New Insights into Normal Pressure Hydrocephalus

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By: Noriana Jakopin and Dr. Jenna Koschnitzky, Director of Research Programs

Idiopathic Normal Pressure Hydrocephalus (iNPH) occurs when there is an abnormal accumulation of cerebrospinal fluid (CSF) which causes ventricular enlargement, often with a limited increase in intracranial pressure (ICP). The core clinical symptoms consist of cognitive impairment, gait disturbance, and urinary incontinence and are generally improved after treatment. However, it is estimated that less than one in five patients receive treatment, and there is limited information on the natural progression of iNPH.

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.

Figure 1

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 click here.

9 Comments for : New Insights into Normal Pressure Hydrocephalus
    • Karen
    • June 22, 2019

    I’m not sure if my husband has NPH but my doctor suggested it. My husband has had an MRI and has seen a neurologist 2 times and he never mentioned Ed it and I feel he has the classic symptoms with wide gait, urinary incontinense and balance issues. He has had multiple concussions but does not have demtia. I heard that sometimes MRI don’t detect NPH but a lumbar puncture can. Is this true? My Husband is only 67 years old.

    • lucretia
    • May 20, 2019

    my mother has NPH what is the life span if u treated ?

    • Betty Branham
    • January 18, 2019

    I have probably had this for about 10 years undiagnosed, then it was misdiagnosed in 2013, they just said you have brain shrinkage, it happens to everyone as they are, doctor said it won’t bother you, bye,bye. Then I mentioned to my new doc and said I have the scan can we do another and compare and we if it has shrink more. We did, but she told me she had never heard of this, this time NPH was the outcome, so I am just starting the process and am really scared, I’m 69, this is getting me down, the neurologist is going over my records they say they will call and make appt when he is done,,,HELP, I’M AFRAID.

    • Margaret Sinn
    • January 1, 2019

    My husband has normal
    Pressure Hydrocephalus. We are not sure how long he had it before it was diagnosed. A PA caught it about 4 years ago. He had a shunt put in. However if he would benefit from it was under question at the time of the surgery. At the last minute the Dr decided to put in the shunt. It lasted for about a year or year and a half. Then he began to regress. In about 6 months time the test showed that the shunt could not be turned down anymore or his brain bleeding would start and he would die. Therefore we moved closer to our daughter about a year ago. Quite honestly, because of his age (83) I feel we are being blown off by the medical world. He is perfect healthy and takes no medications at all except for a baby aspin once a day. I really would like to know the prognosis and how I should be planning for the future. At this point he is incontinent but has control of his bowels. He can use a can but really should be using a walker and progressing to wheelchair. His memory is getting worse steadily. Some times he can remember people in our past names. He is not capable of making decisions about our finances or does not want to. He surprises me sometimes. Like telling me not to move money to our investor until he the Dow was better and being interested in the last 2018 closing telling me it was about time to transfer the money to the investor. He is bright, a retired veterinarian. I really need a understanding about what is to happen in our future and how to plan for it. Can you help me at all about how this will progress and the prognosis. Thank you.

    • Reply

      Margaret, you husband’s case sounds just like my dad… her is in a dementia unit now, since he could no longer use his walked and had to be in a wheelchair and my parents home was not wheelchair accessible. His cognitive functions have spiral downhill severely after being placed in the dementia unit. I think because they are not as vigilant with dehydration, and there is little stimulate in terms of conversation. I am searching for what happens next with this disease. His renal values are climbing.

    • Robert Cesarec, MD
    • July 11, 2017

    We found your study interesting, but would like to know how long the participants were symptomatic before they entered your study. I am 66 year old physician and have had visual perception symptoms for 2 years. Then, the last 6 months I have experienced gait disturbance, mild cognitive loss, and enlarged ventricles. Do you have any idea what my life expectancy is? I currently use a can and walker. Added thought, I have fallen frequently when I lose my balance, but I do not suffer from vertigo. Thank you in advance as I find information on NPH sketchy.

    • July 11, 2017

    My brother and myself have both been diagnosed and shunted for NPH. We are POSITIVE mother had it also, but was never diagnosed. Her death certificate reads, Dementia. My brother was reshunted after about 8 years – his shunt is programable. Mine done 2 years ago and is not programable. Any studies along these lines. I am 75 and brother is 72.

    • Bernadine Kahn
    • July 11, 2017

    My husband was D.C. In 2002 He had aVP shunt INSERTED which relieved his symptoms. It lasted for 10 years then became 2012 he had another shunt. It needed repair this year . He currently is unable to walk and has severe dementia. His neurosurgeon Assures me the shunt is working but the ventricles are still enlarged. He also has seizures. We had good 10 years after being diagnosed. Now it is a full time job caring for him

    • Reply

      I suggest that you consult someone else. I, too, am having trouble. My ETV shows that it is functioning yet I have increasing cognitive disfunction. I am evaluating whether or not a spinal tap could show any improvement. If it, does, I may need a shunt in addition to the ETV Please get a second option because your and your family may be suffering needlessly.

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