The superstar singer-songwriter Billy Joel, a “Piano Man” known for decades of iconic hits like “Uptown Girl,” “She’s Always a Woman,” and “Big Shot,” has been diagnosed with a rare brain condition called normal pressure hydrocephalus (NPH).

The disorder, which mostly affects adults older than 65, led the 76-year-old artist to cancel his upcoming concerts.

What Is Normal Pressure Hydrocephalus (NPH)?

Cerebrospinal fluid is made by the brain and circulates around the brain and spinal cord, says Matthew Potts, MD, a neurosurgeon and an associate professor at the Northwestern School of Medicine in Chicago.

“In normal pressure hydrocephalus, the fluid buildup inside or around your brain disrupts your brain function, which can impact several brain-related abilities, including thinking, memory, and movement,” he says.

Unlike some other neurological conditions, NPH can result in symptoms without causing pressure on the brain — that’s why it’s called “normal” pressure, Dr. Potts adds.

Who Gets Normal Pressure Hydrocephalus?

It’s estimated that about 0.2 percent of people ages 70 to 80 have NPH, and about 6 percent of people over 80 do

“It’s possible there’s a genetic component, but that’s still being researched. Most of the time we don’t know what causes it or why it happens,” says Potts.

What Are the Symptoms of Normal Pressure Hydrocephalus?

NPH can be hard to diagnose because it’s so rare and because there are many other conditions that can cause identical symptoms, particularly in older patients, such as dementia, arthritis, or an enlarged prostate, Potts says.

The three main symptoms of NPH:

• Trouble walking, often described as a shuffling or unsteady gait
• Cognitive issues, including slower thinking or memory problems
• Bladder problems, including an unusually strong urge to pee, or incontinence

How Is Normal Pressure Hydrocephalus Diagnosed?

A diagnosis typically starts with a CT scan or MRI, which can reveal a buildup of cerebrospinal fluid.

“But that doesn’t definitively diagnose NPH, because you can actually have what looks like buildup of fluid for lots of reasons that may not cause any symptoms,” says Potts.

To make a more definitive diagnosis, a healthcare team will drain some of the cerebrospinal fluid using a procedure called a spinal tap (lumbar puncture).

“If the person’s symptoms get better, it’s more likely that they have NPH,” Potts says.

Treatment Options for NPH

The primary treatment for NPH is ventricular shunting, a surgical procedure that redirects excess cerebrospinal fluid away from the brain.

A neurosurgeon drills a small hole in the skull and inserts a shunt (a small tube) which drains fluid into another part of the body, usually the abdomen, where the body can safely absorb it.

“The tubing is completely under the skin, so the patient can then go home and that shunt will continuously drain fluid out of the brain. The goal is that their symptoms improve and they can go about their lives,” says Potts.

Most people will show improvement within hours or days of the procedure, but it is major surgery, so it may take days to weeks to fully recover.

Is Billy Joel’s Brain Condition Life-Threatening?

The prognosis for normal pressure hydrocephalus depends heavily on when doctors diagnose and treat it. The earlier, the better.

NPH isn’t a disorder that causes early death in and of itself, but complications of the condition — for example, an increased risk of falling — may affect life expectancy, says Potts.

Billy Joel Cancels All Scheduled Concerts

The singer has canceled all scheduled concert dates, including shows in football and baseball stadiums in the United States, Canada, and Great Britain.