Creutzfeldt-Jakob Disease: An Overview

Abstract

Creutzfeldt-Jakob disease (CJD) belongs to a family of fatal degenerative disorders of the nervous system known as transmissible spongiform encephalopathy (TSE) or prion diseases, which affect both animals and humans. It is characterized by neuronal loss or neuronal degeneration, spongiform change and astrocyticgliosis which finally results in fatal neurological disorder. CJD affects one million people worldwide each year. The clinical presentation of CJD includes rapidly developing dementia, neurological symptoms, visual impairment, ataxia and development of akinetic mutism and eventually death. The diagnosis is based on clinical presentation and is diagnosed by Magnetic resonance imaging (MRI) findings, electroencephalogram (EEG), cerebrospinal fluid tests (CSF) and genetic tests such as PRNP gene. There have been recent advances in diagnostic techniques, which have allowed more accurate case recognition in all forms of the CJD. The mainstay of treatment is symptomatic and supportive. There have been many medical advances towards a cure for CJD but no definitive drug or therapy has been identified for the disease.