GERSTMANN-STRAUSSLER-SCHEINKER SYNDROME

Course overview

Gerstmann-Sträussler-Scheinker syndrome (GSS) is an extremely rare chronic neurodegenerative brain disorder that leads to dementia. GSS, which is usually inherited, is one of the transmissible spongiform encephalopathies (TSEs), and is also known as a prion disease. Onset of GSS is typically in the third or fourth decade of life with symptoms of ataxia (e.g., clumsiness, unsteadiness on feet). GSS is characterized by progressive cognitive and motor impairment, including memory loss; slurred speech or the inability to speak (i.e., dysarthria); nystagmus (i.e., involuntary eye movement); blindness or deafness; dementia; and accumulation of amyloid plaques in the brain. This continuing education module is designed to give the reader/learner an increased understanding of GSS, its clinical presentation, symptoms, and treatment for patients with this diagnosis. Readers may explore any or all of the topics. One contact hour of continuing education credit is available for those who successfully complete the post-test and evaluation form.