ICPDD: IIH Is Not One Disorder

A Little Whisper:  as the evolution of terms and names for specific types of conditions related to pathologically altered Intracranial Pressure (ICP) continues; it is apparent to me that they share a fundamental cause: the Dysregulation of the physiology that maintains proper ICP.  My neurosurgeon had told me that while it was obvious I had  such a condition, the results of  my diagnostic exams did not fit neatly into any currently existing box.  As such, I had developed a “catch-all” term for a number of seemingly related conditions.  That original term was ICPSD, for “Intracranial Pressure Spectrum Disorder”.  As I continue to study theses conditions, I’ve concluded tha the term “spectrum” does not seem to accurately describe either the relationship between these disorders or indicate their root cause.  I feel more comfortable with the term  ICPDD, or IntraCranial Pressure Dysregulation Disorder.  ICPDD more accurately describes the common fundamental nature of these apparently related disorders we share, and it creates a category under which each particular disorder can be filed.  Sadly, in light of the recent passing if my wife Trina, also an ICPDD warrior, I now feel an urgency to develop a more clear understanding of how  ICPDDs disorders affect the psyche as well as more tangible funtions of our bodies.    

 My purpose in the Shunt Whisperer has been simply to tell my story from the standpoint of  an ICPDD suffer with a modicum of medical training.  I hoped I might develop “unique” insights, and possibly help other ICPDD warriors improve their Quality of Life, and maybe, just maybe provide a small bit of information that might help a doctor make a better decision in the diagnosis, treatment, and management of an ICPDD patient under their care.  Most importantly, a consensus needs to be reached about the urgency with which ICPDDs need to be managed, a topic I will reserve for another secrion.  May God Bless ICPDD patients with comfort and Quality of Life, and may He bless the doctors researching and treating ICPDDs.

-The Shunt Whisperer, October 21, 2017


ICPDD/Intracranial Pressure Dysregulation Disorder. A term that I developed that makes more sense to me everyday. It refers to a number of conditions that share a certain commonality, chiefly adverse or pathologic physical and mental symptoms related to increased Intracranial Pressure (ICP). Here are some of the current condition and the abbreviations associated with them that fall into this category, with explanatory links taken from Google searches with no particular preference:

1) Idiopathic Intracranial Hypertension, or “IIH”
2) Pseudotumor Cerebri, or “PTC” : “false brain tumor”. Patients (myself included), exhibit signs and symptoms that would normally be associated with a brain tumor, but no tumor is found on examination and imaging.
3) Normal Pressure Hydrocephalus, or NPH : This one leaves me scratching my head. In NPH, the ventricles of the brain become enlarged under supposedly “normal” intracranial pressures. A very real disorder that I believe offers insight into the dynamic nature of intracranial pressures.
4) Benign Intracranial Hypertension: (now generally archaic as the process is now know to be far from “benign”.)
5) Chiari: this is what I believed I was suffering from based on my symptoms and review of my medical imaging taken during the course of my illness.
6) Migraine Spectrums Disorder: This is my personal opinion, but the commonality of migraine and migraine-like headaches in ICPDDs is not a coincidence, especially considering that migraines seem to be a result of a backup of arterial blood in the brain.
7) Acute Mountain Sickness/Altitude Sickness: I consider AMS an acute ICPSD. This may seem to be a stretch for some, but AMS presents as a very acute, severe form of IIH. Even the drugs used for treatment are similar. I believe the cause of AMS to possibly be one possible cause of ICPDD, relating to low oxygen tension in the blood and the resulting physiologic response. Damage is caused by what I refer to as a pathologic vicious cycle – sort of redundant, yes, but since it’s my site, it stays. nwcauae like it. In these scenarios, physiologic responses intended to maintain the equilibrium in the body actually end up causing damage due to an inability to adjust to environmental factors. In AMS, the pathologic vicious cycle that interests me is HACE, or High Altitude Cerebral Edema. In HACE, a mountain climber at high altitude has lower oxygen tension in their blood due to reduced oxygen tension in the ambient air. A physiologic response is initiated to pump more blood to the brain to supply it with more oxygen; however, the amount of oxygen that can be delivered in the “thin” air may not be enough, so the body demands even more blood to the brain. A normal response intended to keep the brain well oxygenated, but in the thin air, a buildup of CO2 in the lungs further reduces oxygen tension available to be delivered to the blood and subsequently to the brain. Perversely, as huge amounts of blood flow into the brain, pressure builds in the confines of the cranium; the pressure squeezes thin-walled veins faster than thick-walled arteries, causing a net gain of blood volume. The pressure squeezes the brain against the confines of the skull until its internal vessels are then squeezed down to a fraction of their diameter.  As a result,  blood flow essentially ceases as the brain swells against the confines of the cranium, and deprived of oxygen, the climber passes out and dies if rapid intervention is not available. Much of this is similar to findings in ICPDD patients, just on a much more rapid and deadly timeline. I personally wonder if altered oxygen tension perception in an ICPDD might be an underlying cause, but can offer no explanation at this time as to what the mechanism might be.


There are other conditions which may belong on this list. As I become aware of them, I will add them or remove them. At this time, medicine is only just recognizing ICPDDs, and is struggling to treat the symptoms in patients who have them. I foresee a combined advancement in the understanding, treatment, and management of ICPDDs in our information age where the potential for open sharing of data exists. To the degree that altruism reigns, the field of ICPDD treatment has the potential to advance very rapidly.