Abstract: Some sufferers identified with behavioral-variant frontotemporal dementia (bv-FTD) might as a substitute be affected by a cerebrospinal fluid leak that results in mind sagging.
Supply: Cedars Sinai Medical Middle
A brand new Cedars-Sinai research means that some sufferers identified with behavioral-variant frontotemporal dementia (bvFTD)—an incurable situation that robs sufferers of the flexibility to regulate their conduct and address day by day residing—might 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 Medical Interventions, might level the way in which to a treatment.
“Many of those sufferers expertise cognitive, behavioral and character adjustments 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 remedy is offered. However our research exhibits 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 wire 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 via 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 verify the affected person’s MRI once more to ensure there isn’t any proof for mind sagging,” Schievink stated.
Clinicians may ask a few historical past of extreme complications that enhance when the affected person lies down, vital sleepiness even after enough nighttime sleep, and whether or not the affected person has ever been identified with a Chiari mind malformation, a situation through which 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 tough to find. When the fluid leaks via a tear or cyst within the surrounding membrane, it’s seen on CT myelogram imaging with the help of distinction medium.
Schievink and his workforce just lately found a further reason behind CSF leak: the CSF-venous fistula. In these circumstances, the fluid leaks right into a vein, making it tough 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 via the cerebrospinal fluid.
On this research, investigators used this imaging approach on 21 sufferers with mind sagging and signs of bvFTD, and so they 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 utterly reversed.
“This can be a quickly evolving discipline of research, and advances in imaging expertise have enormously improved our capability 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 isn’t extensively out there, and this research suggests the necessity for additional analysis to enhance detection and treatment charges for sufferers.”
The remaining 12 research individuals, whose leaks couldn’t be recognized, had been handled with nontargeted therapies designed to alleviate mind sagging, equivalent to implantable methods for infusing the affected person with CSF. Nevertheless, solely three of those sufferers skilled reduction from their signs.
“Nice efforts must be made to enhance the detection fee of CSF leak in these sufferers,” Schievink stated.
“We’ve developed nontargeted therapies for sufferers the place no leak might be detected, however as our research exhibits, these therapies are a lot much less efficient than focused, surgical correction of the leak.”
About this dementia analysis information
Authentic Analysis: Open entry.
“The reversible impairment of behavioral variant frontotemporal mind sagging syndrome: Challenges and alternatives” by Wouter I. Schievink et al. Alzheimer’s & Dementia: Translational Analysis & Medical 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) resulting from 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, equivalent to digital subtraction myelography (DSM).
We carried out DSM within the lateral decubitus place below common 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 typical 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 could possibly be detected (P = 0.0011), and who had been handled with non-targeted therapies.
Considerations a few spinal CSF leak shouldn’t be dismissed in sufferers with frontotemporal mind sagging syndrome, even when typical 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.