NPH: Understanding Billy Joel’s diagnosis

Beloved musician Billy Joel, 76, recently announced he was cutting his summer tour short due to serious health concerns. Joel was diagnosed with normal pressure hydrocephalus, which is a rare disorder caused by fluid buildup in the brain. The disorder can cause difficulty walking as well as issues with balance, cognition, memory and bladder control.

“About 1% to 3% of the population develops normal pressure hydrocephalus — NPH for short,” says Dr. Sunil Jeswani, a neurosurgeon with Sharp Community Medical Group and affiliated with Sharp Grossmont Hospital. “And we rarely see it before the age of 60.”

According to the National Institutes of Health (NIH), cerebrospinal fluid, which protects the brain and spinal cord, builds up within the ventricles, or cavities, within the brain. The buildup causes the cavities to widen, putting pressure on the brain's tissues.

NPH can be due to:

• Bleeding in the brain's cerebrospinal fluid

• Head trauma

• Infection

• Tumor

• Complication of surgery

However, NPH can also develop in the absence of any of the above factors.

“The causes of NPH are unknown,” Dr. Jeswani says. “However, we think that the walls of the cavities in the brain, with age, can become flimsy over time, allowing these cavities to expand and hold more fluid.”

Treating NPH

According to Dr. Jeswani, NPH is treatable. The disorder is diagnosed after a neurological exam that may include muscle, reflex, balance, vision, hearing and mental functioning tests. Brain imaging, such as MRI and CT, and a spinal tap to measure cerebrospinal fluid pressure may follow.

“When we suspect a patient has NPH, based on their symptoms and brain imaging, we first temporarily drain some cerebrospinal fluid from the spine,” Dr. Jeswani says. “If their symptoms temporarily improve, then we’re much more convinced that patient has NPH.”

Treatment for NPH is to put in a shunt, Dr. Jeswani says. The shunt permanently diverts fluid from the brain into a cavity somewhere else in the body, usually the abdomen.

“With this surgical treatment, patients can often return to a pretty normal life,” he says. “I have plenty of patients with diagnosed NPH that get a shunt and go back to playing golf, enjoying their normal activities and taking vacations. They can lead a pretty normal, functional life.”

Joel’s fans are sure to hope that this is the case for the performer. While an Instagram statement from his management stated that Joel has been advised not to perform during recovery, he “looks forward to the day when he can once again take the stage.”

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