It’s the condition that affects an estimated 700,000 Americans, but is often mistaken for dementia, Alzheimer’s or Parkinson’s.
And it can be linked to a previous head injury or trauma.
Yet normal pressure hydrocephalus is a condition that doctors are able to treat, especially when diagnosed early — helping someone to avoid long-term effects.
Billy Joel today revealed that he was suffering from the condition, in an abrupt announcement where the 76-year-old also canceled all his upcoming concerts.
It is caused by too much fluid collecting in the ventricles, or spaces in the brain and spinal cord, putting pressure on the areas and triggering its symptoms including struggling to walk, an inability to control the bladder and memory problems.
These happen, doctors say, because the condition affects the functioning of areas of the brain controlling these areas — including the bladder.
Dr Matt Potts, a neurosurgeon at Northwestern University who has treated hundreds of patients with the disease, told DailyMail.com that it is often misdiagnosed initially — with many patients, normally in their 70s, at first treated for other diseases.
‘The challenge with this condition is that, in older people, they can have those things for a lot of different reasons,’ he said.
‘It absolutely can be mistaken for dementia, for example, or another more common condition — leaving patients waiting more than a year for treatment. In some cases, it is never diagnosed.’
Billy Joel has revealed he has been diagnosed with normal pressure hydrocephalus. He is pictured above on stage in February 2025 in Connecticut at his last concert before announcing the diagnosis
In normal pressure hydrocephalus, fluid collects in the ventricles in the brain – or the spaces in the brain – exerting more pressure. The condition, however, does not cause an uptick in brain pressure
Revealing the diagnosis, Joel’s spokesperson said: ‘This condition has been exacerbated by recent concert performances, leading to problems with hearing, vision and balance.
‘Under his doctor’s instructions, Billy is undergoing specific physical therapy and has been advised to refrain from performing during this recovery period.’
The performer’s last concert took place at the Mohegan Sun Arena in Uncasville, Connecticut, on February 22 this year.
It isn’t clear what causes the condition, but previous cases have also seen it linked to brain bleeds, infections and previously having surgery on the brain or suffering a tumor in that area.
It is rarely fatal, but, if left untreated, it can lead to further complications due to damage to the brain.
These may include someone losing the ability to walk, or having progressively more serious memory problems.
What is normal pressure hydrocephalus?
Normal pressure hydrocephalus, or NPH, is a condition that happens where cerebrospinal fluid builds up inside the skull and puts pressure on the brain.
The brain contains ventricles, or cavities, filled with fluid that are used to cushion the organ and the spinal cord — preventing damage.
Normally, the body makes just enough fluid for these areas — called cerebrospinal fluid — and absorbs the same amount each day.
But, in rare cases, too much fluid can build up in the ventricles — putting pressure on the organ and triggering the warning signs of the condition.
Is it becoming more common?
The condition is considered to be rare, but is often misdiagnosed.
Yale Medicine says that although estimates suggest there are as many as 700,000 Americans suffering from the condition — fewer than 20 percent know they have it.
The error can lead to delays in getting proper treatment, raising the risk that the condition will cause permanent damage to the organ.
Joel has abruptly canceled all his upcoming performances, saying he needs to focus on his health (pictured above in October 2021)
In normal pressure hydrocephalus, however, the pressure in the brain remains within normal ranges — hence the name.
It differs from the type typically diagnosed in children, known as hydrocephalus, in which the build-up of fluid leads to much higher pressure on the brain.
What are the warning signs?
Dr Potts said there were three main warning signs for the disease: Memory problems, walking or balance problems and difficulty with urination.
Problems walking that can be signs of the condition include having trouble lifting feet, having shorter or unsteady steps, and rotating toes outward while walking.
Doctors say that freezing or walking uncertainly can also be a sign of the condition.
Urinary issues that may be triggered by the condition include losing control of the bladder, causing someone to urinate unintentionally.
Signs of cognitive problems caused by the condition include someone thinking slower than usual or moving more slowly than normal.
Someone may also have problems with their memory, appearing to be forgetful or absent-minded.
And sufferers may have trouble managing their emotions or could appear apathetic or uninterested in the face of activities they used to enjoy.
How likely are you to survive the condition?
Studies suggest that patients survive on average for 8.8 years following their diagnosis, with a quarter surviving more than 13 years.
Patients are also normally diagnosed around the age of 70 years, doctors say.
Dr Potts emphasized that a diagnosis with the condition was very much not considered to be a death sentence, but something that can be treated.
He said: ‘If it is untreated, it typically progresses but that progression can be very, very slow.
‘Normal pressure hydrocephalus on its own is probably not going to be fatal, but if it progresses in an extreme way, it can leave someone no longer able to walk.
‘On its own, however, we don’t think of it as a fatal disorder.’
The condition can be treated by inserting a device to gradually drain fluid from the brain
How is it diagnosed?
Normal pressure hydrocephalus is normally diagnosed via a combination of neurological and physical exams.
These can include an MRI scan to reveal the ventricles in the brain, and show whether they are expanded — indicating higher pressure and which can be a sign of the condition.
Patients may also be tested for the disease using a spinal tap, which samples cerebrospinal fluid for signs of the condition.
How is it treated?
Normal pressure hydrocephalus is treated by relieving the excess fluid that is causing pressure on the brain.
This may be done via surgery to place a tube, called a shunt, into the brain to drain away excess fluid.
The shunt is normally placed into a ventricle in the brain, and then passed under the skin from your head through the neck and to the chest to your abdomen.
