Abstract: Some sufferers recognized with behavioral-variant frontotemporal dementia (bv-FTD) might as an alternative be affected by a cerebrospinal fluid leak that results in mind sagging.
supply: Cedars Sinai Medical Middle
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 habits and address day by day dwelling—might as an alternative have a cerebrospinal fluid leak, which is commonly treatable.
Researchers say these findings, printed within the peer-reviewed journal Alzheimer’s & Dementia: Translational Analysis and Scientific Interventionsmight level the way in which to a remedy.
“Many of those sufferers expertise cognitive, behavioral and character modifications so extreme that they’re arrested or positioned in nursing properties,” 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 out there. However our examine exhibits that sufferers with cerebrospinal fluid leaks will be cured if we will discover the supply of the leak.”
Cerebrospinal fluid (CSF) circulates in and across the mind and spinal twine to assist cushion them from damage. When this fluid leaks into the physique, the mind can sag, inflicting dementia signs. Schievink stated many sufferers with mind sagging—which will be detected by MRI—go undiagnosed, and he advises clinicians to take a second take 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 isn’t any proof for mind sagging,” Schievink stated.
Clinicians also can ask a couple of historical past of extreme complications that enhance when the affected person lies down, important sleepiness even after enough 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 channel. Mind sagging, Schievink stated, is commonly mistaken for a Chiari malformation.
Even when mind sagging is detected, the supply of a CSF leak will 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 staff not too long ago found a further reason for CSF leak: the CSF-venous fistula. In these circumstances, 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 method 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.
“It is a quickly evolving discipline of examine, and advances in imaging know-how have tremendously improved our potential 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 just isn’t extensively 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 price of CSF leak in these sufferers,” Schievink stated.
“Now we have developed nontargeted therapies for sufferers the place no leak will be detected, however as our examine exhibits, these therapies are a lot much less efficient than focused, surgical correction of the leak.”
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“The reversible impairment of behavioral variant frontotemporal mind sagging syndrome: Challenges and alternatives” 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
Resulting from lack of mind buoyancy, spontaneous spinal cerebrospinal fluid (CSF) leaks trigger orthostatic complications but additionally could cause signs indistinguishable from behavioral variant frontotemporal dementia (bvFTD) as a consequence 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 might require specialised spinal imaging strategies, corresponding to digital subtraction myelography (DSM).
We carried out DSM within the lateral decubitus place below basic 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 not too long ago found kind of CSF leak that can not 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.
Considerations 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.