Manoucher Manoucheri, MD, Internist and Faculty Member at Florida Hospital Internal Medicine Residency and Associate Program Director in charge of the NPH Program at Florida Hospital talks about the signs of NPH and how it is treated.
Interview conducted by Ivanhoe Broadcast News in March 2018.
Is there a title associated with the NPH program?
Dr. Manoucheri: No, I just oversee the program when the patients are in the hospital for the lumbar drain.
The initials NPH, what does that stand for and what are we talking about?
Dr. Manoucheri: Normal Pressure Hydrocephalus stands for increased cerebrospinal fluid accumulation in the brain. Cerebrospinal fluid or CSF is a clear colorless fluid that baths the brain and spinal cord. Some patients have difficulty absorbing the CFS fluid. In other words cerebrospinal fluid is produced in one part of the brain and then circulates around the brain and is reabsorbed. If the patient is unable to reabsorb the fluid appropriately then the fluid accumulates and causes the enlargement of the ventricle hence the terminology normal pressure hydrocephalus.
Does this happen over a period of time or is this something that can pop up rather quickly?
Dr. Manoucheri: Normal pressure hydrocephalus is sort of an insidious problem. It could gradually develop over a period of time. One of the most prominent features of normal pressure hydrocephalus is gait abnormality. Elderly patients find themselves with poor balance, a shuffling gait. It is often misdiagnosed with Parkinson’s disease. Then the cognitive decline could develop also. And some patients also have urinary incontinence that could be kind of confused with overactive bladder.
Does anyone know what causes this?
Dr. Manoucheri: In general normal pressure hydrocephalus, about fifty percent of them have an etiology that could be caused, it could be previous head trauma, a bleed either subarachnoid bleed, intraventricular bleed from head trauma or a hemorrhagic stroke. Also it could be caused by previous episode of meningitis. Inflammation of meninges may affect the areas of the brain that help to reabsorb cerebral spinal fluid. The other fifty percent cause idiopathic but really nobody knows what causes it.
You had mentioned some of the symptoms, is there a concern that patients may be misdiagnosed or may dismiss some of this as normal signs of aging as their gait starts to change?
Dr. Manoucheri: The classic triad of normal pressure hydrocephalus is gait abnormality, cognitive decline and urinary incontinence. Not everybody has that classic presentation. Some do, but you would like to diagnose these patients who are presenting early with symptoms and may not present with the classic triad. There are a number of neurodegenerative conditions of the brain that could be confused with NPH because somehow similarities that represent with gait abnormality, with cognitive decline. One of the examples would be Alzheimer’s disease which is very common. That could present with cognitive decline, memory issues, and executive function decline. Other conditions that could mimic normal pressure hydrocephalus obviously is Parkinson’s disease. Some of these patients also develop dementia. So it is critical to diagnose NPH early because it is the only reversible condition that we can actually help the patient with. Other neurodegenerative conditions unfortunately you can manage symptoms but there’s no reversibility.
Let’s talk about diagnosis. How do you go about determining that this is NPH and not something else?
Dr. Manoucheri: We diagnose normal pressure hydrocephalus based on clinical presentation and also imaging. Imaging modalities that are used for normal pressure hydrocephalus either include an MRI or a CAT scan of the brain. On these imaging’s you have characteristic changes. One most prominent one is disproportionate ventriculomegaly (enlarged ventricles). As we get older our brain shrinks a little bit but usually that atrophy that occurs with aging is proportional. When you see this proportion that enlargement of the ventricles without change in the sulci outside of the brain then that supports the possibility of normal pressure hydrocephalus. And then the program that we’re doing it for the hospital consists of looking at the imaging, looking at the clinical presentation to the neurologist or the clinical physician, primary care physician. And then to enter the program they get complete neuropsychiatric evaluation before the lumbar drain placement. They get a physical therapy assessment including walk tests and gait assessment. And the lumbar drain is placed; we remove fluid periodically for forty eight hours. And then on day three we do an assessment again with a neuro psych evaluation and physical therapy and then determine if there is sufficient improvement in any of those parameters. Diagnostic studies to justify shunt placement.
If a patient has NPH, will you see improvement when some of that fluid comes down?
Dr. Manoucheri: We have seen some dramatic improvement on the second day and for sure on the third day. Some patients develop improvement after a week or so. And those are kind outliers. In our experience a two day drain sufficiently gives us enough diagnostic or positive predictive value to assign patients to be shunted.
After the two day process if doctors are convinced that NPH is the diagnosis what is the next step then?
Dr. Manoucheri: Once the patient completes the lumbar drain all the data are presented to what we call Normal Pressure Hydrocephalus Committee with representatives from nursing, neurosurgery, neuropsychology and physical therapy. And we look at all the data. And then we’ll recommend to the patient that you are not a candidate for shunting. And then they’re scheduled for shunt placement if they agree.
Tell me a little bit about the shunting, what does that involve and again how quickly do the patients symptoms start to reverse?
Dr. Manoucheri: The shunting is done by neurosurgery, it is basically a tube placed in the ventricle and subcutaneously goes in to peritoneal cavity. The amount of drainage is controlled by the device. Most of the shunts that are placed by our neurosurgeons are programmable. So they can control how much CSF fluid is drained. CSF fluid drains from the ventricle cavity in to the abdominal cavity, peritoneal cavity. It keeps the pressure down.
And then the body just rids itself of the fluid?
Dr. Manoucheri: Absorbs it. It takes the pressure off of the brain. And how quickly they respond, some patients respond fairly quickly. This is like I mentioned before we can notice during the program from day one to day three we see significant improvement in the neuro psych evaluation and also gait assessment.
What do patients need to know about this condition? What would you recommend, this will go out to a broad audience across the country and what would you like them to know about what they should be looking for or what family members should be looking for?
Dr. Manoucheri: Our recommendation is that the adult population especially the older population who are predisposed to normal pressure hydrocephalus keep some of the symptoms in mind. Not every gait abnormality is because I’m aging, I’m incontinent because I’m old, I’m losing my memory because I’m elderly. We have to remember a number of these conditions could be caused by a neurodegenerative process that is not reversible. We do not want to miss anyone who has early manifestation of normal pressure hydrocephalus which is potentially reversible.
What’s the frequency in the general population? First of all is this sixty five and older we’re talking about, fifty five and older, is there an age?
Dr. Manoucheri: The majority of the patients who present with idiopathic normal pressure hydrocephalus are older. Younger patients could present with normal pressure hydrocephalus if there was an episode of head trauma, bleed or meningitis. Normal pressure hydrocephalus could occur at any age but the unknown cause tends to be more age related.
More men or more women or is it equally distributed?
Dr. Manoucheri: I couldn’t tell you.
The frequency in the older population, how often is someone diagnosed with normal pressure hydrocephalus, how many cases are there across the United States? Just in a quad.
Dr. Manoucheri: I can’t be sure but the incidence could be anywhere from two to twenty per million incidents. The prevalence is a little bit more. But it is undiagnosed, we don’t know. A lot of people walking funny out there do they have normal pressure hydrocephalus. It could be estimated maybe as much as one or two per hundred thousand. But that may be underestimated because of misdiagnosis. The patient could be diagnosed with Parkinson’s disease or they have Alzheimer’s disease or Louie Body Dementia or Picks disease, other neurodegenerative processes. The prevalence is maybe underestimated. But the new cases that actually diagnose may be anywhere from two to twenty per million and you have to verify that.
Is there a cure for this?
Dr. Manoucheri: Potentially there is a cure for normal pressure hydrocephalus. As I mentioned that’s the only neurodegenerative process that we have a cure for it. Now we have seen patients dramatically improve over a couple of years and then they decline because something else develops. And that something else could be a stroke, could be Alzheimer’s disease or Parkinson’s disease. But clearly if normal pressure hydrocephalus is the only condition it’s curable.
And the shunt is that a onetime process or can it be done again if there is buildup, or would there be buildup again?
Dr. Manoucheri: If the shunt is working it is a onetime placement. And with the newer shunts that are programmable it is very easy to control the amount of flow so they don’t get side effects. If they drain too much fluid headache is one of the complications. Potentially it’s a onetime deal and it’s done.
Is there anything I didn’t ask you that you would want to make sure that people know?
Dr. Manoucheri: Complications, that’s kind of rare usually.
The fact that you have a center here in an area where there’s an elderly population is it unusual to have a center or did Florida Hospital see a need in this area?
Dr. Manoucheri: Actually it was started by somebody else, a previous neurosurgeon. Central Florida being a retirement destination we may be seeing more of these patients. We’re getting approximately fifty plus referrals to our center annually. I’m not sure if this is the biggest center around the country but I’m confident we probably have the most experienced in the country because we’ve had over four hundred seventy patients entering the program during the past eight years. The location of the program being in the middle of Central Florida, older population, retirees and we’re getting more referrals from neurologists and primary care physicians.
I wanted to ask you about your patient Betty, do you remember what she was like when you first saw her?’
Dr. Manoucheri: Betty presented to us with a shuffling, progressive decline in her memory and executive function. She came in and she was entered in the program, the two day program. The lumbar drain was placed, I think totally we removed over three hundred ml of CSF fluid. She had dramatic improvement within the hospital. We quickly referred her to neurosurgery after discussion with our NPH committee meeting. And she has done dramatic improvement, both in her gait assessment. There’s a measure called Berg score which assess gait. And I have the data in front of me. Her Berg score went from thirty four to fifty five in one year. And fifty five is normal, there’s no gait abnormality. Thirty four basically is you need a walker and a cane to get around. Her neuro psych evaluation went from eighty nine to one oh five in two days and to one nineteen which for her age that’s normal. Normal neuro psych evaluation. And she has done quite well.
So this is a case where there was dramatic improvement?
Dr. Manoucheri: Dramatic improvement.
After taking a little bit of fluid out there’s no doubt in your mind that this was a patient with NPH?
Dr. Manoucheri: Yes.
END OF INTERVIEW
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