Normal Pressure Hydrocephalus (NPH): A Highly Treatable Brain Disorder
Ever since Billy Joel revealed on Instagram that he was suffering from normal pressure hydrocephalus (NPH), his condition has become big news, along with the surgical treatment that has eased his symptoms.
With early diagnosis, a skilled neurosurgeon can provide the treatment capable of reversing its harmful effects; a neurosurgeon like Dr. Jared Knopman.
Dr. Knopman wears multiple hats within Weill Cornell Medicine. He’s the Director of Cerebrovascular Surgery and Interventional Neuroradiology—a specialty involving the use of high-tech imaging to guide advanced surgical techniques for vascular disorders of the brain and spinal cord. He’s also an Associate Professor of Neurological Surgery and Fellowship Director for Endovascular Neurosurgery.
Keep reading for answers to your FAQs about a condition that affects from 3 to 5 percent of older adults but can be difficult to diagnose.
What is NPH?
NPH occurs when there’s a buildup of fluid in cavities, called ventricles, that lie deep within the brain. “Normally,” Dr. Knopman says, “cerebrospinal fluid bathes the brain and spinal cord, protecting them from injury and filtering the waste produced by brain metabolism. But when a patient has hydrocephalus, the brain’s plumbing gets clogged. As a result, there’s an imbalance between how much fluid is produced and how much gets absorbed into the bloodstream.”
The build-up of too much fluid can damage brain tissues and cause what he calls a classic triad of symptoms.
What are the characteristic symptoms of NPH?
While NPH can occur in any age group, its symptoms in older adults typically follow a recognizable pattern, including:
- A loss of bladder control
- Gait disturbance
- Cognitive decline
“Patients may have one, two or all three of these,” he says, “and they can easily be mistaken for other age-related issues.” Here’s why.
Urinary incontinence is common among older people. It’s often associated with an enlarged prostate, prostate cancer or weakening of the pelvic floor muscles—all conditions that become more prevalent as people age.
The gait disturbance (abnormal walking) that’s often seen in older people with NPH is characterized by a shuffling quality, and the patient’s feet tend to be spread wide apart as they walk, appearing to have a walk that looks “magnetic” to the floor. But such gait problems can also be caused by back or knee problems, which need to be ruled out before an NPH diagnosis can be confirmed, Dr. Knopman says.
And third, cognitive decline, or dementia, is often associated with neurodegenerative diseases like Parkinson’s and Alzheimer’s. These, too, are fairly common among older adults.
However, when all three of these classic symptoms are seen together, NPH is usually the culprit. An MRI will show the presence of excess fluid in the ventricles, thus suggesting a diagnosis of NPH.
Do these symptoms appear suddenly or gradually?
The symptoms of NPH tend to be progressive and slow-growing, he explains. “It can take several months, a year or even longer for them to become noticeable enough for a family member to bring their loved one in for diagnosis and treatment.”
Is there a difference between normal pressure and “traditional” hydrocephalus?
The short answer is yes. While the symptoms of NPH tend to come on gradually, those associated with traditional hydrocephalus may appear quite suddenly.
The pressure inside your skull that’s associated with NPH is mostly within the normal range, most likely because your brain has time to adjust to the excess fluid. By contrast, the rapid onset of symptoms seen in traditional hydrocephalus creates a higher level of intracranial pressure.
Still, both types of hydrocephalus are treated in the same way, Dr. Knopman says.
What is the gold standard for the treatment of hydrocephalus?
“We insert a ventriculoperitoneal (VP) shunt—a small tube—into the brain, which reroutes the excess fluid into the abdomen, where the body can absorb it,” he says. “The procedure only takes about an hour, and patients usually stay in the hospital for a day or two.
“The shunt includes a valve that can be adjusted over time to meet individual needs,” he adds.
How long does it take to recover from the procedure? From NPH itself?
Recovery is gradual, with most patients experiencing improvement over several months. Sometimes, he says, “patients can even get better within a few weeks after shunting.”
Prospects for a return to normal are excellent when NPH is diagnosed early and treated appropriately. From 80 to 90 percent of patients show significant improvement, if not total resolution, in short order.
Are there ever complications in the aftermath of shunting?
Complications are relatively rare, says Dr. Knopman, occurring in roughly 10 percent of patients. The main risks have to do with the shunt’s malfunctioning, getting clogged with protein or getting infected. In these cases, it will need to be removed and replaced.
A hopeful picture
Although brain disorders are often serious, NPH stands out for its treatability when caught early.
“With timely and proper treatment, most patients go on to live full, active lives,” he says.
Billy Joel’s openness in sharing his condition has raised awareness around an often-overlooked disorder that affects thousands of older adults every year.
At this rate, Dr. Knopman predicts, there’s every reason to believe we’ll see the piano man back on stage”—sooner rather than later!
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