Disease Information for Creutzfeld-Jakob disease: Definition

  • Creutzfeld-Jakob disease is also known as Transmissable Spongiform Encephalopathy Prion Disease; This is a rare progressive, fatal, dementing illness caused by an infectious agent know as a prion. incidence is 1 in a million, usually peak age is 60 years [but range is 16 to 82 years]; 5-10% are familial; cases are spread by corneal transplants, dura mater allografts, and in the past human pituitary extract; Gene for normal prion protein is on chromosome 20; Normal noninfectious prion protein [PrP] is a cellular protein found onsurfaces of neurons; This is converted into a non-soluble precipitate by infection with

    abnormal prions; the disease is fatal; Mean duration of illness is 8 months [range 1-130 months]; Other Prion diseases include kuru, Fatal familial insomnia, Gertsmann-Staussler-Schenker Syndrome, and new variant Creutzfeld-Jakob disease; Animal diseases Include Scrape [sheep] and bovine spongiform encephalopathy [mad cow disease] ; this later can spread to humans ,---------------[Ferri]----------------A rare transmissible encephalopathy most prevalent between the ages of 50 and 70 years; Affected individuals may present with sleep disturbances, personality changes, ATAXIA; APHASIA, visual loss, weakness, muscle atrophy, MYOCLONUS, progressive dementia, and death within one year of disease onset; A familial form exhibiting autosomal dominant inheritance and a new variant CJD (variant ENCEPHALOPATHY, BOVINE SPONGIFORM) have been described; Pathological features include prominent cerebellar and cerebral cortical spongiform degeneration and the presence of PRIONS; (From N Engl J Med, 1998 Dec 31;339(27));---------- Mad cow disease causes accelerated encephalopathy variant in humans seen in younger adults--------------------

    Creutzfeldt Jakob Disease; Also known as : CJD; Jakob"s Disease; Jakob-Creutzfeldt Disease; Subacute Spongiform Encephalopathy ; and Variant Creutzfeldt-Jakob Disease (V-CJD); Creutzfeldt-Jakob Disease (CJD) is an extremely rare degenerative brain disorder (ie, "spongiform" encephalopathy) characterized by sudden development of rapidly progressive neurological and neuromuscular symptoms; With symptom onset, affected individuals may develop confusion, depression, behavioral changes, impaired vision, and/or impaired coordination;