Summary: CSF leak may cause brain sagging and bvFTD symptoms; specialized imaging can detect CSF-venous fistula; surgical ligation can cure bvFTD.
A new study from Cedars-Sinai Medical Center suggests that some patients diagnosed with behavioral-variant frontotemporal dementia (bvFTD) may actually have a cerebrospinal fluid (CSF) leak that leads to brain sagging. This condition is often treatable and can be detected through MRI. Researchers suggest that clinicians should take a second look at patients with telltale symptoms, such as severe headaches that improve when the patient lies down, significant sleepiness even after adequate nighttime sleep, and a diagnosis of a Chiari brain malformation.
The source of a CSF leak can be difficult to locate, but technicians can use a specialized CT scan to observe the contrast medium in motion as it flows through the CSF. In this study, investigators used this imaging technique on 21 patients with brain sagging and symptoms of bvFTD and discovered CSF-venous fistulas in nine of those patients. All nine patients had their fistulas surgically closed, and their brain sagging and accompanying symptoms were completely reversed.
The remaining 12 study participants, whose leaks could not be identified, were treated with nontargeted therapies designed to relieve brain sagging, such as implantable systems for infusing the patient with CSF. However, only three of these patients experienced relief from their symptoms.
These findings suggest the need for further research to improve detection and cure rates for patients. It is important for clinicians to be aware of the possibility of brain sagging due to a CSF leak and to use specialized imaging techniques to detect it. If the leak can be identified, it is possible to surgically close it and reverse the symptoms of bvFTD. For those whose leaks cannot be identified, there are still nontargeted treatments available, although they are not as effective as targeted, surgical correction of the leak.
Overall, this study suggests that some patients diagnosed with bvFTD may instead have a CSF leak that leads to brain sagging. It is important for clinicians to be aware of this possibility, as it could lead to treatments that could reverse the symptoms of bvFTD. Specialized imaging techniques can be used to detect these leaks, and if they are found, they can be surgically closed. If a leak cannot be identified, there are still treatments available, although they are not as effective as targeted surgical correction.
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