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Normal pressure hydrocephalus (NPH) is a neurological disorder that causes dementia. It is characterized by walking abnormalities, cognitive dysfunction (i.e., dementia), and urinary incontinence.
NPH is relatively rare. According to the Hydrocephalus Association, an estimated 375,000 Americans suffer from NPH (Hydro Assoc). However, it is difficult to diagnose NPH because its symptoms resemble other neurological diseases. Doctors must consider your symptoms and run numerous tests to make the diagnosis. For this reason, many sufferers go undiagnosed.
There are two forms of the disorder. The first is idiopathic (iNPH). The cause of iNPH is unknown. The second is caused by secondary disorders or symptoms. The risk of NPH increases after age sixty.
The only effective treatment is ventricular shunting. In cases of severe dementia, the risks of surgery do not outweigh the benefits. Most people show significant improvement after shunt placement. In fact, this condition is one of only a few causes of dementia that are potentially reversible.
The risk of developing NPH increases after the age of 60. As you grow older, you may have more risk factors.
Risk factors of NPH include:
The cause of idiopathic NPH is unknown. Doctors believe it is related to:
The second type of NPH is known as secondary or symptomatic form.
Conditions that may cause this type include:
The three symptoms that characterize NPH are walking abnormalities, cognitive dysfunction (dementia), and urinary incontinence. Minor symptoms may indicate the onset of NPH. Over time these symptoms will become more noticeable.
Minor symptoms in early NPH include:
Examples of walking abnormalities include:
Bladder symptoms caused by NPH include:
Cognitive dysfunction (dementia) symptoms include:
It can be hard to diagnose NPH because it appears similar to other neurodegenerative diseases. Multiple tests must be used to gather sufficient evidence for a diagnosis of NPH.
Neurodegenerative diseases with symptoms similar to NPH:
To help rule out other neurological conditions, your doctor may test your mental function with neuropsychological tests.
Types of neuropsychological tests include:
Imaging tests of the brain are helpful. However, they are insufficient for a diagnosis.
Examples of these include:
Invasive diagnostic tests are another option. These include:
The only successful treatment for NPH is ventricular shunting. Ventricular shunts are surgically implanted to drain excess cerebrospinal fluid (CSF).
Three types of shunts are used:
After the shunt is surgically implanted, it will have valves to control the amount of CSF drainage. The options for valves include:
All surgery carries the risk of complications. Shunt surgery has serious risks that include:
An individual’s outlook will vary according to his or her age, previous health problems, and surgery. Recovery becomes more difficult as age increases.
Surgery is not an option for every patient with NPH. Those with severe dementia are discouraged as the risks of surgery outweigh the benefits. Surgery itself carries over a 30 percent chance of complications in the first year.
A healthy individual who received an early diagnosis has the best outlook. People who are able to have the surgery without complications may see up to a 70 percent improvement in function.
Written by: Lydia Krause
Published on: Jul 16, 2012
Medically reviewed on: Mar 22, 2016: University of Illinois-Chicago, College of Medicine
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