Potential Hidden Explanation for Dementia Found

Potential Hidden Explanation for Dementia Found

Potential Hidden Explanation for Dementia Found

Abstract: Some sufferers recognized with behavioral-variant frontotemporal dementia (bv-FTD) could as a substitute be affected by a cerebrospinal fluid leak that results in mind sagging.

Supply: Cedars Sinai Medical Heart

A brand new Cedars-Sinai examine means that some sufferers recognized with behavioral-variant frontotemporal dementia (bvFTD)—an incurable situation that robs sufferers of the flexibility to manage their conduct and deal with every day residing—could as a substitute have a cerebrospinal fluid leak, which is usually treatable.

Researchers say these findings, printed within the peer-reviewed journal Alzheimer’s & Dementia: Translational Analysis and Scientific Interventionscould level the best way to a remedy.

“Many of those sufferers expertise cognitive, behavioral and character modifications so extreme that they’re arrested or positioned in nursing houses,” stated Wouter Schievink, MD, director of the Cerebrospinal Fluid Leak and Microvascular Neurosurgery Program and professor of Neurosurgery at Cedars-Sinai.

“If they’ve behavioral-variant frontotemporal dementia with an unknown trigger, then no therapy is obtainable. However our examine reveals that sufferers with cerebrospinal fluid leaks might be cured if we are able to discover the supply of the leak.”

Cerebrospinal fluid (CSF) circulates in and across the mind and spinal twine to assist cushion them from harm. When this fluid leaks into the physique, the mind can sag, inflicting dementia signs. Schievink stated many sufferers with mind sagging—which might be detected by MRI—go undiagnosed, and he advises clinicians to take a second have a look at sufferers with telltale signs.

“A educated radiologist, neurosurgeon or neurologist ought to examine the affected person’s MRI once more to ensure there is no such thing as a proof for mind sagging,” Schievink stated.

Clinicians can even ask a couple of historical past of extreme complications that enhance when the affected person lies down, important sleepiness even after ample nighttime sleep, and whether or not the affected person has ever been recognized with a Chiari mind malformation, a situation wherein mind tissue extends into the spinal canal. Mind sagging, Schievink stated, is usually mistaken for a Chiari malformation.

Even when mind sagging is detected, the supply of a CSF leak might be troublesome to find. When the fluid leaks by a tear or cyst within the surrounding membrane, it’s seen on CT myelogram imaging with the help of distinction medium.

Schievink and his group just lately found a further explanation for CSF leak: the CSF-venous fistula. In these instances, the fluid leaks right into a vein, making it troublesome to see on a routine CT myelogram. To detect these leaks, technicians should use a specialised CT scan and observe the distinction medium in movement because it flows by the cerebrospinal fluid.

On this examine, investigators used this imaging approach on 21 sufferers with mind sagging and signs of bvFTD, they usually found CSF-venous fistulas in 9 of these sufferers. All 9 sufferers had their fistulas surgically closed, and their mind sagging and accompanying signs had been fully reversed.

Mind sagging, Schievink stated, is usually mistaken for a Chiari malformation. Picture is within the public area

“It is a quickly evolving discipline of examine, and advances in imaging know-how have tremendously improved our capacity to detect sources of CSF leak, particularly CSF-venous fistula,” stated Keith L. Black, MD, chair of the division of Neurosurgery and the Ruth and Lawrence Harvey Chair in Neuroscience at Cedars-Sinai.

“This specialised imaging shouldn’t be broadly accessible, and this examine suggests the necessity for additional analysis to enhance detection and remedy charges for sufferers.”

The remaining 12 examine contributors, whose leaks couldn’t be recognized, had been handled with nontargeted therapies designed to alleviate mind sagging, corresponding to implantable methods for infusing the affected person with CSF. Nevertheless, solely three of those sufferers skilled aid from their signs.

“Nice efforts should be made to enhance the detection charge of CSF leak in these sufferers,” Schievink stated.

“We’ve got developed nontargeted remedies for sufferers the place no leak might be detected, however as our examine reveals, these remedies are a lot much less efficient than focused, surgical correction of the leak.”

See additionally

Potential Hidden Explanation for Dementia Found

About this dementia analysis information

Creator: Press Office
Supply: Cedars Sinai Medical Center
Contact: Press Workplace – Cedars Sinai Medical Heart
Picture: The picture is within the public area

Authentic Analysis: Open entry.
The reversible impairment of behavioral variant frontotemporal brain sagging syndrome: Challenges and opportunities” by Wouter I. Schievink et al. Alzheimer’s & Dementia: Translational Analysis & Scientific Interventions


The reversible impairment of behavioral variant frontotemporal mind sagging syndrome: Challenges and alternatives


As a consequence of lack of mind buoyancy, spontaneous spinal cerebrospinal fluid (CSF) leaks trigger orthostatic complications but in addition may cause signs indistinguishable from behavioral variant frontotemporal dementia (bvFTD) because of extreme mind sagging (together with the frontal and temporal lobes), as visualized on mind magnetic resonance imaging. Nevertheless, the detection of those CSF leaks could require specialised spinal imaging strategies, corresponding to digital subtraction myelography (DSM).


We carried out DSM within the lateral decubitus place beneath normal anesthesia in 21 consecutive sufferers with frontotemporal dementia mind sagging syndrome (4 ladies and 17 males; imply age 56.2 years [range: 31–70 years]).


9 sufferers (42.8%) had been discovered to have a CSF-venous fistula, a just lately found kind of CSF leak that can’t be detected on standard spinal imaging. All 9 sufferers underwent uneventful surgical ligation of the fistula. Full or near-complete and sustained decision of bvFTD signs was obtained by all 9 sufferers, accompanied by reversal of mind sagging, however in solely three (25.0%) of the twelve sufferers in whom no CSF-venous fistula might be detected (P = 0.0011), and who had been handled with non-targeted therapies.


Issues a couple of spinal CSF leak shouldn’t be dismissed in sufferers with frontotemporal mind sagging syndrome, even when standard spinal imaging is regular. Nevertheless, even with this specialised imaging the supply of the lack of spinal CSF stays elusive in additional than half of sufferers.

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