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Bovine Spongiform Encephalopathy

MedGen UID:
38817
•Concept ID:
C0085209
•
Disease or Syndrome
Synonyms: Bovine Spongiform Encephalitis; BSE (Bovine Spongiform Encephalopathy); BSEs (Bovine Spongiform Encephalopathy); Encephalitis, Bovine Spongiform; Encephalopathy, Bovine Spongiform; Mad Cow Disease; Mad Cow Diseases; Spongiform Encephalopathy, Bovine

Definition

A transmissible spongiform encephalopathy of cattle associated with abnormal prion proteins in the brain. Affected animals develop excitability and salivation followed by ATAXIA. This disorder has been associated with consumption of SCRAPIE infected ruminant derived protein. This condition may be transmitted to humans, where it is referred to as variant or new variant CREUTZFELDT-JAKOB SYNDROME. (Vet Rec 1998 Jul 25;143(41):101-5) [from MeSH]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVBovine Spongiform Encephalopathy

Professional guidelines

PubMed

Creutzfeldt-Jakob disease: updated diagnostic criteria, treatment algorithm, and the utility of brain biopsy.
Manix M, Kalakoti P, Henry M, Thakur J, Menger R, Guthikonda B, Nanda A
Neurosurg Focus 2015 Nov;39(5):E2. doi: 10.3171/2015.8.FOCUS15328. PMID: 26646926
Laboratory activities involving transmissible spongiform encephalopathy causing agents: risk assessment and biosafety recommendations in Belgium.
Leunda A, Van Vaerenbergh B, Baldo A, Roels S, Herman P
Prion 2013 Sep-Oct;7(5):420-33. Epub 2013 Sep 20 doi: 10.4161/pri.26533. PMID: 24055928Free PMC Article
Assessment of the risk factors related to bovine spongiform encephalopathy.
Morley RS, Chen S, Rheault N
Rev Sci Tech 2003 Apr;22(1):157-78. doi: 10.20506/rst.22.1.1384. PMID: 12793778

Recent clinical studies

Etiology

Prion Diseases.
Whitechurch BC, Welton JM, Collins SJ, Lawson VA
Adv Neurobiol 2017;15:335-364. doi: 10.1007/978-3-319-57193-5_13. PMID: 28674988
Bovine spongiform encephalopathy.
Harman JL, Silva CJ
J Am Vet Med Assoc 2009 Jan 1;234(1):59-72. doi: 10.2460/javma.234.1.59. PMID: 19119967
Bovine spongiform encephalopathy.
Prince MJ, Bailey JA, Barrowman PR, Bishop KJ, Campbell GR, Wood JM
Rev Sci Tech 2003 Apr;22(1):37-60. doi: 10.20506/rst.22.1.1389. PMID: 12793773
Prions in dermatology.
Lupi O
J Am Acad Dermatol 2002 May;46(5):790-3. doi: 10.1067/mjd.2002.120624. PMID: 12004327
Prions.
Prusiner SB
Proc Natl Acad Sci U S A 1998 Nov 10;95(23):13363-83. doi: 10.1073/pnas.95.23.13363. PMID: 9811807Free PMC Article

Diagnosis

Bovine spongiform encephalopathy: A review of current knowledge and challenges.
Lestari TD, Khairullah AR, Utama S, Mulyati S, Hernawati T, Damayanti R, Rimayanti R, Wardhani BWK, Fauzia KA, Moses IB, Ahmad RZ, Wibowo S, Fauziah I, Kurniasih DAA, Baihaqi ZA, Wasito W, Kusala MKJ, Lisnanti EF
Open Vet J 2025 Jan;15(1):54-68. Epub 2025 Jan 31 doi: 10.5455/OVJ.2025.v15.i1.5. PMID: 40092198Free PMC Article
A Dissenter's Journey.
James WPT
Annu Rev Nutr 2021 Oct 11;41:1-18. Epub 2021 Jun 11 doi: 10.1146/annurev-nutr-101220-114101. PMID: 34115517
Variant Creutzfeldt-Jakob disease.
Brandel JP, Knight R
Handb Clin Neurol 2018;153:191-205. doi: 10.1016/B978-0-444-63945-5.00011-8. PMID: 29887136
Atypical and classic bovine spongiform encephalopathy.
Casalone C, Hope J
Handb Clin Neurol 2018;153:121-134. doi: 10.1016/B978-0-444-63945-5.00007-6. PMID: 29887132
Prions in dermatology.
Lupi O
J Am Acad Dermatol 2002 May;46(5):790-3. doi: 10.1067/mjd.2002.120624. PMID: 12004327

Therapy

Variant Creutzfeldt-Jakob disease.
Brandel JP, Knight R
Handb Clin Neurol 2018;153:191-205. doi: 10.1016/B978-0-444-63945-5.00011-8. PMID: 29887136
Prospects for safe and effective vaccines against prion diseases.
Mabbott NA
Expert Rev Vaccines 2015 Jan;14(1):1-4. Epub 2014 Sep 30 doi: 10.1586/14760584.2015.965691. PMID: 25266267
Variant Creutzfeldt-Jakob disease.
Ironside JW
Haemophilia 2010 Jul;16 Suppl 5:175-80. doi: 10.1111/j.1365-2516.2010.02317.x. PMID: 20590878
Cross-species infections.
Weiss RA
Curr Top Microbiol Immunol 2003;278:47-71. doi: 10.1007/978-3-642-55541-1_3. PMID: 12934941
Bovine spongiform encephalopathy.
Prince MJ, Bailey JA, Barrowman PR, Bishop KJ, Campbell GR, Wood JM
Rev Sci Tech 2003 Apr;22(1):37-60. doi: 10.20506/rst.22.1.1389. PMID: 12793773

Prognosis

A Dissenter's Journey.
James WPT
Annu Rev Nutr 2021 Oct 11;41:1-18. Epub 2021 Jun 11 doi: 10.1146/annurev-nutr-101220-114101. PMID: 34115517
Atypical and classic bovine spongiform encephalopathy.
Casalone C, Hope J
Handb Clin Neurol 2018;153:121-134. doi: 10.1016/B978-0-444-63945-5.00007-6. PMID: 29887132
Prion diseases.
Johnson RT
Lancet Neurol 2005 Oct;4(10):635-42. doi: 10.1016/S1474-4422(05)70192-7. PMID: 16168932
Prions in dermatology.
Lupi O
J Am Acad Dermatol 2002 May;46(5):790-3. doi: 10.1067/mjd.2002.120624. PMID: 12004327
Beef and bovine spongiform encephalopathy: the risk persists.
Dealler S, Lacey R
Nutr Health 1991;7(3):117-33. doi: 10.1177/026010609100700301. PMID: 1923069

Clinical prediction guides

Atypical and classic bovine spongiform encephalopathy.
Casalone C, Hope J
Handb Clin Neurol 2018;153:121-134. doi: 10.1016/B978-0-444-63945-5.00007-6. PMID: 29887132
Biochemical Characterization of Prions.
Fiorini M, Bongianni M, Monaco S, Zanusso G
Prog Mol Biol Transl Sci 2017;150:389-407. Epub 2017 Aug 8 doi: 10.1016/bs.pmbts.2017.06.012. PMID: 28838671
Bovine spongiform encephalopathy: a tipping point in One Health and Food Safety.
Hope J
Curr Top Microbiol Immunol 2013;366:37-47. doi: 10.1007/82_2012_264. PMID: 22976349
Prion diseases.
Johnson RT
Lancet Neurol 2005 Oct;4(10):635-42. doi: 10.1016/S1474-4422(05)70192-7. PMID: 16168932
Clinical implications of bovine spongiform encephalopathy.
MacKnight C
Clin Infect Dis 2001 Jun 15;32(12):1726-31. Epub 2001 May 7 doi: 10.1086/320760. PMID: 11360215

Recent systematic reviews

Systematic Review of Clinical and Pathophysiological Features of Genetic Creutzfeldt-Jakob Disease Caused by a Val-to-Ile Mutation at Codon 180 in the Prion Protein Gene.
Matsubayashi T, Sanjo N
Int J Mol Sci 2022 Dec 2;23(23) doi: 10.3390/ijms232315172. PMID: 36499498Free PMC Article
Diagnostic value of diffusion-weighted brain magnetic resonance imaging in patients with sporadic Creutzfeldt-Jakob disease: a systematic review and meta-analysis.
Park HY, Kim M, Suh CH, Kim SY, Shim WH, Kim SJ
Eur Radiol 2021 Dec;31(12):9073-9085. Epub 2021 May 12 doi: 10.1007/s00330-021-08031-4. PMID: 33982159
Current evidence on the transmissibility of chronic wasting disease prions to humans-A systematic review.
Waddell L, Greig J, Mascarenhas M, Otten A, Corrin T, Hierlihy K
Transbound Emerg Dis 2018 Feb;65(1):37-49. Epub 2017 Jan 30 doi: 10.1111/tbed.12612. PMID: 28139079
A systematic review of prion therapeutics in experimental models.
Trevitt CR, Collinge J
Brain 2006 Sep;129(Pt 9):2241-65. Epub 2006 Jul 1 doi: 10.1093/brain/awl150. PMID: 16816391
Variant Creutzfeldt-Jakob disease: a cause for concern. Review of the evidence for risk of transmission through abdominal lymphoreticular tissue surgery.
Olsen SB, Sheikh A, Peck D, Darzi A
Surg Endosc 2005 Jun;19(6):747-50. Epub 2005 May 4 doi: 10.1007/s00464-004-9205-2. PMID: 15868249

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