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Chapter 4 - Rare and Unusual Dementias

from Section 1 - Epidemiology and Types of Disorders

Published online by Cambridge University Press:  12 September 2020

Julian C. Hughes
Affiliation:
University of Bristol
Philippa Lilford
Affiliation:
Severn Deanery, University of Bristol
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Summary

Over 95% of cases of dementia are attributable to Alzheimer’s disease (AD), vascular dementia (VaD), dementia Lewy body (DLB), and frontotemporal dementia (FTD), as well as mixed Alzheimer’s and VaD, and so on. In this chapter we consider some of the rare and unusual causes that account for the remaining 5%. Categorizing them according to aetiological group (degenerative, vascular, and infectious causes, including human prion diseases), we discuss the presentation of these forms and reasons for variations in estimated prevalence rates in the general population. We shall then go on to describe toxic, iatrogenic, nutritional, traumatic, metabolic, neoplastic, and autoimmune causes of dementia. Disorders are graded according to their prevalence, to give an idea of the likelihood of their presentation. Guidance is given on the investigation of uncommon cognitive impairment and dementia. We have tried to avoid repeating information available in the Chapter 3 on the causes of young-onset dementias, but inevitably there is some overlap of material.

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Publisher: Cambridge University Press
Print publication year: 2020

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