Understanding Billy Joel’s Brain Condition: Insights from NPR

Billy Joel has canceled his remaining summer concerts and revealed that he has been diagnosed with a brain condition known as normal pressure hydrocephalus.

“I’m truly sorry to let down our audience, and I appreciate your understanding,” Joel, 76, expressed to fans in a post on social media on Friday.

This condition has caused issues with Joel’s “hearing, vision, and balance,” as stated, and his doctors advised him against performing due to the exacerbation of these symptoms. Joel is currently undergoing physical therapy and remains hopeful about returning to the stage in the future.

While treatable, this condition is often underdiagnosed, according to doctors interviewed by NPR.

By sharing his diagnosis publicly, the musician could bring awareness to others, says Dr. Charles Matouk, vice chair of clinical affairs at Yale’s Department of Neurosurgery.

“Billy Joel is incredibly courageous,” Matouk states. “More individuals will become aware of this issue and may seek the help they require sooner.”

Here are three important points to understand about normal pressure hydrocephalus (NPH).

1. It occurs when cerebrospinal fluid accumulates in the brain

In healthy individuals, the body produces roughly half a liter of cerebrospinal fluid daily, says Dr. Gayatri Devi, who directs Park Avenue Neurology in Manhattan. This fluid serves several purposes, including cushioning the brain against the skull, transporting nutrients, and removing waste.

Typically, this fluid is reabsorbed through large veins in the head, but this process can fail in those with normal pressure hydrocephalus.

“Sometimes, certain narrow areas in the system can lead to a gradual buildup of proteins and other substances as we age, causing the fluid to back up,” Devi explains.

This “sludge” that results in the blockage is not well understood but is not a part of normal aging, according to Matouk of Yale.

“Since your skull is a closed box, the fluid needs an exit, or else pressure will build up,” he adds.

Though it results in increased pressure on the brain, it’s termed normal pressure hydrocephalus because the increase occurs so slowly that the pressure remains within the normal range, according to the Cleveland Clinic.

2. Symptoms are often overlooked

Common indicators of normal pressure hydrocephalus include walking difficulties, cognitive issues, and urinary incontinence. However, since it typically affects those over 60, these symptoms might be mistaken for signs of aging.

“Everyone knows grandparents who struggle with mobility due to hip and knee issues or back pain,” Matouk notes. “As both men and women age, they may encounter bladder control problems and memory difficulties as well.”

However, if all three occur simultaneously and worsen over time, it signals that something may be off.

However, that’s not always the case.

“What complicates matters further is individuals don’t need to experience all three symptoms,” Devi remarks. “They might just present with urinary issues, gait disturbances, or memory challenges.”

Additionally, movement problems can sometimes be mistaken for Parkinson’s disease, and cognitive symptoms might be misdiagnosed as Alzheimer’s (though Matouk warns that Alzheimer’s dementia is different from the type of dementia linked to untreated normal pressure hydrocephalus).

When normal pressure hydrocephalus is suspected, diagnosing it typically requires either a CT scan or MRI of the brain. NPR doctors mentioned they haven’t faced issues with insurance covering these tests.

However, some radiologists may not know how to identify it, making diagnosis challenging, according to Dr. Ryan Lee, assistant professor of neurosurgery at Vanderbilt University Medical Center. “It often takes persistent effort from the person who ordered the test or the family and patient” to ensure a specialist evaluates the images, he points out.

The subsequent medical test is usually a lumbar puncture or spinal tap, where doctors use a needle to collect cerebrospinal fluid from the spinal canal. They then inquire with the patient and their family if any symptoms have changed. “We also conduct a physical therapy evaluation before and after,” Lee adds, to prevent any placebo effects.

3. It is treatable

To alleviate pressure on the brain, doctors perform a surgical procedure involving drilling into the skull and placing a shunt into the brain to divert excess fluid for absorption in another area of the body—usually the abdomen.

“Since the fluid is continually produced, it’s essential to have a method to continuously drain it,” states Devi of Park Avenue Neurology.

This surgery isn’t suitable for everyone and does carry risks, such as infection, but patients typically return home the following day, she notes. The sooner normal pressure hydrocephalus is diagnosed and treated, the better the outcome.

While the surgery isn’t a definitive cure, “the results can be exceedingly impressive,” Lee of Vanderbilt states.

“Patients can transition from being wheelchair-bound and struggling with mobility and cognitive clarity to walking almost normally and significantly improving their cognitive function and quality of life,” he explains.

However, Matouk cautioned that cognitive enhancements tend to progress less dramatically than other symptoms.

Nonetheless, Matouk, who is researching a newer, less invasive procedure for these patients, is hopeful that increased awareness will empower patients to advocate for themselves.

“There are very few instances in medicine where we can reverse or improve symptoms, and this condition is one of those rare cases,” he emphasizes. “It would be unfortunate if it’s overlooked—often for months or even years—before a proper diagnosis is established.”