A new Cedars-Sinai study implies that some clients diagnosed with behavioral-variant frontotemporal dementia (bvFTD) — an incurable condition that robs people of the potential to command their conduct and cope with everyday residing — may well in its place have a cerebrospinal fluid leak, which is generally treatable.
Scientists say these findings, revealed in the peer-reviewed journal Alzheimer’s & Dementia: Translational Research and Medical Interventions, may possibly point the way to a remedy.
“Many of these patients knowledge cognitive, behavioral and personality adjustments so significant that they are arrested or positioned in nursing homes,” stated Wouter Schievink, MD, director of the Cerebrospinal Fluid Leak and Microvascular Neurosurgery System and professor of Neurosurgery at Cedars-Sinai. “If they have behavioral-variant frontotemporal dementia with an unidentified trigger, then no cure is obtainable. But our analyze demonstrates that people with cerebrospinal fluid leaks can be healed if we can find the source of the leak.”
Cerebrospinal fluid (CSF) circulates in and around the brain and spinal cord to support cushion them from injuries. When this fluid leaks into the physique, the brain can sag, resulting in dementia signs and symptoms. Schievink explained a lot of people with brain sagging — which can be detected as a result of MRI — go undiagnosed, and he advises clinicians to get a 2nd search at people with telltale indications.
“A professional radiologist, neurosurgeon or neurologist should really examine the patient’s MRI again to make positive there is no proof for brain sagging,” Schievink reported.
Clinicians can also request about a history of significant headaches that enhance when the client lies down, sizeable sleepiness even right after satisfactory nighttime rest, and whether or not the client has at any time been identified with a Chiari brain malformation, a ailment in which brain tissue extends into the spinal canal. Brain sagging, Schievink mentioned, is normally mistaken for a Chiari malformation.
Even when brain sagging is detected, the resource of a CSF leak can be challenging to locate. When the fluid leaks as a result of a tear or cyst in the encompassing membrane, it is obvious on CT myelogram imaging with the help of contrast medium.
Schievink and his group recently found out an further trigger of CSF leak: the CSF-venous fistula. In these situations, the fluid leaks into a vein, generating it tough to see on a program CT myelogram. To detect these leaks, professionals need to use a specialised CT scan and notice the distinction medium in motion as it flows by way of the cerebrospinal fluid.
In this analyze, investigators applied this imaging procedure on 21 people with brain sagging and indications of bvFTD, and they identified CSF-venous fistulas in nine of these individuals. All 9 sufferers experienced their fistulas surgically shut, and their brain sagging and accompanying signs ended up fully reversed.
“This is a rapidly evolving area of analyze, and improvements in imaging know-how have drastically enhanced our potential to detect sources of CSF leak, in particular CSF-venous fistula,” reported Keith L. Black, MD, chair of the office of Neurosurgery and the Ruth and Lawrence Harvey Chair in Neuroscience at Cedars-Sinai. “This specialized imaging is not widely available, and this research suggests the want for even more investigate to improve detection and remedy charges for people.”
The remaining 12 study individuals, whose leaks could not be identified, ended up dealt with with nontargeted therapies developed to decrease brain sagging, these kinds of as implantable methods for infusing the affected person with CSF. However, only a few of these patients skilled relief from their indicators.
“Wonderful efforts want to be created to strengthen the detection rate of CSF leak in these clients,” Schievink claimed. “We have developed nontargeted remedies for clients where no leak can be detected, but as our review demonstrates, these remedies are significantly less successful than qualified, surgical correction of the leak.”
