Courtesy of UB Neurosurgery

Recently, American singer, songwriter, and musician Billy Joel, was diagnosed with normal pressure hydrocephalus (NPH), a brain disorder that can affect balance, vision, and hearing. NPH involves a buildup of cerebrospinal fluid inside the brain. Billy Joel has canceled all of his upcoming concerts, is undergoing physical therapy, and his doctors say that a full recovery is likely.

According to Elad Levy, MD of UB Neurosurgery, NPH is a condition characterized by abnormal accumulation of cerebrospinal fluid (CSF) in the brain’s ventricles, leading to increased pressure within the brain. Despite this increase, the pressure within the ventricles remains within normal ranges, hence the term “normal pressure hydrocephalus.” Dr. Levy says, “NPH typically affects older adults and can lead to symptoms that resemble other neurodegenerative disorders, complicating diagnosis and treatment.”

According to the National Institutes of Health (NIH), the exact cause of NPH remains unclear, but is generally categorized into two types: idiopathic and secondary. Idiopathic NPH occurs without a known cause, while secondary NPH results from conditions that affect the flow and absorption of CSF, such as a head injury, brain surgery, subarachnoid hemorrhage, meningitis, or other infections.

“NPH is associated with symptoms that can significantly impact an individual’s quality of life, such as gait disturbance, urinary incontinence, and cognitive dysfunction. One of the most common and earliest signs of NPH is difficulty in walking. Patients often exhibit a shuffling gait, taking small steps and experiencing balance issues. They may feel as though their feet are glued to the floor. Another hallmark symptom is urinary urgency or incontinence, characterized by frequent urges to urinate or loss of control over bladder function, which can be socially and emotionally distressing. NPH can also lead to cognitive impairment, often manifesting as memory problems, slow thinking, and difficulty in executing tasks. This cognitive decline can be mild to severe and is frequently mistaken for dementia,” says Dr. Levy.

Diagnosing NPH can be challenging due to its overlapping symptoms with other neurological conditions like Alzheimer’s disease and Parkinson’s disease. A comprehensive evaluation includes medical history and physical examination; neuroimaging studies such as MRI or CT scans to detect ventricular enlargement; CSF pressure measurement through lumbar puncture; CSF drainage tests to assess symptom improvement; and neuropsychological testing to evaluate cognitive function

The primary treatment for NPH is surgical intervention, specifically the placement of a ventriculoperitoneal (VP) shunt to divert excess CSF from the ventricles to the abdominal cavity, where it can be absorbed. This can alleviate symptoms and improve the patient’s quality of life. While it is the most common treatment, potential risks and complications include shunt malfunction or blockage, infection, over- or under-drainage of CSF. Early diagnosis and treatment can lead to significant improvement in symptoms, particularly gait disturbance. Cognitive and urinary symptoms may also improve, but the degree of recovery can differ. Post-treatment management involves regular follow-ups to monitor shunt function and address any complications. Physical therapy can help patients regain mobility and balance, while occupational therapy can assist in daily activities and cognitive exercises.

Living with NPH requires a multidisciplinary approach, involving neurologists, neurosurgeons, physiotherapists, and caregivers. Education and support for patients and their families play a crucial role in managing the condition. In Western New York, UB Neurosurgery is at the forefront of diagnosing and treating NPH. If you or a loved one has symptoms of NPH, consider contacting UBNS to make an appointment by calling 716-218-1000, or visiting www.UBNS.com.