| Abstract [eng] |
Dementia is a syndrome which, due to the accelerating demographic ageing, is and will continue to be a major problem, affecting not only health but also other sectors. Dementia symptoms can be prevalent not only in the case of one of the most common diseases, Alzheimer's disease, but also in other rare causes that require greater medical attention to rare diseases. Rare causes of dementia can be divided into neurodegenerative, vascular, infectious, prion, exogenous and endogenous disease groups. Due to the multiplicity of conditions, only a small portion of these groups and possible mechanisms of dementia are described in this paper, with a focus on prion diseases. Prions are still an unsolved mystery in the scientific community even today, and the mechanisms underlying the development of the irregular prion protein in particular remain unclear. Several new possible mechanisms of prion conversion variants have been discussed, which are promising for the future of better understanding and knowledge of these protein structures, which, without any genetic material, are able to transfer conversion information to other prions. Creutzfeldt-Jakob disease is among the fastest-growing and most fatal conditions in the group of prion diseases and, due to its uniqueness, can pose many challenges for both specialist physicians and the patient as well as his or hers relatives. In this paper, three cases of Creutzfeldt-Jakob disease are discussed and their similarities and differences are summarised, differentiating them from other rare causes of dementia, including Hashimoto's encephalopathy, which is characterised by an elevated level of antibodies to thyroid peroxidase (ATPO), and which may mimic Creutzfeldt-Jakob disease due to its similar clinical features. The challenges posed by this disease have led to recommendations and suggestions for rare causes of dementia and, separately, for Creutzfeldt-Jakob disease, not only from a medical or research point of view, but also from an ethical, moral, social and legal perspective. |
