the bloodstream. The increased intracranial pressure of pseudotumor cerebri may be a result of a problem in this absorption process.
In general, your intracranial pressure increases when the contents of your skull exceed its capacity. For example, a brain tumor typically increases your intracranial pressure because there’s no room for it. The same thing happens if your brain swells or if you have too much cerebrospinal fluid.
Recent evidence indicates that the majority of people with pseudotumor cerebri have a narrowing (stenosis) in two large sinuses in the brain (transverse sinuses), but it’s not clear whether the narrowing is a cause or effect of the condition.
No other cause has ever been given to us. She has had a couple MRI’s and they indicated nothing unusual, or nothing that seemed to point to a cause. Thus the whole “ideopathic” part of the name: unknown cause.
The following factors have been associated with pseudotumor cerebri:
Pseudotumor cerebri occurs in about 1 person per 100,000 in the general public. Obese women under the age of 44 are nearly 20 times more likely to develop the disorder.
As I already said, Abi is not obese, though she is under 44. Good to know that she is that special: 1 in 100,000. We always knew she was special… just not a fan of this kind of special.
Substances that have been linked to pseudotumor cerebri include: Ummm… nope, nope, nope, nope… too much milk and cheese? Maybe.
- Growth hormone
- Oral contraceptives
- Discontinuation of steroids
- Excess vitamin A
The following conditions and diseases have been linked to pseudotumor cerebri: Nope to all of these! Never had her parathryoid glands checked though.
- Addison’s disease
- Head injury
- Kidney disease
- Lyme disease
- Polycystic ovary syndrome
- Sleep apnea
- Underactive parathyroid glands
As many as 10 percent of the people with pseudotumor cerebri experience progressively worsening vision and may eventually become blind. Even if your symptoms have resolved, they can recur — months or even years later. Yes, reoccuring… still no vision issues though. Nothing like being in the “lucky” minority!
If pseudotumor cerebri is suspected, a doctor specializing in eye disorders will look for a distinctive type of swelling — called papilledema — in the back of your eye. You will also undergo a visual fields test to see if there are any blind spots in your vision besides your so-called normal blind spot in each eye where the optic nerve enters the retina.
Abi has had these tests. Another cool word to know: papilledema! We do not have eye insurance so this is not covered and it is expensive to have done. Thus, we did not get this done again a the beginning of the summer.
CT or MRI scans can rule out other problems that can cause similar symptoms, such as brain tumors and blood clots. Yes, had this… no real tumor, nothing out of the ordinary. And YES, she has a brain! 😉
Spinal tap (lumbar puncture)
A lumbar puncture — which involves inserting a needle between two vertebrae in your lower back — can determine how high the pressure is inside your skull.
Yes, she had this. She then spend almost a week in the hospital with the spinal headache resulting from it on super high doses of caffeine until it was determined that it wasn’t going away (the writhing teen on the bed wasn’t clue enough) and they did the blood patch. If we have to do this again, we will be certain a blood patch happens at the same time as the pressure check.
Pseudotumor cerebri treatment typically begins with medications to control the symptoms. Weight loss is recommended for obese individuals. If your vision worsens, surgery to reduce the pressure around your optic nerve or to decrease the intracranial pressure may be necessary. Once you’ve had pseudotumor cerebri, you should have your vision checked regularly.
- Glaucoma drugs. One of the first drugs usually tried is acetazolamide (Diamox), a glaucoma drug that reduces the production of cerebrospinal fluid by at least 50 percent. Possible side effects include stomach upset, fatigue, tingling of fingers, toes and mouth, and kidney stones. This is the medicine Abi is on. It has worked for a time, but doesn’t seem to be helping right now. She was told to drink extra water to avoid the kindey stone issues.
- Diuretics. If acetazolamide alone isn’t effective, it’s sometimes combined with furosemide (Lasix), a potent diuretic that reduces fluid retention by increasing urine output.
- Migraine medications. Medications typically prescribed to relieve migraines can sometimes ease the severe headaches that often accompany pseudotumor cerebri.
- Optic nerve sheath fenestration. This procedure cuts a window into the membrane that surrounds the optic nerve. This allows excess cerebrospinal fluid to escape. Vision stabilizes or improves in more than 85 percent of cases. Most people who have this procedure done on one eye notice a benefit for both eyes. However, this surgery isn’t always successful and may even increase vision problems.
- Spinal fluid shunt. Another type of surgery inserts a long, thin tube — called a shunt — into your brain or lower spine to help drain away excess cerebrospinal fluid. The tubing is burrowed under your skin to your abdomen, where the shunt discharges the excess fluid. Symptoms improve for more than 80 percent of the people who undergo this procedure. But shunts can become clogged and often require additional surgeries to keep them working properly. Complications can include low-pressure headaches and infections.
Spinal Shunt was mentioned at one point. I don’t know if this is a good option or not. It might be time, however, to discuss something more permanent for her. What 20 year old wants to continue taking medicine everyday when you are an otherwise healthy person (I know that many have to because they have diseases/problems greater than this). Especially as she moves forward with life and might actually want to bear children someday, knowing that the headaches could worsen might be reason enough not to and I kinda want to have grandchildren! 🙂
I will be calling the neurologist this morning to see what next steps we can take since Abi’s time is limited due to being at camp and then heading back to school. Hopefully, we can reach a solution and get her healthy ASAP!
Thank you for reading this. It seems that it is one of my more popular blog posts. When I posted it I was just trying to vent and educate our own family and friends about what Abi goes through with Pseudotumor. Just in 2016 alone, this post has been read 139 times. WOW!
If you are willing to share, please comment and tell me why you are researching it and what your circumstances are. We would be very interested to know.