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Creutzfeldt–Jakob disease

From Wikipedia
Creutzfeldt-Jakob disease
designated intractable/rare disease, rare disease, class of disease
Subclass oftransmissible spongiform encephalopathy, disease Edit
Dem name afterHans Gerhard Creutzfeldt, Alfons Maria Jakob Edit
Has causeprion Edit
Health specialtyneurology, infectious diseases Edit
Symptoms and signsdementia, myoclonus Edit
Possible treatmentunknown, palliative care Edit
Genetic associationPRNP, MTMR7 Edit
External data available at URLhttp://www.nanbyou.or.jp/entry/5370 Edit
ICD-9-CM046.1, 046.19 Edit
NCI Thesaurus IDC26802 Edit

Creutzfeldt–Jakob disease (CJD) be an incurable, invariably fatal, neurodegenerative disease wey dey belong to de transmissible spongiform encephalopathy (TSE) group.[1][2] Early symptoms dey include memory problems, behavioral changes, poor coordination, visual disturbances den auditory disturbances.[1] Later symptoms dey include dementia, involuntary movements, blindness, deafness, weakness, den coma.[1] About 70% of sufferers dey die within a year of diagnosis.[1] Na Walther Spielmeyer introduce de name "Creutzfeldt–Jakob disease" insyd 1922, after de German neurologists Hans Gerhard Creutzfeldt den Alfons Maria Jakob.[3]

CJD be caused by a prion,[4] an infectious abnormal folding of a protein. Infectious prions be misfolded proteins wey fi cause normally folded proteins make e sanso cam be misfolded.[1] About 85% of cases of CJD dey occur for unknown reasons, while about 7.5% of cases be inherited insyd an autosomal dominant manner.[1][5] Exposure to brain anaa spinal tissue from an infected person sanso fi result in spread.[1] Der be no evidence say sporadic CJD fi spread among people via normal contact anaa blood transfusions,[1] although dis be possible insyd variant Creutzfeldt–Jakob disease.[6][7] Diagnosis dey involve ruling out oda potential causes.[1] An electroencephalogram, spinal tap, anaa magnetic resonance imaging fi support de diagnosis.[1] Anoda diagnosis technique be de real-time quaking-induced conversion assay, wich fi detect de disease insyd early stages.[8]

Der be no specific treatment give CJD.[1] Dem fi use opioids to help plus pain, while clonazepam anaa sodium valproate fi help plus involuntary movements.[1] CJD dey affect about one person per million people per year.[1] Onset be typically around 60 years of age.[1] Na dem first describe de condition insyd 1920.[1] E be classified as a type of transmissible spongiform encephalopathy.[9] Inherited CJD dey account for about 10% of prion disease cases.[5] Sporadic CJD be different from bovine spongiform encephalopathy (mad cow disease) den variant Creutzfeldt–Jakob disease (vCJD).[10]

References

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  1. 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 "Creutzfeldt–Jakob Disease Fact Sheet | National Institute of Neurological Disorders and Stroke". NINDS. March 2003. Archived from the original on 4 July 2017. Retrieved 16 July 2017.
  2. ↑ "Creutzfeldt–Jakob Disease, Classic (CJD)". CDC. 2 October 2018. Retrieved 21 November 2018.
  3. ↑ "Creutzfeldt-Jakob disease". www.whonamedit.com (in American English). Archived from the original on 2024-11-08. Retrieved 2025-11-23.
  4. ↑ "Creutzfeldt–Jakob Disease, Classic (CJD) | Prion Diseases | CDC". www.cdc.gov (in American English). 1 February 2019. Retrieved 17 June 2019. Classic CJD is a human prion disease
  5. 1 2 Manix, Marc; Kalakoti, Piyush; Henry, Miriam; Thakur, Jai; Menger, Richard; Guthikonda, Bharat; Nanda, Anil (2015-11-01). "Creutzfeldt–Jakob disease: updated diagnostic criteria, treatment algorithm, and the utility of brain biopsy". Neurosurgical Focus (in American English). 39 (5): E2. doi:10.3171/2015.8.FOCUS15328. ISSN 1092-0684. PMID 26646926.
  6. ↑ Brandel J, Vlaciu M, Culeux A, Belondrade M, Grznarova K, Plu I, Levasseur M, Haik S (July 2, 2020). "Variant Creutzfeldt–Jakob Disease Diagnosed 7.5 Years after Occupational Exposure". New England Journal of Medicine. 383 (1): 83–85. doi:10.1056/NEJMc2000687. PMID 32609989.
  7. ↑ "Transfusion Handbook/ 5.4: Variant Creutzfeldt–Jakob disease (vCJD)". Joint United Kingdom (UK) Blood Transfusion and Tissue Transplantation Services Professional Advisory Committee. 4 February 2014. Archived from the original on 2017-03-05. Retrieved 29 July 2021.
  8. ↑ Green, Alison J. E. (February 2019). "RT-QuIC: a new test for sporadic CJD". Practical Neurology. 19 (1): 49–55. doi:10.1136/practneurol-2018-001935. ISSN 1474-7766. PMC 6580883. PMID 30282760.
  9. ↑ "About CJD | Creutzfeldt–Jakob Disease, Classic (CJD) | Prion Disease". CDC. 11 February 2015. Archived from the original on 8 August 2017. Retrieved 16 July 2017.
  10. ↑ "Creutzfeldt–Jakob Disease, Classic (CJD) | Prion Diseases". CDC. 6 February 2015. Archived from the original on 18 July 2017. Retrieved 16 July 2017.
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