Medically reviewed by Alan Lucks | MD, Alan Lucks MDPC Private Practice - New York on March 4th, 2024.
This condition primarily affects adults over 60, with cerebrospinal fluid accumulating in brain ventricles despite maintaining normal intracranial pressure (hence "normal pressure").
The classic triad presents as "wet, wobbly, and wacky"—urinary incontinence develops first in 85% of cases, followed by shuffling gait with magnetic steps, then cognitive decline mimicking dementia.
Lumbar puncture provides both diagnostic confirmation and treatment preview—removing 30-50mL of spinal fluid temporarily improves walking and cognition in true NPH cases within 2-4 hours.
Ventriculoperitoneal shunt surgery succeeds in 60-90% of patients when performed early, with walking improvements appearing first (within weeks) followed by bladder control and cognitive gains.
MRI reveals enlarged ventricles with a specific pattern called "Mickey Mouse sign" on coronal views, plus narrowed high convexity spaces that distinguish NPH from normal aging changes.
Normal pressure hydrocephalus (NPH) is a condition that occurs when there is an excessive buildup of cerebrospinal fluid (CSF) in the brain's ventricles. CSF is a clear liquid that surrounds and protects the brain and spinal cord, providing nutrients and removing waste products. In NPH, the natural drainage system for CSF is gradually blocked, causing the ventricles to enlarge and press on various parts of the brain.
NPH usually affects older adults, with the average age of onset being over 60 years. The condition develops slowly over time, and the pressure inside the brain may not be as high as in other types of hydrocephalus. However, the enlarged ventricles can still cause symptoms by pressing on the brain.
The most common symptoms of NPH include:
Dementia-like symptoms: Memory loss, speech problems, apathy, changes in behavior or mood, and difficulties with reasoning, attention, or judgment.
Walking problems: Unsteadiness, leg weakness, sudden falls, shuffling steps, difficulty taking the first step, and "freezing" while walking.
Urinary symptoms: Inability to hold urine (urinary incontinence), frequent urination, and urgency to urinate.
These symptoms can be mistaken for signs of aging or other conditions like Alzheimer's or Parkinson's disease. However, the combination of dementia-like symptoms, walking problems, and urinary issues should alert healthcare providers to the possibility of NPH.
In most cases, the exact cause of NPH is unknown. However, several factors can contribute to the development of the condition, including:
Head injury
Bleeding around the brain (due to a blow to the head)
Stroke
Meningitis (infection of the protective tissue around the brain)
Brain tumor
Surgery on the brain
Diagnosing NPH can be challenging, as the symptoms can resemble those of other conditions. To diagnose NPH, healthcare providers may perform the following tests:
Medical interview and physical exam: To assess symptoms, medical history, and rule out other disorders.
Neuropsychological testing: To accurately document cognitive problems and strengths.
CT scan or MRI of the head: To visualize the brain and detect ventricular enlargement or other changes suggestive of NPH.
Lumbar puncture (spinal tap): To measure CSF pressure and analyze the fluid for abnormalities. Removing excess fluid during this procedure may temporarily relieve symptoms.
The primary treatment for NPH is a surgical procedure called a shunt operation. A shunt is a thin tube implanted in the brain by a neurosurgeon to drain excess CSF from the ventricles to another part of the body, usually the abdomen. The shunt is equipped with a valve that opens when pressure builds up, allowing the fluid to drain harmlessly and be absorbed by the bloodstream.
Shunt surgery is not a cure for NPH, as it does not address the underlying cause of the condition. However, it can help relieve symptoms in many people. The success of the surgery depends on various factors, with earlier diagnosis and milder symptoms generally associated with better outcomes.
For those who are not candidates for surgery, treatment focuses on managing mood and behavioral problems, coping with physical issues like incontinence and walking difficulties, and optimizing overall functioning.
Caring for a person with NPH can be challenging, as the condition affects every aspect of their life. Family members and caregivers may experience a range of emotions, including feeling overwhelmed, depressed, frustrated, or resentful. Support groups can be a valuable resource for coping strategies and connecting with others in similar situations. Organizations like the Family Caregiver Alliance and the Hydrocephalus Association offer resources and support for those affected by NPH.
NPH is a progressive condition, meaning that symptoms generally worsen if left untreated. The outlook for individuals with NPH depends on the underlying cause and whether they are candidates for surgery. While there is no known way to prevent NPH, maintaining a healthy lifestyle and taking steps to avoid head injuries may help reduce the risk of developing the condition.
Early recognition of the wet-wobbly-wacky triad is crucial since shunt surgery can dramatically reverse symptoms when performed promptly, unlike most other causes of dementia. The lumbar puncture test offers both diagnosis and a preview of potential surgical benefits. If you're noticing this symptom combination in yourself or a loved one, Doctronic can help connect you with neurological evaluation quickly.
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