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Celebrity diagnosis sparks new awareness of NPH

A balding man with a beard, glasses and a white coat, poses in an interior setting in front of large medical equipment.
Neurovascular Surgeon Charles Matouk, MD, is director of the Yale Normal Pressure Hydrocephalus Program, which has diagnosed and treated patients for over 10 years. Singer Billy Joel announced in May that he has NPH, prompting increased awareness of this uncommon brain condition.

When singer Billy Joel announced in May that he was canceling concerts due to normal pressure hydrocephalus, he shifted the spotlight to an uncommon brain condition.

“If Billy Joel’s bravery and stardom can have a positive impact by shedding light on this condition, I think that’ll help tons of people get the help they need more quickly,” Charles Matouk, MD, Yale New Haven Hospital neurovascular surgeon and director of the Yale Normal Pressure Hydrocephalus Program (NPH), told The New York Times.

Dr. Matouk – who is not involved in Joel’s care – is among the YNHH and Yale School of Medicine specialists who diagnose and treat NPH, which occurs when cerebrospinal fluid (CSF) builds up in the brain. 

CSF provides cushioning, delivers nutrients and removes waste. Normally ventricles in the brain circulate and drain CSF, which is reabsorbed into the body. With NPH those processes are disrupted – sometimes due to injury, tumors, stroke or other causes. About half of NPH cases are idiopathic, meaning the cause isn’t known.  

At least 700,000 older Americans are estimated to have NPH, but many cases are unrecognized or untreated. Diagnosis can be challenging because the main symptoms – difficulty walking, thinking and controlling the bladder – are also associated with conditions such as Alzheimer’s or Parkinson’s disease or even normal aging. 

The standard treatment for idiopathic NPH involves placing shunts in the brain to help improve circulation and drain excess CSF into the abdomen. In 2022 YNHH became the first U.S. hospital to offer a phase 1 clinical trial for a new device – the CereVasc eShunt System. Implanting this shunt is a minimally invasive procedure in which the surgeon makes a small incision in the leg, guides the equipment through a blood vessel to the base of the skull and creates a new CSF drainage channel. 

The first phase of the trial yielded promising results, with 97 percent of participants experiencing symptom improvement and none reporting serious adverse effects. 

The CereVasc eShunt System clinical trial is just one example of the extensive neurosciences research bolstered by a partnership between YNHH and Yale School of Medicine (YSM). 

“What makes us different as an NPH program is that because of our size, our expertise in treating the condition and being a referral center for the region, we’ve been able to attract large clinical trials,” said Dr. Matouk, professor of Neurosurgery and of Radiology and Biomedical Imaging at YSM. 

When YNHH’s Adams Neurosciences Center opens in 2027 it will expand YNHH’s and YSM’s reach and leadership in clinical trials for NPH and many other neurologic conditions.

“The NPH program Dr. Matouk has developed exemplifies the personalized, mechanism-based care we aspire to offer patients across a broad spectrum of neurologic disorders – including those with neurodegenerative and neurodevelopmental conditions – in the Adams Neurosciences Center,” said Murat Gunel, MD, neurosurgeon-in-chief, Yale New Haven Health, and Sterling Professor and chair of the Department of Neurosurgery, YSM.

Learn more about the Normal Pressure Hydrocephalus Program at YNHH