April 28, 2020 e-Pearl of the Week: Frontotemporal Brain Sagging Syndrome

Frontotemporal Brain Sagging Syndrome

Frontotemporal Brain Sagging Syndrome is a clinicoradiological entity characterized by progressive decline in behavioral and executive functions and headache.1 Average age of onset is 51 years with male predilection. Neuroimaging shows sagging appearance of the frontotemporal region with hypometabolism on PET/SPECT.2 Differentials include behavioral variant frontotemporal dementia and normal pressure hydrocephalus. Treatment involves trial of IV methylprednisolone—followed by epidural blood patches—with surgical repair of the dural tear in refractory cases.3 Spontaneous resolution has rarely been reported,4,5 but overall prognosis remains relatively poor, with none of the treatment modalities resulting in complete reversal of symptoms.

References

  1. Robeson K, Blondin N, Szekely A. Frontotemporal Brain Sagging Syndrome Due to Minor Head Trauma. Neurology 2012;78 (1 Supplement) P03.212.
  2. Wicklund MR, Mokri B, Drubach DA, Boeve BF, Parisi JE, Josephs KA. Frontotemporal brain sagging syndrome: an SIH-like presentation mimicking FTD. Neurology 2011;76:1377–1382.
  3. Mostofi E, Schievink WI, Sim VL. Dural Reduction Surgery: A Treatment Option for Frontotemporal Brain Sagging Syndrome. Can J Neurol Sci 2016;43:593–5.
  4. Kent L, Butterworth R, Butler C. Lessons of the Month 3: Spontaneous resolution of frontotemporal brain sagging syndrome. Clin Med (Lond) 2019;19:336–337.
  5. Slattery CF, Malone IB, Clegg SL, Warren JD, Fox NC. Reversible frontotemporal brain sagging syndrome. Neurology 2015;85:833.

Omar Masihuddin, MPAS, PA-C, Sugar Land Neurology and Sleep (Sugar Land, Texas) and Faisal Khan, MD, DABSM, DABPN, Consultant neurologist, Sugar Land Neurology and Sleep (Sugar Land, Texas)

Omar Masihuddin and Dr. Khan report no disclosures.