Little-known disorder looks just like dementia -- but can be reversed
Published Tuesday, April 18, 2017 10:00PM EDT
Loss of memory, mobility problems and issues with bladder control are often considered early indicators of dementia. But those are the same warning signs for a far lesser-known brain disorder that, in many cases, is completely reversible.
Normal-pressure hydrocephalus (NPH) affects an estimated 770,000 people in the U.S. and Canada, according to recent estimates, and most of those patients go undiagnosed. It happens when spinal fluids build up in the skull and put pressure on the brain.
But doctors have developed a surgical treatment that drains the problematic fluid from the skull. When performed early enough, the procedure can fully restore a patient’s memory.
The problem, doctors say, is that NPH often isn’t correctly identified. In Canada alone, doctors estimate that around 15,000 patients may be misdiagnosed with a more serious condition, such as Alzheimer’s and Parkinson’s disease, that leaves them bedridden or in nursing homes.
“Patients aren’t as aware of it, and doctors aren’t as aware of it. I would estimate that probably less than five per cent of the people who have it actually are getting treatment,” Dr. Mark Hamilton, a neurosurgeon at the University of Calgary, told CTV News.
It’s a scenario that Don Ethell is familiar with. In 2012, the former lieutenant governor of Alberta was beginning to struggle with mobility loss, memory and bladder control.
“I could see there was something terribly wrong,” said his wife, Linda Ethell.
Fortunately for Ethell, he visited Dr. Hamilton, who runs a specialized clinic at the University of Calgary that focuses on hydrocephalus. Hamilton tested Ethell for NPH and spotted the tell-tale fluid in his brain. He underwent surgery, which involved inserting a tube via his abdomen to the bottom of his skull to allow the fluid to drain out.
In fewer than three months, Ethell went from barely being able to lift his feet to walking normally.
“His walking improved dramatically, and his memory tests right now are normal,” Dr. Hamilton said.
This shunt treatment has an 87 per cent success rate, according to a study conducted last year by doctors from the University of Calgary and the University of British Columbia. Researchers also found that symptoms improved for 99 per cent of cases after a second treatment, if necessary.
It’s an astounding transformation that Dr. Hamilton has come to know through treating NPH patients.
“I’ve seen people with moderate dementia, significant short-term memory problems who are functioning at what we consider a normal level,” he said.
The trouble with tackling NPH, Hamilton says, is awareness.
“It’s not as well known a disorder as it should be,” he said. “It is a concern, because patients aren’t as aware of it and doctors aren’t as aware of it. People often get labelled as having Alzheimer’s disease and they don’t have effective treatments. So it is a concern that many of these patients are missed.”
Experts say the number of adults with NPH is increasing as increasing age is a risk factor
NPH is treated at most neurosurgical centres in Canadian cities, but there are only three cities with hydrocephalus specialty clinics -- Calgary, Vancouver and London, Ont. The Calgary clinic is by far the largest, with over 250 new referrals each year and over 1,400 patient visits each year.
Don and Linda Ethell hope that by sharing their story, they can help raise awareness for the unusual but curable neurological disorder.
“It’s just tragic. I hope that this brings awareness to all those out there who are suffering,” Linda said.
The condition, first discovered in 1965, is considered fairly uncommon and affects more than 1 in 200 adults over the age of 55, according to the Spina Bifida and Hydrocephalus Association of Ontario.
For Ethell, getting the proper care has given him another lease on life.
“This has been a breath of fresh air. It’s a second life,” he said.
If you’re concerned that you may have NPH, the Spina Bifida and Hydrocephalus Association of Canada has four recommended steps: record your symptoms, make an appointment with your doctor, ask for a referral for a CT/MRI scan and, once the results are in, request an appointment with a neurosurgeon for more information.
With a report by CTV medical specialist Avis Favaro and producer Elizabeth St. Philip