Cognitive Disorders Other than Alzheimer Disease

Abstract

Higher cognitive functions, such as abstract reasoning, complex decision making, and language, are the mental faculties that separate our species from other animals. When these faculties become impaired as a result of neurologic disease, striking and devastating behavioral changes result. Many neurologic diseases are associated with impaired cognition and behavior, and their etiologies are as varied as their clinical presentations. In this review, the focus is on dementia with Lewy bodies (DLB), the frontotemporal dementias (FTDs), and vascular cognitive impairment (VCI). The review covers the epidemiology and diagnosis of DBB, FTDs, and VCIs, as well as the etiology and genetics. Figures show neuroimaging in DLB, management of DLB, FTLD clinical syndromes, FTLD clinicopathologic correlations: approximate distribution of pathotypes for behavioral-variant FTD and primary progressive aphasia (PPA) variants, PPA-semantic subtype, PPA-logopenic subtype, post-stroke VCI, and subcortical ischemic vascular disease subtype of VCI. Tables list diagnostic criteria for DLB; FTD genetics: gene/protein relationship, clinical syndrome, salient gestures; VCI: clinical and pathologic features of the main subtypes; summary guidance based on VCI prevention studies; and summary guidance based on VCI treatment studies. This review contains 8 highly rendered figures, 5 tables, and 254 references.