Variant creutzfeldt-jakob disease with extremely low lymphoreticular deposition of prion protein

JAMA Neurology

Volumn 71, Issue 3, 2014, Pages 340-343

  Mead, Simon ^a,b   Wadsworth, Jonathan D F ^b   Porter, Marie Claire ^a   Linehan, Jacqueline M ^b   Pietkiewicz, Wojciech ^c   Jackson, Graham S ^b   Brandner, Sebastian ^b   Collinge, John ^a,b  

^a NATIONAL HOSPITAL FOR NEUROLOGY AND NEUROSURGERY   (United Kingdom)

^b UNIVERSITY COLLEGE LONDON   (United Kingdom)

^c THE WALTON CENTRE NHS FOUNDATION TRUST   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

ANTIDEPRESSANT AGENT; DOXYCYCLINE; PRION PROTEIN;

ADULT; ARTICLE; AUTOPSY; BIOPSY; BLOOD ANALYSIS; CASE REPORT; CEREBROSPINAL FLUID ANALYSIS; CHOREA; CLINICAL FEATURE; DEATH; DEPRESSION; DETERIORATION; DISEASE ASSOCIATION; DISEASE COURSE; HUMAN; IMMUNOHISTOCHEMISTRY; MALE; MEDICAL HISTORY; MINI MENTAL STATE EXAMINATION; NEUROLOGIC EXAMINATION; NUCLEAR MAGNETIC RESONANCE IMAGING; PRIORITY JOURNAL; PROTEIN LOCALIZATION; RETICULOENDOTHELIAL SYSTEM; TONSIL BIOPSY; UNEMPLOYMENT; UNITED KINGDOM; VARIANT CREUTZFELDT JAKOB DISEASE; VARIANT CREUTZFELDT JAKOB DISEASE AGENT;

ADULT; CREUTZFELDT-JAKOB SYNDROME; FATAL OUTCOME; GREAT BRITAIN; HUMANS; LYMPHOID TISSUE; MALE; PALATINE TONSIL; PRIONS;

EID: 84896773002     PISSN: 21686149     EISSN: None     Source Type: Journal    
DOI: 10.1001/jamaneurol.2013.5378     Document Type: Article

References (19)

1. Collinge J. Molecular neurology of prion disease. J Neurol Neurosurg Psychiatry. 2005; 76(7): 906-919.
2. Collinge J. Variant Creutzfeldt-Jakob disease. Lancet. 1999; 354(9175): 317-323.
3. Collinge J, Whitfield J, McKintosh E, et al. Kuru in the 21st century: an acquired human prion disease with very long incubation periods. Lancet. 2006; 367(9528): 2068-2074.
4. Hill AF, Zeidler M, Ironside J, Collinge J. Diagnosis of new variant Creutzfeldt-Jakob disease by tonsil biopsy. Lancet. 1997; 349(9045): 99-100.
5. Hill AF, Butterworth RJ, Joiner S, et al. Investigation of variant Creutzfeldt-Jakob disease and other human prion diseases with tonsil biopsy samples. Lancet. 1999; 353(9148): 183-189.
6. Wadsworth JD, Joiner S, Hill AF, et al. Tissue distribution of protease resistant prion protein in variant Creutzfeldt-Jakob disease using a highly sensitive immunoblotting assay. Lancet. 2001; 358(9277): 171-180.
7. Wroe SJ, Pal S, Siddique D, et al. Clinical presentation and pre-mortem diagnosis of variant Creutzfeldt-Jakob disease associated with blood transfusion: a case report. Lancet. 2006; 368(9552): 2061-2067.
8. Gill ON, Spencer Y, Richard-Loendt A, et al. Prevalent abnormal prion protein in human appendixes after bovine spongiform encephalopathy epizootic: large scale survey [published online October 15, 2013]. BMJ. doi:10.1136/bmj.f5675.
9. Hill AF, Joiner S, Linehan J, Desbruslais M, Lantos PL, Collinge J. Species-barrier-independent prion replication in apparently resistant species. Proc Natl Acad Sci USA. 2000; 97(18): 10248-10253.
10. Edgeworth JA, Farmer M, Sicilia A, et al. Detection of prion infection in variant Creutzfeldt-Jakob disease: a blood-based assay. Lancet. 2011; 377(9764): 487-493.
11. Trevitt CR, Collinge J. A systematic review of prion therapeutics in experimental models. Brain. 2006; 129(pt 9): 2241-2265.
12. Collinge J, Sidle KC, Meads J, Ironside J, Hill AF. Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD. Nature. 1996; 383(6602): 685-690.
13. Frosh A, Smith LC, Jackson CJ, et al. Analysis of 2000 consecutive UK tonsillectomy specimens for disease-related prion protein. Lancet. 2004; 364(9441): 1260-1262.
14. Clewley JP, Kelly CM, Andrews N, et al. Prevalence of disease related prion protein in anonymous tonsil specimens in Britain: cross sectional opportunistic survey. BMJ. 2009; 338: b1442.
15. Hilton DA, Ghani AC, Conyers L, et al. Prevalence of lymphoreticular prion protein accumulation in UK tissue samples. J Pathol. 2004; 203(3): 733-739.
16. González L, Dagleish MP, Bellworthy SJ, et al. Postmortem diagnosis of preclinical and clinical scrapie in sheep by the detection of disease-associated PrP in their rectal mucosa. Vet Rec. 2006; 158(10): 325-331. 16531580
17. McCutcheon S, Alejo Blanco AR, Houston EF, et al. All clinically-relevant blood components transmit prion disease following a single blood transfusion: a sheep model of vCJD. PLoS One. 2011; 6(8): e23169.
18. Mathiason CK, Powers JG, Dahmes SJ, et al. Infectious prions in the saliva and blood of deer with chronic wasting disease. Science. 2006; 314(5796): 133-136.
19. Bishop MT, Diack AB, Ritchie DL, Ironside JW, Will RG, Manson JC. Prion infectivity in the spleen of a PRNP heterozygous individual with subclinical variant Creutzfeldt-Jakob disease. Brain. 2013; 136(pt 4): 1139-1145.