A recent study conducted by researchers at Cedars-Sinai found that some patients diagnosed with behavioral-variant frontotemporal dementia (bvFTD) may actually have a treatable condition caused by a cerebrospinal fluid (CSF) leak.
This discovery is significant because bvFTD is a disease that causes patients to lose control of their behavior and their ability to carry out daily activities.
What is Behavioral-Variant Frontotemporal Dementia (bvFTD)?
Behavioral-variant frontotemporal dementia is a type of dementia that affects the frontal and temporal lobes of the brain.
The disease causes patients to lose control of their behavior, personality changes, language difficulties, and loss of the ability to carry out daily activities.
The onset of the disease is usually before the age of 65, and the progression of the disease can be rapid.
The Connection Between CSF Leaks and bvFTD
Cerebrospinal fluid is a clear fluid that surrounds the brain and spinal cord to protect them from injury.
When this fluid leaks into the body, it can cause the brain to sag, leading to dementia-like symptoms. According to the study, many patients with brain sagging caused by a CSF leak go undiagnosed.
This is because the symptoms of a CSF leak can mimic other neurological conditions, such as bvFTD.
Specialized Imaging Techniques
Even when brain sagging is detected, the source of a CSF leak can be difficult to locate.
When the fluid leaks through a tear or cyst in the surrounding membrane, it is visible on CT myelogram imaging with the aid of a contrast medium.
However, the team discovered an additional cause of CSF leak in which the fluid leaks into a vein, making it difficult to see on a routine CT myelogram.
To detect these leaks, technicians must use a specialized CT scan and observe the contrast medium in motion as it flows through the cerebrospinal fluid.
This imaging technique was used on 21 patients with brain sagging and symptoms of bvFTD, and the researchers discovered CSF-venous fistulas in nine of those patients.
All nine patients who were diagnosed with CSF-venous fistulas 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.
This research shows that some patients diagnosed with bvFTD may actually have a treatable condition caused by a CSF leak.
If clinicians take a second look at patients with telltale symptoms and use specialized imaging techniques to detect CSF leaks, patients may be able to receive treatment that could reverse their symptoms.
This study highlights the importance of considering all possible causes of dementia-like symptoms and using specialized imaging techniques to identify treatable conditions.
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The study was conducted by Wouter Schievink et al and published in Alzheimer’s & Dementia: Translational Research and Clinical Interventions.
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