Music legend Billy Joel’s recent diagnosis of normal pressure hydrocephalus or NPH, has brought more attention to this relatively uncommon, debilitating condition. Endeavor Health neurosurgeon William Ares, MD, highlights the symptoms and treatment for this disease that strikes older adults, usually those over 65 and, more commonly, after the age of 70.
NPH happens when an excess of cerebrospinal fluid builds up in the skull and presses on the brain. While it can sometimes, rarely, be caused by a traumatic brain injury, it is more often a disease of aging and the cause remains unclear, explained Dr. Ares.
Classic triad of symptoms
“The symptoms of NPH are very clear, with a classic triad, often known as the three Ws, for wacky, wobbly and wet, meaning elements of dementia, gait disfunction and urinary incontinence,” said Dr. Ares.
One of the challenging elements of characterizing the condition is that it can be occurring in conjunction with other age-related cognitive decline, and slow, insidious onset of symptoms.
“Unfortunately, this triad of symptoms is fairly common in aging, so NPH is not usually the first thing people think of,” said Dr. Ares. “While Alzheimer’s is profoundly common, NPH is not as frequently occurring.”
To move from suspected NPH to confirmed NPH, a patient will undergo a spinal fluid drainage trial where excess spinal fluid is drained. When a person responds positively, and their symptoms improve following the fluid drainage, physicians are able to make an accurate diagnosis for NPH.
While historically a lumbar drain trial has been the mainstay of diagnosis and has been done over a three or four-day hospital stay, Dr. Ares and colleagues have created a one-day protocol with thorough evaluation before and after the lumbar puncture/fluid draining and patients can be diagnosed much more quickly without the discomfort of a multi-day trial.
Brain shunt surgical treatment
Treatment for NPH is surgery to implant a shunt in the brain to drain the excess fluid. Neurosurgeons like Dr. Ares place a tube into the ventricles of the brain, tunneling down to the abdomen where the fluid is drained. A programmable valve controls how much fluid is removed and can be adjusted for more or less volume as needed.
Unfortunately, the shunts do have some risks, including possible infection or failure. But most patients show significant improvements after shunting, particularly with gait and movement issues.
“One thing I tell patients is that NPH is very likely a progressive disease and when we put a shunt in, on average people see benefits for five to seven years,” said Dr. Ares.
A new trial is underway to assess emerging technology with a novel device that drains excess fluid into the cerebral veins, that may represent a less invasive way to treat NPH.
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