overview: Some patients diagnosed with behavioral variant frontotemporal dementia (bv-FTD) may instead suffer from a cerebrospinal fluid leak leading to sagging of the brain.
sauce: Cedars-Sinai Medical Center
A new study from Cedars-Sinai reveals that some patients diagnosed with behavioral variant frontotemporal dementia (bvFTD), an incurable condition that robs them of their ability to control their behavior and cope with daily life suggesting that it is possible. can often be treated.
Researchers Say These Findings Published in Peer-Reviewed Journals Alzheimer’s disease and dementia: translational research and clinical interventionsmay point the way to a cure.
“Many of these patients have cognitive, behavioral and personality changes so severe that they are either arrested or placed in a nursing home,” said Cedars-Sinai’s Cerebrospinal Fluid Leakage and Microvascular Neurosurgery Program. Director and Professor of Neurosurgery, Wouter Schievink, MD, said.
“In the case of behavioral variant frontotemporal dementia of unknown cause, there are no treatments available. It shows what you can do.”
Cerebrospinal fluid (CSF) circulates in and around the brain and spinal cord to relieve injury. When this fluid leaks into the body, it causes the brain to sag, causing symptoms of dementia. Schievink says many patients with sagging brains (detectable on MRI) go undiagnosed and advises clinicians to re-examine patients with clear symptoms.
“A knowledgeable radiologist, neurosurgeon, or neurologist should recheck the patient’s MRI to make sure there is no evidence of brain sagging,” Schievink said.
The clinician also noted a history of severe headaches that improved when the patient lay down, considerable drowsiness even after adequate nighttime sleep, and that the patient had been diagnosed with Chiari brain malformation (a condition in which brain tissue extends into the spine). You can also ask if there is canal. According to Schievink, sagging brains are often mistaken for Chiari’s malformation.
Even when brain sagging is detected, it can be difficult to determine the cause of CSF leakage. When fluid leaks from the surrounding membrane tear or cyst, it can be seen on CT myelogram imaging with a contrast agent.
Schievink and his team recently discovered another cause of CSF leakage, the CSF venous fistula. In these cases, fluid leaks into the veins and is difficult to see on her regular CT myelogram. To detect these leaks, his specialist must use CT scans to observe the movement of the contrast agent as it flows through the cerebrospinal fluid.
In this study, researchers used this imaging technique on 21 patients with symptoms of brain sagging and bvFTD and found CSF venous fistulas in 9 of those patients. All nine patients had their fistulas surgically closed, and the cerebral sagging and associated symptoms were completely reversible.
“This is a rapidly evolving area of research, and advances in imaging technology have greatly improved our ability to detect sources of CSF leakage, particularly CSF venous fistulas,” said Keith L., director of neurosurgery. Dr. Black said. Ruth and Lawrence Harvey Chair of Neuroscience at Cedars-Sinai.
“This specialized image is not widely available. This study suggests the need for further research to improve patient detection and cure rates.”
The remaining 12 study participants, whose leaks could not be identified, were treated with non-targeted therapies designed to relieve brain sagging, including an implantable system for infusing patients with CSF. , only three of these patients experienced symptomatic relief.
“Major efforts are needed to improve the detection rate of CSF leakage in these patients,” Schievink said.
“We have developed non-targeted treatments for patients with undetectable leaks, but as our study shows, these treatments are far less effective than targeted surgical correction of leaks. .”
About this dementia research news
Original research: open access.
“Reversible Impairment of Behavioral Variant Frontotemporal Drooping Syndrome: Challenges and Opportunities” Wouter I. Schievink et al. Alzheimer’s disease and dementia: translational research and clinical interventions
Reversible Impairment of Behavioral Variant Frontotemporal Drooping Syndrome: Challenges and Opportunities
Spontaneous spinal cerebrospinal fluid (CSF) leakage causes orthostatic headache because brain buoyancy is lost, but severe brain sagging (including frontal and temporal lobes), as visualized in the brain ) may cause symptoms indistinguishable from behavioral variant frontotemporal dementia (bvFTD). Magnetic resonance imaging. However, detection of these CSF leaks may require specialized spine imaging techniques such as digital subtraction myelography (DSM).
Twenty-one frontotemporal dementia brain sagging syndrome patients (4 females, 17 males, average age 56.2 years) underwent DSM in the lateral position under general anesthesia. [range: 31–70 years]).
Nine patients (42.8%) were found to have CSF venous fistulas, a recently discovered type of CSF leakage undetectable by conventional spine imaging. All 9 patients successfully underwent surgical ligation of the fistula. Complete or near-complete and sustained resolution of bvFTD symptoms was obtained in all of her 9 patients with reversal of cerebral slack, but he was only 3 people (25.0%) (P. = 0.0011), and those treated with non-targeted therapy.
Concerns about spinal cord CSF leakage should not be ignored in patients with frontotemporal ptosis syndrome, even if conventional spine imaging is normal. However, even with this specialized imaging modality, the cause of spinal cord CSF loss remains unknown in more than half of patients.