This Woman’s “Dementia” Turned Out to Be a Treatable Brain Condition
It took years for doctors to discover that this woman's walking, incontinence, and cognitive problems were due to a treatable—and often misdiagnosed—brain condition called normal pressure hydrocephalus.
The dementia-like symptoms were subtle at first
The changes began slowly for Dorothy Sorlie. Her handwriting grew increasingly illegible, she lost interest in her books and recipes, and she started falling more often.
“The symptoms happened so gradually I’m not sure I was aware that something was happening,” says Sorlie, 79, a retired teacher in Eau Claire, Wisconsin. “It’s not like you get a pain in your knee and know you need to see a doc. It wasn’t that at all.”
What it was, Sorlie later found out, was a little known, poorly understood, and widely underdiagnosed condition called normal pressure hydrocephalus (NPH). Sometimes referred to as “treatable dementia,” NPH is an insidious and mysterious disorder that can mimic many other, more common age-related health problems.
It’s estimated to affect as many as 700,000 people in the United States. Yet some 80 percent of those cases are not properly diagnosed.
For several years, Sorlie was one of those cases.
Searching for answers
“I’m not sure I really thought, ‘Why is this happening?’ until it progressed a little bit,” Sorlie says. And even then, there were no satisfactory answers. “When you have to sign over power of attorney to your husband because you can’t even sign your bloody name—that’s a terrible feeling to get to that point and be without answers.”
Sorlie and her husband, Jim Urness, were nonetheless determined to find answers. They began a quest to understand her symptoms, which included mobility problems, cognitive issues, and urinary incontinence.
They tried a variety of treatments for the symptoms and traveled across the country in search of a diagnosis. “Native American healing in northwestern Alabama. Diagnostic chiropractic in Michigan. Eastern medicine, natural medicine, and Western medicine, of course,” says Sorlie. “I was even sent to Mayo Clinic Rochester for consultative care. They didn’t pick it up.”
All the while, her symptoms were getting worse.
“The mobility issues were significant,” Sorlie says. “My major occupation was falling. I had extensive foot surgery, thinking that might help my mobility. That was not a very good experience.”
Sorlie could no longer walk independently. And despite having always been a good swimmer, she found herself unable to stay afloat in a swimming pool. She was also experiencing mild dementia, she says, and urinary incontinence.
Theories and possible explanations repeatedly fizzled out. “I think I was tested for Parkinson’s maybe 2,700 times,” Sorlie says. Yet she, her husband, and her primary care doctor continued to press for answers.
“I’m blessed with a marvelous primary care physician,” she says. “Never once did he say to me, ‘Dorothy, you’re getting older—adjust.’ Even after I had been to consultative care, he said to me, ‘We’re going to go to a movement specialist again.’ And so he referred me to a movement specialist, who then referred me to a neurosurgeon. Those three docs are my heroes.”
The neurosurgeon ordered a CT scan, and Sorlie had barely gotten back home when he called with the results. “He said, ‘We’ve got it, we can fix it. However, it’s advanced,’ ” recalls Sorlie.
When she returned to his office the next day, he delivered the diagnosis: normal pressure hydrocephalus, a condition she’d never heard of before.
What is normal pressure hydrocephalus?
We all have cerebrospinal fluid protecting our brains. But sometimes too much of the fluid accumulates, which can lead to hydrocephalus. This can occur as a birth defect, or later in life as a result of brain injury. But there’s also a form known as idiopathic NPH, which is mostly shrouded in mystery.
It’s unclear why idiopathic NPH develops or what mechanisms are at work, although it typically strikes people who are in their 60s or older. (“Idiopathic” basically means “cause unknown.”) Those patients tend to exhibit one or more of NPH’s three classic symptoms: mobility problems, dementia, and urinary incontinence.
Because these symptoms are commonly found in elderly people for other reasons, the condition is easy for both patients and doctors to overlook or misinterpret, explains Abhay Moghekar, MD, associate professor of neurology at Johns Hopkins University.
“There are more common explanations for each of these symptoms, and that’s why it’s difficult for a busy internist who has just 15 minutes to see you in clinic,” Dr. Moghekhar says. “It presents with common symptoms of aging. But if you see the three together, your suspicion needs to be raised.”
The primary treatment option for NPH is to implant a shunt, which can help divert cerebrospinal fluid away from the brain. The shunt has a valve that releases cerebrospinal fluid, which drains to a location somewhere else in the body, usually the abdomen, to be absorbed.
This isn’t a good option for every patient, so there’s typically a screening process to determine its suitability. Sorlie was deemed a good candidate for the surgery.
She has little memory of what happened immediately post-surgery. She recalls a relatively slow recovery overall, with lots of physical and occupational therapy. But she was buoyed by support from friends and family, including her husband. Urness played a key role in her rebound, she says, as did Mocha Joe, a rescue golden retriever and therapy dog they liked to take to nearby hospitals.
“That dear dog,” says Sorlie. “When I was at my worst, he would sit by my side—not cuddle with me, but just (be) at attention.”
From his view as a care provider, Urness says he was more impressed with the early stages of Sorlie’s recovery. “She sometimes doesn’t give herself enough credit for the recovery from the most serious aspects of the condition,” he says. “I was greatly relieved within a week, two weeks. It continued to improve for a long time, but the major change happened very quickly.”
“My husband thinks I came back pretty quickly,” says Sorlie, who now walks five miles a day and is free of NPH symptoms. “He says, ‘You’re at 120 percent, and I’d just as soon you take off the 20 percent, please. I can’t keep up.’ ”
Many patients respond well to shunt surgery, Dr. Moghekar says, and the shunt can be adjusted over time with a magnet. Yet even when the shunt works well, he adds, the benefits sometimes begin to wane after a year or so, for reasons that still aren’t well understood.
“I know that there’s no cure for this, and that shunts do have problems,” Sorlie says. “I’m getting older, I realize that. But I’m feeling very healthy.” For now, she is using her dramatic recovery to help spread awareness of this often misdiagnosed condition.
She serves as an outreach volunteer for the Hydrocephalus Association, a role that she says comes naturally to her as a longtime educator. She wants to help others avoid the long, difficult journey she endured before being diagnosed with NPH.
She places no blame on any doctor or anyone else she met along the way, she says, and in fact learned a lot even when feeling hopelessly lost. They were all insightful and positive experiences, she says. And the difficulty of her journey simply illustrates the need for more awareness of NPH.
“If this awareness can help one person avoid what my husband and I endured, then my efforts are worth it,” she says.
- Hydrocephalus Association: "Normal Pressure Hydrocephalus"
- Abhay Moghekar, MD, associate professor of neurology at Johns Hopkins University