New forms of dementia in neurodegenerative diseases: molecular basis, phenomenology, and diagnostic capability

Abstract

In the light of the development of molecular biology and pathomorphology, ideas about degenerative diseases of the nervous system are gradually changing. A clear evidence of this is the description in recent years of new forms of neurodegenerative pathology, manifested by cognitive decline and dementia. These forms include Limbic-Predominant Age-Related TDP-43 Encephalopathy (LATE); dementia associated with Quadruple Misfolded Proteins (QMP), Neuronal Intranuclear Inclusion Disease (NIID). Their appearance in a wide range of neurodegenerative diseases, characterized at the molecular and phenotypic level, raises several questions — from the introduction of new complex terms into the Russian-language scientific literature to the formation of new knowledge among specialists to identify these conditions in practice. The review examines in detail the spectrum of clinical manifestations and genetic characteristics of new forms of neurodegenerative dementia, as well as modern possibilities of their diagnosis.