Summary: Some patients diagnosed with behaviorally-variable frontotemporal dementia (bv-FTD) may instead suffer from cerebrospinal fluid leakage leading to dementia.

Source: Cedars Sinai Medical Center

A new Cedars-Sinai study suggests that some patients with behaviorally variable frontotemporal dementia (bvFTD) — an incurable condition that robs patients of their ability to control their behavior and cope with daily life — may instead have cerebrospinal fluid leakage, which is usually treatable.

Researchers say these findings, published in a peer-reviewed journal Alzheimer’s and Dementia: Translational Research and Clinical Interventionsmay indicate a path to healing.

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

“If the cause of behavioral-dynamic frontotemporal dementia is unknown, there is no treatment. But our research shows that patients with cerebrospinal fluid leaks can recover if we find the source of the leak.

Cerebrospinal fluid (CSF) circulates in and around the brain and spinal cord to help them recover from injury. When this fluid enters the body, the brain can slow down, causing symptoms of dementia. Schevink says many patients with dementia — which can be detected with an MRI — go undiagnosed, and recommends that clinicians take a second look at patients with identifying symptoms.

“A knowledgeable radiologist, neurosurgeon or neurologist should recheck the patient’s MRI to make sure there is no evidence of dementia,” Schievink said.

Clinicians may also ask for a history of severe headaches that improve when the patient sleeps, severe insomnia even after adequate sleep, and whether the patient has been diagnosed with a Chiari brain malformation, a condition in which brain tissue extends into the spine. Canal mental retardation, according to Schievink, is often characterized by a Chiari malformation.

Even when a brain hemorrhage is recognized, the source of the CSF leak can be difficult to locate. As the fluid flows through the tear or the surrounding membrane, it can be seen on a CT myelogram image with the help of contrast media.

Schievink and his team recently discovered an additional cause of CSF leakage: the CSF-venous fistula. In these cases, the fluid enters the vein, making it difficult to see on a conventional CT myelogram. To identify these leaks, technicians must use a special CT scan and monitor their movement as the contrast medium flows through the cerebrospinal fluid.

In this study, investigators used this imaging technique in 21 patients with dementia and symptoms of bvFTD and found CSF-venous fistulas in nine patients. All nine patients had their fistulas closed surgically, and their brain shrinkage and associated symptoms completely resolved.

This shows the brain
Mental retardation, according to Schievink, is often observed with a Chiari malformation. The image is in the public domain.

“This is a rapidly growing field of study, and advances in imaging technology have greatly improved our ability to identify sources of CSF leakage, particularly CSF-venous fistula,” said Keith L. Black, MD, chair of the Department of Neurosurgery. Ruth and Lawrence Harvey Chair in Neuroscience at Cedars-Sinai.

“This unique image is not widely available, and this study suggests that more research is needed to improve the detection and cure rates for patients.”

The remaining 12 study participants, whose flow could not be determined, were treated with non-targeted therapies designed to relieve brain congestion, using implants to pump the patient’s CSF. However, only three of these patients experienced relief of symptoms.

“There is a need to improve the detection rate of CSF flow in these patients,” said Schievink.

“We have developed non-targeted treatments for patients with undetectable leakage, but our study shows that these treatments are less effective than surgically correcting the target.”

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This shows the RTL6 protein

So dementia research news

Author: Press office
Source: Cedars Sinai Medical Center
Contact: Press Office – Cedars Sinai Medical Center
Image: The image is in the public domain.

Preliminary study: Open Access.
A reversible behavioral variant of frontotemporal brain atrophy syndrome: challenges and opportunities.” by Water I. Schievink et al. Alzheimer’s and Dementia: Translational Research and Clinical Interventions


A reversible behavioral variant of frontotemporal brain atrophy syndrome: challenges and opportunities.


Sudden cerebrospinal fluid (CSF) leakage due to loss of brain stimulation can cause bony headache, but severe brain swelling (including frontal and temporal lobes) as seen in the brain can cause symptoms indistinguishable from behavioral dystonia (bvFTD). Magnetic resonance imaging. However, detection of these CSF leaks may require specialized spinal imaging techniques such as digital subtraction myelography (DSM).


We performed DSM in the lateral decubitus position 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%) had a CSF-venous fistula, a recently discovered type of CSF leakage that could not be detected on conventional spinal imaging. All nine patients underwent asymmetric fistula surgery. Complete or complete and persistent symptoms of bvFTD were found in nine patients, accompanied by altered cerebral perfusion, but no CSF-venous fistula could be identified in three (25.0%) of twelve patients (25.0%).P = 0.0011), and treated with non-targeted therapies.


Concern about spinal CSF leakage should not be dismissed in patients with frontotemporal brain sagging syndrome, even if the normal spinal imaging is normal. However, even with this unique imaging, the source of spinal CSF loss remains unclear in more than half of patients.

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