Duke’s Normal Pressure Hydrocephalus (NPH) Clinic was founded in November 2023. In less than two years, the multidisciplinary team representing neurology, neurosurgery, and physical therapy (PT) created a unified protocol for evaluating and treating patients with NPH in the outpatient setting in one day. This coordination is crucial because NPH is commonly undiagnosed or misdiagnosed due to symptoms that mimic other neurodegenerative diseases. 

“Timely testing and diagnosis are important because we can treat and possibly reverse NPH,” says Alexa Bramall, MD, PhD, Duke neurosurgeon. “Some patients experience immediate improvements.” For patients who do not have NPH, the clinic’s comprehensive testing guides patients to the proper diagnosis and team. “If we determine a patient has a different neurodegenerative diagnosis, our pathway quickly routes them to neurology and memory disorders colleagues to get the care they need,” Bramall adds.

Some patients are incidentally identified because scans show ventriculomegaly. Primary care physicians or neurologists identify others based on symptoms, which include gait ataxia, cognitive decline, and urinary incontinence, prompting further workup. Gait disturbances are often the first distinguishable signs. “Changes in the ability to walk, bumping into objects, or frequent falls without signs of cognitive decline in any patient above the age of 65 is a good indication that they might have NPH, and should be further evaluated with brain imaging,” says Bramall. 

“NPH symptoms and age of onset can overlap with Alzheimer's, Parkinson’s, and other movement and memory disorders. Patients also commonly have comorbidities, so having multiple disciplines working together is key for the best patient care,” says Andy Liu, MD, MS, behavioral neurologist.

Patients’ evaluations are typically about two and a half hours and may include a full neurological exam, PT evaluation, an MRI, a high-volume spinal tap, and biomarker testing. “The high-volume spinal tap is a key diagnostic tool to identify if a patient is a candidate for a shunt,” says Bramall. 

The patient’s gait and cognitive function are assessed before and after the tap. “We’re looking for a 30% improvement in gait. If we don’t achieve that with the high-volume lumbar puncture, we may admit the patient for longer testing, or determine NPH isn’t the cause of their symptoms.” 

One unique thing about Duke’s NPH clinic is the comprehensive evaluation protocols that include biomarker testing with a skin biopsy test along with Alzheimer’s Disease biomarkers in the cerebrospinal fluid. “We can detect phosphorylated alpha-synuclein, a key biomarker for Parkinson's disease and other synucleinopathies. Then, we know if one or more factors contribute to the patient’s symptoms and we direct our care accordingly,” says Liu.

If the patient is positive for NPH, patients follow up with Bramall for treatment options, which typically include a programmable shunt to regulate the proper flow and drainage of cerebrospinal fluid. 

“With MRI-compatible programmable shunts, we can adjust the valve to change the drainage rate after surgery,” says Bramall. “NPH can change over time, so programmable shunts allow us to make adjustments non-invasively.” This approach reduces the need for future surgeries in many cases. 

The team has also integrated technology to help patients track and monitor their gait changes at home. “We offer patients a mobile app to record their progress or challenges. It helps give us more information to adjust their shunt or offer more physical therapy when needed,” says Bramall.