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.
My 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:
- 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;
- 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!!!!
- 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.
- 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.
- 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!!
- 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
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.
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).
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
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