Billy Joel cancels shows due to a brain disorder. Here’s what to know

Billy Joel has cancelled all scheduled performances in the United States and Britain because of a brain disorder called normal pressure hydrocephalus, according to a statement posted Saturday on the singer’s social media accounts.

Hydrocephalus refers to a build-up of cerebrospinal fluid, a watery liquid that cushions the brain and spinal cord. With normal pressure hydrocephalus, the fluid accumulates gradually with little to no increase in intracranial pressure. The excess cerebrospinal fluid presses on the brain and can disrupt neural pathways, causing cognitive impairments, difficulty walking and a loss of bladder control.

Joel will be undergoing physical therapy and his doctor has instructed him to refrain from performing as he recovers, the statement said. The Piano Man is cancelling 17 shows and tickets for the cancelled concerts will be automatically refunded.

How is normal pressure hydrocephalus diagnosed and treated? Here’s what to know.

What are the signs and symptoms of hydrocephalus?

There are three main symptoms – cognitive impairment, gait instability and urinary dysfunction – though not everyone has all symptoms.

“People are very forgetful, they don’t remember things or their short-term memory is really poor,” said Kurt Yaeger, an endovascular neurosurgeon and assistant professor of neurosurgery at Houston Methodist Hospital who treats patients, but has not treated Joel.

Other symptoms include mood changes, speaking less often and a loss of interest in daily activities, according to the Hydrocephalus Association, a nonprofit that raises awareness about the condition.

People with normal pressure hydrocephalus may walk with a slow shuffle as if their feet are stuck to the floor, and they may have difficulty turning corners.

“Usually, the gait disability is pretty severe to the point where they’re falling, and they’re hitting their head,” Yaeger said.

In many cases, the normal pressure hydrocephalus is idiopathic, meaning the cause is unknown.

The condition is rare but becomes more common with age. It is estimated that 0.2 per cent to 2.9 per cent of people 65 or older may develop the condition.

“The analogy I use for patients is that it’s like driving around with the parking brake on,” said David Harter, a professor of neurosurgery at the NYU Grossman School of Medicine.

According to the Hydrocephalus Association, the condition is often misdiagnosed as Alzheimer’s disease or Parkinson’s disease. But, in some cases, the symptoms can be reversed by draining excess cerebrospinal fluid.

“If it’s truly normal pressure hydrocephalus, there is a treatment we can offer that can help,” said Matthew Potts, a neurosurgeon at Northwestern Medicine.

“The earlier you address it, the better,” Potts said. However, many people don’t get diagnosed until months or years after their symptoms start because it can take time for doctors to rule out other conditions.

For Joel, the condition was “exacerbated by recent concert performances, leading to problems with hearing, vision and balance,” according to the statement.

How is normal pressure hydrocephalus diagnosed?

The brain disorder can be challenging to diagnose, Potts said.

Neuroimaging may provide a clue. With an MRI, “the fluid spaces of the brain called the ventricles are very enlarged,” but this is not definitive, Yaeger said.

The real test is a lumbar puncture or spinal tap, which drains cerebrospinal fluid. If the patient responds well to getting rid of the excess fluid, it’s a sign they have the condition.

“You use a needle through the [lower] back to get into a space where some of the cerebrospinal fluid is,” Potts said. “You remove some of it, and then you basically see, over the next 30 minutes or an hour, does the patient’s walking get better?”

How is normal pressure hydrocephalus treated?

The mainstay of treatment is implanting a medical device called a ventriculoperitoneal shunt, which removes cerebrospinal fluid from the brain and shunts it into the abdominal cavity, where it is absorbed into the body.

Surgeons drill a small hole in the head to place a catheter into a fluid-filled ventricle in the brain. This catheter is connected to a valve that is hooked up to another catheter that runs underneath the skin leading to the abdomen. The shunt is designed to stay in a patient for the rest of their life, and the valve is adjustable to allow the doctor to change how much fluid is drained as needed, Yaeger said.

“It’s a relatively simple surgical procedure,” Potts said. “And if somebody truly has normal pressure hydrocephalus, it can have just a really profound impact on their quality of life.”

Recovery is “pretty quick” and most patients go home the next day, Potts said. Typically, a patient’s gait will be the first thing to improve after the surgery.

Following treatment, the prognosis is excellent, experts said. “It’s one of the only types of dementia that is treatable and fixable,” Yaeger said.

“I’ve had patients who come in in a wheelchair and, after the surgery, a few weeks down the road, they’re able to walk and exercise and resume normal activities,” he said.

This article originally appeared in The Washington Post.

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