At 79, Tamar wasn’t ready to slow down: She walked three miles a day, was a whiz at Sudoku puzzles, and loved hiking with her husband. But within just a year, she had sharply declined. The once-vibrant septuagenarian could no longer get out of a chair unassisted, didn’t understand how to use a telephone, and had become incontinent. “She was a shell of her former self,” remembers her daughter Iris. “Her physician told us that she had atypical Alzheimer’s disease and that nothing could be done for her.”

Frustrated, Iris and her sisters brought their mother to the Memory Disorders Unit at Mass General, where she failed a series of cognitive tests. The situation seemed hopeless. Then, an ordinary dinner out changed everything. As Iris described her mother’s symptoms, a friend at the table said she thought they sounded familiar. Later, she gave Iris a newspaper clipping describing a man’s struggle with normal pressure hydrocephalus, or NPH. “We’d never heard of NPH before,” says Iris. “But our family decided we wanted Mom to be evaluated for it.”

A Tricky Diagnosis

It may not be a household name, but NPH is increasingly being recognized as a potential cause of symptoms like Tamar’s. In this disorder, excess cerebrospinal fluid accumulates in the ventricles of the brain, leading to a triad of classic symptoms: gait abnormalities, cognitive difficulties, and loss of bladder control. Yet these symptoms often mimic other conditions common in people over age 60, including Alzheimer’s disease, Parkinson’s disease, prostate problems, and overactive bladder, explains Steven E. Arnold, MD, Translational Neurology Head of the Interdisciplinary Brain Center at Mass General. “NPH is a very difficult diagnosis to make,” he says. “But, as one of the only treatable forms of dementia, it’s also an incredibly important diagnosis.”

To address the need for streamlined evaluation and treatment of NPH, Arnold has joined with William T. Curry, MD, an attending in the hospital’s Department of Neurosurgery, to create the Normal Pressure Hydrocephalus Program. Together with nurses Rose Gallagher, RN, and Kara Warner, NP, they provide a team-based approach to diagnosis and care for people with this brain disorder. “Evaluation of NPH by a single specialist can be tricky, because you may have built-in biases based on your previous experiences with patients,” says Curry. “We use an algorithm to assess patients with possible NPH. Our goal is to standardize evaluation follow-up for those who have had a question of NPH or symptoms of it.”

Multidisciplinary Care, All in One Place

A visit to the NPH Program typically begins when a primary care provider or community neurologist refers a patient. These patients may be exhibiting symptoms of NPH, or they may have been told they have the condition and are seeking a second opinion. “We’re not trying to replace the work of other neurologists,” says Curry. “We want to work with them to either rule in NPH or rule it out. What makes the Program different is that we’re centralizing the process and providing evaluation and treatment in the same clinic.”

The first stop in that process is careful evaluation by Arnold, which includes a neurologic examination and medical history. If he deems NPH likely, a lumbar tap test is next. Also known as a spinal tap, the procedure removes a few tablespoon of cerebrospinal fluid from the base of the spine, usually takes less than 20 minutes, and is performed right in the exam room. Because this test acutely lowers pressure in the cerebrospinal system, the results can indicate NPH. “Some patients experience immediate gait improvement after the lumbar tap,” says Arnold. “They come to the hospital in a wheelchair and are able to walk out afterward.”

Others may not have such a dramatic response and are recommended to undergo a more gradual lumbar drain, which requires a three-day hospital stay. Based on the results, they may potentially benefit from the next step—the surgical placement of a shunt by Curry. The programmable shunt, which drains excess cerebrospinal fluid into the patient’s peritoneal cavity, has been shown to improve outcomes and decrease the need for future surgeries. For these patients, Arnold provides aftercare. And because Alzheimer’s disease and other neurologic conditions can be comorbid with NPH, patients receive follow-up for those disorders as well. “Even if we determine that someone doesn’t have NPH, we’re not sending them away without proper care,” says Curry.

Remarkable Potential for Patients

Although the NPH Program at Mass General is just a year old, Arnold and Curry say they have big goals. The pair aims to collect outcomes data on patients, which will inform future studies—the plan is to develop a research component of the program to ultimately improve care of people with NPH. Currently, this multidisciplinary approach is unique to the Boston area, and the program is one of just a handful of dedicated NPH clinics in the United States.

For Tamar and her family, coming to the NPH Program was a life-changing experience. Although she didn’t initially benefit from a lumbar tap, her response to the longer, inpatient cerebrospinal fluid drain was remarkable. “The next day, she suddenly knew where she was—her eyes were different, her voice was different,” remembers Iris. “It was like a flip switched and my mother was back.”

The journey hasn’t always been easy. Tamar, now 81, has no memory of the past year and a half. She’s puzzled when her daughters start crying at the sight of her performing simple tasks like tying her shoes, because she doesn’t remember not being able to do them. But she’s making progress every day, and has returned to her long walks. It’s a transformation that Iris doubts would have been possible without the NPH Program. “Drs. Arnold and Curry understand that not every condition is black and white, and they took the time to see the shades of gray with my mother,” says Iris. “There aren’t always many happy endings in neurology, but this is certainly one.”

To learn more about the NPH Clinic at Mass General, please visit their webpage.