Normal Pressure Hydrocephalus (NPH) – To Treat or Not to Treat & Medical Guidance

Dr. Salomon Hakim explains Normal Pressure Hydrochephalus (NPH)

Dr. Salomon Hakim explains Normal Pressure Hydrochephalus (NPH)

NPH is such a rarely diagnosed dementia disorder that no large population studies have been done.  It is estimated that over 1/3 of American physicians have never even heard of NPH, a disorder that can potentially be reversed or at least diminished and slowed if treated appropriately.

The disease was discovered 50 years ago (1964) by a brilliant and caring researcher, Salomon Hakim, MD, PhD., in Colombia, SA. Unlike other brain diseases there is a dearth of studies that follow NPH patients, treated or untreated.  NPHers exhibit three often misdiagnosed symptoms that continue to diminish quality of life (cognitive impairment, incontinence, balance and gait problems).  Brain scans show dilated ventricles filled with excessive cerebrospinal fluid (CSF).   My feeling is that if you are fortunate NOT to be misdiagnosed then you are one NPHer who has the ability to direct and decide what your immediate and long term future may be.

imageMy sweetie could have been CT scanned diagnosed in 2005 or at least told to get scans on some regular basis, to look out for symptoms, etc. but it took a year of intermittent urine incontinence wrongly attributed to BPH, some bowel incontinence attributed to constipation and then after several falls the traumatic brain injury in 2012 that scans done showed the same enlarged ventricles as 2005 scan. Cognitive stuff also was happening to the point of making me think I no longer want to be in this marriage but I stayed, he fell, rehabbed for months, told he would never work or walk but beat the odds and does have a good quality of life but will never drive his miata, will now watch someone else use his chainsaw to clean up the 8 trees the beavers destroyed last year and probably not walk his beloved dog Chaos again. He was only 67.

Even with the defective shunt, endoscopic third ventriculostomy (ETV) and a subacute subdural hematoma that shifted the midline of his brain 14 mm exactly four weeks after ETV surgery, I would not hesitate to make the same medical decisions as I have. It’s my project to keep him with me for as long as possible. There are no guarantees that shunt will work or that surgical complications won’t happen but not doing it will make your caregiver want to take you to the woodshed eventually and likely reduce caregivers life expectancy since that’s what NPH, Alzheimers and other dementias do to those who have to change adult diapers, clean the bedding too frequently, not be able to leave untreated NPHer alone for a minute, etc.

You will find out that there is really no formula for the doc to follow when first setting your shunt in op room, or to turn shunt up or down if symptoms recur. Doesn’t give us the warm and fuzzies buts that’s where this disease is. So my advice while you have the time, brain power and great fortune to have been diagnosed is:

  1. Find a neurosurgeon that YOU and family can work with. We had no choice because of emergency situation. Ours was a skilled surgeon but Fuggedabout his bedside manner and awareness of his patients predicament. Don’t stop until you find one who treats you like his/her own self;
  2. Before surgery find out how often your neurosurgeon wants to follow you. Ours said come back in a year and our university so called top notch NS had patients return as often as every 2 weeks for office evaluation.  Somewhere in between is the appropriate frequency but it’s baffling that one says 14 days while other says 365!!!!
  3. Don’t leave hospital without prescription for physical therapy. Even if you graduate the day you start it’s better to be evaluated once discharged than by the hospital physical therapists who’s primary goal is to get you to a level that meets discharge criteria. For my sweetie after serious complication he was discharged after walking 20 steps back and forth in the hallway!! The outpatient PT was good for his physical as well as cognitive well being and not to forget his morale since his PTs were all pretty young women gushing over every teeny improvement and even misstep. So spend time identifying a good PT facility that knows neuro rehabilitation because it’s not the same as broken toe PT.
  4. Any upset of your body’s status quo can aggravate mild symptoms and even make long gone symptoms come back for a visit. UTIs are particularly troublesome because one can have them and not know it. That’s why the dementia folks call them silent UTIs. So keep fresh UTI test strips at home and tell family members that if they observe any “odd” behaviors then check for UTI, if positive or negative at home and still behaving off after a day get into your PCP for evaluation.
  5. Educate your PCP if you don’t have one of the very few who even knows what NPH stands for. Ours admitted he had no idea so we talked and stuck with him because he always responds to my calls and fits us in that or the next day.  On one occasion he actually asked what should he do and I said call the neurosurgeon. He stayed late to make sure he got the call back and arranged for a lumbar puncture within 2 hours at local radiology facility.  He even came over to make sure we got set up okay. Turned out neurosurgeon should have said get a scan which I had thought would have been requested. Instead I ceded to neurosurgeon and 2 days later I rushed my sweetie to the ER for an emergency brain surgery!!
  6. Get your docs to talk about you before and after surgery. I can’t tell you how horrible it feels when neurosurgeon says that’s for the other doc or good luck with that issue. No I just deal with your brain. But if you are anything like us your brain is connected to your toe nail eventually.

NOT TO TREAT – WHAT WILL HAPPEN?

Most of the NPH research studies that have been done are on extremely small numbers of patients so results are typically not statistically significant and the focus is on shunt efficacy and complications. The progression of a shunted as well as non-shunted NPHer is simply undocumented in the medical literature. Caregivers report that the whatever symptoms show up just get worse and worse. If you find information on the progression of untreated NPH please send it over.

WHY HAS GETTING A DIAGNOSIS BEEN SO DIFFICULT?

The medical community is not sufficiently educated about NPH.  Reference:  https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2442723/pdf/yjbm_81_1_19.pdf

Click to access yjbm_81_1_19.pdf

WHERE TO FIND USEFUL INFORMATION 

The Hydrocephalus Association has greatly expanded its links to research and other articles about NPH since my journey between 2012-2016.  The links on this page were the ones that helped me the most.  I am no longer a caregiver but occasionally update this page.  Since updates are not frequent the Hydrocephalus Association is a good resource for current NPH information.

YOUTUBE is also replete with good and informative videos about NPH.  

PROGNOSIS RESEARCH 

Quoting directly from: http://www.ahcrn.org/new-insights-into-normal-pressure-hydrocephalus/

“A recently published study from researchers at the University of Gothenburg attempts to shed light on the long term outcomes of untreated iNPH by examining mortality rates, risk of dementia, and symptom progression in individuals with ventricular enlargement. The study was led by Daniel Jaraj, MD, PhD, a member of the HA Network for Discovery Science.

The research group obtained baseline information on the elderly population of Gothenburg, Sweden from 1986-2000. The study included 1,235 participants, all aged 75 or older. No patients with treated iNPH were included. Participants were divided into four groups based on radiological and clinical indications:

Non-iNPH: consisting of participants who had no sign of ventricular enlargement upon computed tomography (CT) scan and did not exhibit any of the core clinical symptoms (cognitive impairment, gait disturbance, and urinary incontinence).

Asymptomatic Hydrocephalic Ventricular enlargement (HVe): consisting of participants who showed ventricular enlargement but did not exhibit the core clinical symptoms.

Possible iNPH: consisting of participants who showed ventricular enlargement and exhibited at least one of the three core clinical symptoms.
Probable iNPH: consisting of participants who showed ventricular enlargement and exhibited gait disturbance as well as either cognitive impairment or urinary incontinence.
Five-year mortality rates were similar in non-iNPH patients, asymptomatic HVe patients, and possible iNPH patients. However, probable iNPH patients had a significantly higher five-year mortality rate of 87.5% (Table 1).

Table 1
Dementia developed in 20.6% of non-iNPH patients while 68% of patients who showed radiological indicators of iNPH (symptomatic HVe, possible iNPH, and probable iNPH) either had dementia at baseline or developed dementia during the study period.

Furthermore, almost all participants with enlarged ventricles showed some sign of symptom progression, and nearly 50% of asymptomatic HVe and possible iNPH patients developed probable iNPH during the time of the study period.

This study was unable to directly compare outcomes of untreated iNPH with treated iNPH. However, the authors hypothesize that radiological indicators (e.g. enlarged ventricles) develop before clinical symptoms (Figure 1). Surgical or other interventions at this early time-point may lead to better outcomes for iNPH patients.”

For more information on this study https://www.ncbi.nlm.nih.gov/pubmed/28238737

MEDICAL GUIDANCE

There are no established standardized protocols for diagnosing, treating and following suspected and/or confirmed NPH patients.   If one is fortunate to have a physician who knows about NPH and who pursues or rules out a diagnosis, treats and monitors, most likely that physician is approaching it differently from any other physician.  Every patient seems to follow a very different path.  Recently, Relkin and others, assembled guidelines for NPH diagnosis and treatment.   Unfortunately they are not recognized by any national or internationl medical authority but they are a very useful tool for patients, caregivers and advocates to use to ensure that the best and most thorough approach is followed.  I implore all NPHers to give copies of these documents to all their providers so the word can get out that NPH can be assessed methodically.

DEVELOPING MEDICAL GUIDELINES – WHY & HOW

http://www.unilim.fr/campus-neurochirurgie/IMG/pdf/part%201%20development%20of%20guidelines%20for%20NPH.pdf

DIAGNOSING NPH

Click to access part%202diagnostic%20of%20NPH.pdf

MEDICAL TESTS

Click to access Value%20of%20Prognostic%20Tests%20for%20INPH.pdf

SURGICAL MANAGEMENT

Click to access Surgical%20Management%20of%20INPH.pdf

SURGICAL OUTCOME AND ASSESSMENT

Click to access part%205%20outcome.pdf

5 thoughts on “Normal Pressure Hydrocephalus (NPH) – To Treat or Not to Treat & Medical Guidance

  1. Somehow I just knew Agent that you would know much more than most. To think 1/3 of physicians had never heard of NPH is incredible. At least the VA radiologist knew of it and it showed up in Dhs report. Since connections on Alz doesn’t seem to work well, I’d love to be able to correspond with you via email. Feel free to send me emails whenever you like.
    Currently Celi on Alz.

  2. Most complete and realistic I have found.
    Mention of family and caregivers shorting their own lives is no joke. One had best be prepared mentally. The stress of dementia. The patient I pcg has been in denial for so long that without training I myself ended up with a disconnected thinking.
    This person I have known over 40 years.
    The forms of dementia I have seen him exibit are varied and seem to come and go…..except the Triad. Best wishes to any and all who end up as attendent.
    Counsuling or seminars maybe a class. This stuff is deadly to everyones mind and body without.

    • Thank you for your comment. I apologize for taking so long to respond.

      You mention that the person with dementia is in denial. That may be the case but there is a neurological condition called anosognosia. Few doctors know about it and I have been “corrected” by one who said it’s agnosia. Well it isn’t.

      Anosognosia is the inability to recognize cognitive deficiencies. It is very common in dementia. It is an outcome of damage to the brain by the dementia process. Here’s a detailed discussion:

      Click to access Anosognosia.pdf

      I hope this information will be helpful in communicating with your patient and with your thinking.

      Also, Teepa Snow is an occupational therapist who has specialized in dementia. There are wonderful videos showing how to caregive for dementia patients. Her focus is mostly Alzheimers but I found the advice very helpful in many situations with my late sweetie.

      If you google Teepa Snow youtube you will find treasure trove of useful information.

      Please find some time/method to destress yourself. It’s very hard to focus on ourselves when caregiving. There’s the guilt, fear, constant tension, etc. My major constant stressor underlying everything else was what would happen to my sweetie if I became incapacitated or died. I had the hardest time trying to figure out a plan for that scenario.

      Please write back if you feel like it. I promise not to take so long to respond. Plus I highly recommend reading and/or participating on the Alzheimers on=line support group. It saved my life during our journey.

      http://Www.alzconnected.org

      All my best,

  3. My brother was recently dx with NPH and he is struggling to have surgery.
    He had open heart surgery over a year ago with a right valve replacement also has Sogren disease..COPD and neuropathy. Do you need he is a good candidate for surgery

    • Hi Elda,

      I’m sorry to hear about your brother’s illnesses and the NPH diagnosis. I am not a doctor so I can’t tell you if he is a good candidate for surgery. The shunt surgery is not very complicated despite being brain surgery. It is quick and been described as easier than tonsillectomy. Typically the patient is discharged within a day or two. Post surgical improvement is may not be immediste. There is no scientific basis for the shunt setting that might reduce symptoms. It is important to have good relationship with the neurosurgeon & frequent followups to assess shunt efficacy.

      Is the neurosurgeon unwilling to do the surgery or have his other doctors warned against it? He should get second opinion from another NPH knowledgeable neurosurgeon. Johns Hopkins has a second opinion process that does not require travel to the hospital if your brother lives far away or can’t travel to Baltimore, MD. They will need copies of his medical records. Here’s a link to the site:

      https://www.hopkinsmedicine.org/second_opinion/neuro/services/

      I hope they can help if you contact them.

      In addition you can contact Debbi Fields who is the founder of the National Hydrocephalus Foundation. She maintains a list of neurosurgeons who are well regarded by other NPHers & caregivers.

      Debbi Fields
      12413 Centralia
      Lakewood, CA 90715-1623
      (562) 402-3523
      hydrobrat@earthlink.net
      debbifields@nhfonline.org
      http://www.nhfonline.org

      Your brother is fortunate that you are his advocate. I know how you & your brother are struggling. My sweetie died in 2016. after the NPH diagnosis/surgery in 2012, his right lung was removed due to lung cancer, he had heart valve surgery, experienced several serious complications from NPH related surgeries and some other serious illnesses.

      Please feel free to continue to contact me. I will be thinking of you & your brother and hoping for the best.

Leave a Reply

Fill in your details below or click an icon to log in:

WordPress.com Logo

You are commenting using your WordPress.com account. Log Out /  Change )

Google photo

You are commenting using your Google account. Log Out /  Change )

Twitter picture

You are commenting using your Twitter account. Log Out /  Change )

Facebook photo

You are commenting using your Facebook account. Log Out /  Change )

Connecting to %s