Reflections on Billy Joel's Normal Pressure Adult Hydrocephalus Diagnosis

Recently international music icon and Long Island favorite son, Billy Joel announced he was suffering from a brain disorder called Normal Pressure Hydrocephalus and cancelled all upcoming concerts. Columbia Neurosurgery’s Dr. Guy McKhann, a world leader in this topic weighed in to share some reflections for this condition and ways to approach treatment.

What is Normal Pressure Hydrocephalus? (Video)

Etiology/Cause: Most NPH patients do not have a known cause, so the full term is iNPH, with "i" standing for idiopathic, as we do not currently understand the reason.

Clinical symptoms/presentation: NPH is predominantly a gait disorder, with patients developing a shuffling gait and either the person or family members note that they start walking like their feet are stuck to the floor. Their gait is usually "magnetic," like they are walking with magnets in their shoes. NPH patients have a variable amount of cognitive decline, including worsening short-term memory and a decrease in their ability to carry out higher cognitive functions. There is also bladder involvement in the majority of patients, with urinary frequency and urgency leading to episodes of incontinence. Other symptoms, such as bowel symptoms, primary cognitive decline/dementia, headaches, and dizziness, are usually not caused by NPH. Patients with primarily cognitive symptoms without prominent gait and balance issues typically do not have NPH. Most NPH patients, unless their diagnosis has been missed for many years, do not have full-blown dementia.

Diagnosis: Diagnosis can be difficult as many conditions can cause similar symptoms in elderly patients. The combination of symptoms and patient/family/physician awareness usually leads to the diagnosis, often after symptoms have been present for months to years.

Treatment: Patients with NPH will usually improve clinically with a trial of spinal fluid drainage from their lower back, significantly increasing the likelihood that they do have NPH. Shunt surgery is recommended if they improve with this drainage trial regarding gait, cognitive function, and sometimes bladder function. A shunt drains spinal fluid from the brain to another body part.

Shunt surgery is generally safe and effective in well-selected patients. At centers such as Columbia University that focus on NPH, other members of the Adult Hydrocephalus Clinical Research Network, the risk of a surgical complication is low single digits.

Response to Treatment: Patient improvement following shunt surgery is primarily one of improved gait and balance, with variable improvement in bladder complaints and cognitive/memory issues. Patients will walk better with less imbalance, lower risk, and fear of falling. Patients and family members often comment on feeling "sharper" or "more alert" following successful shunt surgery. The response of a patient after shunt surgery for NPH is variable. While many patients remain much improved for many years, particularly in gait and balance function, some patients will gradually lose their shunt benefit over time. Having a shunt does not prevent a decline in function due to other causes such as Alzheimer's, Parkinson's, brain vascular disease, or other causes of neurocognitive decline/dementia.

Summary: As aging patients lose function, NPH is a favorable diagnosis, as it is one of the few treatable conditions that cause this loss. However, shunt surgery is not a cure and does not prevent other causes of age-related decline in function.