The entertainment world was rocked recently when superstar singer Billy Joel announced the cancellation of all of his upcoming performances. The reason: he has been diagnosed with a rare but often treatable brain disorder called normal pressure hydrocephalus. 

We saw several articles explaining this news. Many extolled the virtues of Billy Joel, the original “Piano Man,” who has become a megastar with performances that draw tens of thousands in stadium-sized venues. However, in this related Washington Post story, reporters Teddy Amenabar and Richard Sima focus less on Joel’s career and more on the diagnosis itself. Two things really grabbed our attention.  

First, while rare, normal pressure hydrocephalus could affect half a million or more U.S. seniors. Second, the forgetfulness and balance problems this condition triggers are often misdiagnosed as Alzheimer’s disease. But normal pressure hydrocephalus, unlike other forms of dementia, is frequently treatable if diagnosed in time. Let’s take a deeper look. 

Gradual Build-up of Pressure on the Brain Brings Alarming Symptoms 

Billy Joel’s diagnosis of normal pressure hydrocephalus was released in a recent statement on his social media accounts, along with the announcement that the singer was canceling his scheduled performances.  

Washington Post reporters Amenabar and Sima explain, “Hydrocephalus refers to the buildup of cerebrospinal fluid, a watery liquid that cushions the brain and spinal cord. With normal pressure hydrocephalus, the fluid accumulates gradually with little to no increase in intracranial pressure. The excess cerebrospinal fluid presses on the brain and can disrupt neural pathways, causing cognitive impairments, difficulty walking and a loss of bladder control.” 

According to the social media statement, Joel will undergo physical therapy and must refrain from performing while he recovers. Tickets for the canceled 17 shows will be automatically refunded.  

Here is how normal pressure hydrocephalus is diagnosed and treated.  

Signs and Symptoms Often Mimic Other Forms of Dementia 

While not everyone who suffers from normal pressure hydrocephalus has all of the symptoms, there are three main signs of the disease that health care professionals look for: cognitive impairment, gait instability, and urinary dysfunction.   

“People are very forgetful, they don’t remember things or their short-term memory is really poor,” adds Kurt Yaeger, an endovascular neurosurgeon and assistant professor of neurosurgery at Houston Methodist Hospital who treats patients (but has not treated Joel). 

The Hydrocephalus Association—a nonprofit that raises awareness about the condition—states that other common symptoms include mood changes, speaking less often, and loss of interest in daily activities. “People with normal pressure hydrocephalus may walk with a slow shuffle, as if their feet are stuck to the floor, and they may have difficulty turning corners,” the article adds.  

“Usually, the gait disability is pretty severe to the point where they’re falling and they’re hitting their head,” Yaeger says. 

David Harter, a professor of neurosurgery at the NYU Grossman School of Medicine, puts it this way: “The analogy I use for patients is that it’s like driving around with the parking brake on.” 

Cause is Usually Unknown, but Diagnosis Increases with Age 

“In many cases, the normal pressure hydrocephalus is idiopathic, meaning the cause is unknown,” the article explains. “Normal pressure hydrocephalus is rare, but it becomes more common with age. It is estimated that 0.2 percent to 2.9 percent of people 65 or older may develop the condition.” 

Still, we can’t help noting that, if even 1 percent of the 65-plus population were to develop the condition, that represents some 560,000 diagnoses. In other words, the odds are reasonably good that someone you know could be affected by this condition. 

With Early Diagnosis, Symptoms are Reversible 

One of the condition’s unique aspects is that it is—in some cases—treatable, even reversible, despite being commonly misdiagnosed as Alzheimer’s disease or Parkinson’s disease. For some sufferers, the symptoms can be reversed by draining off excess cerebrospinal fluid.  

“If it’s truly normal pressure hydrocephalus, there is a treatment we can offer that can help,” says Matthew Potts, a neurosurgeon at Northwestern Medicine. “The earlier you address it, the better.” 

Notably, it can take time for doctors to rule out other conditions, and therefore many people don’t get the right diagnosis until months or years after their symptoms start. 

For Joel in particular, his condition was “exacerbated by recent concert performances, leading to problems with hearing, vision, and balance,” according to the social media statement. This led to earlier diagnosis. 

Diagnosis Can Be Challenging, Doctors Acknowledge 

Experts agree that this brain disorder can be a challenge to diagnose. Yaeger explains that neuroimaging can help, and that with an MRI, “the fluid spaces of the brain called the ventricles are very enlarged.” However, this enlargement is not definitive, doctors say. 

The article states, “The real test is a lumbar puncture or spinal tap, which drains out the cerebrospinal fluid. If the patient responds well to getting rid of the excess fluid, it’s a sign they have the condition.” 

Potts adds, “You use a needle through the [lower] back to get into a space where some of the cerebrospinal fluid is. You remove some of it and then you basically see, over the next 30 minutes or an hour, does the patient’s walking get better?” 

“Relatively Simple” Procedure Yields Potentially Profound Improvement 

Amenabar and Sima explain, “The mainstay of treatment is surgically implanting a medical device called a ventriculoperitoneal shunt, which removes cerebrospinal fluid from the brain and shunts it into the abdominal cavity, where it is absorbed into the body.” 

This involves drilling a small hole in the patient’s head to “place a catheter into a fluid-filled ventricle in the brain. This catheter is connected to a valve that is hooked up to another catheter that runs underneath the skin leading to the abdomen.” 

This shunt is designed to stay in the patient for the rest of their life, Yaeger says, and the valve is adjustable to allow the doctor to adjust how much fluid is drained.  

“It’s a relatively simple surgical procedure,” Potts says. “And if somebody truly has normal pressure hydrocephalus, it can have just a really profound impact on their quality of life.” 

One of the Only “Treatable and Fixable” Types of Dementia 

Potts adds that recovery is “pretty quick” and most patients go home the next day. Typically, a patient’s gait will be the first thing to improve after the surgery. 

Experts agree that prognosis is excellent after treatment. “It’s one of the only types of dementia that is treatable and fixable,” Yaeger says, concluding the article. “I’ve had patients who come in in a wheelchair and, after the surgery, a few weeks down the road, they’re able to walk and exercise and resume their normal activities.” 

Our thoughts go out to Billy Joel and his family and fans in the wake of this diagnosis. Here’s hoping the prognosis is a good one for the legendary Piano Man.  

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PHOTO CREDIT: Flickr licensed by Creative Commons 

(originally reported at www.washingtonpost.com)