Book contents
- Frontmatter
- Contents
- List of contributors
- Section 1 Introduction
- Section 2 Cognitive impairment, not demented
- Section 3 Slowly progressive dementias
- 18 Semantic dementia
- 19 Progressive non-fluent aphasia
- 20 Cognition in corticobasal degeneration and progressive supranuclear palsy
- 21 Cognitive and behavioral abnormalities of vascular dementia
- 22 CADASIL: a genetic model of arteriolar degeneration, white matter injury and dementia in later life
- Section 4 Rapidly progressive dementias
- Index
- References
18 - Semantic dementia
Published online by Cambridge University Press: 31 July 2009
Book contents
- Frontmatter
- Contents
- List of contributors
- Section 1 Introduction
- Section 2 Cognitive impairment, not demented
- Section 3 Slowly progressive dementias
- 18 Semantic dementia
- 19 Progressive non-fluent aphasia
- 20 Cognition in corticobasal degeneration and progressive supranuclear palsy
- 21 Cognitive and behavioral abnormalities of vascular dementia
- 22 CADASIL: a genetic model of arteriolar degeneration, white matter injury and dementia in later life
- Section 4 Rapidly progressive dementias
- Index
- References
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.
- Type
- Chapter
- Information
- The Behavioral Neurology of Dementia , pp. 264 - 278Publisher: Cambridge University PressPrint publication year: 2009
References
, , (1988). Progressive language impairment without dementia: a case with isolated category specific semantic defect. Journal of Neurology, Neurosurgery and Psychiatry 52:1201–1207.CrossRefGoogle Scholar
, , , (2002a). The influence of personal familiarity and context on object use in semantic dementia. Neurocase 8:127–134.CrossRefGoogle ScholarPubMed
, , , (2002b). When objects lose their meaning: what happens to their use?Cognitive, Affective and Behavioural Neuroscience 2:236–251.CrossRefGoogle Scholar
, , et al. (2003). A duck with four legs: investigating the structure of conceptual knowledge using picture drawing in semantic dementia. Cognitive Neuropsychology 20:27–47.CrossRefGoogle ScholarPubMed
, , (2004). Re-learning object use in semantic dementia. Neuropsychological Rehabilitation 14:351–363.CrossRefGoogle Scholar
, , (1997). The role of semantic memory in object use. Cognitive Neuropsychology 14:219–254.CrossRefGoogle Scholar
, , et al. (2004). Clinical and neuropathologic variation in neuronal intermediate filament inclusion disease. Neurology 63:1376–1384.CrossRefGoogle ScholarPubMed
, , et al. (2001). Patterns of temporal lobe atrophy in semantic dementia and Alzheimer's disease. Annals of Neurology 49:433–442.CrossRefGoogle ScholarPubMed
, , , , (2004). The human perirhinal cortex and semantic memory. European Journal of Neuroscience 20:2441–2446.CrossRefGoogle ScholarPubMed
, , et al. (2005). The pathological basis of semantic dementia. Brain 128:1985–1995.CrossRefGoogle ScholarPubMed
, , et al. (1997). The temporal variant of frontotemporal dementia. Brain 120:1027–1040.CrossRefGoogle ScholarPubMed
, , , (1995). Progressive prosopagnosia associated with selective right temporal lobe atrophy: a new syndrome?Brain 118:1–13.CrossRefGoogle ScholarPubMed
, , (2003). Slowly progressive defect in recognition of familiar people in a patient with right anterior temporal atrophy. Brain 126:792–803.CrossRefGoogle Scholar
, , et al. (2001). Differing patterns of temporal atrophy in Alzheimer's disease and semantic dementia. Neurology 57:216–225.CrossRefGoogle ScholarPubMed
, (2000). Semantic dementia: clinical, radiological and pathological perspectives. Journal of Neurology 247:409–422.CrossRefGoogle ScholarPubMed
, (1998). Tool use and mechanical problem solving in patients with apraxia. Neuropsychologia 36:581–589.CrossRefGoogle ScholarPubMed
, , (2004). Cognition and anatomy in three variants of primary progressive aphasia. Annals of Neurology 55:335–346.CrossRefGoogle ScholarPubMed
, (1997). Differentiating the roles of the hippocampal complex and the neocortex in long-term memory storage: evidence from the study of semantic dementia and Alzheimer's disease. Neuropsychology 11:77–89.CrossRefGoogle Scholar
, (1999). Episodic memory in semantic dementia: implications for the roles played by the perirhinal and hippocampal memory systems in new learning. Behavioural and Brain Sciences 22:452–453.CrossRefGoogle Scholar
, , (1995). Progressive pure anomia: insufficient activation of phonology by meaning. Neurocase 1:25–38.CrossRefGoogle Scholar
, , , (1999a). Relearning and subsequent forgetting of semantic category exemplars in a case of semantic dementia. Neuropsychology 13:359–380.CrossRefGoogle Scholar
, , (1999b). Episodic memory: new insights from the study of semantic dementia. Current Opinion in Neurobiology 9:245–250.CrossRefGoogle Scholar
, , , , (2000). Insights from semantic dementia on the relationship between episodic and semantic memory. Neuropsychologia 38:313–324.CrossRefGoogle ScholarPubMed
, , , , (2002). Multiple inputs to episodic memory in semantic dementia: words tell another story. Neuropsychology 16:380–389.CrossRefGoogle ScholarPubMed
, , (1995). Autobiographical memory and executive function in early dementia of Alzheimer type. Neuropsychologia 33:1647–1670.CrossRefGoogle ScholarPubMed
, (1998). A reversal of the temporal gradient for famous person knowledge in semantic dementia: implications for the neural organisation of long-term memory. Neuropsychologia 36:803–825.CrossRefGoogle ScholarPubMed
, (2001). Episodic memory: insights from semantic dementia. Philosophical Transactions of the Royal Society of London Series B: Biological Sciences 356:1423–1434.Google ScholarPubMed
, (2001a). The neuropsychology of frontal variant FTD and semantic dementia. Introduction to the special topic papers: Part II. Neurocase 7:113–121.CrossRefGoogle ScholarPubMed
, (2001b). The classification, genetics and neuropathology of frontotemporal dementia (FTD). Introduction to the special topic papers: Part I. Neurocase 7:31–35.Google Scholar
, (1995). Is semantic memory consistently impaired early in the course of Alzheimer's disease? Neuroanatomical and diagnostic implications. Neuropsychologia 33:441–459.CrossRefGoogle ScholarPubMed
, (1996). Non-fluent progressive aphasia and semantic dementia: a comparative neuropsychological study. Journal of the International Neuropsychological Society 2:511–524.CrossRefGoogle Scholar
, , , (1992). Semantic dementia: progressive fluent aphasia with temporal lobe atrophy. Brain 115:1783–1806.CrossRefGoogle ScholarPubMed
, , , (1996). Naming and knowing in dementia of Alzheimer's type. Brain and Language 54:302–325.CrossRefGoogle ScholarPubMed
, , et al. (1999a). The differentiation of semantic dementia and frontal lobe dementia (temporal and frontal variants of frontotemporal dementia) from early Alzheimer's disease: a comparative neuropsychological study. Neuropsychology 13:31–40.CrossRefGoogle ScholarPubMed
, , (1999b). What and how: evidence for the dissociation of object knowledge and mechanical problem solving skills in the human brain. Proceedings of the National Academy of Sciences USA 96:9444–9448.CrossRefGoogle ScholarPubMed
, , , , (2000). The role of conceptual knowledge in object use: evidence from semantic dementia. Brain 123:1913–1925.CrossRefGoogle ScholarPubMed
, , , , (2003). Survival in frontotemporal dementia. Neurology 61:349–354.CrossRefGoogle ScholarPubMed
, , et al. (2004). Clinicopathological correlates in frontotemporal dementia. Annals of Neurology 56:399–406.CrossRefGoogle ScholarPubMed
, (1992). Pyramids and Palm Trees: A Test of Semantic Access From Pictures and Words. Bury St Edmunds, UK: Thames Valley Test Company.Google Scholar
, , , , (2006). “A horse of a different colour”: do patients with semantic dementia recognise different versions of the same object as the same?Neuropsychologia 44:566–575.CrossRefGoogle ScholarPubMed
, , et al. (1998). MR volumetric analysis of the human entorhinal, perirhinal, and temporopolar cortices. American Journal of Neuroradiology 19:659–671.Google ScholarPubMed
, (1999). Impaired knowledge of famous people and events and intact autobiographical knowledge in a case of progressive right temporal lobe degeneration: implications for the organization of remote memory. Cognitive Neuropsychology 16:589–607.CrossRefGoogle Scholar
, , et al. (2005). Antemortem diagnosis of frontotemporal lobar degeneration. Annals of Neurology 57:480–488.CrossRefGoogle ScholarPubMed
, (2000). Gogi aphasia or semantic dementia? Simulating and assessing poor verbal comprehension in a case of progressive fluent aphasia. Cognitive Neuropsychology 17:437–465.CrossRefGoogle Scholar
, , , (1999). Is a picture worth a thousand words? Evidence from concept definitions by patients with semantic dementia. Brain and Language 70:309–335.CrossRefGoogle Scholar
, , (2000). Classical anomia: a neuropsychological perspective on speech production. Neuropsychologia 38:186–202.CrossRefGoogle ScholarPubMed
, , , , (2001). No right to speak? The relationship between object naming and semantic impairment: neuropsychological evidence and a computational model. Journal of Cognitive Neuroscience 13:341–356.CrossRefGoogle Scholar
, , (1997). Modality-specific operations in semantic dementia. Cortex 33:593–622.CrossRefGoogle ScholarPubMed
, , (1995). Why there are complementary learning systems in the hippocampus and neocortex: insights from the successes and failures of connectionist models of learning and memory. Psychological Review 102:419–457.CrossRefGoogle ScholarPubMed
, , et al. (2001). Clinical and pathological diagnosis of frontotemporal dementia: report of the Working Group on Frontotemporal Dementia and Pick's Disease. Archives of Neurology 58:1803–1809.CrossRefGoogle Scholar
, , , (1986). Primary dysphasic dementia: clinical, pathological, and biochemical studies. Annals of Neurology 20:126.Google Scholar
(1982). Slowly progressive aphasia without generalised dementia. Annals of Neurology 11:592–598.CrossRefGoogle Scholar
(1913). On aphasia due to atrophy of the cerebral convolutions. Brain 36:493–524.CrossRefGoogle Scholar
, , et al. (2005). Neuropathologic, biochemical, and molecular characterization of the frontotemporal dementias. Journal of Neuropathology and Experimental Neurology 64:420–428.CrossRefGoogle ScholarPubMed
, , et al. (1999). Disrupted temporal lobe connections in semantic dementia. Brain 122:61–73.CrossRefGoogle ScholarPubMed
(1994). Neuropsychological correlates of frontotemporal cerebral atrophy. Japanese Journal of Neuropsychology 10:11–17.Google Scholar
, , et al. (1998). Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology 51:1546–1554.CrossRefGoogle ScholarPubMed
, , , , (2002). Memory consolidation and the hippocampus: further evidence from the study of autobiographical memory in semantic dementia and the frontal variant of frontotemporal dementia. Neuropsychologia 40:633–654.CrossRefGoogle Scholar
, (2006). Sweet nothings: narrative speech in semantic dementia. In (ed.) From Inkmarks to Ideas: Challenges and Controversies about Word Recognition and Reading. Hove, UK: Psychology Press, pp. 299–317.Google Scholar
, , et al. (2006). “Pre-semantic” cognition in semantic dementia: six deficits in search of an explanation. Journal of Cognitive Neuroscience 18:169–183.CrossRefGoogle Scholar
, , , (2008). Varieties of silence: the impact of neurodegenerative diseases on language systems in the brain. In (ed.) Topics in Integrated Neuroscience: From Cells to Cognition. Cambridge, UK: Cambridge University Press, pp. 181–205.CrossRefGoogle Scholar
, , et al. (2001). Hemispheric dominance for emotions, empathy and social behaviour: evidence from right and left-handers with frontotemporal dementia. Neurocase 7:145–160.CrossRefGoogle ScholarPubMed
(1892). Uber die Beziehungen der senilen Hirnatrophie zur Aphasie. Prager Medische Wochenschrift 17:165–167.Google Scholar
(1904). Zur symptomatologie der linksseitigen Schlafenlappenatrophie. Monatschrift für Psychiatrie und Neurologie 16:378–388.CrossRefGoogle Scholar
, , et al. (2003). Autobiographical memory and autonoetic consciousness: triple dissociation in neurodegenerative diseases. Brain 126:2203–2219.CrossRefGoogle ScholarPubMed
, (1988). Slowly progressive aphasia in three patients: the problem of accompanying neuropsychological deficit. Brain 111:151–168.CrossRefGoogle ScholarPubMed
, , , (2002). The prevalence of frontotemporal dementia. Neurology 58:1615–1621.CrossRefGoogle ScholarPubMed
, , , (2003). Object recognition under semantic impairment: the effects of conceptual regularities on perceptual decisons. Language and Cognitive Processes 18:625–662.CrossRefGoogle Scholar
, , et al. (2004a). The structure and deterioration of semantic memory: a neuropsychological and computational investigation. Psychological Review 111:205–235.CrossRefGoogle ScholarPubMed
, , , (2004b). Natural selection: the impact of semantic impairment on lexical and object decision. Cognitive Neuropsychology 21:331–352.CrossRefGoogle ScholarPubMed
, , , (2006). Semantic memory in Alzheimer's disease and the fronto-temporal dementias: a longitudinal study of 236 patients. Neuropsychology 20:319–335.CrossRefGoogle Scholar
, , et al. (2002). Patterns of brain atrophy in frontotemporal dementia and semantic dementia. Neurology 58:198–208.CrossRefGoogle ScholarPubMed
(1909). Die partielle Gorsshirnatrophie. Journal für Psychologie und Neurologie 14:115–130.Google Scholar
, , (1991). A cognitive neuropsychological model of limb praxis. Cognitive Neuropsychology 8:443–458.CrossRefGoogle Scholar
(1927). Uber Picksche Krankheit. Monatschrift für Psychologie und Neurologie 65:230–275.CrossRefGoogle Scholar
, , et al. (2005). The natural history of temporal variant frontotemporal dementia. Neurology 64:1384–1390.CrossRefGoogle ScholarPubMed
, , , , (2001). Semantic knowledge and episodic memory for faces in semantic dementia. Neuropsychology 15:101–114.CrossRefGoogle ScholarPubMed
, , (2002). Perceptual and semantic contributions to episodic memory: evidence from semantic dementia and Alzheimer's disease. Journal of Memory and Language 47:197–213.CrossRefGoogle Scholar
, , (1989). Semantic dementia: a form of circumscribed cerebral atrophy. Behavioural Neurology 2:167–182.Google Scholar
, , (1994). Semantic dementia: autobiographical contribution to preservation of meaning. Cognitive Neuropsychology 11:265–288.CrossRefGoogle Scholar
, , (1995). Autobiographical experience and word meaning. Memory 3:225–246.CrossRefGoogle ScholarPubMed
(1926). Uber die Picksche atrophie. Aeitschrift für die Gesamte Neurologie und Psychiatrie 101:729–747.CrossRefGoogle Scholar
, , , , (2002). Is knowledge of famous people disproportionately impaired in patients with early Alzheimer's disease?Neuropsychology 16:344–358.CrossRefGoogle Scholar
, , (2003). Left/right asymmetry of atrophy in semantic dementia: behavioural cognitive implications. Neurology 61:1196–1203.CrossRefGoogle ScholarPubMed
(1995). Organization of memory: quo vadis. In (ed.) The Cognitive NeurosciencesCambridge, MA: MIT Press, pp. 839–847.Google Scholar
(1975). Selective impairment of semantic memory. Quarterly Journal of Experimental Psychology 27:635–657.CrossRefGoogle ScholarPubMed
, , , (2004). The contribution of autobiographical significance to semantic memory: evidence from Alzheimer's disease, semantic dementia, and amnesia. Neuropsychologia 42:25–48.CrossRefGoogle ScholarPubMed
, , (2005). The neural correlates of semantic and behavioural deficits in frontotemporal dementia. NeuroImage 24:1042–1051.CrossRefGoogle ScholarPubMed
- 2
- Cited by