ABSTRACT
Gerstmann-Sträussler-Scheinker Syndrome (GSS) is a rare, infectious syndrome related to a mutation in the prion protein. A 60-yr-old, 152-cm, 31-kg woman with GSS was scheduled for open gastrostomy. This is the first report about an anesthetic experience in a patient with GSS. We describe our experience and precautions in the anesthetic management of a GSS patient.
Subject(s)
Anesthesia/methods , Gerstmann-Straussler-Scheinker Disease/surgery , Mutation/genetics , Prions/genetics , Airway Obstruction/prevention & control , Equipment Contamination/prevention & control , Female , Gerstmann-Straussler-Scheinker Disease/genetics , Humans , Intubation, Intratracheal/instrumentation , Intubation, Intratracheal/methods , Middle AgedABSTRACT
A 50-year-old patient with a 6-month history of progressive cognitive and motor disability is presented. There were no myoclonic jerks on examination and no periodic sharp waves by electroencephalography. Imaging showed high signal on T2-weighted scans in the basal ganglia and posterior limbs of the internal capsules, with no restricted diffusion and parenchymal volume loss. A brain biopsy was performed. Western blot analysis revealed a protease-resistant prion protein fragment (PrP7-8), the molecular hallmark of Gerstmann-Straussler-Scheinker disease.
