Billy Joel and Normal Pressure Hydrocephalus: Shining a light on a little known but potentially treatable condition

You may have seen in the news that Billy Joel has been diagnosed with normal pressure hydrocephalus (NPH), but what is it?  

NPH is a progressive neurological condition that falls under the dementia umbrella. It occurs when excess cerebrospinal fluid (CSF) builds up in chambers of the brain called ventricles. The ventricles expand and encroach on the surrounding tissues, causing the neurological symptoms of NPH. Unlike other forms of hydrocephalus, NPH does not result in significantly raised intracranial pressure. As a condition, it is more similar to other neurodegenerative conditions, such as Parkinson’s disease, and other causes of dementia, such as Alzheimer's disease. However, unlike many dementia causes, there are effective treatment options for NPH that can potentially reverse or improve symptoms or slow disease progression. 
 
NPH primarily affects older adults. Estimates vary, but it's thought that NPH could affect around 0.8% of people over 65 (1,2). In the UK, that would be approximately 100 thousand people.

NPH is characterised by a triad of symptoms: 

• Gait disturbances – Difficulty walking, shuffling steps, or feeling as if the feet are stuck to the floor. Difficulty balancing and falls, particularly backwards, are common. 
• Cognitive impairment – Memory loss, confusion, and difficulty concentrating. 
• Urinary incontinence – Loss of bladder control, which may worsen over time.

Because these symptoms can resemble those of Alzheimer’s or Parkinson’s disease, diagnosis is often missed, mistaken, or delayed. 
 
NPH is diagnosed through a combination of clinical presentation and the results of imaging and diagnostic tests. Investigations may include: 

• CT or MRI scans to detect enlarged ventricles and brain changes relating to NPH. 
• Lumbar puncture (spinal tap) to assess CSF pressure and response to fluid removal. 
• Gait analysis and cognitive tests to evaluate movement and memory function.

The most effective treatment for NPH is shunt surgery, where a small tube (shunt) is implanted to drain excess CSF from the brain to the abdomen. This procedure can significantly improve symptoms, especially if performed early. Evidence suggests that 76% of people experience some improvement after shunt surgery (3). Gait (walking) improves in 75% of patients, cognitive function improves in 61%, and improvement of incontinence can be seen in 55%. Where surgery isn't an option, for example, due to frailty, symptoms can be managed with multidisciplinary support including physiotherapy, medication, occupational therapy, and neuropsychology. 
 
Early diagnosis and treatment can greatly enhance quality of life for individuals with NPH. If you or a loved one experience symptoms, consulting a neurologist for evaluation is crucial. Shine can offer advice and support before, during, and after diagnosis of NPH. 
 
Shine has been raising awareness about NPH throughout NPH Awareness Week, which purposefully coincides with Dementia Action Week to help shine a light on this little-known condition. We want to help ensure everyone who needs it has access to an early and accurate NPH diagnosis to facilitate timely treatment and symptom management. 

Shine’s NPH support services are here for anyone in need in England, Wales, and Northern Ireland. You can access support by phone at 01733 555988 and by email at firstcontact@shinecharity.org.uk. We have an NPH information and resource hub and peer support Whatsapp groups you can join.  

For professionals wanting to know more about NPH we recommend joining Shine and completing our CPD accredited e-learning module on the condition. Your welcome email will contain a link to register for the Shine Academy e-learning platform. As a Shine professional member, you can also request advice, information, and resources.  

We were sorry to learn during Awareness Week that Billy Joel is struggling with symptoms of NPH and, as a result, has needed to cancel tour dates. We hope he has access to all the help he requires back in the USA, the Hydrocephalus Association there has lots of great NPH information.  

Shine wishes Billy Joel all the best and hopes he will be able to return to his music soon and "hold on till that old second wind comes along." 

References

1) O Conn H. (2011). Normal pressure hydrocephalus (NPH): more about NPH by a physician who is the patient. Clinical Medicine 11(2): 162–5. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5922741/ 

2) Kiefer M, Unterberg A. (2012). The differential diagnosis and treatment of normal-pressure hydrocephalus. Dtsch Arztebl Int. 109(1-2):15-25; https://pmc.ncbi.nlm.nih.gov/articles/PMC3265984/ 

3) Giordan, E., Palandri, G., Lanzino, G., Murad, M. H., & Elder, B. D. (2019). Outcomes and complications of different surgical treatments for idiopathic normal pressure hydrocephalus: a systematic review and meta-analysis. Journal of Neurosurgery JNS, 131(4), 1024-1036. https://doi.org/10.3171/2018.5.JNS1875