Johns Hopkins neurologist Abhay Moghekar and neurosurgeon Mark Luciano have seen it again and again — patients who were completely incapacitated, crippled by headaches, unable to walk or bewildered by declining vision. They often went years before they received help, passed around from doctor to doctor unable to diagnose or treat their mysterious neurological conditions. Many of these patients ultimately found relief and recovery at Johns Hopkins’ Hydrocephalus and Cerebral Fluid Center, which brings together a multidisciplinary team of neurologists, neurosurgeons, geriatricians, neuroradiologists and physical therapists to treat those with conditions that affect cerebrospinal fluid across the continuum of life.
Most patients seen at the center have one of four conditions: adult normal pressure hydrocephalus, adult congenital hydrocephalus, pseudotumor cerebri, or cerebrospinal fluid leaks from the nose, ears or spine. Each of these conditions is marked by disruption of production, absorption, and/or flow of cerebrospinal fluid (CSF) that ultimately affects brain function.
For all of these conditions, early diagnosis and treatment are key to preserving neurological function, says Moghekar. “We’re the only center in the country that sees this breadth of CSF disorders,” he adds.
Once a week, the team comes together to see patients in clinic, working as a cohesive unit. Each of these conditions has features that can complicate diagnosis, treatment or both, Luciano explains. For example, finding the exact source of a CSF leak can be difficult; operations to correct pseudotumor cerebri can be tricky because of the small size of patients’ ventricles; and the symptoms of normal pressure hydrocephalus can mimic many that accompany natural aging.
However, Moghekar says, the team’s wealth and breadth of experience and use of cutting-edge tools help patients achieve the best possible outcomes. In the last few years, the team has started taking detailed images of the brain’s venous system for patients with pseudotumor cerebri, since research has shown that many have stenosis in key blood vessels. Consequently, these patients might benefit from stenting rather than shunting. Similarly, Moghekar and other neurologists at the center have started using biomarkers in collected CSF samples from patients with suspected normal pressure hydrocephalus to look for recently discovered Alzheimer’s disease biomarkers. Patients with these biomarkers, he explains, have less favorable outcomes than those who don’t when they receive standard hydrocephalus treatments.
While many patients seen at the center require no follow-up after their conditions have been resolved, some continue to receive care there for years. These include adult patients with congenital hydrocephalus who had shunts implanted in infancy. Although congenital hydrocephalus is often considered a pediatric condition, patients don’t outgrow it, says Luciano, who began his career as a pediatric neurosurgeon. Rather, these patients need continual care for the rest of their lives.“Some of my patients have my cell number and know that if there’s a problem, they can reach me directly,” Luciano says. “It’s a privilege to be entrusted with their care and be able to nurture those relationships long term.”
