Discovering the possible hidden cause of dementia Neuroscience News

summary: Some patients diagnosed with behaviorally variable frontotemporal dementia (bv-FTD) may instead experience cerebrospinal fluid leakage that causes the brain to sag.

Source: Cedars Sinai Medical Center

A new study from Cedars-Sinai suggests that some patients diagnosed with behaviorally variable frontotemporal dementia (bvFTD) — an incurable condition that denies patients the ability to control their own behavior and cope with daily life — may instead experience fluid leakage. myeloid, which is often treatable.

The researchers say the findings are published in the peer-reviewed journal Alzheimer’s disease and dementia: translational research and clinical interventionsmay 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 Schievink, 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.

Chevink said sagging of the brain is often mistaken for Chiari malformation. The image is in the public domain

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 treatment rates for patients.”

The remaining 12 study participants, whose leakage could not be determined, were treated with nontargeted therapies designed to reduce brain sagging, such as implantable systems to inject the patient with 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,” Schievink said.

“We have developed non-targeted therapies for patients where a leak cannot be detected, but as our study showed, these therapies are much less effective than targeted surgical correction of a leak.”

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About this research dementia news

author: press office
Source: Cedars Sinai Medical Center
Contact: Press Office – Cedars Sinai Medical Center
picture: The image is in the public domain

Original search: open access.
“Reversible impairment of the behavioral variant of frontotemporal flabby brain syndrome: challenges and opportunities” by Wouter I. Schievink et al. Alzheimer’s disease and dementia: translational research and clinical interventions


Reversible vulnerability to the behavioral variant of frontotemporal flabby brain syndrome: challenges and opportunities

an introduction

Due to the loss of brain buoyancy, spontaneous leaks of cerebrospinal fluid (CSF) cause orthostatic headaches but can also cause symptoms indistinguishable from behavioral variable frontotemporal dementia (bvFTD) due to severe sagging of the brain (including the frontal and temporal lobes), Brain as visualized by magnetic resonance imaging. However, detection of CSF leaks may require specialized spinal imaging techniques, such as digital subtraction myelography (DSM).


We performed DSM in the lateral decubitus position under general anesthesia in 21 consecutive patients with flaccid brain syndrome in frontotemporal dementia (4 women, 17 men; mean age 56.2 years). [range: 31–70 years]).


Nine patients (42.8%) were found to have a CSF venous fistula, a recently discovered type of cerebrospinal fluid leak that cannot be detected on conventional spinal imaging. All nine patients underwent surgical ligation of the fistula. Complete or near complete and sustained resolution of bvFTD symptoms was obtained by all nine patients, accompanied by a reversal of cerebral sagging, but in only three (25.0%) of the twelve patients no CSF ​​fistula was detected (s = 0.0011), and those treated with non-target therapies.


Concerns about cerebrospinal fluid leakage in patients with FTSE should not be ignored, even when conventional imaging of the spine is normal. However, even with this specialized imaging, the source of CSF loss remains elusive in more than half of patients.

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