Aim:
Creutzfeldt – Jakob disease (CJD) is a transmissible spongiform encephalopathy that manifests as a rapidly progressive dementia. In this article, we described two clinical cases of CJD observed in St. Panteleimon Hospital during 2023 year.

Material and methods:
Diagnosis of this prion disease remains a challenge because of the large variability of the clinical symptoms especially in its early stages of disease. The Centers for Disease Control and Prevention (CDC) listed recognizable clinical features and paraclinical tests to supplement the replicable diagnostic criteria in vivo. There are three main clinical subtypes of CJD: Heidenhain, Brownell-Oppenheimer, Stern.

Case report:
A 68 y.o. woman delivered to the emergency department with severe speech impairment in a somnolent state - 13 points Glasgow Coma Scale. Her relatives described a clinical manifestation: rapidly progressive dementia, visual disturbances, abnormal gait and coordination, retrograde amnesia. A 67 y. o. man delivered to the emergency department with headache, vertigo, abnormal gait and coordination, progressive extremities weakness, disorientation, memory and concentration impairment. His relatives told about extremely rapidly progression of symptoms during last three months. First clinical case presents a Heidenhain variant, second case – Brownell - Oppenheimer variant.

Summary:
Described clinical cases correspond to the criteria of possible CJD according to CDC’s Diagnostic Criteria for Creutzfeldt - Jakob Disease (2018). The most recommended approaches to in vivo diagnosis of CJD are strict application of diagnostic criteria, careful interpretation of neurovisualization, EEG.