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Living With a Pseudotumor Cerebri (Idiopathic Intracranial Hypertension) Diagnosis

I was diagnosed with pseudotumor cerebri in 2008 and want to share some tips that have helped me manage the symptoms.

What Is Pseudotumor Cerebri?

After my diagnosis in October of 2008, I was surprised and dismayed to find so little information on this disease—it is also known as benign intracranial hypertension (BIH) or more commonly idiopathic intracranial hypertension. Sure, you can find medical reports and statistics, but the human side has been lost. Nowadays, there is a lot more information than what could be found back then. This may be because of its rarity—less than 1 in a 100,000 people diagnosed—although, these numbers have grown over the years I have had it. Another reason may be the fact that doctors and scientists still do not know the cause.

IIH mainly affects women of a childbearing age who are obese, although I was not overweight. There are also young men and women and older men who are getting the disease—the numbers are considerably lower, but the cases exist nonetheless.

It has many stages and treatments. However, I would like to touch more on the daily experiences of living with the disease and the ways I have found to alleviate some of the symptoms. If you've been diagnosed, maybe this can give you a heads up on what to expect in the months or years to come.

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I Woke Up With an Unbearable Headache

I started to notice a headache one day in October—I woke up with it and could never really seem to shake it throughout the day. The next day, it was still there and getting worse, it seemed. Not being one for having headaches and being pregnant, I put it out on stress.

In the following days, it continued worsening to the point where I was puking every morning, and I literally felt like my head was going to explode. It wasn't like any headache I had ever had; it was a pressure that feels like your head was in a vice.

I finally went to the emergency room—I had not been able to keep anything down for days. I just wanted someone to screw a hole in my head to relieve the pressure.

Misdiagnosis

The first hospital I went to was CAMC in Charleston, WV. I have no qualms about putting their name out there as a place not to visit to diagnose this disease; maybe their treatment would be better than their diagnosis skills. But, alas, I will never put myself in their hands again.

I won't describe the horrid treatment and unethical behavior. However, I will touch on the misdiagnosis—or maybe lack of one. First, I was told it may be meningitis, so I was given a lumbar puncture. The proper body position—lying on your side—for this procedure is imperative to a proper diagnosis. They were trying to give me one hunched over with an ever growing belly! After about an hour of failed attempts and, "Oops, I keep hitting bone!", I was pretty much done. I was given a half-hearted talk about checking cultures, which was pretty much BS, and sent home.

The good news is, I felt a bit of relief from the headache; the seven failed attempts at a lumbar puncture produced a small amount of fluid that probably released some of the pressure. In hindsight, the pain caused to my spine from repeated puncture may have also lessened the head pain from a comparative standpoint.

However, the next day, I woke up to the same pain. Being literally scared to go to another doctor, due to the treatment I got at the aforementioned hospital, I stayed home another week in pain. I couldn't eat or sleep; I just lied there with my eyes closed. I have never been such a baby with pain, but this is the most I have ever felt in my life—and I have birthed two children—so I know the pain is very real and excruciating.

Finally, a Proper Diagnosis

Out of desperation, I went to another hospital after two failed attempts at others. I went to Cabell in Huntington, WV: hands down the best hospital, although I may be a little biased.

I was first given a CT scan to look for tumors since this is what my symptoms would indicate. Seeing nothing there, they gave me a lumbar puncture. Although patients are usually given Valium to reduce any anxiety about the procedure, I was pregnant and was unable to take it. The purpose of the lumbar puncture was to take a pressure sample of my spinal fluid through my back, although I'm still not quite sure how that one figures out. My pressure ws high, although I could have just told them that, and the diagnosis could have been made.

My doctor diagnosed me with pseudotumor cerebri. The first time I heard "tumor" and "cerebri"—I knew the latter had to do with the head—I was scared. I had a child growing inside of me, and I felt like I was just handed a death sentence. The doctor explained a bit of what it was. I was told to look up the disease when I got home and was urged to stay away from forums. She also prescribed me acetazolamide (Diamox) three times a day.

After Your Diagnosis, What Appointments Can You Expect?

First Appointment Is With a Neurologist

After getting the diagnosis, a visit to a neurologist will be the next step in your journey. It shouldn't take long for this to occur—my visit was actually three days later. They will surely do some blood work, and, depending on the medication prescribed, they will debate what they are actually going to be looking for. Since I was prescribed Diamox, my neurologist, Dr. Hasan Ercan, checked my protein levels for liver function approximately every three months.

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When in the neurologist office, they will give you a battery of mental and physical tests. To me, it felt almost like a sobriety test. They will check your reflexes, coordination, and legs for any swelling (I believe this is another symptom of this disease). These are all painless tests that usually take about 20-30 minutes. Your doctor will also want to check your optic nerve. This involves a light being shone close to your eyes while you stare at a distant spot. They have to dilate the eyes to look through your pupils.

There will also be questions about other symptoms, such as nausea and sight or vision problems. You must understand that although the pain is in your head, the real danger is the pressure in your eyes—kind of like glaucoma. Although death is a possibility if left untreated, from what I have researched, the immediate concern is widening blind spots (papilledema), or even worse, vision loss and blindness.

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The Next Appointment Is With an Ophthalmologist

This one will take a few weeks and won't be too pleasant—not really painful but uncomfortable nonetheless. They will first dilate your eyes with a yellow solution and then check the pressure of your eyes. While you rest your chin on a strap, a nurse will blow air very close to your eyeball. I have a tendency to pull back when things come towards my eyes, so I actually had to have another nurse hold my head in place.

After a period of time in a room waiting for your eyes to dilate, you will be taken into another room where they will test your vision function. You will be told to look at a screen with one eye covered and to push a button every time you see a light speck. This will gauge your peripheral vision and any loss thereof. They'll repeat the test with the other eye. This took me about 10-15 minutes for each eye.

The last test will take you to another machine to take a picture of your optic nerve to look for any permanent damage there.

Finally, you will see your ophthalmologist to go over the test results. Hopefully, you will not get any bad news. If you do, there will be a few more tests.

Tips:

  • Like I said, none of these are particularly painful, however, your eyes will feel strained and sore.
  • The dilation will obscure your vision markedly, and light will be very uncomfortable. Having another person to drive you home is optimal, but I have always driven myself home after these visits.
  • Usually, if there is no pressure in your eyes and everything looks good, you can expect to have these appointments every six months or so. If you are not doing all of these tests at least every six months, you are jeopardizing your health, particularly your eyesight.
  • Find a specialist that you are comfortable with that will go through all of the steps that are needed.
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If Your Case Is Serious, You May Need Further Tests

Magnetic Resonance Imaging (MRI)

You may also be sent to your local hospital to get an MRI. This is to look at the blood vessels in your brain to see if there are any areas of concern. The test will last 45-60 minutes. You will have to answer a few question about any metal you may have in your body, old tattoos, etc. because the machine is essentially a huge magnet.

After changing into a gown, you will get into a large circular machine on a rolling bed. The first part is around 30-40 minutes. You will have to remain perfectly still, even while the machine makes loud, piercing sounds. For the second part, you will be rolled out, and dyes will be injected before you go back in for another 10-15 minutes.

Emergency or Periodic Lumbar Punctures

In some cases, you will have to get emergency and/or periodic lumbar punctures. You may have already had one since that is usually the first test done. Do find a capable person to do this; I have had good ones and bad ones, and there is no need for any extra pain. Do not be afraid or embarrassed to speak up if the local anesthetic is not applied deep enough or if the pain is too much. You may be given Valium through an IV, although if you are pregnant, a different pain medication or no pain medication is used.

Tips:

  • A stressful situation will cause headaches due to the raised cerebrospinal fluid pressure, which may lead to an emergency room visit. Know your hospitals and always stick to one you trust. I have had one of these episodes in the past and was given a pain medication through an IV.
  • Some doctors will opt for a lumbar puncture, although if you do not want one done on a regular basis, and you have no vision difficulties, you can always request for less invasive treatment.
  • It's is your body and your health. Research your disease as much as you can, and be proactive.
  • I also recommend keeping a notebook of questions for your doctor. The average time spent with your actual doctor is 15 minutes, so make the most of it. Thoughts and questions will go through your head in the months between appointments, and I have found it is good to have them all written down.

What Can You Do to Manage Your Symptoms?

First and foremost, take control of your health—know what the steps of the treatment process are and which one you are at. The steps are as follows:

  1. Lumbar puncture
    • In some cases, the first one will alleviate the excess pressure and end your symptoms.
  2. Oral diuretics like acetazolamide (Diamox) or furosemide
  3. Optic nerve sheath decompression.
    • This is primarily used in cases of vision loss and severe pressure buildup behind the eyes. This is a surgical intervention to increase blood flow to the optic nerve.
  4. Cerebrospinal fluid shunt
    • This technique is used is a last resort. It is usually very effective for short-term relief. However, in the long run, further surgeries are likely.

There are several other options for medical interventions, and they are coming up with more as the disease is researched more.

If you are at the earlier stages of the disease, there are some things you can do to try to stunt its progression, or in some cases, put it in remission for good.

  1. If you have any excess body fat, it is imperative that you do what you can to lose it. Extra weight adds extra pressure; this is usually why most of the cases are overweight or obese women. More often than not, symptoms occur during periods of weight gain. This is your best chance of stopping the symptoms for good. Last time I checked, a third of patients will experience remission with enough weight loss.
  2. For pain relief, in some cases, migraine medication will be prescribed.
    • OTC ibuprofen and/or Tylenol can be used for minor, dull headaches. However, please keep in mind that taking OTCs more than twice a week can cause residual headaches.
    • I have found that wrapping a scarf tightly around my temples for about 15 minutes can sometimes do wonders.
  3. Try changing your diet.
    • Lower your salt intake. This will help stop water retention.
    • I worked with a specialist from Arizona named Dr. Foster who wanted to use the concept she used for migraine patients on my condition. The basis was the removal of foods that raise adrenaline levels, such as citrus, and replacement of premade foods with whole foods to reduce consumption of a lot of chemicals. This is combined with at least 30 minutes of exercise daily to raise your serotonin levels.
    • I will admit, this is a hard diet to stick to, but if followed strictly, I believe you could really find a benefit with regards to your headache and anxiety levels. I found results in as little as two days. In adding all the whole foods and cutting processed foods, you will also be able to slim-down your figure.

Conclusion

I hope that this was helpful to you—to let you know that you are not alone if not to give you a useful tip or two. There will be times when you wake up when you just wish your headache wasn't dull and throbbing or you didn't have nausea. Just take that to drive you to the weight loss you may need to put this into remission. If you have used these tips and still have symptoms, just take it day by day. Every day, try to make it a little better by lowering your stress and being mindful of what we put in our bodies. I urge you to constantly look for new information on this disease and learn anything new that may help you. My best wishes to you.

Update: 4/20/2018 (Ten Years Later)

I would like to make an update. It’s been almost ten years since I wrote this article and a lot has happened since then. Three years after diagnosis, I started getting low pressure headaches. This is when your headache gets worse as the day goes on and it’s more of a pulling sensation. I went off of Diamox and was in remission for almost five years.

Pressure started to raise a couple of years ago and I got a leak from a hole in my skull, made from long term pressure. (CSF Rhinorrhea) Since then, I’ve seen several neurologists and surgeons, though after careful research, I haven’t found the proper one to do brain surgery. I run low pressure now, sometimes really low and there are a whole slew of issues with low pressure, including meningitis risk, but the pain is so much more manageable now then it was in high pressure.

I’m supposed to have surgery, but for now I drip CSF through the nose. It keeps my pressure from rising, but I’m unsure how long this will last. If you find that you are leaking clear fluid through your nose, eyes or ears (full feeling) get it checked out and the fluid checked for CSF. It is more prevalently tied to IIH then is recorded and diagnosis can be a pain.

I also found that MMJ helped greatly in slowing down production of CSF fluid, as well as for pain. I think, paired with Diamox, it was what put me in remission finally. I’ve been off Diamox for almost seven years and though my IIH is active again, it is more manageable than it was in the beginning.

Good luck on your journey and I hope relief for you all.

This content is accurate and true to the best of the author’s knowledge and does not substitute for diagnosis, prognosis, treatment, prescription, and/or dietary advice from a licensed health professional. Drugs, supplements, and natural remedies may have dangerous side effects. If pregnant or nursing, consult with a qualified provider on an individual basis. Seek immediate help if you are experiencing a medical emergency.

Comments

hope35 on August 26, 2019:

Thank you for the information. Hopefully more medical advances will be made. It is a struggle daily- - but I am trying to stay postive.

kaiyan717 (author) from West Virginia on August 24, 2019:

I have heard of that as well. Many facebook forum particpants have done that. Mrv will show blockages that

could be helped with stenting.

hope35 on August 24, 2019:

I was diagnosed with IIH recently(about 8 months ago). I am not over weight. I feel birth control might have caused my problem. I have had headaches -- migraines, nausea. I had a MRI's and Lumbar Puncture-- which showed that I have high pressure. . I hear hissing in my ears on and on and o.f I have some permanent vision in my left eye. I had a optic nerve sheath fenestration it saved me from not loosing all my vision in left eye. It help reduce my headaches. It has been a struggle. I am continuing to see a neuro ophthalmologist to monitor my condition. I have been looking into treatments that I may need in the future. I saw on the internet a procedure that involves using a stent in brain-- instead of shunt.

kaiyan717 (author) from West Virginia on August 08, 2018:

I'm sorry you've had this for so long WetaFly. I've had it a decade and it feels like a lifetime. I wish there were better options. I've avoided surgeries for all this time, but with a cranial leak, one is now inevitable. I wish there was something better out there. I hope maybe in another five or ten years, something better will come along, more understanding. It's pretty shocking to think I wrote this ten years ago and we're not much better off now, then we were back in 2008. I can't imagine the options in the early nineties. I hope for you relief and peace of mind. Thank you for commenting.

WetaFly on August 08, 2018:

My final comment was not because i feel IIH will be what takes me out but as a viet nam veteran exposed to agent orange i have already out lived my own brother and several friends all on the same hill exposed simultaneously, i consider my IIH successes a conquest and testimony to modern technology.

Aug- 8 -2018, I will have my shunt adjusted to a new pressure setting as the cupping of the optic nerve has become darker( more noticable.) Kayian is correct as I have had five shunt revisions, two were complete failures the other three were pressure adjustments. With the new non invasive adjustable shunts invasive renisions will be greatly reduced. I believe that the programmable ventricular or LP

kaiyan717 (author) from West Virginia on August 06, 2018:

I ended up with a leak through my nose. Body shunted itself in a way.

WetaFly on July 31, 2018:

I was diagnosed in 1991, I had been suffering severe headaches for a couple weeks but due to work obligations (Sr. Eq Eng) in a Semiconductor fabrication plant I did not go to Dr. Alarm goes off 0000hrs finally time ER Deb drives, conclusion-misdiagnosid, severe ear infections causing the headaches cure antibiotics. The following morning 0800 big problem! Extremely distorted vision wife escortes me to Dr. Johnston he looked in my eyes called 911 and this began a 13 week stay starting in ICU, LP confirmed CSF was so high they drained it till it was readable, my retinas had detached, they were successfully reattached. I failed diamox and every other meds tried so resolved to LP's every couple days. So 11 weeks later they found an experimental LP shunt and this was promptly surgically implanted. Fast forward 23 yrs and 5 shunt revisions I got what I feel will be my last one at the VA in Little Rock. A good neurologist and functioning CSF shunts have saved most of my eye sight and has made quality of life worth living. By all means ask your Dr. about an Ventricular or Lumbar Peritoneal shunt, mine is a Medtronics Strata programmable LP shunt. I wish all the best to those suffering this all to often misdiagnosed disease, I sometimes think maybe agent orange or the myriad of chemicals I used in the wafer fabs? It will be over in the next dozen or so years. Aloha

Alexis on April 21, 2018:

Thank you so much for this, your words have truly been a comfort.

kaiyan717 (author) from West Virginia on March 12, 2018:

Sorry to hear that anyone else has this,especially at such a young age. I was blessed with remission for almost five years. I hope the same relief to you soon. Yes, it does suck

Chelsey on March 12, 2018:

I went through basically all the same thing, been diagnosed since 15, I'm 18 now and it sucks to deal with it and wish it would just go away.

kaiyan717 (author) from West Virginia on October 08, 2017:

I am sorry to hear you have had it so young. Many get misdiagnosed with migraines or meningitis. It is rare, so it is one of the last things thought of. I wrote this many years ago because there wasn't a lot of information out there. Times are changing and getting better. I would invite you to check out some forums. Go on Facebook and search IIH. There are many with hundreds and even thousands of others that are going through it as well. There are boys, men, kids, every demographic you can think of. Sometimes talking to people that get exactly what you are going through can help so much. It has helped me out, as well as given me some great coping tips. There is always hope of remission and of life getting better. This disease will give as much as it takes and I hope you can find some silver lining. It will get better and I hope for you some relief.

Jared on September 30, 2017:

Reading all of these comments, all these stories, and everyone's perspective is very interesting. I myself was diagnosed with IIH at the age of 10 in 2012, I'm now 15 and have been through multiple LP's and have tried 2 different medications (topamax and acetazolamide). I'm incredibly worried as I am every opposite of the "normal" for this disease. I am very young, very healthy and a Male. My first indications of IIH were when I was 8, I would wake up with these headaches that would render me useless. I would go to school not being able to think straight, feeling nauseous and not even being able to see straight. It took 2 years of doctor hopping before someone told me something other than Meningitis or Chronic Migraines. The first LP they performed came back with tests showing my spinal fluid pressure was at 38, they told me the normal was around 12. Since then the highest it's been was 41, and there are no signs of it going away any time soon. I have been given no cause, and no explanation for how or why I developed IIH. It has left me wondering, debating and contemplating many things. My education has been utterly destroyed because of this. I went from being a top student in the country to being years behind. Along with many other problems and diseases I have been robbed of my childhood, and unfortunately I go to sleep most nights in tears. I've never opened up about this before, but after seeing everyone else's stories I just had to. I just hope I dont go blind, I cant even imagine not being able to see my future children, my future wife, or anything. Thank you so much to everyone in this forum.

kaiyan717 (author) from West Virginia on August 01, 2017:

Sorry to hear you got this diagnosis. It can be a hard one. When I wrote this years ago it was a lot less prevalent. I have high blood pressure that I think is due to constant pain. The worse it is, the worse it gets. I have had it for almost 9 and a half years now, though I have a leak through my nose. (Yes it can blow) So my symptoms are now low cranial pressure. Check out some forums, they have great ones on facebook. They will help you feel not so alone, as well as giving you ideas for coping better. Good luck and God bless!

Petalos90 on August 01, 2017:

I was diagnosed a week ago. But before, it was few years of nightmares. The headaches, dizziness, blurry vision, etc... The part of my head most affected is my right side, my eye, ear, the ringing and pulsation, sometimes it's overwhelming. After the lumbar puncture, that week I felt a relief, but it looks like my symptoms are coming back, including high blood pressure. Did you experienced high blood pressure as well? The Cardiologist has already ruled out anything heart related, an extensive check-up of my arteries was done and he confirmed that my heart and veins are healthy, that the only thing that he could think of is of something Neurological. I am guessing that my high blood pressure is tied up to the symptoms of Pseudotumor Cerebri. This blog has been a blessing. Thank you all for sharing your stories. Wish you all and everyone else the best!

kaiyan717 (author) from West Virginia on July 07, 2017:

That's great Amanda. I love to hear good news!

Amanda on July 07, 2017:

I was diagnosed in the summer of 2013 after persistent blurred vision sent me to my eye doctor. I had an emergency MRI to rule out a brain tumor, then an emergency lumbar puncture to confirm diagnosis of IIH.

I spent several years taking the common meds and getting lumbar punctures.

An angel of a neurologist told me about a stenting procedure that could help widen veins in my brain to allow fluids to flow better and could potentially reduce or eliminate my symptoms.

Diagnostic CT scans showed that my intracranial transverse sinuses were only 30% opened thus making me a good candidate for stenting. My team of neurologists agree that this narrowing is the cause of my IIH, but the cause of the narrowing cannot be defined.

I've had my stent only for a few months, but I am off all IIH meds and am regaining some semblance of normalcy.