Creutzfeldt Jakob Disease: An Incurable Malady

Abstract

Creutzfeldt Jakob Disease is a rare and fatal neurodegenerative disorder characterized by rapidly worsening dementia, myoclonic jerks and akinetic mutism. It accounts for more than 90% of all human prion diseases. Over 90% of patients progress from normal function to death in under a year. There is no definitive treatment and it must be distinguished from other causes of rapidly progressive dementia such as viral encephalitis, autoimmune and paraneoplastic encephalitis which will respond to appropriate therapy. In the following 2 cases, we describe patients with presentation of rapidly progressive dementia which was finally diagnosed as Probable sporadic CJD. It is essential to make early diagnosis as it will allow patient and family to understand the course of disease and prognosis