ArticleSurgical neurology international2020
Frontotemporal brain sagging syndrome: Craniospinal hypovolemia secondary to a T6-T7 cerebrospinal fluid-venous fistula.
Article in Surgical neurology international, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers.
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Who cites it
5 citing papers in PubMed, 10 citations in OpenAlex.
- Spinal dementia: Don't miss it, it's treatable.Neuroradiology · 2024Article
- Brain Sagging Dementia.Current neurology and neuroscience reports · 2023Review
- [Spontaneous intracranial hypotension with brain sagging and reversible frontotemporal dementia : Case report and review of the literature].Der Nervenarzt · 2022Review
- The reversible impairment of behavioral variant frontotemporal brain sagging syndrome: Challenges and opportunities.Alzheimer's & dementia (New York, N. Y.) · 2022Article
- Frontotemporal Brain Sagging Syndrome as a Treatable Cause Mimicking Frontotemporal Dementia: A Case Report.Case reports in neurologyArticle
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Authors and funding
9 authors at 1 institution in 1 country.
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Abstract
backgroundThe frontotemporal brain sagging syndrome (FTBSS) is defined as an insidious/progressive decline in behavior and executive functions, hypersomnolence, and orthostatic headaches attributed to cerebrospinal fluid (CSF) hypovolemia. Here, a T6 CSF-venous fistula (e.g., between the subarachnoid CSF and a paraspinal vein) resulted in a CSF leak responsible for craniospinal hypovolemia. CASE DESCRIPTION: A 56-year-old male started with orthostatic headaches and fatigue after scuba diving. His symptoms included progressive, vertigo, tinnitus, nausea, lack of judgment, inappropriate behavior, memory dysfunction, apathy, tremor, orofacial dyskinesia, dysarthria, dysphagia, and hypersomnolence. The lumbar puncture revealed an opening pressure of 0 cm H2O. Magnetic resonance imaging (MRI) findings included brain sagging, bilateral temporal lobe herniation, and pachymeningeal enhancement. The computed tomography (CT) myelogram showed a thoracic diverticulum and a CSF-venous leak at the T6-T7 level. Surgery, which comprised a T6-T7 laminotomy, allowed for dissecting, clipping, and ligating the diverticulum/fistula. The patient improved postoperatively (e.g., cognitive, behavioral, and brainstem symptoms). The follow-up MRI's showed the reversion of the sagging index/uncal herniation.
conclusionThe FTBSS should be considered in the differential diagnosis of an early onset frontotemporal dementia. Establishing the diagnosis and localizing the site of a spinal CSF/venous leak warrant both MRI and myelogram CT studies, to pinpoint the CSF leak site for proper surgical clipping/ligation of these thoracic diverticulum/CSF-venous leaks.
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