Neurológia pre prax 1/2024

Comorbid Creutzfeldt-Jakob disease as a diagnostic diffuculty: a case report of combination of four different neurodegenerations with advanced vascular changes

Creutzfeldt-Jakob disease (CJD) is an irreversibly fatal disease caused by a pathologically conformed prion protein. Although it can also occur at a younger age, it is found in a surprisingly high percentage in all age groups in comorbidity with other neuropathological entities. In many cases, the comorbidities can change the typical clinical symptomatology of patients and thus significantly complicate clinical diagnosis. We present the case report of a 75-year-old female patient, in whom definitive neuropathological examination revealed a combination of sporadic Creutzfeldt-Jakob disease, fully developed dementia with Lewy bodies, fully developed Alzheimer’s disease, frontotemporal lobar degeneration with predominance of features of age-related astrogliopathy, and relatively advanced vascular dementia. Although comorbidities of other neurodegenerative diseases are common in CJD, in this case it was a very unusual combination of multiple developed neurodegenerations.

Keywords: Creutzfeldt‑Jakob disease, PrP, prionoses, prion diseases, comorbid neurodegenerations