NPH: A Treatable, Sometimes Reversible, Form of Dementia.

Singer Billy Joel has cancelled his 2025 planned concerts, announcing he’s been diagnosed with normal pressure hydrocephalus (NPH), a rare brain disorder. Frequently mistaken for Alzheimer’s or Parkinson’s disease, NPH can cause problems with cognition, mobility, and continence.

NPH occurs when too much cerebrospinal fluid (CSF) builds up in the brain’s ventricles—a network of cavities that cushion the brain. Excess fluid can press against the brain tissue, causing damage and dementia-like symptoms. 

With early diagnosis, treatment to drain the ventricles along with physical therapy and rehabilitation can improve or reverse NPH symptoms. If left untreated, it can be fatal. 

NPH mostly affects those in their 60s and 70s. The Hydrocephalus Association estimates that as many as 800,000 people have NPH, but more than 80% of them are unrecognized or untreated, often due to misdiagnosis. 

Representatives for the “Piano Man” say Joel doesn’t intend to retire and is optimistic that treatment will allow him to return to the concert stage. Knowing the symptoms of NPH and identifying it early can help many people get effective treatment that can often reverse symptoms. 

Three main symptoms of NPH.

Most of the time, doctors don’t know what causes NPH. Sometimes, it can be caused by having had a head injury, brain surgery, tumor, cyst, hematoma, or a brain infection such as meningitis. This is known as secondary NPH.

NPH is known by three classic symptoms:

• Gait disturbances: Usually the first symptom to develop, people with NPH can have difficulty walking. Often, this means taking slow, short steps with trouble going down stairs. Some people report that their feet feel heavy or stuck to the floor. This is known as “magnetic gait.” 

• Cognitive impairment: Difficulty thinking can mean a loss of interest in activities, mood changes, trouble with decisions and paying attention, forgetfulness, and short-term memory loss, among other symptoms.

• Urinary incontinence: People with NPH can have a hard time controlling their bladder. The result can be feeling like they need to go often and right away or, in more severe cases, total loss of bladder control.

These symptoms can be different for everyone, and they mimic signs of aging and other cognitive or movement conditions such as Alzheimer’s disease or Parkinson’s disease. 

Related reading: Deep Brain Stimulation for Movement Disorders: Improved Quality of Life, Less Medication.

Early, accurate diagnosis of NPH.

Early diagnosis of NPH is important, because it is one of the only forms of dementia that can be improved with treatment. We see the best outcomes among patients who are accurately diagnosed and treated early—within 6-12 months of symptom onset. The longer NPH goes untreated, the more likely it is to cause permanent brain damage. 

Neurologists can perform imaging exams and tests to confirm or rule out a diagnosis of NPH. These can include:

• CT brain scan: To examine the size of the ventricles to see if they take up more than 50% of the brain, a sign of NPH.

• MRI: To see detailed images of the brain and how much fluid is flowing.

• Gait assessment: Analysis of walking to help determine how much a person is impaired. 

• Neuropsychological testing: Tests to learn about a person’s thinking and functions such as memory, language, visual information skills, and attention

One of the most impactful tests to learn if surgery can help is a high-volume lumbar tap. This test allows us to simulate how a patient might respond to a surgically-implanted shunt to drain CSF. 

First, we conduct careful gait and cognitive analysis to learn how well a patient is functioning before the test. After the patient gets anesthetic to numb the area, we insert a long needle into the spine at the lower back. We remove about 50cc (about a quarter cup) of CSF. Then, we test how well the patient is walking and thinking. 

If symptoms improve after we remove CSF, this is a good sign that surgery can help relieve symptoms of NPH. 

Effective treatment for NPH is available.

If we agree that surgery is likely to help, a neurosurgeon can implant a device called a shunt. This thin, flexible tube placed in the brain’s ventricles diverts the flow of CSF to the abdomen, where it is absorbed by the body. 

Research estimates that more than 80% of people with NPH who respond to a high-volume lumbar tap will see rapid improvement after shunt placement. Gait improvement is usually first, followed by cognition. Physical therapy can help patients regain safe and independent mobility. 

Our safety record at MedStar Washington Hospital Center is excellent. Complications are rare and can include shunt infection or malfunction over time, causing symptoms to return. We use X-rays and other imaging to monitor patients for complications.

Related reading: Research Explores Virtual Reality Floor Test for Early Alzheimer’s Disease.

Standardized diagnosis & other advances on the horizon.

At MedStar Washington Hospital Center, neurosurgeons and neurologists collaborate to help patients achieve the best outcomes. Our teams coordinate care, provide accurate clinical diagnosis and imaging, and develop custom treatment plans for each patient. 

To improve NPH care, standardized diagnostic criteria and AI-assisted algorithms can help us better identify NPH and tell it apart from other conditions sooner, when treatment is most effective.

My research, for example, is focused on biomarkers – signs of disease in a patient’s blood samples. For instance, MedStar Health offers a blood-based test for Alzheimer’s disease that identifies the presence of certain proteins that accumulate in the brain and impact its function. In the future, blood tests could help us differentiate between NPH and Parkinson’s disease, which can appear similar on a brain scan.

Forthcoming technologies such as smart programmable shunts and more pressure-sensitive adaptive valves could improve NPH treatment. New rehabilitation strategies such as robotic-assisted gait training, which uses robotic devices to help patients relearn walking, are in the wings, too.

There are many exciting advances on the horizon, but first we must make sure we’re accurately identifying patients with this often-reversible condition. More accurate diagnosis and earlier treatment can help more people like Billy Joel return to the lives they love, whether that’s playing sold-out concerts or enjoying more healthy years with their families.