Brain Sagging Dementia

Author:

Lashkarivand Aslan,Eide Per Kristian

Abstract

Abstract Purpose of Review Brain sagging dementia (BSD) is a rare but devastating form of early-onset dementia characterized by intracranial hypotension and behavioral changes resembling behavioral variant frontotemporal dementia. This review aims to provide a comprehensive overview of BSD, highlighting its pathomechanism, diagnostic tools, and available treatment options. Recent Findings BSD exhibits a complex clinical manifestation with insidious onset and gradual progression of behavioral disinhibition, apathy, inertia, and speech alterations. Additionally, patients may exhibit brainstem and cerebellar signs such as hypersomnolence and gait disturbance. Although headaches are common, they may not always demonstrate typical orthostatic features. Recent radiological advances have improved the detection of CSF leaks, enabling targeted treatment and favorable outcomes. Summary Understanding the pathomechanism and available diagnostic tools for BSD is crucial for a systematic approach to timely diagnosis and treatment of this reversible form of early-onset dementia, as patients often endure a complex and lengthy clinical course.

Funder

University of Oslo

Publisher

Springer Science and Business Media LLC

Subject

Neurology (clinical),General Neuroscience

Reference97 articles.

1. •• Lashkarivand A, Eide PK. Brain sagging dementia – diagnosis, treatment, and outcome: a review. Neurology. 2022;25:25. First systematic review on brain sagging dementia, covering its characteristics, diagnosis, treatment, and outcomes. The authors present a diagnostic tool to facilitate accurate diagnosis and treatment.

2. • Lashkarivand A, Eide PK. The first report on brain sagging dementia caused by a cranial leak: a case report. Frontiers Neurol [electronic resource]. 2022;13:1006060. First case report of a patient with BSD due to a cranial CSF leak, successfully treated with full recovery. Remarkably, the initial injury causing the leak occurred approximately 50 years before the onset of BSD symptoms.

3. Schievink WI, Maya MM, Barnard ZR, Moser FG, Jean-Pierre S, Waxman AD, et al. Behavioral variant frontotemporal dementia as a serious complication of spontaneous intracranial hypotension. Operative Neurosurg. 2018;15(5):505–15.

4. •• Schievink WI, Maya M, Barnard Z, Tache RB, Prasad RS, Wadhwa VS, et al. The reversible impairment of behavioral variant frontotemporal brain sagging syndrome: challenges and opportunities. Alzheimers Dement (N Y). 2022;8(1):e12367. The authors conducted a comprehensive literature review on brain sagging and bvFTD symptoms, emphasizing the significance of accurate recognition, diagnosis, and treatment. In a study involving 21 patients exhibiting these symptoms, DSM in the lateral decubitus position revealed a CSF-venous fistula in nine patients, with complete symptom resolution following treatment.

5. Vetrugno R, Mascalchi M, Chierichetti F, Gallassi R, Alessandria M, Guerrini L, et al. Hypoactive-hypoalert behavior (“psychic akinesia”) in intracranial hypotension syndrome. Neurology. 2008;71(18):1452–4.

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