Billy Joel's condition: What to know about normal pressure hydrocephalus

Billy Joel has announced that he will be canceling all scheduled concerts after being diagnosed with normal pressure hydrocephalus.

Here's what to know about the condition.

What is normal pressure hydrocephalus?

Normal pressure hydrocephalus is a condition where too much cerebrospinal fluid builds up in the chambers of the brain, called ventricles or cavities, according to Johns Hopkins Medicine. Each day a person’s body makes enough cerebrospinal fluid to cushion the brain and the spinal cord. Under normal conditions, that fluid is absorbed so it does not build up. NPH is considered rare and generally is seen in older people.

What causes NPH?

NPH may be caused by conditions such as head injury, cranial surgery, subdural hematomas or meningitis, according to the Hydrocephalus Association. But in many cases doctors aren’t sure why a person develops it.

NPH is found in less than 3% of adults 65 and over, according to the Yale Medicine website. One estimate says over 700,000 people in the United States have the condition but not even 20% are diagnosed with it. Experts believe that NPH could be the cause of 5% of dementia cases in the United States.

What are the symptoms of NPH?

"Someone with normal pressure hydrocephalus may have memory problems, short-term memory, primarily, but it can be long-term memory as well," said Dr. David Chesler, a neurosurgeon and director of Pediatric Neurosurgery at Stony Brook Children’s Hospital. "They have this slow, shuffling, somewhat unsteady walk — they almost look like they have Parkinson’s disease. They can also develop urinary incontinence."

What is the treatment for NPH?

In some cases, surgeons can place a shunt, or tube, into the brain to drain the excess fluid.

"Your brain is making fluid and your brain's soaking the fluid back up. That fluid is coming from the plasma, the water content of your blood," Chesler said. "So we are putting the fluid back either directly into the blood ... or a space where there's a large surface area that can soak the fluid back up and return it back to the circulation," such as the abdomen.

He said the surgery usually takes between 30 and 60 minutes with two or three incisions on the body.

What is the prognosis for someone with NPH?

Chesler said generally about one-third of patients see an improvement in symptoms after surgery to place a shunt to drain the fluid.

"They may not be like they were in their 30s, but their gait will get better, their memory will get better," he said. 

Another one-third of patients may remain stable but they continue to decline slowly, he said. The final third actually decline after shunt surgery because the brain does not respond well to lowering the pressure.

Chesler said another option is to remove fluid a little at a time with a high volume lumbar puncture and see if the patient's symptoms improve before undergoing surgery to place a shunt.

By Lisa L. Colangelo

lisa.colangelo@newsday.com@lisalcolangelo

Lisa joined Newsday as a staff writer in 2019. She previously worked at amNewYork, the New York Daily News and the Asbury Park Press covering politics, government and general assignment.