The human spongiform encephalopathies

Abstract

The human spongiform encephalopathies are a group of heterogenous, usually fatal diseases, characterized by a unique pathogenetic mechanism and distinct clinical presentation. They are classified into sporadic, familial and acquired forms. The diagnosis of spongiform encephalopathies is based on the combination of the neuropathological examination, with the clinical presentation, the laboratory findings and genetic tests. The most common sporadic form is Creutzfeldt-Jakob disease, while the main familial types are the familial types of Creutzfeldt-Jakob disease, Gerstmann-Straussler-Scheinker disease, prion disease with cerebral amyloid angiopathy, the fatal familiar insomnia. In the present study we aim to review the main features and types of human spongiform encephalopathies, including the sporadic, familiar, and acquired forms.

Keywords

• human spongiform encephalopathies
• cjd
• human prion diseases