What is NPH, the condition sidelining Billy Joel?
Singer-songwriter Billy Joel, the renowned “Piano Man,” recently canceled all of his upcoming concerts due to a rare brain disorder known as Normal Pressure Hydrocephalus (NPH).
NPH occurs when excessive cerebrospinal fluid (CSF) accumulates, and the brain shows enlargement of ventricles (fluid chambers in the brain).
“There is a normal amount of cerebrospinal fluid we all have, which gets produced regularly and gets drained back into the body. When it doesn’t, that excess CSF can lead to a clinical triad of symptoms," said Cape Cod Hospital neurologist Mathew Pulicken, MD, MHS.
NPH symptoms include trouble walking, difficulty with bladder control and memory problems.
What causes NPH?
According to the Hydrocephalus Association, in most cases, doctors cannot pinpoint the exact cause of NPH. The majority of cases are referred to as idiopathic, arising spontaneously.
The condition is most commonly seen in adults aged 60 or over. A recent study estimates that 800,000 older Americans may be living with NPH, and more than 80 percent of cases remain unrecognized or untreated, according to the Hydrocephalus Association.
Testing is Important
Joel, 76, said in a social media statement posted by his representatives that his condition had been “exacerbated by recent concert performances, leading to problems with hearing, vision and balance.”
Without appropriate testing, said Dr. Pulicken, NPH can be misdiagnosed as Alzheimer’s disease or other dementias and diseases that can affect walking, including Parkinson’s disease. In addition, the symptoms – mobility, bladder control issues and memory problems – often emerge at different stages of the disease and can be mistakenly attributed to normal aging, or other conditions, which makes NPH diagnosis challenging.
How is NPH diagnosed?
Tests to confirm NPH include magnetic resonance imaging (MRI) and CT scans. If a patient has NPH, the imaging studies will show enlargement of the brain’s ventricles.
“That alone is not a conclusive diagnosis, as with normal aging and brain atrophy there is compensatory enlargement of ventricles,” he said. “However, it is a sign that raises suspicions. It’s important to follow the protocols. If there is clinical suspicion, and the imaging shows an enlargement of the ventricles, the gold standard test then is a lumbar puncture.”
Also known as a spinal tap, a lumbar puncture involves a needle inserted into the lower back and cerebrospinal fluid removed. As the name suggests, in NPH there is no increase in CSF pressure, but patients can show improvement in walking speeds and stability when CSF is removed temporarily. If diagnosed early enough – and treated correctly – patients with NPH can show a dramatic and rapid improvement in their gait.
“Walking speed/posture is something we can measure, and show improvement,” said Dr. Pulicken.
Bladder and cognitive progress can be slower to improve, he added, which adds to the importance of earlier diagnosis.
How is NPH treated?
The most common treatment is the surgical implantation of a shunt into the brain to drain the fluid, usually into the abdomen, where it can be absorbed. The procedure creates a path for the fluid to flow properly from the ventricles bypassing the blocked CSF drainage.
Dr. Pulicken said a new procedure is in the pipeline and being tried that’s less invasive. Instead of drilling a hole in the skull, a shunt is inserted into a blood vessel and progressed to the base of the brain to drain the fluid, but it has not yet been approved by the Food and Drug Administration.
Joel’s willingness to go public with his condition will be beneficial in bringing more attention to NPH and perhaps increased funding for research and treatments.
“It’s good to have more information out there and raise awareness on rarer syndromes,” said Dr. Pulicken. “NPH is rare, but in an older population like we have on the Cape, NPH can be seen in up to 5 percent of patients over 80, and early detection with approved treatments can help prevent worsening.”
