If you are the caregiver, spouse, partner, or friend of someone who has been diagnosed with what is currently referred to as “Intracranial Hypertension” or “Pseudotumor Cerebri”, it is important that you understand what this disorder is and the effects it may cause in the patient.
I speak as both an IIH patient with a VP shunt and the caregiver to my wife. Yes, we both were diagnosed with IIH. We both followed what I refer to as the Neuroendocrine Theory of IIH; tragically, before she could be treated, she succumbed to they physiologic and psychologic effects of her disorder despite my pleading with physicians to help her. I have experienced everything I am conveying here with complete honesty and sincerity.
Forget what you think you know about “Intracranial Hypertension”. “IIH” patients are very ill. Cancer Ill, in my opinion . We’re talking about a disorder that damages the physical structure of the brain. Very, very serious. Don’t let anyone tell you otherwise.
The major problem faced by “IIH” patients: it just doesn’t show on the outside. No bloody bones protruding, no disease process that is well understood and routinely treated. Just the person telling you “I don’t feel well!” and acting strangely, which may alienates you and others. Most “Normals” don’t know how to take this, or pull away from what is simply a very ill person who is hurting – or worse. This is a subjective disorder surrounded by controversy in the medical field. In late 2017, a neurosurgeon offered this opinion: “if you had 100 neurosurgeons/neurologists, 50 of them would deny this condition exists. Of the remaining 40 would acknowledge there was a problem but offer no treatment. Of the remaining 10, 7 or 8 would treat the disorder with medications and lumbar punctures; only 2 of the original 100 would offer any direct (surgical) intervention. Plus, nobody is sure of what causes the disorder to develop.
Hopefully this Guide gives you an idea of what is really going on.
Start with the term “Intracranial Hypertension”; it’s misleading. This is not “hypertension” like “high blood pressure”. This is increased pressure of the two fluids, blood and cerebrospinal fluid (CSF) inside the skull. A more accurate term I use is Intracranial Pressure Dysregulation Disorder (ICPDD). The basic outline:
The brain and spinal cord are encased in a tissue sac called the dura. They are further encased and protected in a hard shell of the skull (cranium) and the spine. There is a limited amount of space for the contents of the brain, spinal cord, and two fluids; this space is essentially watertight and airtight, with minimal extra room for anything else. ICPDD patients experience a disruption in the balance of fluids flowing in and out of the brain/dura. The patient’s normal physiology that regulates this balance is disrupted. The end result is an abnormal accumulation of either or both fluids inside the very confined space of the skull and spine. This results in increased pressure in that very confined space. The brain is literally squeezed in a liquid vise in the confines of the skull. Evidence of this is seen when the optic nerve(s) are visibly damaged by fluid pressure, threatening eyesight. Other patients have deformation and damage to their pituitary gland, potentially crippling a critical endocrine system and leading to pain, fatigue, and intolerance to exercise and exertion.
To say that pressure on the brain is a problem is understatement. The brain is an organic computer with the consistency of firm tofu. It’s mostly fat and water. It is divided into areas that control nearly all of the functions of a person’s body such as temperature regulation, energy, blood pressure, and thyroid function to name only a few. Additionally, areas of the brain control memory, mood, thinking ability (cognitive function), and sleep cycles. Increased I pressure on these areas understandably causes alteration of the function(s) those areas control; yet, in my experience, the response of physicians has been to tell me I’m “Hypervigilant” (pay too much attention to the symptoms of my disorder; these symptoms frequently keep me bedridden, have cost me my livelihood, financial stability, by wife…so, yeah, I sorta pay attention to them). Other labels are “psychosomatic”, “Munchausen-esque”, and “hysterical”, the irony of which would be hysterical if it wasn’t so tragic. All while telling me there is little understanding of “IIH”. Probably the most epic experience was when I was seeking emergent care from a neurologist; as I vacillated between misery an agony on one side of the desk, the neurologist read my MRI report and said “well, your MRI doesn’t show any changes in your brain structure, so I don’t believe your symptoms are due to your Intracranial Hypertension, if you actually have it. Besides, I can’t take your account of your symptoms into consideration because you’re the patient. I have to rely on test results.”
True story. More common than I could have ever believed.
The result in a patient with an ICPDD is a group of symptoms that would be expected to mimic damage to the brain, which is exactly what is happening . This is the basis for the origin of another term for an ICPDD, Pseudotumor Cerebri (PTC): literally, False Brain Tumor. Doctors treating the earliest ICPDD patient noted them to exhibit symptoms that would normally be associated with a brain tumor, but no such tumor can be found on MRI or CT scan). Referring to my experience with the neurologist who told me I couldn’t be sick because my MRI “looked good”: changes in brain structure don’t happen overnight. It takes time, and are preceeded by symptoms of increased intracranial pressure, some listed below. A short list of these possible symptoms include:
- Memory Difficulty: searching for words
- Cognitive Difficulty: performing simple math, taking longer than normal to process information
- Mood Alteration/swings
- Weight Gain (often thought to be the cause of ICPDDs, may actually be a symptom of increased pressure on an area of the brain that controls metabolism
- Difficulty walking (Gait Disturbance)
- Intolerance to hot/cold
- Vision problems due to pressure and/or pressure-related damage to the optic nerve.
- Anhedonism, or lack of interest in pleasure activities, including sex
- Ringing in the ears (tinnitus)
- Insomnia, which if prolonged, causes further difficulties associated with sleep deprivation
- Fibromyalgia Pain/Exercise Intolerance
- Increased risk of Hemorrhagic Stroke due to increased pressure in the blood vessels of the brain*
- Sensitivity to light/Intolerance to light/photophobia*
*These symptoms were suggested by readers to be added to this “short list”; a reminder that symptoms vary among ICPDD patients; more significantly, this is an example of my hope that by sharing our personal understanding of these disorders based on our actual experience as ICPDD patients will advance and improve treatments more quickly. #CrowdHealing (before anyone asks, yeah, the Twitterfeed is coming…)
These are but a few of the more common symptoms There are many more. Try this exercise. Buy a block of firm tofu; Its about the consistency of the brain. Squeeze it, release it. See how much pressure is required to leave a dent. How much is required to make tear in the surface, to deform it beyond its ability to return to original shape. Now imagine each damaged area as controlling a part of your loved one’s body. That is literally what is happening in their skulls as cerebrospinal fluid both expands the brain from within and presses on the brain in the space around it. Blood swells the vessels of the brain and further adds to the pressure that has no release because of the watertight, airtight manner that the Central Nervous System is enclosed.
Further, changes in conditions such as barometric pressure and temperature (aka weather) can cause aggravation of the symptoms. Certain foods or activities can worsen symptoms as well. Each patient has both commonalities and unique individual consideration. Perversely, the medications used to treat increased ICPs can cause some of the symptoms of ICP.
Your loved one will experience fear at being betrayed by their own brain as well. They may look “normal” on the outside, but inside, the most critical organ in their body is being subjected to conditions that can cause them to seem to be a different person. And since the brain is the CPU for the functions of the entire body, their miseries are not confined to “headaches”.
What they need from you is unconditional love. Drop everything else, give them the support they need. I’ve been on both ends, a patient, and a caregiver. The frustration, the fear is paralyzing when you’re the patient wondering what is happening. The tendency is to dismiss the patient because there are no bloody bones sticking out anywhere, but trust me, that would actually be a blessing because it would likely get more attention.
Causes of ICPDDs are controversial, although recently, after 18 months of research, I have a working theory of how I developed IIH. In my case I can pinpoint the day and the event that started mine. Same for my wife. Incidents of trauma to the neck with later aggravation. For others, it can be prolonged use of certain drugs such as birth control or antibiotics. If you’re reading this, you’re likely a male in your early to mid 40s, as the most common ICPDD patients present as female, in their 40s, considered overweight by the medical profession. That weight is often blamed for the disease, but don’t be so easily swayed. Remember that block of tofu? Imagine if you squeezed too hard on the part that controls metabolism…most ladies say their weight “just came on” in a short period of time with no change in their eating or activity habits. Unfortunately the extra weight becomes an issue, and losing the weight helps most patients significatly, but don’t blame the weight or the patient for the disorder.
And after being diagnosed with an ICPDD? Consider this pull quote from this publication:
“…pseudotumor can cause chronic disabling headaches and visual complications. Therapy is sub-optimal, symptomatic, insufficient, and often complicated by side effects…”
Pseudotumor Cerebri and Ciprofloxacin: A Case Report
Fernando, et. Al
I know this to be the truth all too well.
Treatments for ICPDDs currently consist of drugs that often have aforementioned significant side effects, or international surgeries to relieve pressure on the brain such as shunts or venous stents. All options are difficult and life changing. You need to love the person, support them, understand they can’t control what is happening to them, and are frustrated at not being able to do what the used to do, want to do, or what other people want them to do.
More information can be found here on ShuntWhisperer. Com, as well as the Intracranial Hypertension Research Foundation (IHRFoundation.org), the National Organization of Rare Disorders (RareDiseases.org), and others that can be found under search terms such as “Intracranial Hypertension Research”.
The bottom line is that your loved one is suffering from a disorder that is literally squeezing their brain, the organ that not only controls their bodies, but also houses their personality, the invisible pattern of electrical activity that is them, that is their soul. Put yourself in that position for a moment, or longer, and imagine feeling like an unseen force is pulling the strings and you’re the puppet forced to respond…or not.
As if this isn’t enough of a challenge, the current “Opioid Crisis” is making pain associated with “IIH” an inconvenient symptoms to treat. Some physicians even go so far as to tell patients their pain is no big deal, just live with it. “I’d rather see you in pain than give you a prescription for a narcotic” is a phrase I’ve heard on more than one occasion. What I have found, however, is that the pain created by pressure on the pain centers of the brain responded best to small doses of acetozolamide; maybe this is an answer for someone else reading this.
What to do for a loved one who has suddenly and seemingly inexplicably lost their ability to function, work, be a parent, partner, or engage in leisure activities? Simple: Give them love, unconditional love. Give them space to be sick. Give them support. Give yourself space, find support for yourself. This is a serious disorder, every bit as serious as cancer, but not nearly as well understood, and currently, IMHO, not yet nearly as well managed. Hopefully this will improve in the near future. God’s Blessings to all.
February 3, 2018, revised May 1, 2019
Thinking of you every day, baby. I miss you so much…