Skull defect – Frontotemporal dementia sagging brain syndrome
Abstract Objective Frontotemporal dementia (FTD) sagging brain syndrome is a disabling condition. An underlying spinal Cerebrospinal fluid leak can be identified in only a minority of patients and the success rate of non‐directed treatments is low. Some of these patients have a remote history of cra...
Saved in:
| Main Authors: | , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wiley
2025-01-01
|
| Series: | Annals of Clinical and Translational Neurology |
| Online Access: | https://doi.org/10.1002/acn3.52277 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1832592673311031296 |
|---|---|
| author | Wouter I. Schievink Marcel M. Maya Robin Babadjouni Angelique Sao‐Mai S. Tay Rachelle B. Taché |
| author_facet | Wouter I. Schievink Marcel M. Maya Robin Babadjouni Angelique Sao‐Mai S. Tay Rachelle B. Taché |
| author_sort | Wouter I. Schievink |
| collection | DOAJ |
| description | Abstract Objective Frontotemporal dementia (FTD) sagging brain syndrome is a disabling condition. An underlying spinal Cerebrospinal fluid leak can be identified in only a minority of patients and the success rate of non‐directed treatments is low. Some of these patients have a remote history of craniectomy/cranioplasty and we report a positive response to custom implant cranioplasty revision many years after their initial cranioplasty. Methods We reviewed medical records and imaging studies of 61 consecutive patients with FTD sagging brain syndrome. A SIH Disability Assessment Score (SIHDAS) questionnaire was completed to assess the severity of the symptoms before and after custom implant cranioplasty. Pre‐ and post‐operative brain MRI was obtained to assess degree of brain sagging. Results Eight (13.1%) of the 61 patients had a history of craniectomy/cranioplasty 1.5–13.5 years prior to onset of symptoms of FTD sagging brain syndrome. The mean age of the one woman and seven men at the time of presentation to our medical center was 50 years (range, 26–68 years). None had sinking scalp flap syndrome. Prior treatments included epidural blood patching and dural reduction surgery. Custom cranial implant surgery was performed in four patients and resulted in prompt and remarkable improvement of symptoms in three patients (SIHDAS: very severe disability to no or mild disability) and mild improvement in one patient. Brain MRI showed improvement of brain sagging. Interpretation A disproportionate number of patients with FTD sagging brain syndrome have a remote history of supratentorial craniectomy/cranioplasty and revision cranioplasty should be considered. |
| format | Article |
| id | doaj-art-d05e37eba9314d6587d33e3da3ead10c |
| institution | Kabale University |
| issn | 2328-9503 |
| language | English |
| publishDate | 2025-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Annals of Clinical and Translational Neurology |
| spelling | doaj-art-d05e37eba9314d6587d33e3da3ead10c2025-01-21T05:41:42ZengWileyAnnals of Clinical and Translational Neurology2328-95032025-01-0112122623410.1002/acn3.52277Skull defect – Frontotemporal dementia sagging brain syndromeWouter I. Schievink0Marcel M. Maya1Robin Babadjouni2Angelique Sao‐Mai S. Tay3Rachelle B. Taché4Department 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 Neurosurgery Cedars‐Sinai Medical Center Los Angeles California USAAbstract Objective Frontotemporal dementia (FTD) sagging brain syndrome is a disabling condition. An underlying spinal Cerebrospinal fluid leak can be identified in only a minority of patients and the success rate of non‐directed treatments is low. Some of these patients have a remote history of craniectomy/cranioplasty and we report a positive response to custom implant cranioplasty revision many years after their initial cranioplasty. Methods We reviewed medical records and imaging studies of 61 consecutive patients with FTD sagging brain syndrome. A SIH Disability Assessment Score (SIHDAS) questionnaire was completed to assess the severity of the symptoms before and after custom implant cranioplasty. Pre‐ and post‐operative brain MRI was obtained to assess degree of brain sagging. Results Eight (13.1%) of the 61 patients had a history of craniectomy/cranioplasty 1.5–13.5 years prior to onset of symptoms of FTD sagging brain syndrome. The mean age of the one woman and seven men at the time of presentation to our medical center was 50 years (range, 26–68 years). None had sinking scalp flap syndrome. Prior treatments included epidural blood patching and dural reduction surgery. Custom cranial implant surgery was performed in four patients and resulted in prompt and remarkable improvement of symptoms in three patients (SIHDAS: very severe disability to no or mild disability) and mild improvement in one patient. Brain MRI showed improvement of brain sagging. Interpretation A disproportionate number of patients with FTD sagging brain syndrome have a remote history of supratentorial craniectomy/cranioplasty and revision cranioplasty should be considered.https://doi.org/10.1002/acn3.52277 |
| spellingShingle | Wouter I. Schievink Marcel M. Maya Robin Babadjouni Angelique Sao‐Mai S. Tay Rachelle B. Taché Skull defect – Frontotemporal dementia sagging brain syndrome Annals of Clinical and Translational Neurology |
| title | Skull defect – Frontotemporal dementia sagging brain syndrome |
| title_full | Skull defect – Frontotemporal dementia sagging brain syndrome |
| title_fullStr | Skull defect – Frontotemporal dementia sagging brain syndrome |
| title_full_unstemmed | Skull defect – Frontotemporal dementia sagging brain syndrome |
| title_short | Skull defect – Frontotemporal dementia sagging brain syndrome |
| title_sort | skull defect frontotemporal dementia sagging brain syndrome |
| url | https://doi.org/10.1002/acn3.52277 |
| work_keys_str_mv | AT wouterischievink skulldefectfrontotemporaldementiasaggingbrainsyndrome AT marcelmmaya skulldefectfrontotemporaldementiasaggingbrainsyndrome AT robinbabadjouni skulldefectfrontotemporaldementiasaggingbrainsyndrome AT angeliquesaomaistay skulldefectfrontotemporaldementiasaggingbrainsyndrome AT rachellebtache skulldefectfrontotemporaldementiasaggingbrainsyndrome |