Open AccessCreutzfeldt-Jakob Disease Phenotype and Course: Our Experience from a Tertiary Center
Author(s) -
Sadanandavalli Retnaswami Chandra,
Thomas Gregor Issac,
Mariamma Philip,
Veeranna Gadad
Publication year - 2016
Publication title -
indian journal of psychological medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.417
H-Index - 24
eISSN - 0975-1564
pISSN - 0253-7176
DOI - 10.4103/0253-7176.191376
Subject(s) - transmissible spongiform encephalopathy , medicine , myoclonus , dementia , pediatrics , disease , creutzfeldt jakob syndrome , scrapie , pathology , psychiatry , prion protein
Prion diseases are protein conformation disorders and neither caused by viroid or virus but is a transmissible particle labeled a prion by Pruisner. Normal prion protein becomes infectious by a different folding, but the triggers are not known. Based on the characteristic brain pathology, they are grouped under spongiform encephalopathy affecting both man and animals. Estimated prevalence is one per million. Creutzfeldt-Jakob disease (CJD) registry from National Institute and Neurosciences (NIMHANS), Bengaluru, reported 69 cases in 30 years.