What Brain Disorder Does Billy Joel Have? A Neurosurgeon Explains

Rock legend Billy Joel, aged 76, has been diagnosed with normal pressure hydrocephalus (NPH), a neurological condition that has forced him to cancel his upcoming tour. NPH, often mistaken for dementia, can profoundly affect walking, balance, and cognitive function.

What is NPH? How is is treated? And what lies ahead for the iconic musician?

What Is Normal Pressure Hydrocephalus?

The brain is composed of three primary components: brain tissue, blood, and cerebrospinal fluid. CSF, a clear liquid, circulates through the brain and spinal cord, cushioning neural structures and delivering nutrients. It flows through chambers called ventricles and is absorbed by specialized structures known as arachnoid granulations.

In NPH, generally the absorption of CSF is impaired, leading to fluid accumulation in the ventricles. This buildup exerts pressure on surrounding brain tissue, causing symptoms that may resemble dementia but are distinct from it.

NPH is relatively rare, affecting approximately 0.2% of individuals aged 70–80 and up to 6% of those over 80. While it predominantly occurs in older adults, its prevalence increases with age..

How Does Normal Pressure Hydrocephalus Occur?

CSF is continuously produced and circulates throughout the neuroaxis, the brain and spinal cord. For the system to function properly, CSF must be absorbed efficiently. In NPH, the primary issue is inadequate absorption, often due to dysfunction in the arachnoid granulations. Arachnoid granulations or Pacchionian granulations are microscopic body parts responsible for the absorption of spinal fluid back into the venous system.

Unlike other forms of hydrocephalus, where blockages obstruct CSF flow, NPH typically involves normal intracranial pressure, hence the term “normal pressure.” However, the excess gradual fluid still disrupts brain function within the rigid confines of the skull, leading to neurological symptoms.

What Are The Symptoms of Normal Pressure Hydrocephalus?

NPH manifests gradually, often mimicking other conditions such as Parkinson’s disease or dementia. Its hallmark is Hakim’s triad: gait disturbance, urinary incontinence, and cognitive impairment. Notably, only 50–75% of patients exhibit all three symptoms simultaneously. Gait difficulties are the most prevalent, affecting 80–95% of patients. These issues present as shortened steps, poor coordination, and trouble with complex movements, sometimes described by patients as their feet “sticking” to the ground. To observers, it may simply appear as difficulty walking.

Cognitive symptoms are subtler, including slowed thinking, forgetfulness, and challenges with executive functions such as planning or impulse control. Urinary issues, when present, typically involve urgency or incontinence.

How Is Normal Pressure Hydrocephalus Treated?

NPH does not respond to medication.

Instead, treatment focuses on removing excess CSF to alleviate symptoms. The diagnostic process often begins with a lumbar puncture, where a needle is inserted into the lower back to measure CSF pressure and remove a significant volume of fluid. Physicians then assess whether this improves symptoms, particularly gait. This test may be repeated to confirm the diagnosis.

If the lumbar puncture yields positive results, patients may be candidates for a ventriculoperitoneal (VP) shunt. This surgical procedure involves placing a catheter into the brain’s ventricles through a small hole in the skull. The catheter connects to a valve that regulates CSF flow and tubing that diverts the fluid to the abdominal cavity, where it is safely absorbed. The surgery, which typically lasts 15–60 minutes, may employ neuronavigation technology or a type of surgical GPS system to ensure precise catheter placement.

What Is The Prognosis?

NPH is partially treatable, with shunt surgery improving some symptoms, particularly gait, in many patients. However, not all symptoms resolve, and outcomes vary. A critical aspect of NPH is its potential to mimic dementia, making accurate diagnosis essential. Approximately 30% of NPH patients also have a concurrent neurodegenerative condition, such as Alzheimer’s disease, complicating the clinical picture. I have personally treated patients labeled with dementia who were later found to have NPH.

What Are The Next Steps For Billy Joel?

Billy Joel is prioritizing his health.

According to his social media, “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.”

We wish him a swift and successful recovery.