title: Young-onset CJD: age and disease phenotype in variant and sporadic forms.
abstract: Since 1996, there have been over 140 reports of a Creutzfeldt-Jakob disease variant (vCJD), with a striking prevalence of these cases in the UK. The main peculiarity of vCJD is its onset in young people, but other features also distinguish it from sporadic CJD. Despite epidemiological data suggesting a link with bovine spongiform encephalopathy, the origin of vCJD is not completely understood. We hypothesized that the onset of vCJD at a young age might contribute to the disease phenotype. We searched the Medline/PubMed database for all case reports of CJD in the under-30s and selected 20 sporadic CJD patients with a median age at onset of 25.5 years. Our series displays a long disease duration and other vCJD-like features, suggesting that CJD in young people is different from classic CJD and that the vCJD phenotype may be partly related to young age.
authors: Laboratory of Experimental Neurobiology, IRCCS C. Mondino Institute of Neurology Foundation, Pavia, Italy.
authors: Manuel
authors: M
authors: Corato
authors: Cristina
authors: C
authors: Cereda
authors: Emanuela
authors: E
authors: Cova
authors: Carlo
authors: C
authors: Ferrarese
authors: Mauro
authors: M
authors: Ceroni
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000375:Aging / Q000502:physiology; D007562:Creutzfeldt-Jakob Syndrome / Q000235:genetics / Q000503:physiopathology*; D004569:Electroencephalography; D005260:Female; D005838:Genotype; D006801:Humans; D008297:Male; D010641:Phenotype; D058225:Plaque, Amyloid / Q000473:pathology; D011328:Prions / Q000235:genetics; D013534:Survival; D016019:Survival Analysis
chemical_list: D011328:Prions