Singer Billy Joel, 76, recently announced he's been diagnosed with a brain disorder after having trouble with his hearing, vision, and balance.
Dr. Ryan Cheung, a neurohospitalist and medical director of the Stroke Program at Texas Health Plano, says normal pressure hydrocephalus begins with a slow buildup of fluid in the brain.
Dr. Cheung: The brain has these fluid-filled spaces in the middle. And so your ventricles, these fluid-filled spaces, can enlarge very slowly. This process happens so gradually that the pressure within the brain doesn't change. And so, hence the name normal, but it puts pressure on surrounding brain structures. And that's what causes the symptoms.
The symptoms are a classic triad of what's commonly referred to as wet, wacky, and wobbly:
- Wet: Meaning you can get urinary incontinence
- Wacky: Memory and thinking, cognitive changes. You can be more forgetful over time.
- Wobbly: You can get walking problems and changes in your gait. It can look a lot like Parkinson's disease, where you can have a magnetic gait where it almost seems like your feet are stuck to the ground, and you have trouble picking them up.
Baker: What causes this to happen, especially since this happens slowly, as you say?
Dr. Cheung: It’s idiopathic, meaning we haven't found a clear cause.
It is more common in older patients, typically 70 years and older. The incidence varies based on study to study, but it could be as small as about 0.2% of patients above 70. And above 80, probably in the 4 to 5%.
I think it is perhaps underdiagnosed and not as recognized. There are a lot of overlapping features with Parkinson's disease, as well as other dementias and cognitive impairments. And so it does take some careful teasing out and diagnosis with a neurologist, a neurosurgeon, and a medical team.
Baker: How is it diagnosed? What's the telltale sign that you look for to know that it's hydrocephalus?
Dr. Cheung: The memory changes, the walking problems, and the urinary incontinence kind of increase your suspicion for it. And so that usually presents to the patient's primary care physician who may then refer the patient to perhaps a neurologist for some of those other cognitive and gait symptoms.
After a neurologist evaluates the patient and gets a careful history, the next step would be an MRI imaging or a CT scan of the brain. This reveals the evidence of a buildup of fluid in the brain.
This often leads to another procedure called a spinal tap or a lumbar puncture, where a small needle is placed in the spine in the same sort of space where epidural anesthesia is introduced, but we draw out fluid, and we also measure the pressure within the brain. So that pressure should be normal, hence the name normal pressure hydrocephalus, and the fluid is sent off to the lab to look for any other causes of their symptoms, like infection or any other evidence of inflammation.
But after some fluid is removed, the patients are tested again. And patients who do have normal pressure hydrocephalus often notice a transient improvement of their symptoms, particularly in their walking speed and their gait pattern. And so that's where having physical therapists who can assess the gait is very helpful. And you can also repeat some cognitive tests. And these changes are typically seen sometimes hours and sometimes days after the procedure.
Baker: This is treatable.
Dr. Cheung: Once you make the diagnosis with a temporary relief in that pressure by draining off some of that fluid, then those patients are sent for neurosurgical evaluation of something called a ventral peritoneal shunt, a VP shunt. There's a small tube that's placed surgically within the ventricles, these cisterns within the brain, and it drains the excess fluid into the abdomen so that it's no longer pushing in on brain structures.
Baker: Once you have the treatment, are you done with normal pressure hydrocephalus, or is this one of those things you're never totally rid of?
Dr. Cheung: So, for a lot of the patients who do have a lot of improvement with the shunting, it does, for the most part, correct a lot of their symptoms. Down the line, they may need ongoing adjustment of the shunt, so the shunts are adjustable, and you can change the settings on that. So if they need more or less drainage, sometimes that can come up in the future. But in general, we would expect their gait, their walking, to improve, as well as their cognitive abilities.
Baker: So then the question becomes, because none of this sounds like a great experience to go through, is normal pressure hydrocephalus preventable at all?
Dr. Cheung: Unfortunately, we don't know of any ways to prevent NPH, nor do we know what causes the underlying etiology. I would recommend to my patients, though, staying active and maintaining a healthy lifestyle to maintain brain health, because there are a lot of other things associated or that can look similar to NPH. Things like dementia and cognitive impairments, and strokes, these things that some of them are preventable, or things that we can do to delay the onset of these things.
Baker: The reason why I ask in part is this illness, this condition, has gained a lot of attention because of Billy Joel. And so it makes you wonder, since we're not quite sure what causes it, what might cause it? And with someone like that, an active entertainer, is it your lifestyle? Is it how much you might move on stage, or perhaps overdoing it with some activity?
Dr. Cheung: As far as we can tell, it's not related to any sort of activity patterns or anything that he may have done or any of the other patients with NPH have done.
The biggest risk factor is age, and Billy Joel is 76 years old, and that puts him right in the middle of that category where those patients are at highest risk for potentially developing NPH.
Baker: So it's just one of those things that can come with age. Anything else you want to add about this?
Dr. Cheung: I would just say that perhaps if your loved one is having some changes in their gait, maybe they're more forgetful or having some urinary incontinence, and it seems to be coming on very, very gradually, then it could be something that you could talk to your doctor about.
But otherwise, there are many other medical causes for any of these symptoms that can happen in older adults.
Baker: So, the underlying rule is when anything comes along that seems unusual, check it out.
Dr. Cheung: Absolutely, partner with your doctor because together you two are in charge of your health.
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What is normal pressure hydrocephalus? Billy Joel's condition is often treatable
