Normal Pressure Hydrocephalus

15-42 Normal Pressure Hydrocephalus

Reed Pence: Seventy-three-year-old JD Cain is a former aerospace inspector who lives in southern Indiana. A little more than 15 years ago he started having some medical problems.

JD Cain: I had a little shakage in my hand and got a little concerned about it. It seemed to be getting worse so we went to a neurologist. I was [also] having bad headaches. That first [neurologist] diagnosed me with Parkinson’s, so he treated me. It just kept getting worse and worse and my quality of life was going downhill pretty bad.

Pence: JD’s wife, Donna, says the shaking in her husband’s hand was just the start. JD started having bathroom accidents, and nothing the doctors did could help.

Donna Cain: The incontinence just kept getting worse until finally he had to wear a diaper. He went to one neurologist and he went to the other one and every time he’d go back and tell him he was weaker. [The doctor] just kept upping the medicine. They had him on nine pills a day on one medicine and three pills a day on another medicine. It was not doing him any good.

JD Cain: It was so horrible it was like taking care of a baby. I was in diapers and I couldn’t control my bladder. It was a mess. Also, I was shuffling. I fell down and crawled into the bedroom trying to get up and I was down on the floor for six and a half hours. I did not have any strength at all. I fell down our stairs three or four times. It’s a wonder I hadn’t killed myself. It was like that constantly.

Pence: JD says he also started forgetting things as if he had dementia. He started losing his temper at the slightest provocation. But the Parkinson’s medication did nothing for any of his symptoms. In fact, switching to a new drug seemed to trigger even more problems.

JD Cain: I had [gone] to Texas about work and when I stopped on the way back I couldn’t control my gambling. I always did like to play a few cards, but never anything like it was getting to be. I dumped about four or five thousand dollars.

Pence: Eventually the Cain’s went to yet another neurologist, who wondered why JD had never had an MRI or CT scan to help confirm the diagnosis. So after 13 years of struggle, they got an MRI. It showed that JD didn’t have Parkinson’s at all. He had a condition that may look like Parkinson’s or Alzheimer’s disease. It’s called “normal pressure hydrocephalus.”

Marvin Bergsneider: Hydrocephalus is a condition of excessive water on the brain. That’s what the word literally means in terms of what we see as physicians. We all have fluids basis within our brains called ventricles. If they balloon up and become too large you get a condition called hydrocephalus.

Pence: That’s Dr. Marvin Bergsneider, Professor of Neurosurgery and head of the Adult Hydrocephalus and ICP Disorders program at UCLA. He says “water on the brain,” as many people know it, is common in children, but it wasn’t until the 1960’s that doctors recognized it could also be present in older adults.

Bergsneider: We don’t understand why this particular form of hydrocephalus occurs in the geriatric population. It may be that it’s just a form of degeneration of the brain that affects more the circulation of spinal fluid. Think of it as pipes in your house: as pipes get old they tend to get clogged up and the circulation is impaired. We see patients with this condition often also have vascular disease of the brain. You have tiny little strokes, not large strokes, and many of them are clinically silent. But more patients with normal pressure hydrocephalus do have vascular disease. Whether one is a risk factor for the other, which one’s the cart and which one’s the horse, we don’t know.

Pence: Bergsneider says as a result of the clog, cerebrospinal fluid can’t drain from the inside of the brain as it normally does. It’s a little like a normally fast-flowing stream.

Bergsneider: If a beaver puts a dam you get a pond or a lake above the stream. Presumably something like that is occurring where there is a blockage of the normal flow of fluid and the ventricles violate up just like the lake does.

Pence: When those ventricles overfill they affect the nerves nearby, such as those controlling movement of the legs.

Bergsneider: If these ventricles balloon up, the connection from those brain cells to your spinal cord get stretched the most. Those connections have to loop around those large ventricles versus the control of the hand, for example, is on the side of the brain and as the ventricles enlarge those fibers and nerve cells just get pushed to the side, but they didn’t get stretched compared to what the leg fibers do. That’s a theory of why difficulty with walking is the predominant finding with normal pressure hydrocephalus because the leg fibers are preferentially stretched.

Pence: Bergsneider says neurons responsible for bladder control are also in the same area.Blood flow to those nerves can also be impaired by overfilled ventricles, cutting nutrients and leading to an even further impairment. Incontinence and dementia are often hallmarks of normal pressure hydrocephalus or NPH, b.ut troubles with walking show up most often. Many patients say they have a shuffling walk with extremely small steps.

Bergsneider: Patients with this disorder — often it’s as if their automatic walking system is failing. They aren’t able to do those simple tasks. They have difficulty initiating gait. When they get going they take very short steps. If they make a turn they take very small steps and take more steps than other people of matched age would do to make a turn. They have difficulty going up a step or walking in gravel. But the same patient, if you stop them and you say, ‘okay think about what you’re doing. Take a large step with your left foot and then your right foot,’ they can march right down the hallway.  But if they get distracted then these short steps come about. The original description was that of a magnetic gait. It’s a good description for these patients who really have this as if their feet are stuck to the ground.

Pence: Bergsneider says “short steps” are also part of Parkinson’s disease. But it’s rare for normal pressure hydrocephalus to include Parkinson’s characteristic tremor. Still, the Hydrocephalus Association estimates that as many as five percent of people diagnosed with Parkinson’s or Alzheimer’s disease actually have NPH and could be treated.

Bergsneider: Difficulty with walking, difficulty with bladder control and difficulty with memory — if you walk into any nursing home or talk to patients who are aging, those are very common symptoms of aging in general. But a real challenge for physicians and patients is to figure out what is it about their characteristic findings or the symptomatology that they’re having?  Can [these symptoms] alert them that this is not just normal aging or this is just not some other cause that’s causing this.

Pence: However, Bergsneider says there’s a significant lag between the start of symptoms and irreversibility. Often, before the disorder is seen, it’s been present for some time eating away at the brain’s reserves.

Bergsneider: You don’t become clinically symptomatic until you start losing that reserve where you can no longer compensate. That’s when the symptoms become apparent. When your mother or grandmother starts saying, ‘Wow there’s something wrong,’ well she’s probably had this long before. You’re noticing it because the reserves have been used up. It’s very typical for NPH that when the patient’s tired, they haven’t had a good night’s sleep or they have a minor illness like a bladder infection, that that’s when their symptoms come out.

Pence: If NPH continues untreated, eventually the reserves are completely gone, and damage becomes irreversible.

Bergsneider: It’s a progressive disorder and one that if you don’t treat you will end up in a wheelchair incontinent of urine and demented.

Pence: Bergsneider says a CT scan or MRI can often clearly show whether the ventricles of the brain are swollen. But that doesn’t always mean a patient will respond to draining off cerebrospinal fluid. So he says at UCLA, they do a test first.

Bergsneider: We bring them into the hospital, we put in the very small catheter into their spine, a drainage catheter. We literally drain spinal fluid for a couple days. I call that the “WYSIWYG” procedure — what you see is what you get — because patients will improve right away. But once you have the disorder they will go from some of them unable to walk to two days later walking around happy as clams. Their bladder control has returned, the family says that they’re cognitively much clearer, they’re able to play cards again. Once we pull out the drain the affect will last for about a week, then it goes away. For those patients who improve and then get worse again, in essence, we’ve done a test shunt, but if you take that picture, 90% of those patients will get better with a shunt.

Pence: A shunt is a tube-like device implanted under the scalp that drains excess spinal fluid from the brain into the abdomen. It needs to be closely monitored, so the right amount of fluid is removed. Having too little cerebrospinal fluid can be fatal. But once it’s adjusted correctly, the Cain’s can tell you it works.

Donna Cain: It was almost immediate to me. By the time he left the hospital his incontinence, that was the first thing, it went completely away. I don’t think I ever hear him say ‘I don’t feel good’ in the morning now when he wakes up. And that was every morning “I don’t feel good”. But it was almost instantaneous after he had that surgery; it was just remarkable.

JD Cain: You cannot believe how much better and how fast I felt better. I have no doubt that they hit the nail right on the head because life isn’t a dark bleak thing anymore. I look forward to getting up. I am back, back to a good life.

Pence: Bergsneider says the earlier NPH is treated, the better. Some patients respond well to a shunt, but their brain reserves are so depleted that they backslide within a year or two. Others show no more symptoms for 15 or 20 years. And it can be a major turnaround for someone who had thought they had Alzheimer’s or Parkinson’s disease and were getting worse by the day. You can find out much more about normal pressure hydrocephalus online at lifenph.com… Or through a link on our webpage, radiohealthjournal.org. I’m Reed Pence.