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Deficits of semantic cognition in semantic dementia and in aphasia consequent on CVA (stroke) are qualitatively different. Patients with semantic dementia are characterised by progressive degradation of central semantic representations, whereas multimodal semantic deficits in stroke aphasia reflect impairment of executive processes that help to direct and control semantic activation in a task-appropriate fashion [Jefferies, E., & Lambon Ralph, M. A. (2006). Semantic impairment in stroke aphasia vs. semantic dementia: A case-series comparison. Brain 129, 2132-2147]. We explored interactions between these two aspects of semantic cognition by examining the effects of cumulative phonemic cueing on picture naming in case series of these two types of patient. The stroke aphasic patients with multimodal semantic deficits cued very readily and demonstrated near-perfect name retrieval when cumulative phonemic cues reached or exceeded the target name's uniqueness point. Therefore, knowledge of the picture names was largely intact for the aphasic patients, but they were unable to retrieve this information without cues that helped to direct activation towards the target response. Equivalent phonemic cues engendered significant but much more limited benefit to the semantic dementia patients: their naming was still severely impaired even when most of the word had been provided. In contrast to the pattern in the stroke aphasia group, successful cueing was mainly confined to the more familiar un-named pictures. We propose that this limited cueing effect in semantic dementia follows from the fact that concepts deteriorate in a graded fashion [Rogers, T. T., Lambon Ralph, M. A., Garrard, P., Bozeat, S., McClelland, J. L., & Hodges, J. R., et al. (2004). The structure and deterioration of semantic memory: A neuropsychological and computational investigation. Psychological Review 111, 205-235]. For partially degraded items, the residual conceptual knowledge may be insufficient to drive speech production to completion but these items might reach threshold when they are bolstered by cues.

Studies of semantic dementia and PET neuroimaging investigations suggest that the anterior temporal lobes (ATL) are a critical substrate for semantic representation. In stark contrast, classical neurological models of comprehension do not include ATL, and likewise functional MRI studies often fail to show activations in the ATL, reinforcing the classical view. Using a novel application of low-frequency, repetitive transcranial magnetic stimulation (rTMS) over the ATL, we demonstrate that the behavioral pattern of semantic dementia can be mirrored in neurologically intact participants: Specifically, we show that temporary disruption to neural processing in the ATL produces a selective semantic impairment leading to significant slowing in both picture naming and word comprehension but not to other equally demanding, nonsemantic cognitive tasks.

Within the connectionist triangle model of reading aloud, interaction between semantic and phonological representations occurs for all words but is particularly important for correct pronunciation of lower frequency exception words. This framework therefore predicts that (a) semantic dementia, which compromises semantic knowledge, should be accompanied by surface dyslexia, a frequency-modulated deficit in exception word reading, and (b) there should be a significant relationship between the severity of semantic degradation and the severity of surface dyslexia. The authors evaluated these claims with reference to 100 observations of reading data from 51 cases of semantic dementia. Surface dyslexia was rampant, and a simple composite semantic measure accounted for half of the variance in low-frequency exception word reading. Although in 3 cases initial testing revealed a moderate semantic impairment but normal exception word reading, all of these became surface dyslexic as their semantic knowledge deteriorated further. The connectionist account attributes such cases to premorbid individual variation in semantic reliance for accurate exception word reading. These results provide a striking demonstration of the association between semantic dementia and surface dyslexia, a phenomenon that the authors have dubbed SD-squared., ((c) 2007 APA, all rights reserved.)

Patients with semantic dementia can show superior immediate recall of words that they still understand relatively well, as compared with more semantically degraded words, suggesting that conceptual knowledge makes a major contribution to phonological short-term memory. However, a number of studies have failed to show such a recall difference, challenging this view. We examined the effect of several methodological factors on the recall of known and degraded words in 4 patients with semantic dementia, in order to investigate possible reasons for this discrepancy. In general, our patients did exhibit poorer recall of the degraded words and made more phonological errors on these items. In addition, set size affected the magnitude of the recall advantage for known words. This finding suggests that semantic degradation influenced the rate of learning in the immediate recall task when the same items were presented repeatedly. The methods used to select known and degraded items also impacted on the recall difference. List length, however, did not affect the advantage for known words. The coherence of items in phonological short-term memory was affected by their semantic status, but not by the length of the material to be retained. The implications of these findings for the role of semantic and phonological representations in verbal short-term memory are discussed.

Several laboratory experiments assessing the ability of patients with semantic dementia to use familiar objects have revealed deficits corresponding directly to the patients' remaining conceptual knowledge for the same items. The same patients, however, were reported to demonstrate normal use of some objects relevant to their everyday lives. The study reported here was designed to explore this apparent discrepancy by examining the influence of personal familiarity with object exemplars, and of the contexts in which they are typically used. Two patients with severe semantic impairment were given single objects that they were still using at home on a regular basis and asked to demonstrate the use of each. Performance on these items was compared with use of perceptually similar and perceptually different exemplars of the same objects. All three sets were tested in the patients' own homes and also in the laboratory. Both patients were significantly more successful at using their own objects than the perceptually different exemplars, while an advantage for 'own' relative to similar exemplars characterized one of the two patients. Familiar home context had no impact on performance. The results suggest that repeated experience with personally familiar objects helps to maintain appropriate responses to them in the face of severely degraded conceptual knowledge.

Semantic dementia patients make numerous phoneme migration errors in their immediate serial recall of poorly comprehended words. In this study, similar errors were induced in the word recall of healthy participants by presenting unpredictable mixed lists of words and nonwords. This technique revealed that lexicality, word frequency, imageability, and the ratio of words to nonwords all influence the stability of the phonological trace. These factors affected phoneme migrations and phoneme identity errors for both the words themselves and the nonwords they were presented with. Therefore, lexical/semantic knowledge encourages the phonological segments of familiar words to emerge together in immediate serial recall. In the absence of such knowledge, the elements of a particular item are more likely to recombine with the phonemes of other list items. These findings demonstrate the importance of lexical and semantic binding in verbal short-term memory.

Language impairments caused by stroke (post-stroke aphasia, PSA) and neurodegeneration (primary progressive aphasia, PPA) have overlapping symptomatology, nomenclature and are classically divided into categorical subtypes. Surprisingly, PPA and PSA have rarely been directly compared in detail. Rather, previous studies have compared certain subtypes (e.g. semantic variants) or have focused on a specific cognitive/linguistic task (e.g. reading). This study assessed a large range of linguistic and cognitive tasks across the full spectra of PSA and PPA. We applied varimax-rotated principal component analysis to explore the underlying structure of the variance in the assessment scores. Similar phonological, semantic and fluency-related components were found for PSA and PPA. A combined principal component analysis across the two aetiologies revealed graded intra- and intergroup variations on all four extracted components. Classification analysis was used to test, formally, whether there were any categorical boundaries for any subtypes of PPA or PSA. Semantic dementia formed a true diagnostic category (i.e. within group homogeneity and distinct between-group differences), whereas there was considerable overlap and graded variations within and between other subtypes of PPA and PSA. These results suggest that (i) a multidimensional rather than categorical classification system may be a better conceptualization of aphasia from both causes; and (ii) despite the very different types of pathology, these broad classes of aphasia have considerable features in common.

The core clinical feature of semantic dementia is a progressive yet selective degradation of conceptual knowledge. Understanding the cognitive and neuroanatomical basis for this deficit is a key challenge for both clinical and basic science. Some researchers attribute the deficit to damage to pan-modal conceptual representations that are independent of any particular sensory–motor modality and are represented in the ventrolateral anterior temporal lobes. Others claim that damage to modality-specific visual feature representations in the occipitotemporal ‘ventral stream’ is responsible. In the present study, we tested the hypothesis that concept degradation in semantic dementia involves a combination of these pan-modal and modality-specific elements. We investigated factors influencing knowledge of object concepts by analysing 43 sets of picture-naming data from patients with semantic dementia. We found a strong influence of two pan-modal factors: highly familiar and typical items were named more accurately than less familiar/atypical items at all stages of the disorder. Items associated with rich sensory-motor information were also named more successfully at all stages, and this effect was present for sound/motion knowledge and tactile/action knowledge when these modalities were studied separately. However, there was no advantage for items rich in visual colour/form characteristics; instead, this factor had an increasingly negative impact in the later stages of the disorder. We propose that these results are best explained by a combination of (i) degradation of modality-independent conceptual representations, which is present throughout the disorder and is a consequence of atrophy focused on the ventrolateral anterior temporal lobes; and (ii) a later additional deficit for concepts that depend heavily on visual colour/form information, caused by the spreading of atrophy to posterior ventral temporal regions specialized for representing this information. This explanation is consistent with a graded hub-and-spoke model of conceptual knowledge, in which there is a gradual convergence of information along the temporal lobes, with visual attributes represented in the posterior cortex giving way to pan-modal representations in the anterior areas. [ABSTRACT FROM PUBLISHER]

Despite a vast literature examining semantic impairment in Alzheimer's disease (AD), consensus regarding the nature of the deficit remains elusive. We re-considered this issue in the context of a framework that assumes semantic cognition can break down in two ways: (1) core semantic representations can degrade or (2) cognitive control mechanisms can become impaired [1]. We hypothesised and confirmed that the nature of semantic impairment in AD changes with disease severity. Patients at mild or severe stages of the disorder exhibited impairment across various semantic tasks but the nature of those deficits differed qualitatively for the two groups. Commensurate with early dysfunction of the cognitive control, temporoparietal-frontal-cingulate network, characteristics of deregulated semantic cognition were exhibited by the mild AD cases. In contrast, the severe AD group reproduced features of additional degradation of core semantic representations. These results suggest that spread of pathology into lateral anterior temporal lobes in later stage AD produces degradation of semantic representations, exacerbating the already deregulated system. Moreover, the dual nature of severe patients' impairment was highlighted by disproportionately poor performance on tasks placing high demand on both conceptual knowledge and control processes - e.g., category fluency. [ABSTRACT FROM AUTHOR]

The most selective disorder of central conceptual knowledge arises in semantic dementia, a degenerative condition associated with bilateral atrophy of the inferior and polar regions of the temporal lobes. Likewise, semantic impairment in both herpes simplex virus encephalitis and Alzheimer's disease is typically associated with bilateral, anterior temporal pathology. These findings suggest that conceptual representations are supported via an interconnected, bilateral, anterior temporal network and that it may take damage to both sides to produce an unequivocal deficit of central semantic memory. We tested and supported this hypothesis by investigating a case series of 20 patients with unilateral temporal damage (following vascular accident or resection for tumour or epilepsy), utilizing a test battery that is sensitive to semantic impairment in semantic dementia. Only 1/20 of the cases, with a unilateral left lesion, exhibited even a mild impairment on the receptive semantic measures. On the expressive semantic tests of naming and fluency, average performance was worse in the left- than right-unilateral cases, but even in this domain, only one left-lesion case had scores consistently more than two standard deviations below control means. These results fit with recent parallel explorations of semantic function using repetitive transcranial magnetic stimulation as well as functional imaging in stroke aphasic and neurologically intact participants. The evidence suggests that both left and right anterior temporal lobe regions contribute to the representation of semantic memory and together may form a relatively damage-resistant, robust system for this critical aspect of higher cognition. [ABSTRACT FROM PUBLISHER]

Disorders of semantic cognition in different neuropsychological conditions result from diverse areas of brain damage and may have different underlying causes. This study used a comparative case-series design to examine the hypothesis that relatively circumscribed bilateral atrophy of the anterior temporal lobe in semantic dementia (SD) produces a gradual degradation of core semantic representations, whilst a deficit of cognitive control produces multi-modal semantic impairment in a subset of patients with stroke aphasia following damage involving the left prefrontal cortex or regions in and around the temporoparietal area; this condition, which transcends traditional aphasia classifications, is referred to as ‘semantic aphasia’ (SA). There have been very few direct comparisons of these patient groups to date and these previous studies have focussed on verbal comprehension. This study used a battery of object-use tasks to extend this line of enquiry into the non-verbal domain for the first time. A group of seven SA patients were identified who failed both word and picture versions of a semantic association task. These patients were compared with eight SD cases. Both groups showed significant deficits in object use but these impairments were qualitatively different. Item familiarity correlated with performance on object-use tasks for the SD group, consistent with the view that core semantic representations are degrading in this condition. In contrast, the SA participants were insensitive to the familiarity of the objects. Further, while the SD patients performed consistently across tasks that tapped different aspects of knowledge and object use for the same items, the performance of the SA participants reflected the control requirements of the tasks. Single object use was relatively preserved in SA but performance on complex mechanical puzzles was substantially impaired. Similarly, the SA patients were able to complete straightforward item matching tasks, such as word-picture matching, but performed more poorly on associative picture-matching tasks, even when the tests involved the same items. The two groups of patients also showed a different pattern of errors in object use. SA patients made substantial numbers of erroneous intrusions in their demonstrations, such as inappropriate object movements. In contrast, response omissions were more common in SD. This study provides converging evidence for qualitatively different impairments of semantic cognition in SD and SA, and uniquely demonstrates this pattern in a non-verbal expressive domain—object use. [ABSTRACT FROM PUBLISHER]

This study addresses continuing controversies concerning the nature of semantic impairment in early dementia of the Alzheimer type (DAT), and the relationship between conceptual knowledge and picture naming. A series of analyses of fine-grained feature knowledge data show that: (1) distinctive features of concepts were more vulnerable than shared; (2) the amount of attribute knowledge about a concept was associated reliably, and in a graded fashion, with the ability to name a picture of that item; (3) sensory features were differentially important in naming; and (4) the degree of disruption to different types of attribute knowledge did not vary between items from living and nonliving domains. These findings are discussed in the context of contemporary cognitive and computational models of semantic memory organisation. [ABSTRACT FROM AUTHOR]

Patients with semantic dementia, the temporal variant of frontotemporal dementia, are relevant to both the neuroanatomical and neuropsychological debates in the category-specific literature. These patients present with a selective and progressive semantic deficit consequent on circumscribed atrophy of the inferolateral polar temporal lobes bilaterally, including the inferotemporal gyrus. In this study, a patient KH with a significant advantage for artefacts over living things was compared to five other semantic dementia patients with commensurate levels of semantic impairment. KH demonstrated a consistent category difference in favour of artefacts across all the expressive and receptive semantic tests. This difference was reliable even when familiarity, frequency, and other potential confounding factors were controlled. While KH demonstrated an association between poor knowledge of sensory attributes and a consistently greater impairment on living things than artefacts, the other patients did not. As observed in a number of previous studies, all five of the patients, contrasted to KH, exhibited an advantage for functional/associative over sensory attributes but without demonstrating the category-specific deficit that the sensory-functional theory (and the locus of their atrophy) might predict. The results of this and other studies are discussed in relation to four accounts of category specificity: the sensory-functional theory, domain-specific knowledge systems, intercorrelated features, and individual differences. [ABSTRACT FROM AUTHOR]

Presents a study on patients with semantic dementia in cognitive neuropsychology. Drawings of concrete concepts made by semantic dementia patients from dictation of their names; Importance of semantic memory in humans; Use of feature analysis to evaluate the drawings made by the patients.

In our earlier article, we proposed that recent episodic experiences in patients with semantic dementia support the production of nongeneralisable, autobiographically constrained, "semantic-like" facts (Graham, Lambon Ralph, & Hodges, 1997). We argued that this type of "semantic-like" knowledge was distinguishable from true semantic information because our two patients with semantic dementia showed no facilitatory effect of recent autobiographical experiences on their knowledge of golf and bowls; information which was presumably learnt prior to the onset of their disease. In this paper, we discuss the implications of these results for current views relating to the nature and organisation of long-term memory. [ABSTRACT FROM AUTHOR]