Prion diseases are rare in children. Three types are known:
kuru,
variant Creutzfeldt-Jakob disease (CJD), and iatrogenic CJD. All three affect children and young adults, and are transmitted by infectious contamination.
Kuru was the result of ritual funeral practices similar to cannibalism; variant CJD affects young people who have eaten meat from cows with
mad cow disease (mostly in the UK); and iatrogenic CJD is secondary to graft of human tissues performed in the 1980s (dura mater, pituitary extracted
growth hormone). The disease appears after 4-30 years of incubation. The initial symptomatology is frequently neurological (
cerebellar ataxia, oculomotor disturbance, peripheral nerve
pain, pyramidal syndrome) followed by
dementia. There is no
biological test available that can give a definite diagnosis of
prion disease apart from neuropathology, although
prion accumulation in vCJD can be demonstrated in pharyngeal tonsil by immunohistochemical techniques. This devastating disease results inevitably in death. No specific treatment is available.