Potential Hidden Cause of Dementia Discovered

Summary: Some patients diagnosed with behavioral variant frontotemporal dementia (bv-FTD) may have cerebrospinal fluid leakage that causes brain sag.

Source: Cedars Sinai Medical Center

A new Cedars-Sinai study suggests that some patients diagnosed with behavioral variant frontotemporal dementia (bvFTD) – an incurable condition that robs the patient of the ability to control their behavior and cope with daily life – may have cerebrospinal fluid leakage, which is often treatable.

The researchers reported their findings, published in a peer-reviewed journal Alzheimer & Dementia: Translational Research and Clinical Interventionscan point the way to a cure.

“Many of these patients have such severe cognitive, behavioral and personality changes that they are arrested or placed in a nursing home,” said Wouter Schievink, MD, director of the Cerebrospinal Fluid Leak and Microvascular Neurosurgery Program and professor of Neurosurgery at Cedars-Sinai.

“If they have behavioral variant frontotemporal dementia with an unknown cause, then there is no treatment available. But our study shows that patients with cerebrospinal fluid leakage can be treated if we can find the source of the leak.

Cerebrospinal fluid (CSF) circulates around the brain and spinal cord to help prevent injury. When this fluid leaks into the body, the brain can shrink, causing symptoms of dementia. Schievink said many patients with brain sagging-which can be detected through MRI-go undiagnosed, and advised doctors to take another look at patients with telltale symptoms.

“A radiologist, neurosurgeon or neurologist should re-examine the patient’s MRI to make sure there is no evidence of brain sagging,” says Schievink.

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The doctor may also ask about the patient’s history of severe headaches that worsen during sleep, significant sleepiness despite a good night’s sleep, and whether the patient has ever been diagnosed with Chiari brain malformation, a condition in which the brain tissue extends beyond the spinal cord. continue. Brain sagging, Schievink said, is often mistaken for a Chiari malformation.

Even if a brain leak is detected, the source of the CSF leak can be difficult to find. When the fluid leaks through tears or cysts in the surrounding membrane, it is visible on CT myelogram imaging with the help of a contrast medium.

Schievink and his team recently discovered an additional cause of CSF leakage: CSF venous fistulas. In these cases, the fluid leaks into the vein, making it difficult to see on a routine CT myelogram. To detect these leaks, the technician must use a special CT scan and watch the contrast medium move as it flows through the cerebrospinal fluid.

In this study, the researchers used this imaging technique in 21 patients with cerebral palsy and symptoms of bvFTD, and found CSF-venous fistulas in nine of these patients. All nine patients had surgical closure of the fistula, and brain slack and associated symptoms had resolved.

This shows the brain
Brain sagging, Schievink said, is often mistaken for a Chiari malformation. Images are in the public domain

“This is a rapidly growing field of study, and advances in imaging technology have improved our ability to detect the source of CSF leaks, particularly CSF-venous fistulas,” said Keith L. Black, MD, chairman of the Department of Neurosurgery and the Ruth and Lawrence Harvey Chair at Neuroscience at Cedars-Sinai.

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“This specific imaging is not available, and this study suggests the need for further research to improve detection rates and treat patients.”

The remaining 12 study participants, whose leak could not be identified, were treated with nontargeted therapies designed to reduce brain swelling, such as an implanted system for infusing patients with CSF. However, only three of these patients experienced relief from their symptoms.

“Major efforts should be made to improve the detection rate of CSF leaks in these patients,” Schievink said.

“We have developed a non-targeted treatment for patients with no detectable leak, but as studies have shown, this treatment is less effective than targeted, surgical correction of the leak.”

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About this dementia research news

Author: Press Office
Source: Cedars Sinai Medical Center
Ignition: Press Office – Cedars Sinai Medical Center
Picture: The image is in the public domain

Original Research: Open access.
“Reversible damage from behavioral variant frontotemporal brain sagging syndrome: Challenges and opportunities” by Wouter I. Schievink et al. Alzheimer & Dementia: Translational Research & Clinical Interventions


Reversible damage from behavioral variant frontotemporal brain sagging syndrome: Challenges and opportunities

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Due to the loss of brain buoyancy, spontaneous leakage of spinal cerebrospinal fluid (CSF) causes orthostatic headache but can also cause symptoms that are indistinguishable from behavioral variant frontotemporal dementia (bvFTD) due to severe brain slack (including the frontal and temporal lobes), such as depicted in the brain. magnetic resonance imaging. However, the detection of this CSF leak requires special 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 frontotemporal dementia brain sagging syndrome (4 women and 17 men; mean age 56.2 years). [range: 31–70 years]).


Nine patients (42.8%) were found to have CSF-venous fistula, a recently discovered type of CSF leak that cannot be detected on conventional spinal imaging. All nine patients underwent uneventful surgical ligation of the fistula. Complete or near-complete and persistent resolution of bvFTD symptoms was obtained by all nine patients, accompanied by reversal of brain swelling, but only three (25.0%) of twelve patients had no detectable CSF venous fistula (P = 0.0011), and those treated with non-targeted therapy.


Concerns about spinal CSF leakage should not be dismissed in patients with frontotemporal brain slack syndrome, even when conventional spinal imaging is normal. However, even with this specialized imaging, the source of spinal CSF loss remains elusive in more than half of patients.


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