ArticleNeurology. Clinical practice2024
Compulsive Repetitive Flexion With Breath-Holding in Sagging Brain Syndrome.
Article in Neurology. Clinical practice, 2024. 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.
- Delayed Presentation of Diencephalic-Mesencephalic Junction Dysplasia With Compulsive Truncal Movements and Blepharospasm: A Case Report From India.Journal of movement disorders · 2026Article
- Azygos Vein Stenosis in Frontotemporal Dementia Sagging Brain Syndrome.AJNR. American journal of neuroradiology · 2025Article
- Reply: Dementia in spontaneous intracranial hypotension: look at the spine.Neuroradiology · 2025Article
- Skull defect - Frontotemporal dementia sagging brain syndrome.Annals of clinical and translational neurology · 2025Article
- Movement Disorders in Brain Sagging Syndrome Due To Spontaneous Intracranial Hypotension: A Review.Tremor and other hyperkinetic movements (New York, N.Y.) · 2024Review
Corrections and comments
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Authors and funding
2 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Background and Objective: Spontaneous intracranial hypotension (SIH) from CSF leak commonly produces headache. It also may produce sagging brain syndrome (SBS), often with neurocognitive symptoms indistinguishable from behavioral-variant frontotemporal dementia (bvFTD). The authors describe a new clinical sign that appears to be pathognomonic of SBS. Methods: We reviewed medical records and brain imaging in patients seen at our 2 centers who presented with SIH, SBS, and bvFTD symptoms. Results: There were 51 patients (12 women, 39 men) with mean age 55.5 years (range, 26-70 years). MRI showed severe brain sagging in all. Thirteen patients displayed repetitive flexion with breath-holding at the time of clinical presentation. Five patients had repetitive flexion with breath-holding, which resolved before presenting for evaluation. Thus, 35.3% (18) of 51 patients with SBS displayed seemingly compulsive repetitive flexion with breath-holding. Discussion: Compulsive repetitive flexion with breath-holding appears to be pathognomonic of SBS, deserving the acronym CoRFBiS (compulsive repetitive flexion with breath-holding in SBS). CoRFBiS should alert the clinician to SBS with SIH as the proximate cause of the clinical constellation, rather than bvFTD.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.