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  • Cited by 2
  • Print publication year: 2009
  • Online publication date: July 2009

18 - Semantic dementia

Summary

Introduction

Semantic dementia (SD; also known as progressive fluent aphasia) is regarded as a part of the spectrum of non-Alzheimer dementias that produce selective atrophy of the anterior temporal and/or orbitomedial frontal lobes; these conditions are referred to collectively as either the frontotemporal dementias (FTD) or frontotemporal lobar degeneration (FTLD) (Neary,1994; Neary et al., 1998). Although previously thought to be rare, FTD in fact has about the same prevalence as Alzheimer's disease (AD) below the age of 65 (Ratnavalli et al., 2002). Three clinical presentations of FTD are commonly described: a behavioral variant (bv-FTD), and two language variants, SD and progressive non-fluent aphasia (PNFA) (Hodges and Miller, 2001a,b). Since the mid 1990s, research on SD has produced a great deal of information about the clinical and neuropsychological features, progression, anatomy and neuropathology of the condition, which we attempt to review and synthesize here.

Early history

Although the term “semantic dementia” is recent (Snowden et al., 1989), the syndrome has been recognized under different labels for over a century. Between 1892 and 1904, Arnold Pick (1892, 1904) reported a series of remarkable cases characterized by progressive amnesic aphasia and changes in behavior; at autopsy these patients had marked atrophy of the left temporal lobe. Pick was perhaps the first neuroscientist to draw attention to the fact that progressive brain atrophy may lead to focal symptoms.

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