Sporadic fatal insomnia in an adolescent.
The occurrence of sporadic prion disease among adolescents is extremely rare. A prion disease was confirmed in an adolescent with disease onset at 13 years of age. Genetic, neuropathologic, and biochemical analyses of the patient's autopsy brain tissue were consistent with sporadic fatal insomnia, a type of sporadic prion disease. There was no evidence of an environmental source of infection, and this patient represents the youngest documented case of sporadic prion disease. Although rare, a prion disease diagnosis should not be discounted in adolescents exhibiting neurologic signs. Brain tissue testing is necessary for disease confirmation and is particularly beneficial in cases with an unusual clinical presentation.
Division of High-Consequence Pathogens and Pathology, National Center for Emerging and Zoonotic Infectious Diseases, Centers for Disease Control and Prevention, Atlanta, Georgia; and.
Jennifer L
JL
Blase
Laura
L
Cracco
Lawrence B
LB
Schonberger
Ryan A
RA
Maddox
Yvonne
Y
Cohen
Ignazio
I
Cali
Ermias D
ED
Belay
D000293:Adolescent; D001921:Brain / Q000473:pathology*; D017809:Fatal Outcome; D006801:Humans; D034062:Insomnia, Fatal Familial / Q000150:complications / Q000175:diagnosis*; D008297:Male
Creutzfeldt-Jakob disease; adolescent; prion disease; sporadic fatal insomnia; transmissible spongiform encephalopathy