Creutzfeldt-Jakob Syndrome (Creutzfeldt-Jakob Disease)

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 (potentially associated with 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))
Also Known As:
Creutzfeldt-Jakob Disease; Encephalopathy, Subacute Spongiform; Subacute Spongiform Encephalopathy; Syndrome, Creutzfeldt-Jakob; CJD (Creutzfeldt-Jakob Disease); Creutzfeldt Jacob Disease; Creutzfeldt-Jakob Disease, Familial; Creutzfeldt-Jakob Disease, New Variant; Creutzfeldt-Jakob Disease, Variant; Familial Creutzfeldt-Jakob Disease; Jakob-Creutzfeldt Disease; Jakob-Creutzfeldt Syndrome; V-CJD (Variant-Creutzfeldt-Jakob Disease); Variant Creutzfeldt-Jakob Disease; CJD (Creutzfeldt Jakob Disease); Creutzfeldt Jakob Disease; Creutzfeldt Jakob Disease, Familial; Creutzfeldt Jakob Disease, New Variant; Creutzfeldt Jakob Disease, Variant; Creutzfeldt Jakob Syndrome; Creutzfeldt-Jakob Diseases, Familial; Disease, Creutzfeldt Jacob; Disease, Creutzfeldt-Jakob; Disease, Familial Creutzfeldt-Jakob; Disease, Jakob-Creutzfeldt; Encephalopathies, Subacute Spongiform; Familial Creutzfeldt Jakob Disease; Familial Creutzfeldt-Jakob Diseases; Jacob Disease, Creutzfeldt; Jakob Creutzfeldt Disease; Jakob Creutzfeldt Syndrome; New Variant Creutzfeldt Jakob Disease; Spongiform Encephalopathies, Subacute; Subacute Spongiform Encephalopathies; Syndrome, Jakob-Creutzfeldt; V CJD (Variant Creutzfeldt Jakob Disease); Variant Creutzfeldt Jakob Disease; New Variant Creutzfeldt-Jakob Disease; Spongiform Encephalopathy, Subacute
Networked: 2215 relevant articles (13 outcomes, 122 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Creutzfeldt-Jakob Syndrome (Creutzfeldt-Jakob Disease)
2. Prion Diseases (Transmissible Spongiform Encephalopathies)
3. Scrapie
4. Bovine Spongiform Encephalopathy (Mad Cow Disease)
5. Chronic Wasting Disease


1. Ironside, James W: 54 articles (01/2021 - 01/2002)
2. Collinge, John: 48 articles (01/2022 - 12/2002)
3. Kitamoto, Tetsuyuki: 43 articles (03/2022 - 06/2002)
4. Zerr, Inga: 41 articles (01/2022 - 06/2002)
5. Parchi, Piero: 34 articles (01/2022 - 10/2003)
6. Head, Mark W: 31 articles (01/2019 - 01/2002)
7. Brandner, Sebastian: 28 articles (01/2022 - 03/2003)
8. Mead, Simon: 25 articles (01/2022 - 03/2006)
9. Haïk, Stéphane: 25 articles (10/2021 - 06/2002)
10. Gambetti, Pierluigi: 25 articles (11/2019 - 03/2003)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Creutzfeldt-Jakob Syndrome:
1. PrionsIBA
2. 14-3-3 Proteins (14-3-3 Protein)IBA
3. Prion ProteinsIBA
4. Growth Hormone (Somatotropin)IBA
5. PrPSc ProteinsIBA
6. ParaffinIBA
7. Peptide Hydrolases (Proteases)FDA Link
05/21/2004 - "Previous studies using post-mortem human brain extracts demonstrated that PrP in Creutzfeldt-Jakob disease (CJD) brains is cleaved by a cellular protease to generate a C-terminal fragment, referred to as C2, which has the same molecular weight as PrP-(27-30), the protease-resistant core of PrP(Sc) (1). "
09/01/2014 - "Western blot analysis of proteinase Kresistant PrP showed a characteristic band pattern with a small molecular band of 6 kDa. The reason for the similarity in clinicopathologic findings between the present case and Creutzfeldt-Jakob disease is uncertain; however, the existence of an unknown disease-modifying factor is suspected. "
04/01/2013 - "To determine the incidence and phenotype of variably protease-sensitive prionopathy within a single well-defined cohort, we have conducted a retrospective review of patients referred to the National Creutzfeldt-Jakob Disease Research & Surveillance Unit during the period 1991-2008. "
01/01/2013 - "However, a prominent molecular characteristic of PrP(Sc) in the recently identified variably protease-sensitive prionopathy (VPSPr) is the absence of a diglycosylated form, also notable in familial Creutzfeldt-Jakob disease (fCJD), which is linked to mutations in PrP either from Val to Ile at residue 180 (fCJD(V180I)) or from Thr to Ala at residue 183 (fCJD(T183A)). "
11/07/2008 - "We characterized a novel protease-resistant PrP fragment migrating 2-3 kDa faster than PrP27-30 in Creutzfeldt-Jakob disease (CJD) brains. "
8. Formaldehyde (Formol)FDA Link
9. Antiviral Agents (Antivirals)IBA
10. Anti-Bacterial Agents (Antibiotics)IBA

Therapies and Procedures

1. Surgical Instruments (Clip)
2. Blood Transfusion (Blood Transfusions)
3. Therapeutics
4. Operative Surgical Procedures
5. Nursing Care