A recent Cedars-Sinai study shows that some clients identified with behavioral-variant frontotemporal dementia, an incurable condition triggering loss of habits control and daily living abilities, may really have a treatable cerebrospinal fluid leak instead.
A Cedars-Sinai study suggests doctors dealing with dementia should search for cerebrospinal fluid leak– a treatable cause of an otherwise incurable condition.
Wouter Schievink, MD, director of the Cerebrospinal Fluid Leak and Microvascular Neurosurgery Program, said lots of patients with brain sagging– which can be spotted through MRI– go undiagnosed, and he advises clinicians to take a review at patients with telltale signs.
A new Cedars-Sinai research study recommends that some clients diagnosed with behavioral-variant frontotemporal dementia (bvFTD)– an incurable condition that robs clients of the capability to control their behavior and deal with daily living– may rather have a cerebrospinal fluid leak, which is frequently treatable.
” Many of these clients experience cognitive, behavioral, and character modifications so serious that they are detained or put in nursing houses,” said Wouter Schievink, MD, director of the Cerebrospinal Fluid Leak and Microvascular Neurosurgery Program and professor of Neurosurgery at Cedars-Sinai. Our study reveals that clients with cerebrospinal fluid leakages can be cured if we can find the source of the leakage.”
When this fluid leakages into the body, the brain can droop, causing dementia signs. Schievink said lots of clients with brain sagging– which can be discovered through MRI– go undiagnosed, and he advises clinicians to take a 2nd look at patients with telltale signs.
To find these leaks, service technicians need to use a specialized CT scan and observe the contrast medium in motion as it streams through the cerebrospinal fluid.
Scientists say these findings, released in the peer-reviewed journal Alzheimers & & Dementia: Translational Research and Clinical Interventions, may point the method to a cure.
” Many of these clients experience cognitive, behavioral, and personality modifications so extreme that they are arrested or placed in retirement home,” stated Wouter Schievink, MD, director of the Cerebrospinal Fluid Leak and Microvascular Neurosurgery Program and teacher of Neurosurgery at Cedars-Sinai. “If they have behavioral-variant frontotemporal dementia with an unidentified cause, then no treatment is readily available. But our research study reveals that patients with cerebrospinal fluid leaks can be cured if we can discover the source of the leakage.”
On the Left: A picture of brain sagging. On the right: Post-op resolution of brain sagging. Credit: Cedars-Sinai Medical Center
Cerebrospinal fluid (CSF) flows around the brain and spine cord to help cushion them from injury. When this fluid leakages into the body, the brain can sag, triggering dementia symptoms. Schievink stated lots of patients with brain sagging– which can be spotted through MRI– go undiagnosed, and he encourages clinicians to take a second look at clients with telltale signs.
” An experienced radiologist, neurosurgeon or neurologist ought to check the clients MRI again to make sure there is no proof for brain sagging,” Schievink stated.
Wouter Schievink, MD. Credit: Cedars-Sinai Medical Center
Clinicians can also ask about a history of serious headaches that improve when the patient lies down, significant drowsiness even after appropriate nighttime sleep, and whether the client has actually ever been identified with a Chiari brain malformation, a condition in which brain tissue extends into the spine canal. Brain sagging, Schievink said, is often incorrect for a Chiari malformation.
Even when brain sagging is discovered, the source of a CSF leak can be hard to locate. When the fluid leakages through a cyst or tear in the surrounding membrane, it is noticeable on CT myelogram imaging with the aid of contrast medium.
Schievink and his group recently discovered an extra cause of CSF leak: the CSF-venous fistula. In these cases, the fluid leakages into a vein, making it challenging to see on a routine CT myelogram. To detect these leaks, professionals must utilize a specialized CT scan and observe the contrast medium in movement as it streams through the cerebrospinal fluid.
In this research study, investigators used this imaging technique on 21 clients with brain sagging and symptoms of bvFTD, and they discovered CSF-venous fistulas in 9 of those patients. All 9 clients had their fistulas surgically closed, and their brain drooping and accompanying signs were entirely reversed.
” This is a quickly developing field of study, and advances in imaging technology have actually greatly improved our ability to find sources of CSF leak, especially CSF-venous fistula,” stated Keith L. Black, MD, chair of the department of Neurosurgery and the Ruth and Lawrence Harvey Chair in Neuroscience at Cedars-Sinai. “This specialized imaging is not widely offered, and this research study suggests the need for additional research study to improve detection and cure rates for clients.”
The staying 12 study individuals, whose leakages could not be determined, were treated with nontargeted treatments developed to ease brain sagging, such as implantable systems for infusing the client with CSF. Just three of these patients experienced relief from their signs.
” Great efforts require to be made to improve the detection rate of CSF leak in these clients,” Schievink stated. “We have developed nontargeted treatments for patients where no leakage can be found, but as our research study reveals, these treatments are much less efficient than targeted, surgical correction of the leak.”
Reference: “The reversible disability of behavioral alternative frontotemporal brain drooping syndrome: Opportunities and challenges” by Wouter I. Schievink, Marcel Maya, Zachary Barnard, Rachelle B. Taché, Ravi S. Prasad, Vikram S. Wadhwa, Franklin G. Moser and Miriam Nuño, 18 December 2022, Alzheimers & & Dementia: Translational Research and Clinical Interventions.DOI: 10.1002/ trc2.12367.
