While other dementias including Alzheimer’s disease cannot be reversed, Normal Pressure Hydrocephalus (NPH) is an illness with dementia symptoms that can dramatically improve after surgery. NPH is caused by excessive spinal fluid in the brain, but identifying it is difficult and people with the disease are often misdiagnosed as having Alzheimer’s or Parkinson’s. The surgical insertion of a device called a shunt, however, can offer relief for people with NPH, and for this reason it’s important to know whether your loved one is among the five to 10 percent of people with dementia who have NPH.
This article describes the causes and symptoms of NPH, with details about the surgery and advice for caregivers of people with NPH.
People with Normal Pressure Hydrocephalus (NPH) have too much cerebrospinal fluid in pathways called “ventricles” that connect the brain to the spinal cord. Fluid in these ventricles usually keeps the brain floating and protected, and also provides nutrients to maintain brain chemistry and a functioning nervous system. NPH causes the ventricles to enlarge and put pressure on surrounding brain tissue. When brain tissue around the ventricles compresses due to the pressure, the body reacts with symptoms including difficulty thinking that is similar to dementia, as well as trouble walking and the loss of bladder control.
The disease has “normal” in its name because if doctors take a spinal tap, drawing cerebrospinal fluid from a space in the spine, the results of that test will read as normal despite the pressure in the brain.
How a person gets NPH in the first place is usually unknown. Someone can develop the sickness because of a tumor, head injury, hemorrhage, or infection. Meningitis, which is inflammation in the spinal cord caused by an infection, has been linked with NPH.
About 700,000 Americans have NPH, and the typical age of onset is in someone’s 60s or 70s. The symptoms of normal pressure hydrocephalus (NPH) are:
– Inability to walk normally. The gait problems are described sometimes as unsteadiness similar to “walking on a boat.” People with NPH will also say they feel like their feet are stuck to the ground.
– Dementia, usually beginning with the loss of motivation, loss of short-term memory, and difficulty completing normal tasks. Changes in personality and an inability to concentrate are also common with NPH.
– Urinary incontinence. An inability to control the bladder means someone with NPH will urinate at inappropriate times and/or feel the need to relieve themselves constantly.
These symptoms might not all occur. The walking troubles come first and are most common, followed by dementia. Loss of bladder control typically is the last symptom to arrive after years with the disease.
NPH is often misdiagnosed because these symptoms are similar to other dementias like Alzheimer’s disease and Parkinson’s disease with dementia. A correct diagnosis requires a doctor to run numerous tests including neurological exams, a CT scan or MRI, and a gait analysis that times a person’s walking pace. Even though the “normal” in normal pressure hydrocephalus comes from the “normal” readings that appear when a spinal tap is taken from someone with the illness, a spinal tap is still likely to be part of testing. While pressure readings may not indicate fluid building up in the brain, doctors will still perform a tap to analyze for abnormalities.
NPH can be fatal if not treated, but treatment can significantly reduce symptoms.
Normal Pressure Hydrocephalus is much rarer than Alzheimer’s disease, affecting about 700,000 Americans compared to more than 5.5 million people with Alzheimer’s. NPH also differs from Alzheimer’s, and other dementias, because there is a surgical procedure that is successful in lessening symptoms, sometimes significantly. With Alzheimer’s and others, the only treatment is therapies and medications that help manage symptoms but cannot reverse them. The dementia that comes from NPH is considered “mild,” similar to the early stages of Alzheimer’s. In the later stages of Alzheimer’s, the disease can become so severe that a person forgets friends and family, and may ultimately forget how to swallow. NPH does not affect thinking ability this dramatically.
There is no cure for Normal Pressure Hydrocephalus, but the disease can be treated with surgery, and symptoms often reduce dramatically. The operation begins with doctors performing a spinal tap to remove a large amount of cerebrospinal fluid. If this is tolerated, and symptoms improve fairly quickly, then a thin tube called a “shunt” is inserted into the brain to relieve pressure. The shunt drains excess spinal fluid into another part of the body for absorption. Shunts can be implanted with valves that are controlled without additional surgery. Valves may also be gravity-controlled, responding to pressure and adjusting to avoid over-drainage.
This operation is the only known treatment for NPH, and has been successful for reducing symptoms. Some subjects have reported such dramatic improvement that they almost feel cured but, again, there is no cure.
There is no medication that helps with symptoms of NPH. Drugs cannot reduce the fluid around the brain to relieve pressure and stop the symptoms.
Most important for family members of someone with NPH is to stay updated on medical procedures and find out if your loved one qualifies for surgery to insert a shunt. Not everyone with NPH can have the surgery, but the benefit for those who qualify might be a dramatic reduction in symptoms. After the shunt, some sufferers have seen full recovery of their ability to walk, with improved thinking.
Before surgery, as symptoms begin to show, it’s important for family members to stay supportive. Someone with NPH can find the loss of focus and short-term memory (and independence) incredibly frustrating. Bouts of depression and aggressive behaviors may become more common, and symptoms will get worse if a caregiver doesn’t empathize. The best way to handle frustrations is to communicate openly and offer hope and support.
A person with mild dementia is unlikely to wander or forget friends and family, but communication ability will suffer. A caregiver needs to practice hearing skills like anticipating which words might be dropped or mixed up as a person speaks. It’s also a good idea to minimize distractions. For more on communicating with someone with dementia, click here.
The difference is the level of care provided. Assisted living is more independent—an apartment-style environment providing meals and personal care including help with activities of daily living like eating and getting dressed. Memory care is specifically for people with dementia who have lost some independence and require more routine and structure. Staff in memory care is specially trained to help people whose illnesses prevent them from being able to communicate properly. Memory care offers more supervision and security, including surveillance and special locks to prevent wandering.
Consulting with doctors and visiting the residential communities in your area is the only way to know which option is best for your loved one. An assisted living or memory care home may be a perfect fit for someone with NPH whose symptoms are not relieved with surgery. Click here for more reading on how to find the right home.
A nursing home, which offers assisted-living-style housing with full-time nursing care, may be a better option if your loved one has additional debilitating medical issues. More on nursing homes.