Wernicke (1900, as cited in G. H. Eggert, 1977) suggested that semantic knowledge arises from the interaction of perceptual representations of objects and words. The authors present a parallel distributed processing implementation of this theory, in which semantic representations emerge from mechanisms that acquire the mappings between visual representations of objects and their verbal descriptions. To test the theory, they trained the model to associate names, verbal descriptions, and visual representations of objects. When its inputs and outputs are constructed to capture aspects of structure apparent in attribute-norming experiments, the model provides an intuitive account of semantic task performance. The authors then used the model to understand the structure of impaired performance in patients with selective and progressive impairments of conceptual knowledge. Data from 4 well-known semantic tasks revealed consistent patterns that find a ready explanation in the model. The relationship between the model and related theories of semantic representation is discussed.

The clinical presentation of patients with semantic dementia is dominated by anomia and poor verbal comprehension. Although a number of researchers have argued that these patients have impaired comprehension of non-verbal as well as verbal stimuli, the evidence for semantic deterioration is mainly derived from tasks that include some form of verbal input or output. Few studies have investigated semantic impairment using entirely non-verbal assessments and the few exceptions have been based on results from single cases ([3]: Breedin SD, Saffran EM, Coslett HB. Reversal of the concreteness effect in a patient with semantic dementia. Cognitive Neuropsychology 1994;11:617-660, [12]: Graham KS, Becker JT, Patterson K, Hodges JR. Lost for words: a case of primary progressive aphasia? In: Parkin A, editor. Case studies in the neuropsychology of memory, East Sussex: Lawrence Erlbaum, 1997. pp. 83-110, [21]: Lambon Ralph MA, Howard D. Gogi aphasia or semantic dementia? Simulating and assessing poor verbal comprehension in a case of progressive fluent aphasia. Cognitive Neuropsychology, (in-press). This study employed sound recognition and semantic association tasks to investigate the nature of the verbal and non-verbal comprehension deficit in 10 patients with semantic dementia. The patients were impaired on both verbal and non-verbal conditions of the assessments, and their accuracy on these tasks was directly related to their scores on a range of other tests requiring access to semantic memory. Further analyses revealed that performance was graded by concept and sound familiarity and, in addition, identified significant item consistency across the different conditions of the tasks. These results support the notion that the patients' deficits across all modalities were due to degradation within a single, central network of conceptual knowledge. There were also reliable differences between conditions. The sound-picture matching task proved to be more sensitive to semantic impairment than the word-picture matching equivalent, and the patients performed significantly better on the picture than word version of a semantic association test. We propose that these differences arise directly from the nature of the mapping between input modality and semantic memory. Words and sounds have an arbitrary relationship with meaning while pictures benefit from a degree of systematicity with conceptual knowledge about the object.

Objectives-To investigate the prevalence of changes in mood, personality, and behaviour in frontotemporal dementia (FTD) and Alzheimer's disease (AD) and hence, which features reliably distinguish between them. To establish whether the frontal and temporal variants of FTD are characterised by diVerent behavioural changes. Methods-A questionnaire was designed to assess a wide range of neuropsychiatric changes; it incorporated features reported in previous studies of FTD and components of the neuropsychiatric inventory. Results-Factor analysis showed four robust and meaningful symptom clusters: factor 1-stereotypic and eating behaviour; factor 2-executive dysfunction and self care; factor 3-mood changes; factor 4-loss of social awareness. Only stereotypic and altered eating behaviour and loss of social awareness reliably diVerentiated AD from FTD with no eVect of disease severity. By contrast, executive dysfunction, poor self care, and restlessness showed a significant eVect of disease severity only, with the more impaired patients scoring more highly. Changes in mood were found to be equally prevalent in the three patient groups. Analysis of individual symptoms showed increased rates of mental rigidity and depression in the patients with semantic dementia compared with those with fv FTD. Conversely, the latter group showed greater disinhibition. Discriminant function analysis correctly classified 71.4% overall and 86.5% of the patients with AD. Conclusions-This questionnaire disclosed striking diVerences between patients with FTD and AD, but only stereotypic behaviour, changes in eating preference, disinhibition, and features of poor social awareness reliably separated the groups. The patients with fv FTD and semantic dementia were behaviourally very similar, reflecting the involvement of a common network, the ventral frontal lobe, temporal pole, and amygdala. Dysexecutive symptoms and poor self care were found to be aVected by the severity of the disease, reflecting perhaps spread to dorsolateral prefrontal areas relatively late in the course of both FTD and AD. This questionnaire may be of value in the diagnosis and the monitoring of therapies.

It has been reported that patients with semantic dementia function well in everyday life and sometimes show striking preservation of the ability to use objects, even those specific objects for which the patient has degraded conceptual information. To explore this phenomenon in nine cases of semantic dementia, we designed a set of semantic tests regarding 20 everyday objects and compared performance on these with the patients' ability to demonstrate the correct use of the same items. We also administered a test of mechanical problem solving utilizing novel tools, on which the patients had completely normal ability. All but the mildest affected patient showed significant deficits of naming and on the visually based semantic matching tasks. Object use was markedly impaired and, most importantly, correlated strongly with naming and semantic knowledge. In a small number of instances, there was appropriate use of an object for which the patient's knowledge on the semantic matching tasks was no better than chance; but this typically applied to objects with a rather obvious relationship between appearance and use, or was achieved by trial and error. The results suggest that object use is heavily dependent upon object-specific conceptual knowledge, supplemented to some degree by a combination of visual affordances and mechanical problem solving.

No abstract

The 8 patients involved in this study were impaired on tests assessing knowledge of objects and on the demonstration of their use. The patients' success in object use was significantly correlated with their knowledge about the objects, providing further evidence that conceptual knowledge plays a key role in object use. Having a recipient present improved performance in the moderately impaired patients, suggesting that a certain level of conceptual knowledge must remain for the additional information to be beneficial. Although overall accuracy in using the target objects was not related to our measures of affordance, the specific aspects of use afforded by the objects' structures were relatively impervious to semantic impairment, suggesting a role for affordance information when object-specific knowledge is disrupted. The patients' familiarity with the objects was an important predictor of performance. Finally, despite good performance on tests of mechanical problem solving, the patients showed very little evidence of employing these skills in their interactions with real objects.

In Study 1, six patients with semantic dementia were asked to produce drawings of concrete concepts from dictation of their names. The drawings were characterised by a loss of distinctive features. In the artefact domain, this feature loss resulted in representations that were increasingly box-like. In the living domain, as well as distinctive features being lost, there was a tendency for patients to include incorrect features that resulted in more familiar and "prototypical" representations.A second study included two further conditions in the drawing assessment: immediate and delayed copying of line drawings of concrete concepts. Analysis of the drawings produced by three patients with semantic dementia confirmed that overall performance was significantly influenced by the task condition (immediate>delayed) and severity of disease. The rate of intruding features, but not of omitted ones, was influenced by the domain of the item, with a greater proportion of intrusions in the living than in the nonliving domain. There was also a significant effect of feature distinctiveness on the proportions of these error types: Intruded features were most likely to come from the pool of properties that are shared across domain.

The aims of this study were (a) to explore the utility of, and make more widely available, an updated and extended version of the Cambridge Semantic Memory test battery, and (b) to use this battery in conjunction with other tests to characterise the profile of several different forms of progressive cognitive impairment: semantic dementia (SD, n = 15), mild cognitive impairment (MCI, n = 7), established Alzheimer's disease (AD) (n = 8), all in comparison to normal controls (n = 45). The semantic battery is useful in a variety of ways for exploring the nature of semantic deficits; on its own, however, it does not provide sensitive differentiation between patients with AD and SD. An assessment including measures of episodic memory and visuospatial abilities as well as the semantic battery is recommended for good characterisation of the cognitive profiles associated with SD and AD.