The reversible impairment of behavioral variant frontotemporal brain sagging syndrome: Challenges and opportunities
Abstract Introduction Due to loss of brain buoyancy, spontaneous spinal cerebrospinal fluid (CSF) leaks cause orthostatic headaches but also can cause symptoms indistinguishable from behavioral variant frontotemporal dementia (bvFTD) due to severe brain sagging (including the frontal and temporal lo...
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| Format: | Article |
| Language: | English |
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Wiley
2022-01-01
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| Series: | Alzheimer’s & Dementia: Translational Research & Clinical Interventions |
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| Online Access: | https://doi.org/10.1002/trc2.12367 |
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| author | Wouter I. Schievink Marcel Maya Zachary Barnard Rachelle B. Taché Ravi S. Prasad Vikram S. Wadhwa Franklin G. Moser Miriam Nuño |
| author_facet | Wouter I. Schievink Marcel Maya Zachary Barnard Rachelle B. Taché Ravi S. Prasad Vikram S. Wadhwa Franklin G. Moser Miriam Nuño |
| author_sort | Wouter I. Schievink |
| collection | DOAJ |
| description | Abstract Introduction Due to loss of brain buoyancy, spontaneous spinal cerebrospinal fluid (CSF) leaks cause orthostatic headaches but also can cause symptoms indistinguishable from behavioral variant frontotemporal dementia (bvFTD) due to severe brain sagging (including the frontal and temporal lobes), as visualized on brain magnetic resonance imaging. However, the detection of these CSF leaks may require specialized spinal imaging techniques, such as digital subtraction myelography (DSM). Methods We performed DSM in the lateral decubitus position under general anesthesia in 21 consecutive patients with frontotemporal dementia brain sagging syndrome (4 women and 17 men; mean age 56.2 years [range: 31–70 years]). Results Nine patients (42.8%) were found to have a CSF‐venous fistula, a recently discovered type of CSF leak that cannot be detected on conventional spinal imaging. All nine patients underwent uneventful surgical ligation of the fistula. Complete or near‐complete and sustained resolution of bvFTD symptoms was obtained by all nine patients, accompanied by reversal of brain sagging, but in only three (25.0%) of the twelve patients in whom no CSF‐venous fistula could be detected (P = 0.0011), and who were treated with non‐targeted therapies. Discussion Concerns about a spinal CSF leak should not be dismissed in patients with frontotemporal brain sagging syndrome, even when conventional spinal imaging is normal. However, even with this specialized imaging the source of the loss of spinal CSF remains elusive in more than half of patients. |
| format | Article |
| id | doaj-art-ac0de9199b374857a9dd960242bf9168 |
| institution | Kabale University |
| issn | 2352-8737 |
| language | English |
| publishDate | 2022-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Alzheimer’s & Dementia: Translational Research & Clinical Interventions |
| spelling | doaj-art-ac0de9199b374857a9dd960242bf91682024-12-03T12:37:32ZengWileyAlzheimer’s & Dementia: Translational Research & Clinical Interventions2352-87372022-01-0181n/an/a10.1002/trc2.12367The reversible impairment of behavioral variant frontotemporal brain sagging syndrome: Challenges and opportunitiesWouter I. Schievink0Marcel Maya1Zachary Barnard2Rachelle B. Taché3Ravi S. Prasad4Vikram S. Wadhwa5Franklin G. Moser6Miriam Nuño7Department of Neurosurgery Cedars‐Sinai Medical Center Los Angeles California USADepartment of Imaging Cedars‐Sinai Medical Center Los Angeles California USADepartment of Neurosurgery Cedars‐Sinai Medical Center Los Angeles California USADepartment of Neurosurgery Cedars‐Sinai Medical Center Los Angeles California USADepartment of Imaging Cedars‐Sinai Medical Center Los Angeles California USADepartment of Imaging Cedars‐Sinai Medical Center Los Angeles California USADepartment of Imaging Cedars‐Sinai Medical Center Los Angeles California USADepartment of Public Health Sciences University of California Davis California USAAbstract Introduction Due to loss of brain buoyancy, spontaneous spinal cerebrospinal fluid (CSF) leaks cause orthostatic headaches but also can cause symptoms indistinguishable from behavioral variant frontotemporal dementia (bvFTD) due to severe brain sagging (including the frontal and temporal lobes), as visualized on brain magnetic resonance imaging. However, the detection of these CSF leaks may require specialized spinal imaging techniques, such as digital subtraction myelography (DSM). Methods We performed DSM in the lateral decubitus position under general anesthesia in 21 consecutive patients with frontotemporal dementia brain sagging syndrome (4 women and 17 men; mean age 56.2 years [range: 31–70 years]). Results Nine patients (42.8%) were found to have a CSF‐venous fistula, a recently discovered type of CSF leak that cannot be detected on conventional spinal imaging. All nine patients underwent uneventful surgical ligation of the fistula. Complete or near‐complete and sustained resolution of bvFTD symptoms was obtained by all nine patients, accompanied by reversal of brain sagging, but in only three (25.0%) of the twelve patients in whom no CSF‐venous fistula could be detected (P = 0.0011), and who were treated with non‐targeted therapies. Discussion Concerns about a spinal CSF leak should not be dismissed in patients with frontotemporal brain sagging syndrome, even when conventional spinal imaging is normal. However, even with this specialized imaging the source of the loss of spinal CSF remains elusive in more than half of patients.https://doi.org/10.1002/trc2.12367behavioral variant frontotemporal dementiabrain saggingcerebrospinal fluid leakfrontotemporal dementiaspontaneous intracranial hypotension |
| spellingShingle | Wouter I. Schievink Marcel Maya Zachary Barnard Rachelle B. Taché Ravi S. Prasad Vikram S. Wadhwa Franklin G. Moser Miriam Nuño The reversible impairment of behavioral variant frontotemporal brain sagging syndrome: Challenges and opportunities Alzheimer’s & Dementia: Translational Research & Clinical Interventions behavioral variant frontotemporal dementia brain sagging cerebrospinal fluid leak frontotemporal dementia spontaneous intracranial hypotension |
| title | The reversible impairment of behavioral variant frontotemporal brain sagging syndrome: Challenges and opportunities |
| title_full | The reversible impairment of behavioral variant frontotemporal brain sagging syndrome: Challenges and opportunities |
| title_fullStr | The reversible impairment of behavioral variant frontotemporal brain sagging syndrome: Challenges and opportunities |
| title_full_unstemmed | The reversible impairment of behavioral variant frontotemporal brain sagging syndrome: Challenges and opportunities |
| title_short | The reversible impairment of behavioral variant frontotemporal brain sagging syndrome: Challenges and opportunities |
| title_sort | reversible impairment of behavioral variant frontotemporal brain sagging syndrome challenges and opportunities |
| topic | behavioral variant frontotemporal dementia brain sagging cerebrospinal fluid leak frontotemporal dementia spontaneous intracranial hypotension |
| url | https://doi.org/10.1002/trc2.12367 |
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