TY - CHAP M1 - Book, Section TI - Gerstmann-Sträussler-Scheinker (GSS) Disease A1 - Bissonnette, Bruno A1 - Luginbuehl, Igor A1 - Engelhardt, Thomas PY - 2019 T2 - Syndromes: Rapid Recognition and Perioperative Implications, 2e 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. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/28 UR - accessanesthesiology.mhmedical.com/content.aspx?aid=1164070699 ER -