Singer-songwriter Billy Joel has said he has been diagnosed with a neurological condition that prevents him from performing for some time. He is suffering from normal pressure hydrocephalus (NPH), where the fluids inside your brain cavities are overproduced, cannot be absorbed and accumulate over time. This is a medical malady that is both under-recognised and frequently misinterpreted. Joel has cited disturbances in balance, hearing and vision.
What Is Normal Pressure Hydrocephalus?
At first glance, the term normal pressure hydrocephalus appears oxymoronic — how can a condition characterised by fluid accumulation in the brain occur under “normal” pressure? Your body makes just enough cerebro-spinal fluid (CSF) that nourishes the brain and spinal cord and can be absorbed. Here CSF is in excess and cannot be absorbed by the body. Hence it settles in the ventricles or chambers of the brain, enlarging them and compressing adjacent brain tissue. Yet the pressure within the skull remains seemingly within normal limits, thus eluding easy detection.
What about symptoms?
The condition manifests through a triad of symptoms that are as disruptive as they are subtle:
● Gait disturbances, often mistaken for clumsiness or frailty;
● Cognitive impairment, echoing the early murmurings of dementia;
● Urinary incontinence, a loss of bladder control.
Most patients have trouble walking, develop balance problems and have falls. They complain of forgetfulness, confusion, depression and cannot follow questions.
What causes normal pressure hydrocephalus?
The normal flow of CSF throughout the brain and spinal cord gets blocked. This could be because of past injuries or bleeding, infection, brain tumor, or surgery. The extra fluid and pressure can lead to brain damage over time.
Why diagnosis is difficult?
It is difficult to diagnose this condition because its symptoms overlap with many other conditions. In older adults, diagnosis is difficult because it is assumed to be Alzheimer’s or Parkinson’s. Yet unlike these neurodegenerative conditions, normal pressure hydrocephalus is treatable.
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It can be diagnosed with neuroimaging, followed by a lumbar puncture that, if relieving symptoms even temporarily,
What about treatment?
Neurosurgeons implant a shunt under your scalp, a slender conduit that diverts excess fluid from the brain to the gut where it is absorbed. The shunt is inserted into the affected ventricle in the brain and then placed under your skin from your head through your neck and chest to your abdomen. When effective, the patient recovers quickly, regaining their cognitive and motor faculties. But it doesn’t work well with some patients and has limited benefits. Diuretics usually don’t work in this condition.
Why proper diagnosis matters
Cognitive decline is too often dismissed as an age-related problem and even treatable conditions are left undiagnosed. So if you see somebody’s steps faltering, their thoughts clouding, or their continence waning, do not dismiss it as an old age problem. It might just be a reversible neurological condition.
(Dr Chafale is Interventional Neurologist, Apollo Hospitals, Navi Mumbai)
