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Frontotemporal brain sagging syndrome: an SIH-like presentation mimicking FTD
M R Wicklund1, B Mokri, D A Drubach
1Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA.
Neurology
|April 20, 2011
Summary
Frontotemporal brain sagging syndrome (FBSS) can mimic behavioral variant frontotemporal dementia (bvFTD). This condition, characterized by brain sagging, may be treatable and warrants recognition.
Area of Science:
- Neurology
- Neuroimaging
- Neurodegenerative Diseases
Background:
- Behavioral variant frontotemporal dementia (bvFTD) is a recognized syndrome linked to structural and metabolic changes in the frontal and temporal lobes.
- Frontotemporal brain sagging syndrome (FBSS) presents with features overlapping bvFTD, alongside evidence of brain sagging.
Purpose of the Study:
- To describe the clinical and imaging characteristics of patients with frontotemporal brain sagging syndrome (FBSS).
- To differentiate FBSS from typical bvFTD and highlight its potential treatability.
Main Methods:
- Retrospective chart review of patients diagnosed with FTD and imaging evidence of brain sag between 1996 and 2010.
- Analysis of clinical symptoms, functional imaging (hypometabolism), brain MRI findings (brain sagging, brainstem distortion), CSF analysis, and neuropathology.
Main Results:
- Eight patients (7 male, 1 female) diagnosed with FBSS, with median onset at 53 years.
- Common symptoms included behavioral/cognitive dysfunction, somnolence, and headache. Imaging showed frontotemporal hypometabolism and brain sagging.
- Neuropathology in two cases excluded neurodegenerative disease, suggesting FBSS is distinct from typical FTD.
Conclusions:
- Frontotemporal brain sagging syndrome (FBSS) can clinically and radiographically mimic bvFTD.
- Recognition of FBSS is crucial as it represents an unusual presentation that is potentially treatable.
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