Normal Pressure Hydrocephalus is a disorder in the brain where an excess of cerebrospinal fluid accumulates in the brain’s ventricles, causing added pressure to the brain. The brain ventricles are enlarged due to the excess CSF, disrupting and damaging brain tissue. When this happens, it leads to difficulty walking, thinking, reasoning, and loss of bladder control.
Normal Pressure Hydrocephalus can occasionally be treated with surgery by inserting a shunt, a long thin tube, that will go and drain the excess cerebrospinal fluid from the brain to the abdomen. Surgery typically will help correct walking, but thinking, reasoning, and bladder issues will be less likely to see improvements. Shunting does not help everyone living with Normal Pressure Hydrocephalus. There is a lack of data on how long the benefits last from shunting.
Normal Pressure Hydrocephalus affects individuals in their 60s and 70s. The Hydrocephalus Association estimates that around 700,000 adults have Normal Pressure Hydrocephalus. Still, it is often misdiagnosed with Alzheimer’s Disease or Parkinson’s Disease.
What Is Normal Pressure Hydrocephalus?
Normal Pressure Hydrocephalus is a disorder that occurs when you have too much cerebrospinal fluid in certain parts of your brain.
Your brain and spinal cord are surrounded by a clear liquid called Cerebrospinal fluid, or CSF. It is made and stored in brain cavities called ventricles. This fluid moves around your brain from ventricle to ventricle. When extra fluid is in the ventricles, it usually drains away from the brain and is absorbed by the veins at the top of the brain. The function of the Cerebrospinal fluid is to protect your brain and spinal cord and supply them with the nutrients they need. Not only that, but it works to remove some of the waste products.
Hydrocephalus occurs when the natural draining and absorption of Cerebrospinal fluid does not function properly. This causes the ventricles to enlarge to account for the extra fluid, which then presses on other brain parts, causing different symptoms. Hydrocephalus can occur for many various reasons, some are born with it, and some develop it in their life.
Normal Pressure Hydrocephalus is a type of Hydrocephalus that happens in older adults in their 60s or 70s. Normal Pressure Hydrocephalus differs from other types of Hydrocephalus because it develops at a slower rate. The Cerebrospinal fluid drainage is blocked gradually, causing the fluid to back up slowly. Since this happens slowly, the ventricles also enlarge at a much slower pace. This still causes pressure to other parts of the brain, causing symptoms from Hydrocephalus.
The most common parts of the brain affected by Normal Pressure Hydrocephalus help control the legs, bladder, and the cognitive processes that help with reasoning and thinking. If the mental decline is severe enough, it will affect your day-to-day activities and cause Dementia.
The Dementia symptoms from Normal Pressure Hydrocephalus are similar to those living with Alzheimer’s Disease. The walking impairments are almost identical to those living with Parkinson’s DIsease. This is why so many cases are misdiagnosed for those living with Normal Pressure Hydrocephalus. Surgical procedures can restore your Cerebrospinal fluid to the correct levels. With proper treatment of Normal Pressure Hydrocephalus, it can be reversed and stop the progression of symptoms. Still, the first step is getting the appropriate diagnosis from your doctor.
At What Age Does Hydrocephalus Occur?
Hydrocephalus can occur at any age when there is too much Cerebrospinal fluid in the brain, which can not be flushed out by natural and normal processes of the body, which cause the ventricles to expand, adding pressure to the brain. Hydrocephalus is broken down into the following age categories.
- Infants.
- Toddlers and older children.
- Young and middle-aged adults.
- Older adults, 60 and over. This is the age group that we refer to as Normal Pressure Hydrocephalus.
Does Any Other Name Refer To Normal Pressure Hydrocephalus?
Doctors and healthcare providers will sometimes use other names when referring to Normal Pressure Hydrocephalus. These include:
- Normal Pressure Hydrocephalus
- NPH
- Adult-onset Hydrocephalus
How Common Is Normal Pressure Hydrocephalus?
It is hard to know how many individuals live with Normal Pressure Hydrocephalus because symptoms are similar to Alzheimer’s and Parkinson’s Disease. It is estimated that about 10 percent of people with Dementia may actually have Normal Pressure Hydrocephalus.
What Causes Normal Pressure Hydrocephalus?
Normal Pressure Hydrocephalus results from having too much cerebrospinal fluid that gets built up in the ventricles causing the ventricles to enlarge and put extra pressure on the brain. This swelling can lead to brain damage if it isn’t caught early enough.
The cause of Normal Pressure Hydrocephalus is often unknown. It may be caused by obstruction, poor absorption, overproduction, injury, bleeding, infection, brain tumor, or brain surgery.
Normal Pressure Hydrocephalus is rare and usually affects those 60 years of age and older. Symptoms are similar to those with Parkinson’s Disease or Alzheimer’s. Healthcare providers who specialize in Normal Pressure Hydrocephalus can usually tell the difference by doing special testing.
Cerebrospinal fluid plays a vital role in brain function. It does the following:
- It keeps the brain buoyant. The brain is heavy, and the Cerebrospinal fluid allows it to float within the skull.
- Adds a layer of cushioning to the brain to help prevent injury.
- Removes waste products of the brain.
- It helps maintain constant pressure in the brain by continually flowing back and forth between the brain cavity and the spinal column. This helps compensate for changes in the brain caused by blood pressure.
What Causes Too Much Cerebrospinal Fluid In The Ventricles?
The following factors contribute to too much Cerebrospinal fluid in the ventricles.
- Obstruction. The most common problem is a partial blockage that causes the cerebrospinal fluid to not flow properly from one ventricle to the other or around the brain.
- Poor Absorption. A less common cause is due to poor absorption of cerebrospinal fluid, often related to inflammation of brain tissue from disease or injury.
- Overproduction. On rare occasions, there is too much cerebrospinal fluid caused by overproduction. This means more fluid is being produced than can be absorbed or discarded properly.
- Injury, Bleeding, infection, Brain Tumor, or brain surgery. Any of these injuries can cause the cerebrospinal fluid not to be absorbed or appropriately discarded.
What Are The Risk Factors Of Normal Pressure Hydrocephalus?
The following risk factors increase your chance of being diagnosed with Normal Pressure Hydrocephalus. These include:
- Older than 60 years old.
- Have a brain infections
- Suffered a head injury
- Have a brain tumor
- Have undergone brain surgery.
Symptoms of Normal Pressure Hydrocephalus
Normal Pressure Hydrocephalus symptoms make it difficult to diagnose because they are similar to the beginning stages of Dementia.
The symptoms of Normal Pressure Hydrocephalus can be categorized into three different sections, walking abnormalities, Dementia, and impaired bladder control.
Walking Symptoms Of Normal Pressure Hydrocephalus
Walking symptoms of Normal Pressure Hydrocephalus include:
- Feeling unsteady
- Slowness in short steps
- Shuffling
- Halting steps
Bladder Symptoms Of Normal Pressure Hydrocephalus
Bladder symptoms of Normal Pressure Hydrocephalus include:
- Frequent urination
- Urgency in needing to use the bathroom
- Complete incontinence
Dementia Symptoms Of Normal Pressure Hydrocephalus
Dementia symptoms of Normal Pressure Hydrocephalus include:
- Lack of attention
- Lack of concentration
- Loss of complex thinking such as multi-step actions
- Loss of motor control
- Difficulty understanding
- Difficulty expressing ideas
- Forgetting things more often
- Extreme mood changes
- Lack of interest in things they used to love
When someone is living with Normal Pressure Hydrocephalus, it is often misdiagnosed as early-stage Dementia.
You may also see minor symptoms such as:
- Dizziness
- Difficulty walking up slopes and stairs
- Difficulty in rising and sitting on a chair.
- As Normal Pressure Hydrocephalus worsens, all the symptoms will become more noticeable.
Stages Of Normal Pressure Hydrocephalus
Since Normal Pressure Hydrocephalus is one of the causes of Dementia, it is possible for those living with Normal Pressure Hydrocephalus to worsen over time caused by the effects of too much cerebrospinal fluid. There are three stages of Normal Pressure Hydrocephalus. They are as follows:
- Stage 1- Progressive cognitive impairment (Dementia)
- Stage 2- Walking/balance issues
- Stage 3- Bladder Complications/ incontinence
Early treatment of Normal Pressure Hydrocephalus can vastly improve symptoms and lifespan. If Normal Pressure Hydrocephalus goes untreated, symptoms will worsen over time. Those who have been living with Normal Pressure Hydrocephalus with symptoms for years without proper treatment can still see improvements and live a longer life if they seek medical attention and get treated.
Diagnosis Of Normal Pressure Hydrocephalus
Diagnosing those living with Normal Pressure Hydrocephalus can be a little more difficult because it shares similar symptoms of Parkinson’s Disease and Alzheimer’s Disease. When a doctor thinks you may have Normal Pressure Hydrocephalus, they may refer you to a neurologist. One or more of the following tests may be done to confirm a diagnosis and assess if you are a good candidate for surgery.
Medical Interview
It is common for your doctor to ask you a series of questions to understand your symptoms better. Questions they may ask are:
- What are your symptoms, and when did they start?
- What are your medical problems now and in the past?
- What are your mental issues now and in the past?
- Family medical history
- Medications you are currently taking and medications you have taken.
- Lifestyle and habits
- Work and travel experiences
Physical Examination
Your doctor will perform a thorough medical examination to rule out other conditions with similar symptoms. The examination may include
- Tests of mental condition.
- Neuropsychological testing to test dementia symptoms.
- Lab tests to rule out other conditions or causes.
Gait Assessment
A Physical therapist may conduct a gait assessment. This helps evaluate the degree of impairment.
Your healthcare provider may order a few of the following tests and scans.
Brain Imaging
Through brain imaging, doctors will be able to identify if you have enlarged ventricles.
CT Scan
A CT Scan is a reliable way to determine if someone is living with Normal Pressure Hydrocephalus. It is a sophisticated technique when an x-ray beam is passed through the body, creating a picture of all the internal structures. In this case, the brain is displayed in the image. This can show enlargements in the ventricles and other changes that may lead to a Normal Pressure Hydrocephalus diagnosis.
MRI
An MRI uses radio signals and a strong magnet to create a detailed picture of the brain. This takes about 30 minutes and allows your doctor to see inside the brain to see if there are enlargements or other abnormalities.
Cisternography
Cisternography is not commonly used. They are much more involved than a CT scan or an MRI. This test will highlight absorption of the cerebrospinal fluid.
Spinal Tap
A spinal tap will measure your cerebrospinal fluid pressure and remove some of the fluid that is around the spinal cord. This fluid is then tested, looking for abnormalities to understand or give clues to what might be the problem.
Your doctor may remove more fluid than is required to see if this will relieve some of your symptoms. This is usually a temporary fix. If the removal of extra fluid does help, it is commonly recommended that a surgical shunt be helpful.
Tips For Visiting Your Doctor
You want to get the most out of the visits with your doctor. These tips can help you ensure that you get the most out of each visit.
- Know why you are seeing your doctor.
- Write down all your questions and concerns beforehand. This helps you to remember to ask all your questions.
- Bring someone with you to help you remember your questions and all the treatment options that may be available to you.
- Write down any new diagnosis, treatment option, recommended test, or medication.
- If medication or tests are recommended, understand why these are being recommended.
- Ask about new treatments that may be available. If you have a confirmed diagnosis of Normal Pressure Hydrocephalus, see if you are a good candidate for surgery.
- Write down all upcoming appointments, including the date, time, location, and the purpose for each visit.
- Be sure you know how to contact your doctor if questions come up before your next appointment.
- Get a care summary report from your visit.
Treatment Of Normal Pressure Hydrocephalus
Shunt System
The most common treatment for Normal Pressure Hydrocephalus is surgery. The doctor will place a shunt, a long tube, into the brain that will drain the excess fluid. Typically the shunt is inserted into a ventricle in the brain and then passed under your skin down through your neck, then chest to your abdomen. This is where the excess fluid is absorbed. The ventricles return to normal size. The shunt will stay in place as needed to help remove the extra cerebrospinal fluid.
Implanting a shunt is a huge benefit to many people; however, it does not work for everyone. Getting diagnosed early and getting proper treatment will help.
It is important to note that a shunt is not a cure. It does not treat the problem that causes the cerebrospinal fluid not to drain properly but does help dispose of the excess fluid to prevent further damage.
Endoscopic Third Ventriculostomy
Another surgical procedure called Endoscopic Third Ventriculostomy may be another option for individuals diagnosed with aqueductal stenosis. During this procedure, the neurosurgeon will use a special endoscope to create different paths for the cerebrospinal fluid to go to avoid the obstruction that is currently preventing the fluid from moving around correctly. There are still clinical trials investigating Endoscopic Third Ventriculostomy as a treatment for Normal Pressure Hydrocephalus.
What Are The Risks Of Placing A Shunt?
Placing a shunt to help eliminate the excess cerebrospinal fluid is very safe; however, complications can occur as with any procedure. Risks include:
- Excessive bleeding
- Infection
- Adverse reaction to anesthesia
- Difficulty breathing
- Changes in heart rate
- Changes in blood pressure.
There are rare risks of placing a shunt that can be serious and life-threatening if not treated. These include:
- Infection of the shunt or brain
- Blood clots
- Bleeding within the brain
- Damage to Brain tissue
- Swelling in the brain
Suppose you have a fever, headache, abdominal pain, fatigue, a spike in blood pressure, or the small symptoms you had before your shunt. In that case, you want to contact your doctor immediately. This is a sign of infection or malfunction that needs to be addressed quickly.
Management Of Normal Pressure Hydrocephalus
A shunt system can help treat those living with Normal Pressure Hydrocephalus. However, there can be complications after surgery. These can be within days or seen years later. The most common shunt complications are malfunction and infection. You want to look for the following signs and symptoms of malfunction.
Signs And Symptoms Of A Malfunction Of A Shunt
Shunt malfunction symptoms vary from person to person. You must be aware of all the signs of symptoms in a shunt malfunction. If these appear, contact your healthcare provider. These symptoms include:
- Difficulty walking
- Cognitive challenges/ mild Dementia
- Urinary problems (urgency or incontinence)
- Swelling along the shunt track
- Fever (a sign of failure or infection of the shunt)
- Redness along the shunt track (a sign of failure or condition of the shunt)
Progression Of Normal Pressure Hydrocephalus
Many factors contribute to the progression of Normal Pressure Hydrocephalus.
For those who seek medical care early, symptoms can remain mild and controlled with proper therapy and treatment. For those living with Normal Pressure Hydrocephalus who do not seek medical care, their symptoms will worsen. These include deteriorating Dementia as the brain tissue continues to be damaged caused by the extra cerebrospinal fluid.
Most of the time, conditions will worsen if symptoms go untreated. Shunt therapy can drastically improve outcomes in individuals that are good candidates for the shunt treatment.
If individuals living with Normal Pressure Hydrocephalus go without treatment, conditions will worsen, including worsening signs and symptoms of Dementia. Normal Pressure Hydrocephalus can lead to death when the brain tissue becomes significantly damaged.
Are There Ways To Prevent Normal Pressure Hydrocephalus?
There are no known ways to prevent Normal Pressure Hydrocephalus. However, if you think you are living with Normal Pressure Hydrocephalus, you will want to seek treatment right away.
What Is The Prognosis Of Normal Pressure Hydrocephalus?
The prognosis of Normal Pressure Hydrocephalus can be fatal if it is not treated. Detecting Normal Pressure Hydrocephalus early with a proper diagnosis and successful treatment plan dramatically increases your chance of a good recovery.
With surgery, therapies, and other interventions, those who have Normal Pressure Hydrocephalus can go on to live pretty normal lives. However, this varies from person to person.
If Normal Pressure Hydrocephalus goes untreated, symptoms will worsen over time.
What Are The Complications Of Normal Pressure Hydrocephalus?
The complications of Normal Pressure Hydrocephalus include:
- Difficulty walking
- Trouble thinking
- Loss of bladder control
When Should You Contact Your Healthcare Provider?
Suppose you or a loved one in their 60s or 70s have changes in your walking, thinking, or have a loss of bladder control. In that case, you will want to schedule an appointment with your health care provider as soon as possible. You will want to tell your doctor about the changes and when they started. Early diagnosis is key in helping prevent other long-term effects caused by Normal Pressure Hydrocephalus.
Key Points of Normal Pressure Hydrocephalus
- Normal Pressure Hydrocephalus is caused when there is an excess of cerebrospinal fluid in the ventricles, which causes them to enlarge, adding extra pressure to the brain.
- It may be corrected with a shunt. The long-term benefits of this are still unknown. Suppose you are a candidate for a shunt. In that case, you want to make sure you are aware of the signs of malfunction of the shunt and contact your doctor immediately if they occur, as they can be life-threatening.
- Symptoms of Normal Pressure Hydrocephalus include difficulty walking, difficulty thinking, or loss of bladder control.
- Early diagnosis may prevent other long-term problems.
- Normal Pressure Hydrocephalus is often misdiagnosed because it has similar symptoms to Alzheimer’s and Parkinson’s Disease.
- Normal Pressure Hydrocephalus is seen in those typically in their 60s and 70s.
- Normal Pressure Hydrocephalus may be caused by obstruction, poor absorption, overproduction, injury, bleeding, infection, brain tumor, or brain surgery.
- There are three stages of Normal Pressure Hydrocephalus. Stage 1 is signs of Dementia; stage 2 includes walking and balance issues; Stage 3 is bladder complications, including incontinence.
Resources
- https://www.alz.org/alzheimers-dementia/what-is-dementia/types-of-dementia/normal-pressure-hydrocephalus
- https://www.hopkinsmedicine.org/health/conditions-and-diseases/hydrocephalus/normal-pressure-hydrocephalus
- https://www.webmd.com/brain/normal-pressure-hydrocephalus
- https://www.mayoclinic.org/diseases-conditions/hydrocephalus/symptoms-causes/syc-20373604
- https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Hydrocephalus-Fact-Sheet
- https://thebrielle.com/resources/normal-pressure-hydrocephalus/
- https://my.clevelandclinic.org/health/diseases/15849-normal-pressure-hydrocephalus-nph
- https://www.healthline.com/health/ventriculoperitoneal-shunt#outlook
