Frontotemporal Brain Sagging Syndrome

1. Ade Wijaya, MD – June 2023

2.  A progressive disorder characterized by
apathy, behavior, and cognitive dysfunction,
hypersomnolence, and orthostatic headache,
is diagnosed via a sagging appearance of the
brain on imaging, similar to that observed in
spontaneous intracranial hypotension (SIH)
Gustafson L. Frontal lobe degeneration of non-Alzheimer type. II. Clinical picture and
differential diagnosis. Arch Gerontol Geriatr. 1987;6(3):209-223.
Neary D, Snowden JS, Gustafson L, et al. Frontotemporal lobar degeneration: a
consensus on clinical diagnostic criteria. Neurology. 1998;51(6):1546-1554.

3.  Insidious onset, slow progressive course, and
symptoms similar to that of a behavioral
variant of frontotemporal dementia (FTD);
however, it is a potentially treatable disease
 Early diagnosis, treatment, and appropriate
neuropsychological assessment for the
evaluation of treatment efficacy are
important
Gustafson L. Frontal lobe degeneration of non-Alzheimer type. II. Clinical picture and
differential diagnosis. Arch Gerontol Geriatr. 1987;6(3):209-223.
Neary D, Snowden JS, Gustafson L, et al. Frontotemporal lobar degeneration: a
consensus on clinical diagnostic criteria. Neurology. 1998;51(6):1546-1554.

4.  Mechanical forces (stretching) on the frontal
and temporal cortices and (or) their circuits
 Spontaneous intracranial hypotension
Schievink WI, Maya MM, Barnard ZR, et al. Behavioral variant frontotemporal dementia as
a serious complication of spontaneous intracranial hypotension. Oper Neurosurg.
2018;15(5):505-515.

5. Schievink WI, Maya M, Barnard Z, Taché RB, Prasad RS, Wadhwa VS, Moser FG, Nuño M.
The reversible impairment of behavioral variant frontotemporal brain sagging syndrome:
Challenges and opportunities. Alzheimer's & Dementia: Translational Research & Clinical
Interventions. 2022;8(1):e12367.

6. Brain magnetic resonance images. a Axial
T1-weighted postcontrast image
demonstrating pachymeningeal
enhancement and effacement of the basal
cisterns with distortion of the
midbrain. b Coronal fluid-attenuated
inversion recovery image demonstrating
bilateral subdural collections
(arrows). c Sagittal T1-weighted image
demonstrating severe sagging of the brain
with downward displacement of the
cerebellar tonsils, downward drooping
splenium, collapsed widened midbrain, and
flattening of the ventral pons with
effacement of the basal cisterns. d Sagittal
T1-weighted postcontrast image
demonstrating the enlargement of the
venous sinus and pituitary gland.
Sugiyama A, Tamiya A, Yokota H, Mukai H, Otani R, Kuwabara S. Frontotemporal Brain
Sagging Syndrome as a Treatable Cause Mimicking Frontotemporal Dementia: A Case
Report. Case Reports in Neurology. 2022;14:82-7.

7.  Frontotemporal Brain Sagging Syndrome mimicking
BvFTD
 Due to spontaneous intracranial hypotension
 Diagnosis: Clinical and Radiology
 Treatable condition. Early diagnosis and treatment
is important