When Billy Joel announced he had to cancel his upcoming tour, he revealed he was diagnosed with a rare neurological condition called normal pressure hydrocephalus (NPH).

The announcement followed a fall he took during a show in February in Connecticut. NPH, which can be treated by our expert neurology team, affects balance and walking, as well as thinking and bladder control.

What is NPH? What causes it? How is it diagnosed and treated?

Our expert, WellSpan neurosurgeon Dr. Pawel Ochalski, has cared for patients with the condition. He breaks it down for you.

NPH and its causes

NPH occurs when excess cerebrospinal fluid accumulates in the brain. It can be caused by a past injury, bleeding, an infection or certain types of brain tumors.

“In most cases, the cause is not known,” Dr. Ochalski said.

The condition is rare, impacting less than 1% of those ages 65 to 80, but its chances increase with age. As many as 6% of those over the age of 80 may develop the condition. Joel is 76 years old.

The condition is called “normal pressure” because even though the brain has more fluid than usual, a spinal tap may show a normal reading. While there is no elevated pressure, the extra fluid stretches the brain, resulting in changes in how the brain functions.

NPH symptoms and diagnosis

It can be challenging to diagnose NPH because its symptoms often look like they are the result of dementia, Parkinson’s disease or even normal aging, Dr. Ochalski said.

These symptoms include:

• Unsteady walking. People with NPH often walk like they are on a rocking boat: bent over, legs wide apart, with their feet sticking to the “deck.” Sometimes they walk with their toes pointing outward in what resembles a penguin’s gait. They also may shuffle or freeze.
• Bladder control. People may need to urinate more often or lose control of their bladder.
• Cognitive difficulties. People may show signs of slower thinking, memory problems and emotional changes.

Doctors can diagnose NPH with a variety of methods, including a physical exam, an MRI scan and a spinal tap.

Dr. Ochalski said the spinal tap can be the most helpful tool because it allows a doctor to drain excess fluid, which may cause a temporary decrease in symptoms.

Once the diagnosis is made, doctors can treat NPH by implanting a device called a shunt, which drains the excess fluid from the brain into the chest or abdomen, where the body can absorb it.

“Relieving the pressure on the brain helps to reduce symptoms and makes life easier for patients,” Dr. Ochalski said.

Patients also can get physical therapy to help with their walking, which Joel said he is doing.

NPH prognosis

If NPH is detected and treated early enough, symptoms can greatly improve, particularly in regard to walking and bladder control. Cognitive symptoms also can decrease, however about a third of patients also have a degenerative neurological condition such as Alzheimer’s disease, which may impact these symptoms.

Overall, it is important to seek treatment early if you are exhibiting the three main symptoms of NPH: unsteady walking, loss of bladder control and cognitive problems.

“Get to your doctor and get checked out,” Dr. Ochalski said. “Sooner is always better when it comes to treating this condition.”

To learn about our neurology and neurosurgery services, and find an expert WellSpan neurologist, go here.