Understanding the brain disorder with a confusing name that struck Billy Joel

As Billy Joel’s family rallies around him, a lot of fans are left wanting more information about the brain disorder that has taken him off the road and into treatment. Joel announced on May 23 that he’s canceling all concerts after being diagnosed with normal pressure hydrocephalus. 

MUSC Health neurologist Lidia Yamada, M.D., explains what that is, the symptoms it causes, treatments and more in this Q&A.

Q: What is normal pressure hydrocephalus? 

A: Normal pressure hydrocephalus is a condition where excess spinal fluid builds up in the brain’s ventricles (the spaces normally filled with clear fluid inside the brain), stretching them out. That fluid puts pressure on areas responsible for walking, thinking and bladder control. The term “normal pressure” refers to the fact that the fluid builds up slowly over time, giving the brain tissue a chance to adapt, so pressure readings often appear normal even though the ventricles are enlarged and the brain is being compressed.

Q: What was your reaction to hearing that Billy Joel has it?

A: I appreciated that Billy Joel and his team were open about the diagnosis, since most people have never heard of normal pressure hydrocephalus (NPH). It’s often underrecognized, and public awareness could help people who are struggling with similar symptoms. 

 

But I also have some concerns. NPH is sometimes described as a “treatable” or “reversible” cause of dementia –and while treatment is a potential option for those rare individuals who truly have NPH, that’s not the case for most other causes of memory loss. I worry that the headlines might make it sound like most forms of dementia can be fixed with a shunt, and that’s not the case. NPH is a rare condition compared to more common causes of gait problems and dementia, like Alzheimer’s or Parkinson’s disease.

And even among patients who do have NPH, not everyone improves with treatment. So while I’m glad this is starting a conversation, we have to be careful not to oversimplify a complex medical issue, especially when it involves something as serious as brain surgery.

Q: You referred to dementia. Is that one of the effects of NPH?

A: The three “hallmark” symptoms are:

• Difficulty walking (typically, but not always, a slow, shuffling gait, like the person’s feet are stuck to the floor).
• Memory problems (often described as forgetfulness, difficulty with tasks or “foggy thinking”).
• Urinary incontinence.

Sometimes all three are present. Sometimes it’s just one or two. Patients often come to us with different combinations of these symptoms and at various stages. 

Because these issues also show up in much more common conditions, like Parkinson’s, Alzheimer’s disease or what we call vascular dementia, it can be hard to pinpoint the exact cause. That’s part of what makes diagnosing NPH so tricky.

Q: Is it treatable?

A: It is, in some patients. In the evaluation we do in our NPH clinic, after making sure there isn’t another diagnosis that better explains the patient’s symptoms, we usually admit them to the hospital and start with something called a lumbar drain trial. We slowly and continuously remove spinal fluid through a small catheter in the lower back and observe whether the person’s walking or thinking improves over the next couple of days. If there’s noticeable improvement, we might recommend a VP shunt. This is a surgically implanted tube that drains excess fluid from the brain into the belly, where it can be safely absorbed.

But it’s not a magic fix, and it doesn’t work for everyone. Even in those who improve with the VP shunt, the benefit is typically temporary, not a cure. Research is still ongoing to understand who will benefit most from shunting. We don’t yet have perfect predictors, which is why evaluation by a team experienced in NPH is so important. This type of individualized approach really matters.

Q: What causes NPH?

A: The most common form of NPH in the elder population is called “primary” or “idiopathic” NPH, meaning we still don’t know what causes it. It tends to develop gradually over time, which is part of what makes it so easy to miss early on. People who have had brain surgery, bleeding in the brain, a tumor or certain infections may also be at higher risk for what we call “secondary” NPH, which tends to occur more often in a younger population compared to idiopathic cases.

Age is the main risk factor for idiopathic NPH. Most patients we diagnose are between 60 and 80 years old, and the likelihood increases significantly after age 65. That said, it’s still considered rare. Research suggests a prevalence of about 0.2% in people between 70 and 80, with some studies showing it could rise to 5% to 6% in those over 85. 

Q: A statement on Billy Joel’s social media said his condition has been exacerbated by recent concert performances, leading to trouble with hearing, vision and balance. Can you talk about why his performances might have made his condition worse? 

A: Performing takes a toll on the body. There’s the stress, the lights, the travel and the exhaustion. Any kind of underlying brain dysfunction, including NPH, can become more noticeable under that kind of stress. So it makes sense that his symptoms might have shown up more during concerts.

That said, it’s important to note that hearing and vision issues are not typically idiopathic NPH symptoms. The condition primarily affects gait, cognition and bladder control. If someone is experiencing sensory problems, we usually look for other explanations as well.

Q: What else would you want people to know about NPH?

A: Not every gait or memory issue is NPH. But if someone you care about is walking differently, getting more forgetful or having bladder issues, they deserve a proper evaluation. That starts with seeing a neurologist and getting brain imaging. 

We created a dedicated NPH clinic at MUSC Health because we were seeing too many patients being misdiagnosed, some overlooked entirely and others getting surgery without enough evidence to support it. 

We don’t want to miss a diagnosis that could change someone’s life, but we also don’t want people undergoing brain surgery without clear indication. I hope Billy Joel’s story brings more attention to NPH but also reminds people that diagnosing and treating it require experience. Awareness is powerful but so is getting the diagnosis right.