RT Book, Section A1 Bissonnette, Bruno A1 Luginbuehl, Igor A1 Engelhardt, Thomas SR Print(0) ID 1164070699 T1 Gerstmann-Sträussler-Scheinker (GSS) Disease T2 Syndromes: Rapid Recognition and Perioperative Implications, 2e YR 2019 FD 2019 PB McGraw-Hill Education PP New York, NY SN 9781259861789 LK accessanesthesiology.mhmedical.com/content.aspx?aid=1164070699 RD 2024/03/29 AB GSS is characterized by cerebellar ataxia, pyramidal and extra-pyramidal features, and progressive dementia, eventually leading to death. GSS is invariably fatal and there is no proven treatment or prophylaxis. It is a rare infectious syndrome related to a mutation in the prion protein. It is familial form of subacute spongiform encephalopathy resulting in widespread degeneration of the nervous system, usually beginning in the fourth or fifth decade of life. Special consideration must be given to potential contamination of the attending personnel, other patients, and medical materials.