Journal
PEDIATRICS
Volume 133, Issue 3, Pages E766-E770Publisher
AMER ACAD PEDIATRICS
DOI: 10.1542/peds.2013-1396
Keywords
adolescent; Creutzfeldt-Jakob disease; prion disease; sporadic fatal insomnia; transmissible spongiform encephalopathy
Categories
Funding
- Centers for Disease Control and Prevention [UR8/CCU515004]
- Charles S. Britton Fund
- National Institutes of Health [P01 AG-14359]
- National Institutes of Health (NIH)
Ask authors/readers for more resources
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.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available