An Overview of Primary Dementias as Clinicopathological Entities

Arash Salardini, MD


Semin Neurol. 2019;39(2):153-166. 

In This Article


Primary dementias are predominantly proteinopathies where misfolded proteins accumulate and lead to neuroinflammation, synaptic loss, cell death, and gliosis. The exception is vascular dementia, which accumulates vascular lesions. Each clinical-pathological entity is defined by the type of misfolded protein which causes it, and by its clinical presentation. Among the primary dementias, AD is the best characterized with good biomarkers for accurate diagnosis. Frontotemporal dementia clinical phenotypes, on the other hand, overlap and do not have the benefit of reliable biomarkers for confirmation. The diagnostic complexity for the rest of the conditions falls somewhere between these two extremes. A knowledge of primary dementias is the basis of the practice of dementia medicine.