Lessons of the month 3: Spontaneous resolution of frontotemporal brain sagging syndrome

Kent, Louisa; Butterworth, Richard; Butler, Chris

doi:10.7861/clinmedicine.19-4-336

Cited by 10 publications

(17 citation statements)

References 4 publications

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“…Upon further scrutiny, one may reveal that the symptoms started with severe headaches several months to years before cognitive decline. 22,27,29,30,35 Like patients with SIH who may feel worsening of headaches towards the end of the day, patients with BSD may feel behavioral and cognitive decline towards the evening. 16 However, we are not sure whether an acute (within minutes) positional worsening and improvement of behavioral and cognitive symptoms are related to BSD.…”

Section: Discussionmentioning

confidence: 99%

“…Some common characteristics were outlined in most reports: antegrade amnesia, 22,[24][25][26] distractibility and impersistence, 23,24,[27][28][29][30] preservative and stereotyped behavior, 7,8,21,[30][31][32][33] and altered speech output. 8,16,21,[27][28][29][30][31][32][33] A decline in social and interpersonal conduct, including bizarre behavior and social and sexual inappropriateness, was a prominent future in most reports. [5][6][7][8]16,17,[29][30][31][32][33][34][35][36][37] In contrast to bvFTD, 60% of patients with BSD also presented with cerebellar symptoms such as ataxia, gait disturbance, and dysarthria.…”

Section: Clinical Presentationmentioning

confidence: 99%

“…8,16,19,[21][22][23] Although signs and symptoms mimicking bvFTD were an inclusion criterion, most cases with an insidious onset and gradual progression, 1 without an acute event that triggered immediate medical attention (more than 3-4 weeks), were included in the analysis. Some common characteristics were outlined in most reports: antegrade amnesia, 22,[24][25][26] distractibility and impersistence, 23,24,[27][28][29][30] preservative and stereotyped behavior, 7,8,21,[30][31][32][33] and altered speech output. 8,16,21,[27][28][29][30][31][32][33] A decline in social and interpersonal conduct, including bizarre behavior and social and sexual inappropriateness, was a prominent future in most reports.…”

Section: Clinical Presentationmentioning

confidence: 99%

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Brain Sagging Dementia—Diagnosis, Treatment, and Outcome

Lashkarivand

Eide

2022

Neurology

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Brain sagging dementia (BSD), caused by spontaneous intracranial hypotension (SIH), is a rare syndrome that is only recently recognized, mimicking the clinical findings of behavioral variant frontotemporal dementia (bvFTD). Being aware of its signs and symptoms is essential for early diagnosis and treatment in this potentially reversible form of dementia. Our objective was to identify cases with BSD in the literature and present its clinical characteristics, diagnostic workup, treatment options, and outcome.The review was reported according to PRISMA guidelines and registered with the PROSPERO database (CRD42020150709). MEDLINE, EMBASE, PsychINFO, and Cochrane Library were searched. There was no date restriction. The search was updated in April 2021.A total of 983 articles were screened and assessed for eligibility. Twenty-nine articles (25 case reports and four series) and 70 patients were selected for inclusion. No cranial leak cases were identified. BSD diagnosis should be made based on clinical signs and symptoms and radiological findings. There is a male predominance (F: M ratio 1:4) and a peak incidence in the 6th decade of life. The main clinical manifestation is insidious onset, gradually progressive cognitive and behavioral changes characteristic for bvFTD. Headache is present in the majority of patients (89%). The presence of brain sagging and absence of frontotemporal atrophy is an absolute criterion for the diagnosis. The CSF leak is identified with myelography and digital subtraction myelography. The treatment and repair depend on the etiology and extent of the dural defect, although an epidural blood patch is the first-line treatment in most cases. With treatment, 81% experienced partial and 67% complete resolution of their symptoms. This review highlights the most important clinical aspects of BSD. Due to the sparse evidence and lack of BSD awareness, many patients are most likely left undiagnosed. Recognizing this condition is essential to provide early treatment to reverse the cognitive and behavioral changes that may otherwise progress and fully impair the patient. Moreover, patients with longstanding SIH must be carefully assessed for cognitive and behavioral changes.

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Section: Discussionmentioning

confidence: 99%

Section: Clinical Presentationmentioning

confidence: 99%

Section: Clinical Presentationmentioning

confidence: 99%

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Brain Sagging Dementia—Diagnosis, Treatment, and Outcome

Lashkarivand

Eide

2022

Neurology

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“…However, given that the clinical nadir of neurological symptoms was reached and followed by a plateau phase before the treatment and that the improvements after treatment were exceedingly gradual, we cannot exclude the possibility that the latent improvement occurred spontaneously. Cases with spontaneous resolution of FBSS have been reported and spontaneous resolution of CSF leakage and “auto-blood patch” resulting from lumbar puncture or trauma have been postulated as mechanisms for spontaneous improvement in FBSS [ 9 , 10 ].…”

Section: Discussion/conclusionmentioning

confidence: 99%

Frontotemporal Brain Sagging Syndrome as a Treatable Cause Mimicking Frontotemporal Dementia: A Case Report

et al. 2022

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Frontotemporal brain sagging syndrome (FBSS) is a progressive disorder characterized by symptoms similar to the behavioral variant of frontotemporal dementia (FTD), with a sagging appearance of the brain on imaging similar to that observed in spontaneous intracranial hypotension (SIH). The onset of behavioral and cognitive symptoms of FBSS is insidious and progressive, similar to those of FTD. Here, we report a case involving a 53-year-old man with progressive hypersomnolence, apathy, forgetfulness, and personality changes but without headache or auditory symptoms. The combination of frontotemporal dysfunction, hypersomnolence, and the appearance of a sagging brain on magnetic resonance imaging suggested a diagnosis of FBSS. Although a definite site of cerebrospinal fluid leakage could not be identified in our case, clinical symptoms and imaging findings were improved after an epidural blood patch. Considering FBSS as a differential diagnosis of FTD is important even in the absence of typical SIH symptoms, such as headache or auditory symptoms.

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“…Editor -Like Dr Kent and colleagues, 1 I have recently seen a patient with apparently spontaneous resolution of neuroradiological features of frontotemporal brain sagging syndrome (FTBSS), but with different clinical outcome.…”

Section: Spontaneous Resolution Of Frontotemporal Brain Sagging Syndromementioning

confidence: 99%

Spontaneous resolution of frontotemporal brain sagging syndrome

Larner

2019

Clinical Medicine

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Lessons of the month 3: Spontaneous resolution of frontotemporal brain sagging syndrome

Cited by 10 publications

References 4 publications

Brain Sagging Dementia—Diagnosis, Treatment, and Outcome

Brain Sagging Dementia—Diagnosis, Treatment, and Outcome

Frontotemporal Brain Sagging Syndrome as a Treatable Cause Mimicking Frontotemporal Dementia: A Case Report

Spontaneous resolution of frontotemporal brain sagging syndrome

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