Three symptoms that strike when you walk, talk and urinate that could mean you have rare brain disorder suffered by Billy Joel
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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.
A spokesperson for the singer said that his condition had been 'exacerbated' by recent performances, leading to problems with hearing, vision and balance. He is now taking a break and undergoing 'physical therapy' to treat the condition.
Normal pressure hydrocephalus is caused by too much fluid collecting in the ventricles, or spaces in the brain and spinal cord, putting pressure on these areas and triggering its symptoms, including struggling to walk, an inability to control the bladder and memory problems.
This happens because the excess fluid compresses and stretches the brain tissue, interfering with the control of muscles and communication between nerve cells.
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.
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
'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.'
Joel'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.
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.
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 actually know they have it.
The error can lead to delays in getting proper treatment, raising the risk of a patient suffering permanent damage to their brain.
Joel has abruptly canceled all his upcoming performances, saying he needs to focus on his health (pictured above in October 2021)
Dr Potts said patients often have to wait at least a year to finally get a diagnosis, and often much longer. In some cases, he said, the condition is never diagnosed.
The condition does not cause pressure in the brain to rise markedly, but can still be detected on brain scans which show enlarged ventricles.
It differs from the form of hydrocephalus that more commonly affects children, which leads to much higher pressure levels in the brain than normal.
Dr Potts said there were three main warning signs for the disease: Memory problems, walking or balance problems and difficulty with urination.
For movement, signs of the condition include having trouble lifting the feet, short or unsteady steps, rotating toes outward while walking, freezing or walking uncertainly.
For bladder issues, doctors say that losing control of the bladder is a signal, with this causing someone to urinate unintentionally.
And for cognitive issues, signs of the condition include thinking more slowly than normal, having problems with memory or appearing to be forgetful or absentminded.
Sufferers may also have trouble managing their emotions, or could appear apathetic or uninterested in activities that they used to enjoy.
It can also cause hearing problems, according to some studies — which found an association between the condition and this complication. Doctors believe this is due to changes in the fluid pressure that affect the inner ear.
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
Normal pressure hydrocephalus is 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.
Dr Potts said diagnosis often comes years after symptoms first appear, saying it takes time for patients to seek help and then for healthcare providers to reach the right diagnosis.
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.
Daily Mail
