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Spreading the Word about NPH
By: Dean Olsen, Coppley News
CNS Photo by Kevin German
Six years ago Betty Boardman was successfully treated for normal pressure hydrocephalus.
It scares Betty Boardman to think what life would be like now if she hadn't found doctors in Chicago who spotted a brain disease that interfered with her sense of balance and forced her to use a walker.
The condition, known as normal pressure hydrocephalus, could have led to dementia, urinary incontinence and full-time dependence on a wheelchair.
"I was guided by the Lord," said Boardman, 84, a Springfield, Ill., resident who underwent surgery in Chicago in 1997 and found the results to be miraculous and almost immediate. A shunt that was inserted into her brain relieved symptoms of NPH and allowed her to walk without assistance only two days later.
NPH occurs when excess fluid accumulates in the cavities within the brain, but it often goes undiagnosed. It can cause symptoms similar to those caused by Alzheimer's and Parkinson's diseases.
Even when surgeons suspect the disease, they have been hesitant to conduct the routine operation to treat it, said NPH researcher Anthony Marmarou, vice chairman of the neurosurgery division at Virginia Commonwealth University.
Surgeons have been wary because the procedure carries a risk of infection, and they often are uncertain how many patients will benefit.
That's why Marmarou and others who treat NPH patients are looking forward to the publication of guidelines that will help doctors more confidently diagnose and deal with the disease.
They also are trying to spread information to the general public so patients and their families will put pressure on their doctors to investigate all the options if any or all of the classic symptoms - gait problems, urinary incontinence or mild memory loss -- are present. The disease typically strikes people older than 60.
"You wonder how many more are out there who could conceivably benefit," Marmarou said. "That's part of the mission -- trying to get the word out."
Only 20 percent of NPH patients are properly diagnosed and treated, according to the doctors who cared for Boardman at the Chicago Institute of Neurosurgery and Neuroresearch.
Marmarou estimated that NPH affects up to 10 percent, or 750,000, of the 7.5 million Americans thought to have Alzheimer's disease and other forms of dementia. In fact, he said as many as one in seven residents of nursing homes and assisted-living centers have the disease.
Marmarou said many people would improve so much after the surgery that they wouldn't need to stay at those facilities.
"The physician on the street sees so many disease processes," said Marmarou, a Ph.D.-level brain expert who collaborates with doctors in treating patients. "It may be equally effective to inform the lay public and let them alert the physicians."
For about a month in 1995, Boardman struggled to keep her balance while walking. The problem went away as suddenly as it started, only to return in a worse form in 1997.
The lifelong homeowner, married to the retired former owner of a funeral home, was frustrated in her search for a doctor in Springfield and at the Mayo Clinic who would diagnose her problem or attempt a treatment.
"There were quite a few doctors who checked me over, but there was just no definite decision," Boardman said.
In desperation, she and her husband, Robert Boardman, drove to Chicago on the advice of a relative who had a good experience with surgeons in a group practice called the Chicago Institute of Neurosurgery and Neuroresearch.
Doctors at the institute diagnosed NPH after tests that included an MRI, spinal tap and sonogram. Betty Boardman then underwent the $30,000 operation, which was covered by Medicare.
Neurosurgeon Dr. Gail Rosseau inserted a plastic tube into Boardman's brain through a small hole in the skull. This tube then was attached to a shunt placed under her skin.
As a result of the surgery, excess fluid in her brain's ventricles travels up through the plastic tube and to the shunt valve, which regulates the flow. The fluid continues its route through the flexible plastic tube as the tube snakes under her skin around the back of her ear, down her neck and across her chest.
The tube, still under the skin, then deposits the fluid in her abdominal cavity, where the fluid is absorbed into the body with no harmful effects.
Boardman's skin has healed over the incision on her head. She combs her hair over the bumps caused by the surgery, making the scar unnoticeable; the tubing below her skin is virtually invisible.
The surgery "was a piece of cake," Boardman said. "The only bummer was that I was allergic to the anesthesia" and sick for several days because of that. I never had a headache -- nothing. It was a very simple operation."
Boardman spoke recently at a medical seminar in Chicago to help spread the word about the disease and treatment. She said she is amazed that doctors aren't more aware of the condition, but one of her Chicago doctors wasn't surprised that the NPH wasn't diagnosed in Springfield or at the Mayo Clinic.
"In the past, the diagnosis was a little difficult to establish because there were a variety of ways to do it, but none of them was really very good," said Dr. Len Cerullo, a neurosurgeon and founder of the Chicago institute.
NPH can damage or destroy brain tissue because doctors believe that the cavities in the brain enlarge to handle the extra fluid. The surrounding brain tissue becomes compressed as a result. If the condition isn't relieved, permanent brain damage can occur.
Marmarou said experts involved in producing the new guidelines have learned that if NPH is suspected, cerebral spinal fluid should be drained with a spinal tap to determine if the patient would benefit from shunt surgery. The entire surgical procedure typically takes less than two hours.
Marmarou said the best test to pinpoint the problem involves draining small amounts of cerebral fluid for three days while the patient is hospitalized and closely observed. If the patient shows any positive benefits from drainage, it's likely he or she will benefit in the long term from a shunt.
The guidelines likely will be published within six months to a year in the Journal of Neurosurgery.
Dr. Jose Espinosa, a neurosurgeon at Southern Illinois University School of Medicine, agreed that the guidelines should be of assistance in treating NPH, and they also could help convince public and private insurers that they should pay for the three-day test in the hospital.
"We need a better way to find out who has it and who doesn't," he said.
Espinosa hopes the general public becomes more informed about NPH and asks primary-care doctors about it. He said many patients who have the disease probably never make it beyond a family practitioner or internist to reach a specialist like himself.
Espinosa said patients and their families are savvier nowadays and have searched the Internet before they quiz him about procedures and conditions.
"It keeps you on your toes," he said.
Betty Boardman, who was diagnosed and treated without the benefit of the new testing procedure, said she has a "new outlook on life since mine was almost taken away from me."
"So many people have this condition, and it goes undiagnosed for so long -- sometimes forever," she said. "Patients should explore all their options because there is always hope."
What is Hydrocephalus?
Aspects of Hydrocephalus
Causes of Hydrocephalus
Facts about Hydrocephalus
How is Hydrocephalus Diagnosed?
Signs of Hydrocephalus & Shunt Malfunctions
Normal Pressure Hydrocephalus
Types of Seizures