Variant Creutzfeldt-Jakob disease: first two indigenous cases in Republic of Ireland. Case report and perspective.
The first two cases of indigenous variant Creutzfeldt-Jakob disease (vCJD) in the Republic of Ireland are reported in two men, neither of whom had lived outside Ireland. Both diagnoses were made ante-mortem based on clinical presentation, brain imaging, positive 14-3-3 protein in one case, and tonsillar biopsy. We discuss some of the clinical aspects of vCJD and the significance of these cases in the Irish context.
McHugh
J C
JC
Department of Neurology, St Vincent's University Hospital, Elm Park, Dublin, Ireland.
Bradley
D
D
Hutchinson
M
M
Brett
F
F
Heffernan
J
J
Howley
R
R
Farrell
M
M
Tubridy
N
N
D048948:14-3-3 Proteins / Q000134:cerebrospinal fluid; D000328:Adult; D001921:Brain / Q000473:pathology*; D007562:Creutzfeldt-Jakob Syndrome / Q000473:pathology*; D005838:Genotype; D006801:Humans; D007494:Ireland; D008279:Magnetic Resonance Imaging; D008297:Male; D000072002:Prion Proteins; D011328:Prions / Q000235:genetics
D048948:14-3-3 Proteins; C512656:PRNP protein, human; D000072002:Prion Proteins; D011328:Prions