UMass Memorial team puts spotlight on disorder.
Janet M. Arcieri, an active retired social worker from Webster, didn't think anything was wrong three years ago, at age 72, except that she was having difficulty walking.
"I had to hold onto the walls," she said.
But the urinary incontinence Mrs. Arcieri experienced had increased, and her family was starting to worry that she was forgetting things.
Many older people and clinicians alike might have attributed these symptoms to early Alzheimer's disease, neurological conditions like Parkinson's disease, or even just aging. But when Mrs. Arcieri mentioned her concerns to her primary care physician at UMass Memorial Medical Center - University Campus, Dr. John Solomonides, he asked if he could do an MRI scan of her brain.
"I was a little taken aback," Mrs. Arcieri said. "We led a very full life. ... So why all the hoopla?"
What Dr. Solomonides found led him to refer Mrs. Arcieri to UMass Memorial neurologist Dr. Banu Sundar and Dr. Oguz Cataltepe, director of the center's pediatric neurosurgery. After further studies, they diagnosed Mrs. Arcieri with normal pressure hydrocephalus, or NPH.
The two specialists are part of a multidisciplinary team that two months ago launched the UMass Memorial adult hydrocephalus clinic, one of several surgical specialty clinics at the Lake Avenue campus. The clinical team includes specialists in neurosurgery, neurology, neuropsychology, ophthalmology and neuroradiology.
NPH is a condition in which the spaces, or ventricles, inside the brain fill with cerebrospinal fluid that doesn't get absorbed as it should, Dr. Cataltepe explained. The fluid-filled ventricles press on brain tissue and cause symptoms, which typically include a wobbly gait and imbalance, urinary incontinence and mild dementia.
NPH typically occurs in people older than age 60, when the brain begins to shrink and the ventricles enlarge. Unlike congenital forms of hydrocephalus affecting newborns, or hydrocephalus caused by tumors or infections, the pressure from the fluid in the brain isn't abnormally high but can still affect body functions.
Dr. Cataltepe said NPH accounts for 5 percent to 10 percent of all cases of dementia. But the condition is often misdiagnosed as something else, so the real number may be higher.
The Hydrocephalus Association estimates that at least 350,000 Americans have the condition.
"The same symptoms can be seen in other pathologies, which are neurological," Dr. Cataltepe said.
When Dr. Sundar first saw Mrs. Arcieri, she thought that her symptoms might be linked to Parkinson's disease, which also affects the ability to walk.
To rule out Parkinson's or other conditions, patients in the hydrocephalus clinic receive special MRIs and a multidisciplinary assessment. One of the tests is a lumbar puncture, or spinal tap, to remove some of the cerebrospinal fluid and see whether symptoms improve. A drain may be inserted for a day or two to fully assess changes.
Improvement from the lumbar puncture can occur almost immediately in patients with NPH.
Mrs. Arcieri said, "I got off that bed and walked down that long, long hallway, holding onto nothing. She (Dr. Sundar) made me do it five times."
Dr. Cataltepe showed Mrs. Arcieri images of her brain, which indicated she hadn't suffered brain tissue loss but may have been living with accumulating fluid for some time.
"The balance is minute," Mrs. Arcieri said. "Just taking those two vials that she (Dr. Sundar) took and I was walking. It's magnificent what they can do."
Mrs. Arcieri said she keeps her brain active with lots of reading and doing crossword puzzles.
She was a perfect candidate for a permanent shunt inserted through a hole drilled into her skull. The shunt continually drains excess fluid from her brain into her abdominal cavity, where it is absorbed.
The shunt is checked once a year and adjusted, if necessary, by magnets.
Dr. Cataltepe said that no more than 10 medical centers in the United States have multidisciplinary hydrocephalus clinics such as the one at UMass Memorial. Much of the research in this field comes from Europe and Scandinavia.
The UMass Memorial clinic has seen approximately 10 patients since it was organized, although doctors had previously seen hydrocephalus patients individually and referred them to each other.
"It just gives more continuity of care for these patients, and the diagnosis comes more quickly," Dr. Sundar said.
Mrs. Arcieri has "starred" in a recorded interview Dr. Sundar uses with medical students and residents on neurology rounds.
But she attributes the initial step to diagnosis to her primary care physician, Dr. Solomonides. She said he was tuned into the possibility of hydrocephalus because he is also a pediatrician and has encountered a different form of the condition with children.
Mrs. Arcieri said she told Dr. Solomonides: "We need to instruct our primary care physicians because they're the first ones that see this."
Ronald Arcieri, Mrs. Arcieri's husband, said the diagnosis and treatment of his wife's hydrocephalus by the UMass Memorial team clearly "picked up her spirits" as well as restored her normal activity.
"That was our miracle," he said.
Contact Susan Spencer by email at firstname.lastname@example.org. Follow her on Twitter @SusanSpencerTG.
CUTLINE: Dr. Oguz Cataltepe, director of pediatric neurosurgery at UMass Memorial Medical Center in Worcester, talks with patient Janet M. Arcieri of Webster, who was diagnosed with normal pressure hydrocephalus, or NPH, three years ago.
PHOTOG: T&G Staff/TOM RETTIG