Sporadic Creutzfeldt Jakob disease in two adolescents

Abstract

The cases in this report indicate that sporadic CJD can develop at a very young age, that variant CJD is not the only form of CJD occurring in this age group and that neuropathological examination is essential for accurate diagnosis of human prion disease.

Sporadic Fatal Insomnia in an Adolescent

“…[17][18][19][20][21][22] Deaths from sporadic prion disease in those aged ,20 years are extremely rare, with only 7 cases of sporadic CreutzfeldtJakob disease previously recorded. 23 In addition to his age, the patient' s multiple concussions were diagnostically deceptive. Although the early clinical signs could overlap with those found in postconcussive syndrome, their progressive worsening and development of additional neurologic signs led to considerations of alternate diagnoses later in the disease course.…”

Kuru prions and sporadic Creutzfeldt–Jakob disease prions have equivalent transmission properties in transgenic and wild-type mice

“…Although the genes responsible for mouse incubation time quantitative trait loci have not yet been identified, orthologous human genes are likely to be globally polymorphic with significant differences within and between Europe and the Fore. Furthermore, age is an important determinant of survival in sporadic and inherited prion diseases (68,69) and youth may be associated with an atypical sporadic CJD neuropathology (70). The marked difference in mean age of kuru and sporadic CJD patients might thus account for some of the neuropathological differences that distinguish kuru from the majority of sporadic CJD cases.…”

Bovine spongiform encephalopathy

“…22,[114][115][116] The newly recognized vCJD was distinguishable from sporadic CJD, even in the rare instance when sporadic CJD occurred in an adolescent. 117 Analysis of the prion isoform deposits in vCJD patients revealed the same biochemical properties (eg, electrophoretic banding patterns and antibody staining affinities) as those associated with prion deposits in BSE prion-infected cattle; the properties were distinct from those of prions associated with any other known spongiform encephalopathy, including prions in patients with sporadic CJD. 118,119 Inoculation of nonhuman primates with brain material from vCJD patients induced a disease indistinguishable from that caused by inoculation with brain material from cattle with clinically evident BSE.…”