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New Program Recognizes Hydrocephalus' Many Faces

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Winter 2001, Volume 14, Number 1

A patient comes to see you in a wheelchair. She’s fuzzy-minded, incontinent, has poor balance and can barely walk. Two weeks later, she’s doing the chicken dance at a wedding. The dramatic turnaround in some hydrocephalus patients after shunts drain excess cerebrospinal fluid never fails to delight neurologist Michael Williams, M.D.  Neurosurgeon Daniele Rigamonti, M.D., finds similar drama in draining CSF from patients with severe subarachnoid hemorrhage (SAH). It literally wakes them from a coma.

Drs. Daniele Rigamonti and Michael Williams
Williams (front) and Rigamonti check a patient's

But most adults with hydrocephalus, the two colleagues say, show more subtle symptoms; they need an involved diagnostic workup and finely tailored treatment. That, plus a swell in patient population - up from 44 last year to 100 this year - has prompted Rigamonti and Williams to launch the Johns Hopkins Hydrocephalus Program. One of four in this country, it’s the only such operation shared by neurosurgery and neurology. A urologist, cognitive neurologist, physical and occupational therapist round out the staff.

"Adult hydrocephalus is underdiagnosed and undertreated," says Williams. "We’ve long assumed this is a disorder of the elderly", he says, "but we’re finding that it’s not only poorly diagnosed in that age bracket, but it’s also rarely considered in younger and middle-aged patients, whose lives can be greatly disrupted.

"Hydrocephalus in elderly people can be insidious," he says. Their poor bladder control, balance and memory problems mimic old age. In people 18 to 55, symptoms are more subtle. Gait typically isn’t a problem, says Williams. But patients urinate frequently. They can’t keep mentally organized - they live by post-it notes - and may act depressed. Years of experience led the clinicians to write a diagnostic protocol now in use at Hopkins.

The typical treatment for the disorder is a shunt, implanted beneath the skin, to route CSF from ventricles in the brain to the peritoneal cavity. While CT scans may show the enlarged ventricles that hint at hydrocephalus, says Williams, "they don’t tell if a shunt would solve the problem." So Center staff use a conservative diagnostic technique, a two-day continuous monitoring of spinal fluid pressure followed by slow drainage of CSF. Patients who improve from the draining become shunt candidates.

"We’re cautious about giving shunts," says Rigamonti, "because they’re not without risk." Older patients apprehensive about unnecessary shunt surgery find the test relieving - although it’s time-consuming," adds Williams.

Rigamonti often sees acute cases where surgery’s a necessity. He’s seen many SAH patients whose intracranial hemorrhaging creates pressure so high it blocks CSF from being reabsorbed. A temporary catheter from the ventricles can drain excess fluid, he says, and, later, a shunt’s implanted if necessary. Recently, Rigamonti developed a ventriculostomy, an endoscopic operation for patients whose CSF pathway is blocked by tumor or another obstruction. A tiny hole in the wall of the third ventricle lets the CSF drain slowly.

Meanwhile, research on the syndrome continues. "When baby boomers show up," says Williams, "we hope to have better diagnostic techniques and outcomes for this often-ignored disease."


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