Billy Joel has canceled his upcoming concerts because of a brain disorder that has affected his hearing, vision and balance, the singer-songwriter announced on Friday.
The condition, known as normal pressure hydrocephalus, or N.P.H., is estimated to affect hundreds of thousands of older Americans. Here’s what to know about the disorder.
What is normal pressure hydrocephalus?
N.P.H. occurs when excess cerebrospinal fluid builds up in the brain, causing symptoms that include trouble walking, balancing and controlling one’s bladder. It can also lead to memory problems.
The bladder symptoms can include not just incontinence, but also waking up at night to urinate with increasing frequency.
A statement posted to Mr. Joel’s social media accounts on Friday said his condition had been “exacerbated by recent concert performances.”
N.P.H. is rare, but risk increases with age. Dr. Charles Matouk, a neurosurgeon at Yale University and director of the Normal Pressure Hydrocephalus Program, estimated that less than 1 percent of the population ages 65 to 80 might get it, but that the number was likely 5 percent or more after age 80.
Dr. Matouk said the condition was likely underdiagnosed because its symptoms can easily be dismissed as normal effects of aging. He urged people not to make that assumption, and to see a doctor if they experienced trouble walking, controlling their bladder and remembering things.
How is it diagnosed?
Doctors often suspect N.P.H. when a patient shows up with the triad of gait, bladder and memory problems.
The first test is likely to be a CT scan or M.R.I. In patients with the condition, that imaging will show enlargement of the brain’s fluid-filled ventricles. But the conclusive test is a spinal tap: Because that procedure removes cerebrospinal fluid, patients with N.P.H. experience a temporary alleviation of symptoms, confirming the diagnosis, Dr. Matouk said.
How is it treated?
Treatment typically involves a surgery called ventricular shunting, which is often very effective, though it doesn’t resolve symptoms for every patient.
The disease occurs when the channel that cerebrospinal fluid normally drains through is blocked. So to treat it, a surgeon drills a hole in the skull and inserts a shunt that bypasses the blocked channel and lets fluid drain into a part of the body that can absorb it, like the lining of the abdomen or the lung.
Dr. Matouk leads a group of researchers working on a less invasive procedure in which a shunt is inserted into a blood vessel and threaded up through a vein in the neck to a ventricle at the base of the brain, from which fluid can drain into the bloodstream.
The researchers recently completed a small, preliminary trial that Dr. Matouk said showed the procedure to be safe. However, the results of that work have not been published, and the procedure has not yet been approved by the Food and Drug Administration. They are now enrolling patients for the next clinical trial stage.
Some patients may also opt for physical therapy to improve their gait stability. Mr. Joel’s statement said that he was undergoing physical therapy, “and has been advised to refrain from performing during this recovery period.”
What’s the prognosis?
It depends on when the disease is diagnosed. If it’s caught early enough, surgery can largely or fully reverse the symptoms in many patients. But for some patients, especially in later stages, symptoms can become irreversible.
“If you get to me in a wheelchair, we can only expect the shunt to help so much,” Dr. Matouk said. “The earlier it’s diagnosed, the better patients will do — or at least the more information we’ll have to make appropriate, deliberate decisions about your health care.”
Gait and bladder symptoms tend to respond better to treatment than cognitive symptoms, he said, but the cognitive symptoms can still improve.
Maggie Astor covers the intersection of health and politics for The Times.
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