What is normal pressure hydrocephalus (NPH), the brain condition Billy Joel was diagnosed with?

On Friday, singer Billy Joel announced that he is canceling his entire tour due to a rare diagnosis that can cause issues with walking and even thinking clearly.

The 76-year old posted on Instagram that he has been diagnosed with a condition called normal pressure hydrocephalus (NPH), a rare brain condition which affects about 5.9% of people over the age of 80, according to the Cleveland Clinic. Joel said that performing concerts worsens the effects, causing problems with “hearing, vision, and balance”.

What is normal pressure hydrocephalus (NPH)?

NPH is a condition in which extra fluid builds up in the brain, called cerebrospinal fluid (CSF). That fluid would typically cushion and clean the brain and spinal cord, but with NPH, it doesn’t properly drain and instead collects in the brain’s ventricles, causing them to swell. The swelling puts pressure on the nearby brain tissue, particularly in the areas which control movement, memory, and bladder function.

Billy Joel fell on stage during a performance in February. This is those with NPH experience unsteadiness and difficulty lifting their feet.

NPH is typically diagnosed in older adults, usually showing up around 70 years old.

Causes and diagnosis of NPH

There are two man types of NPH - Idiopathic and Secondary. With Idiopathic NPH, there is no clear cause and shows up randomly in older adults. Secondary NPH is caused by an outside factor like a head injury, brain surgery, bleeding in the brain, or certain infections such as meningitis.

Diagnosing NPH can be difficult because it’s often mistaken for Alzheimer’s or Parknson’s due to the similarities in the symptoms. Typically, doctors will look at the patient’s medical history and perform a physical to examine their walking and balance. They will also do an MRI or CT scan to look for enlarged ventricles. A spinal tap is sometimes performed as well in order to see if the symptoms improve temporarily.

Good news: NPH is treatable

The good news is, if it’s caught early, NPH can be treated and even reversed. If a patient responds well to a spinal tap, it is a good sign that the NPH may be treatable. It is one of the few causes of dementia-like symptoms that is actually treatable.

The most common treatment is a shunt surgery. The shunt is a small tube implanted in the brain that drains the excess fluid to another part of the body (usually the abdomen), where it can be absorbed. This helps relieve pressure and improve symptoms - especially walking.

A drug called acetazolamide is another form of treatment, which helps the body decrease the fluid levels on its own. However, it doesn’t work for everyone.

Joel’s post said that he “is undergoing specific physical therapy”, but did not specify which sort of treatment he is receiving.

The key with NPH is early diagnoses. If it goes on too long without being treated, it can lead to more permanent brain damage, which is why it’s imperative to do the tests and not assume it’s dementia, or simply just aging.

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