A new Cedars-Sinai study suggests that some patients diagnosed with behaviorally variable frontotemporal dementia (bvFTD) — an incurable condition that denies patients the ability to control their behavior and cope with daily life — may instead experience fluid leakage. myeloid, which is often treatable.
The researchers say these findings have been published in the peer-reviewed journal Alzheimer’s disease and dementia: translational research and clinical interventions. It may point the way to treatment.
“Many of these patients experience cognitive, behavioral, and personality changes so severe that they are arrested or placed in nursing homes,” said Wouter Schevenck, MD, director of the Neurosurgery and Microvascular Surgery Program and professor of neurosurgery at Cedars-Sinai. “If they have behaviorally variable frontotemporal dementia with an unknown cause, there is no treatment available. But our study shows that patients with CSF leaks can be treated if we can find the source of the leak.”
Cerebrospinal fluid circulates in and around the brain and spinal cord to help protect them from injury. When this fluid leaks into the body, the brain can hang, causing symptoms of dementia. Many patients with sagging of the brain — which can be detected on an MRI — go undiagnosed, Schievink said, and advised doctors to take a second look at patients with telltale symptoms.
“The radiologist, neurosurgeon, or neurologist should look at the patient’s MRI again to make sure there is no evidence of brain sagging,” Chevink said.
Doctors may also ask about a history of severe headaches that improve when the patient lies down, extreme sleepiness even after adequate night’s sleep, and whether the patient has ever been diagnosed with Chiari cerebral malformation, a condition in which brain tissue extends into the spine. Channel. Chevink said sagging of the brain is often mistaken for Chiari malformation.
Even when sagging of the brain is detected, it can be difficult to determine the source of the CSF leak. When the fluid leaks through a tear or cyst in the surrounding membrane, it is visible on a CT scan of the spinal cord with the help of contrast medium.
Schievink and his team recently discovered an additional cause of a CSF leak: a CSF-venous fistula. In these cases, fluid leaks into the vein, making it difficult to see on a routine spinal CT scan. To detect these leaks, technicians must use specialized CT scans and observe the contrast medium in motion as it flows through the cerebrospinal fluid.
In this study, the researchers used this imaging technique on 21 patients with sagging of the brain and symptoms of bvFTD, and detected CSF-venous-venous fistulas in nine of these patients. All nine patients had their fistulas closed surgically, and their cerebral sagging and associated symptoms were completely reversed.
said Keith L. Black, MD, chief of neurosurgery and the Roth and Lawrence Harvey chair in neurology at Cedars-Sinai. “Such specialized imaging is not widely available, and this study suggests the need for more research to improve detection and cure rates for patients.”
The remaining 12 study participants, whose leakage could not be determined, were treated with off-target therapies designed to reduce brain sagging, such as implantable systems to infuse the patient’s cerebrospinal fluid. However, only three of these patients experienced relief from their symptoms.
“Significant efforts need to be made to improve the detection rate of CSF leaks in these patients,” Chevink said. “We have developed non-targeted therapies for patients where no leak can be detected, but as our study showed, these therapies are much less effective than targeted surgical correction of the leak.”