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 regulate their conduct and deal with each day residing — might as an alternative have a cerebrospinal fluid leak, which is usually treatable.
Researchers say these findings, revealed within the peer-reviewed journal Alzheimer’s & Dementia: Translational Analysis and Medical Interventions, might 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 houses,” mentioned 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 examine reveals 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 mentioned many sufferers with mind sagging — which will be detected by way of 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 verify there isn’t any proof for mind sagging,” Schievink mentioned.
Clinicians may also ask a couple of historical past of extreme complications that enhance when the affected person lies down, vital sleepiness even after satisfactory 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 mentioned, is usually mistaken for a Chiari malformation.
Even when mind sagging is detected, the supply of a CSF leak will be tough to find. When the fluid leaks by way of 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 lately found a further explanation for CSF leak: the CSF-venous fistula. In these instances, 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 by way of 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 have been fully reversed.
“This can be a quickly evolving discipline of examine, and advances in imaging know-how have drastically improved our means to detect sources of CSF leak, particularly CSF-venous fistula,” mentioned 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 is just not 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, have been handled with nontargeted therapies designed to alleviate mind sagging, resembling implantable methods for infusing the affected person with CSF. Nonetheless, solely three of those sufferers skilled aid from their signs.
“Nice efforts have to be made to enhance the detection fee of CSF leak in these sufferers,” Schievink mentioned. “We’ve developed nontargeted remedies for sufferers the place no leak will be detected, however as our examine reveals, these remedies are a lot much less efficient than focused, surgical correction of the leak.”