THE NEUROENDOCRINE THEORY – HOW I BELIEVE I DEVELOPED IIH

I’m away from my computer at the moment. Mixed feelings, that…at any rate, I wanted to start getting this idea out for discussion. I’ve been wanting to get it out for four months, but I was just too sick to drive my computer after performing the basic tasks of paying bills, laundry, opening a can of Campbell’s Kettle Cooked soup (not a plug, Campbells isn’t paying me a darn thing, but for $2.50 from Amazon, the Jambalaya and White Chicken Chili is not too bad).

I will only be posting the bullet point outline of my theory today due to limitations of typing on a phone as well as the need to present aspects of each point in greater detail in the proper order. And, while this is MY theory of how I developed “IIH”, I have witnessed it play out personally in two people I know personally (both female), as well as seen it as a theme in many other “IIH” patients in various support groups. I suspect this process may likely apply to others as well due to commonalities in physiology between human beings, but I don’t represent it as the ultimate explanation for all Adult Onset Acquired/Secondary IIH/PTC/NPH.

I put this theory together through months of research, review of my medical records, family interviews, and the following publications I urge everyone coping with this disorder to read:

1) “The Driscoll Theory” by Dr. Diana Driscoll, available on her website PrettyIll.com (thank you Renee for bringing this to my attention). While Dr. Driscoll’s focus is primarily related to complications off Ehlers-Danlos Syndrome in which altered intracranial pressure is a common symptom, I believe much of her theory applies to non-EDS patients with altered ICP as well, and will discuss this in detail in the near future.

2) “Adrenal Fatigue Syndrome” by Dr. Michael and Dorine Lam. Adrenal Fatigue Syndrome (AFS) is a condition that develops in cases of prolonged stress. This can result in a blunting of the endocrine response to stress, and Dr. Lam details what the result of this altered endocrine response can cause. I have been dealing with AFS most of my professional life, and it has been a significant obstacle in my recovery.

Even with these contributions, I still didn’t understand how stress causes increased intracranial pressure. However, shortly after reading Dr. Lam’s book, a research paper from 2013 literally fell into my lap, a veritable Rosetta Stone tying everything together.

I need to state this theory is based around my initial postulate that ICP in adult onset secondary “IIH” is governed primarily by cerebrovascular/blood dynamics. Blood is the ONLY fluid that flows into the brain/skull at a rate of 750-1000 cc/minute at rest. CSF is made from arterial blood in the brain at a mere 0.35 cc/minute. While CSF can make a significant contribution to ICP, failing to take cerebrovascular dynamics into account ignores the 800 lb gorilla in the head. I arrived at this in a rather “side-loaded” manner, but I’m not the first to make this observation:

Dr. Mark Wilson, Neurosurgeon, Royal Academy of Medicine: Monro-Kellie 2.0: The dynamic vascular and venous pathophysiological components of intracranial pressure

Thus, without further rambling, The ShuntWhisperer’s Neuroendocrine Theory of Adult Onset Acquired/Secondary Intracranial Hypertension:

IIH IS NOT A PRIMARY DISORDER. IT IS A SYMPTOM OF ENDOCRINE DYSREGULATION AS A RESULT OF PROLONGED STRESS. I don’t consider this to be the only possible cause of Acquired ICP, but I believe it is how I developed my particular disorder. Thus my contention that endocrine evaluation and stress reduction are as important to optimal treatment outcome as is reduction of pathologic ICPs.

1) Pre-existing condition(s), developmental or acquired, which predisposes a patient’s intracranial pressure physiology to be higher than “normal”. Examples include but are not limited to:

a. Underdevelopment of the Venous Sinus Network, resulting in reduced absorption of CSF

b. Reduced Intracranial Volume, including Chiari Spectrum findings with or without symptoms.

2) Past Physical Trauma, especially cervical trauma

3) Autoimmune/Inflammatory conditions with or without symptoms, especially IBS, Crohn’s Disease, gastroparesis, endometriosis, and Polycystic Ovary Disorder

4). Female Sex

5). Exposure to prolonged stress with alteration of endocrine stress response, especially “blunting” of the Recovery Phase from initial Fight/Flight Response.

and finally:

6) Precipitating Stressor. This critical event can be physical and/or emotional, and pushes a patient’s quiescently challenged physiology into an area where increased ICP and associated serious consequences develop. If, as in my case, it results in a dramatic and unexplained loss of function which threatens livelihood, relationships, and pleasure activities, this can initiate a vicious cycle of increasing intensity until the patient has very limited or no ability to function.

The Good News: based on observation of the early onset of IIH in several patients, I believe that ICP is reduced into a normal zone while the patient’s body still has the ability to heal the damage caused, remission is possible when accompanied by lifestyle changes to reduce stress, dietary changes, and monitoring. Reduction of ICP at this time may be able to accomplished with very small doses of Diamox/acetozolamide.

So there it is, for what it’s worth. Over the next few weeks I will fill out pertinent details, with research references. Again, this is my theory based on my experience; I have no idea how broadly it applies to any other patient, but I’m beginning to see similar notions from medicine.

As always, prayers for comfort and food days to all & May God Bless each of us, our families, and our doctors.

April 30, 2019

The Shunt Whisperer

4 thoughts on “THE NEUROENDOCRINE THEORY – HOW I BELIEVE I DEVELOPED IIH

  1. This speaks volumes to me…
    – Past physical trauma, yes to the head.
    – Auto-immune/Inflammatory conditions, yes, psoriasis, and I believe I have some level of metabolic disorder and/or hypothyroidism
    – female, yes
    – Exposure to prolonged stress with alteration of endocrine stress response, especially “blunting” of the Recovery Phase from initial Fight/Flight Response….. I was in a long term emotionally abusive relationship, so yes.
    – precipitating stressor… I left said relationship and changed my entire lifestyle (moved states, quit smoking, changed diet, etc) within months of my initial symptom onset….
    I look forward to reading more of your theorising 🙂

    1. Kelly: I’m sorry to hear about these challenges. An ultimate point in this theory is that I believe involvement of an endocrinologist is necessary to evaluate the FUNCTIONAL status of your endocrine system’s condition, especially the HPA Axis (Hypothalamus/PituitaryAdrenal System). The HPA Axis is the “master control” of the endocrine system, and typically the adrenal segment weakens first, taking the other systems down with it. When I say “functional” status, I’m referring to testing that assesses individual hormone levels over their particular period of normal highs and lows. One endocrine blood panel gives a “snapshot” of the endocrine system, but as the saying goes, even broken clocks show the right time twice a day. Cortisol is a critically important stress control hormone that waxes and wanes throughout the day, so proper evaluation requires measuring its levels during at least 5 critical periods in 24 hours. You might want to start looking at Dr. Lam’s website, DrLam.com, and familiarize yourself with Adrenal Fatigue; “The Driscoll Theory” addresses issues of autoimmune conditions with regards to stress as well.

      My belief is that my IIH is a symptom of Adrenal Fatigue, not a primary disorder. Treating Intracranial Pressure Dysregulation without looking at how endocrine function may have been affected by ICP alterations seems less than ideal. I will be filling in the blanks regularly, even daily if possible, in hopes that it helps others such as yourself. Prayers to you for comfort and better days.

  2. * I had head/neck trauma at age 18.
    * Viral meningitis at age 19, 24, and 36. Yes-viral meningitis 3x
    * Total hysterectomy at 30 for severe endometriosis. The intense headaches and vision changes started in 1991 but I wasn’t diagnosed until 2007 and by that time I’d lost 30% of my optic disc in both eyes.
    * LP and VP shunt and 6 optic nerve decompressions…2000mg of Diamoxx ER and 160mg of Nadolol a day for the last 8yrs

    Some days are better than others! Currently training for a 1/2 marathon with my daughter-I had given up my running after the 3rd meningitis round-that’s what REALLY pushed my body over the edge! But I’ve GOT to train and cross that finish line cuz I’m so tired of this disorder…

    The reason I am posting/sharing is because your observations make such SENSE! I’m a retired combat medic from the AF and a licensed massage therapist so I have extensive schooling in A&P, pathophysiology and the endocrine system. To read your testimony and observations make me feel validated! There is an article that you wrote about the caregiver on Facebook and I posted that due to the fact you stated everything so descriptively and accurately. I’ve had such positive feedback from my friends/family after they read it-THANK YOU!

    1. Neck trauma shows up regularly in ICPDD patients. Cervical Dysautonomia including Pure Autonomic Failure, changes in venous outflow as a result of endothelial damages to vertebral and internal jugular veins, Starling Effect restriction of IJV drainage by the Internal Carotid, as well as vagus tone alteration due to acute or chronic trauma all have the potential to change cerebrovascular drainage in patients predisposed to reduced ICP change compliance. It is also very telling how structures following the course of the ventricles are also the structures at greatest risk for damage from increased ICP pressure/duration. Optic nerves are next to the pituitary gland, arguably one of the two most critical endocrine glands controlling bodily functions, yet while optic nerve deformation gets the attention it well deserves, Empty/Partial Empty Sella Syndrome is dismissed out of hand as “common” and “probably” unimportant.

      “Probably” is OK unless you happen to be one of the “probably is important” – like it is in my case. Life and exercise with HGH is rough, but to date no neuro doc I’ve “consulted” with cares to consider what I have to say or my very real, very valid, very documented experiences.

      This response marks a shift in my attitude on Shuntwhisperer and towards the medical profession in general. I have attempted to be cautious in my responses and subject matter, fearing that rubbing NeuroDocs the wrong way would be counterproductive. An appointment with a new neurologist necessitated by Dr. Kenneth Liu’s unexpected and unfortunate (for all) indefinite sabbatical has left me without hope of finding a neuro who possesses the diagnostic skills I was fortunate to witness in my residency, skills which so impressed me that I modeled my dental and oral surgical career after them, doctors who would NEVER tell a suffering patient that their account of their disease was “unimportant”, thereby never learning the true nature of the patient’s condition. As a result, symptoms are treated, but underlying causes are ignored. Patients are seen as annoying pathologies who whine and whimper over their discomforts, with little concern over whether the patient can return to being a parent, a husband, live a life with some semblance of dignity and self sufficiency. “Patient made a satisfactory recovery” usually means they have a pulse…and that’s not a joke.

      I’m going to come right out and say that shunts are poorly performed. As a surgeon familiar with bone healing, you can’t flap a patient’s scalp over a 14.5mm burr-hole in a skull and expect bone to magically heal in. The soft scar tissue that forms around the silicone catheter penetrating the meninges does not allow cells to heal to it – only around it. This sets the stage for unwanted CSF leakage from the subdural space ABOVE the brain, not the Third Ventricle/Foramen of Monro. CSF seeps along the tract of the catheter, allowing unregulated and unwanted loss of CSF with resultant unstable ICP and development of Intracranial HYPOtension, slit ventricle syndrome, and a new set of neurological symptoms.

      Ask me how I know.

      Lumbar Shunts? I suspect even worse. How many shunts are deemed to fail because of “leakage”, CSF filled abdominal cysts adjacent the vertebra where the shunt is placed.

      The shame of it all is that neurologic surgeons have everything at their fingertips in the OR to mitigate this with a quick and relatively cheap procedure known a s Guided Tissue Regeneration. Orthopedists pioneered it decades ago, and the development of viable dental implants made GTR a common, predictable procedure in bone wound management. It has been stated to me that CSF leakage around shunt catheters – aka “peritubal leakage” – “isn’t much of a problem.”

      Until it is, when it can be a big problem. Given that fact, the only reason I can see NOT to repair the bone around shunt catheters when they are placed is some type of corporate ignorance and lack of concern for what constitutes a “satisfactory recovery.”.

      To this day, no neurosurgeon or neurologist has cared to discuss the injury that precipitated the last 12 years of decline in my health. I will
      call that piss-poor history taking and case-workup. I have heard ad nauseum “we don’t know anything about why ICP increases/decreases/changes”, and then when I try to respectfully offer an observation about my condition, they are routinely disregarded. News Flash: while you are bowing at the altar of Evidence Based Medicine, people are suffering. Even Dying. All because no doctor wants to follow any procedure that hasn’t been the subject of a double blinded study and published in a peer-reviewed journal (if they even read them). Which, by the way, takes about 10 years.

      Conservatively.

      Diamoxx and Naldolol. Both antihypertensives. Diamox, a carbonic anhydrase inhibitor, reduces CSF production, but it also reduces systemic blood pressure/Mean Arterial Pressure. Naldolol is a beta blocker that reduces adrenaline’s effects on BP. Since the ONLY fluid flowing in and out of the cranium is about 1 liter of blood per minute (yes, only blood – THINK), from which less than 1/2cc of CSF is produced in the ventricles, which effect – blood pressure/volume reduction or CSF production – likely has a greater effect on ICP (absent a non-communicating hydrocephalus)?

      I drove 160 miles last week to consult with a new neurologist about a very specific, very significant problem. After an hour of examination in which I was only allowed to answer questions asked by his holiness (“forget what you think you know or read on Google” – by the way, why is something wrong if I read it on Google, but OK if Dr. holiness reads it?). After his exam, he actually said he was going to dismiss my written and verbalized chief complaint as “unimportant”, and told me all my meds (3) were obscuring his well developed ability to determine my problem, he’d send a letter to my PCP (hopelessly clueless about ICPDDs), and made no follow up appointment.

      Just so you know, Dr. Phil wanna-be, the only thing that kept me from expressing my displeasure was the resident following you around. I didn’t want to prove you a fool in front of him. BTW, he already thinks you’re a pompous jerk.

      Oh, and the MRI that you said looked “so great – you have a great brain.”- the radiologist report was remarkable for evidence of chronic Intracranial Hypotension and/or S/P subarachnoid hemorrhage.

      Which I could have told you, but you wouldn’t let me.

      Lori, I’m sorry for all this, bit I have a feeling you are of a similar opinion. I will no longer carry the water for such an ill-behaved group of professionals who ate tasked with the very sacred task of caring for the organ and systems that is the seat of our souls. Offended? Own it. Google 3 words: “Why are Neurosurgeons”….

      Yeah.

      Lori, myself, and others are textbook cases of what I suspect to be institutionalized ignorance and apathy by a group of physicians widely held to be the “best”. With one exception, the three neurosurgeons and two neurologists I’ve consulted with have been condescending, arrogant, cruel, even resorting to outright lying when caught red handed. The pace of neurologic/neurosurgical treatment is absolutely glacial; there is no follow up after treatment “unless there is a problem”, so all the potential retrospective treatment data gets lost and no improvements are made.

      As a dentist, I get a lot of snide remarks from physicians -“you’re not a real doctor, just a dentist.”. I submit you should aspire to be what I am. I learned early in my career that few diseases/disorders survive the onslaught of trained internal medicine doctors.

      I’m still right.

      As a dentist, I had to assess, examine, accurately diagnose, and treat patients at the same appointment. I wasn’t pulling pituitary adenomas out of a nostril, but I was doing way more than polishing teeth. I was a very good diagnostician, especially when I learned the three words “I don’t know.”. I didn’t leave patients without answers or the promise of an answer in the near future.

      Yeah. I once aspired to be a neurosurgeon. I admire your surgical skill set, but in general the humanity is lacking. Oh – the presence of a shunt with “good ventricles” absolutely does NOT mean I’m cured, anymore than an insulin pump cures diabetes. There is no research to show how quickly ventricles change in size, or when symptomologies affect the patient. Stop judging a patient’s condition by an MRI.

      Learn what the source of Intracranial Pressure is.

      And please, stop blaming CSF for all Intracranial Pressure Alterations, and stop thinking of your patients as obsessive, Munchausenesque, “hypervigilant” hypochondriacs, and start treating them like you would want someone you love dearly to be treated.

      Wes
      The Shuntwhisperer
      Sept 3, 2019

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