What to know about normal pressure hydrocephalus as Billy Joel cancels tour

The brain disorder that forced Billy Joel to cancel his concert tour can cause trouble walking and thinking straight — but is often treatable and can even be reversed, doctors said.

A post on the 76-year-old singer’s Instagram on Friday said that he was recently diagnosed with normal pressure hydrocephalus and that concert performances exacerbated problems with his “hearing, vision, and balance.”

The condition is relatively uncommon, according to the Cleveland Clinic, affecting “about 5.9% of people over 80.”

It’s most commonly diagnosed in older adults, usually showing up around age 70.

What is normal pressure hydrocephalus?

The human body is constantly trying to keep a steady level of cerebrospinal fluid to cushion the brain and spinal cord, either by making more or absorbing it.

That fluid tends to take up a little more space in older adults because brain volume shrinks as a normal part of the aging process, said Dr. Wajd Al-Holou, a neurosurgeon at the University of Michigan Health.

“But what happens in normal pressure hydrocephalus is an abnormal buildup of fluid that causes brain dysfunction because of the pressure it’s putting on the brain,” he said.

What are the symptoms and how is it diagnosed?

Patients are often diagnosed after they see a doctor because they — or a family member — are concerned they might be developing dementia, which is also more common as people age.

Memory loss, bladder control issues and trouble walking are also common. Patients can have trouble lifting their feet, putting them at risk for stumbling and falling.

Doctors often learn about normal pressure hydrocephalus and its three hallmark symptoms with this catchphrase: ‘wet, wobbly and weird,’ said Dr. Vikram Udami, a neurosurgeon in private practice in San Diego.

“The ‘weird’ is the cognitive issue, typically things like memory problems or confusion, and the ‘wobbly’ stands for gait instability,” he said. “The ‘wet’ is the urinary incontinence.”

Brain imaging, either a CT scan or an MRI, is generally used to look for the buildup of fluid, as well as a variety of neurological testing.

Doctors may also do what’s called a lumbar puncture to drain the excess fluid. If the person’s symptoms improve, the condition is often diagnosed as normal pressure hydrocephalus, or NPH.

Udami said no one knows what causes the condition, but added that it’s highly unlikely that loud music or concert touring would trigger NPH.

How is it treated?

It can look like dementia, but there’s a key difference: normal pressure hydrocephalus is treatable and in many cases reversible if it’s caught early.

Commonly, neurosurgeons implant a ventriculoperitoneal shunt, or tube, into the brain that helps drain the fluid buildup into another part of the body, like the abdomen. The patient then essentially pees the excess fluid out, Udami said.

A drug called acetazolamide can also be used, as it may help the body decrease the level of fluid on its own. The medicine doesn’t work for everyone, Al-Holou said.

It was unclear Friday what kind of treatment Joel would receive. His social media post said that the singer “is undergoing specific physical therapy,” but did not give details.

That kind of therapy is usually given, doctors said, to help patients regain balance.

Any treatment is more effective if a diagnosis is made quickly. If the condition goes on too long unchecked, it could lead to permanent brain damage, Al-Holou said.

If a person seems to be experiencing early signs such as troublesome memory loss and difficulty walking, “they should see a primary care physician or neurologist to investigate further,” he said.