2024/11/14 by Serejo Portugal, Rita, F.Silva, Maria Helena, Matos Gonçalves, André +3
#14-3-3 Proteins #Acitretin #Creutzfeldt-Jakob Syndrome/blood #Creutzfeldt-Jakob Syndrome/diagnosis #Creutzfeldt-Jakob Syndrome/diagnostic imaging
paper · doi:10.60591/crspmi.249
Creuztfeldt-Jakob disease (CJD) is a rare and fatal prion neurogenerative disease that affects humans and other mammals, with a global prevalence of 1: million habitants. It occurs mainly between the ages of 50 and 70 and is characterized by rapidly progressive dementia. It is divided into 3 main forms: sporadic, genetic, and acquired. The definitive diagnosis is made through anatomopathological examination of the brain, although a highly probable diagnosis can be made through clinical criteria and complementary diagnostic tests. We present three clinical cases of CJD diagnosed in a Hospital Unit. The average age was 61 years old, and all presented initial behavioral symptoms. The mortality rate was 100% with anatomopathological examination, which confirmed the diagnosis of sporadic Creutzfeldt-Jakob disease. Rapidly progressive dementia with atypical signs/symptoms should be investigated. Although rare, the sporadic form has been increasing, meaning greater attention and recognition are needed.