Category-specific deficits in semantic dementia: links between perception and semantic knowledge

The nature of knowledge and its relationship with the perceptual processes are among the most central issues in the study of human cognition. Should knowledge be abstract, then semantic memory and perception should be relatively independent. On the contrary, if knowledge is sensory-dependent, then memory and perception should be very close. The first view is supported by the multiple system approach of memory, whereas the second view is supported by the single-store memory theories. One way to study these links is through the category-specific impairment and the sensory-functional theory (SFT). Category-specific impairment is generally observed for living items compared to artefacts. The SFT explains this deficit by defining living items as essentially based on perception. In the abstract view of knowledge, a living deficit should be related to a deficit in processing sensory knowledge. On the opposite, the sensory-dependent view states that this deficit results from perception impairment. This article focuses on the relations between knowledge and perception in semantic dementia (SD). SD is characterized by a progressive loss of semantic knowledge, making it particularly interesting to study. This article first focuses on the SFT, to explain the category-specific impairment. The issue of perceptual processing in SD is then reviewed from the lowest level (senses) to the highest level of perception (multimodal integration). The data demonstrated normal perception for these patients. However, visual integration appeared to be impaired for existing knowledge. This result is discussed in relation with a possible involvement of the anterior temporal lobes. These regions are known to be the most vulnerable in SD. Recently these regions have also been shown to be involved in the multimodal integration. Taken together, these data suggest that perception and knowledge could be linked and partially explained by the SFT. Finally, the data support the sensory-dependent approaches of memory.     

Specialization of phonological and semantic processing in Chinese word reading

The purpose of this study was to examine the neurocognitive network for processing visual word forms in native Chinese speakers using functional magnetic resonance imaging (fMRI). In order to compare the processing of phonological and semantic representations, we developed parallel rhyming and meaning association judgment tasks that required explicit access and manipulation of these representations. Subjects showed activation in left inferior/middle frontal gyri, bilateral medial frontal gyri, bilateral middle occipital/fusiform gyri, and bilateral cerebella for both the rhyming and meaning tasks. A direct comparison of the tasks revealed that the rhyming task showed more activation in the posterior dorsal region of the inferior/middle frontal gyrus (BA 9/44) and in the inferior parietal lobule (BA 40). The meaning task showed more activation in the anterior ventral region of the inferior/middle frontal gyrus (BA 47) and in the superior/middle temporal gyrus (BA 22,21). These findings are consistent with previous studies in English that suggest specialization of inferior frontal regions for the access and manipulation of phonological vs. semantic representations, but also suggest that this specialization extends to the middle frontal gyrus for Chinese. These findings are also consistent with the suggestion that the left middle temporal gyrus is involved in representing semantic information and the left inferior parietal lobule is involved in mapping between orthographic and phonological representations.     

Restoration of conceptual knowledge in a case of semantic dementia

Patients with semantic dementia (SD) may undergo successful relearning of object names, but these gains are usually restricted to the trained exemplars, demonstrating poor generalization. We hypothesized that generalization could be improved by restoring an item’s semantic network through specific strategies that recruit the remaining personal semantic memories (conceptual enrichment therapies). We describe the case of a patient with SD who showed greater generalization of learning following a conceptual enrichment therapy than when learning items in a word-retrieval therapy. Our results suggest that enhancing an item’s semantic network connections may result in improved generalization of learning in SD. A learning mechanism in the presence of compromised hippocampi is also discussed.     

The role of the temporal lobe semantic system in number knowledge: evidence from late-stage semantic dementia

Previous reports have demonstrated that many aspects of number knowledge remain unimpaired in semantic dementia, despite severe comprehension problems in other domains. It is argued that this advantage for numbers arises because the disease spares the parietal lobe magnitude system thought to be critical for number processing. Models of numerical cognition that favour a separation between verbal and magnitude representations of number might, however, predict a restricted impairment of the verbal number code in this condition. We obtained support for this hypothesis in a patient with late-stage semantic dementia. She was impaired at a variety of tasks tapping the verbal number code; for example, reading and writing Arabic numerals, naming and word-picture matching with dot pictures, reading aloud number words, digit span and magnitude comparison/serial ordering tasks with number words. In contrast, she demonstrated good understanding of the magnitude and serial order of numbers when tested with Arabic numerals and non-symbolic representations. These findings suggest that although the magnitude meaning of numbers is isolated from the temporal lobe semantic system, the anterior infero-temporal lobe may play a critical role in binding English number words to their non-symbolic magnitude meaning.     

Grapheme to phoneme conversion: the basis of reading disability?

Although there have been many attempts to discriminate subtypes of reading disabled children, this study provides evidence of two common factors that seem to generalize to the vast majority of children with specific reading disability. The first, a right hemisphere cognitive style, may have some diagnostic utility. The second, a grapheme to phoneme deficit, may be at the heart of the disability. Techniques for diagnosis and remediation are discussed.     

Colour knowledge in semantic dementia: it is not all black and white

In three experiments we assessed the colour knowledge of patients with semantic dementia, a neuro-degenerative condition that gradually erodes conceptual knowledge. In Experiment 1, the patients’ colour naming performance correlated strongly with their object naming for frequency-matched items, with no patient showing better-than-expected naming of colours relative to objects. In Experiment 2, where patients were asked to colour black-and-white line drawings of common objects, all patients were impaired relative to controls, and performance correlated strongly with degree of semantic deficit. The fact that patients often erroneously selected green for fruits or vegetables, and brown for animals, suggests some preservation of general knowledge about the colours that typify a given domain. In Experiment 3, patients were given pairs of identical line drawings of familiar animals, fruits and vegetables—one of each pair coloured correctly, and one incorrectly—and were asked to choose the correct one. When the target's colour was characteristic of the domain, patients scored well; but when the distractor had a typical hue and the target's colour was unusual (e.g. a green versus an orange carrot), performance was far poorer. The results are discussed with reference to alternative theories about the neural basis of conceptual knowledge.     

Differential impairment of person-specific knowledge in a patient with semantic dementia

We report the case of ST, a 61-year-old gentleman of superior intelligence, who presented with a five-year history of slowly progressive word finding difficulty for proper names. MRI (magnetic resonance imaging) showed marked atrophy in the left anterior temporal lobe and SPECT (single photon emission computed tomography) demonstrated hypoperfusion in the left anterior and inferior temporal region. A detailed behavioral analysis of the integrity of his semantic system revealed naming deficits for both proper and common names. ST also showed severe impairments in person-specific semantic knowledge, but preservation of knowledge for famous places and common objects. ST's semantic knowledge deficit for people emerged whether he was tested with the faces or the names of famous individuals. Variables that affect name retrieval, such as familiarity, age of acquisition, and frequency of concept encounter, do not account for the selective impairment of person-specific knowledge evidenced by ST. Qualitative differences between the nature of knowledge representations for people and for common objects may underlie this effect. Disruptions in left temporal regions associated with semantic processing may be sufficient to produce a selective deficit in person-specific knowledge.     

Developmental differences of neurocognitive networks for phonological and semantic processing in Chinese word reading

Developmental differences in the neurocognitive networks for phonological and semantic processing in Chinese word reading were examined in 13 adults and 13 children using functional magnetic resonance imaging (fMRI). Rhyming and semantic association judgments were made to two-character words that were presented sequentially in the visual modality. These lexical tasks were compared with a nonlinguistic control task involving judgment of line patterns. The first main finding was that adults showed greater activation than children in right middle occipital gyrus on both the meaning and rhyming task, suggesting adults more effectively engage right hemisphere brain regions involved in the visual-spatial analysis of Chinese characters. The second main finding was that adults showed greater activation than children in left inferior parietal lobule for the rhyming as compared with the meaning task, suggesting greater specialization of phonological processing in adults. The third main finding was that children who had better performance in the rhyming task on characters with conflicting orthographic and phonological information relative to characters with nonconflicting information showed greater activation in left middle frontal gyrus, suggesting greater engagement of brain regions involved in the integration of orthography and phonology.     

Lexicality effects in word and nonword recall of semantic dementia and progressive nonfluent aphasia

Background: Verbal working memory is an essential component of many language functions, including sentence comprehension and word learning. As such, working memory has emerged as a domain of intense research interest both in aphasiology and in the broader field of cognitive neuroscience. The integrity of verbal working memory encoding relies on a fluid interaction between semantic and phonological processes. That is, we encode verbal detail using many cues related to both the sound and meaning of words. Lesion models can provide an effective means of parsing the contributions of phonological or semantic impairment to recall performance.

Methods & Procedures: We employed the lesion model approach here by contrasting the nature of lexicality errors incurred during recall of word and nonword sequences by three individuals with progressive nonfluent aphasia (a phonological dominant impairment) compared to that of two individuals with semantic dementia (a semantic dominant impairment). We focused on psycholinguistic attributes of correctly recalled stimuli relative to those that elicited a lexicality error (i.e., nonword → word OR word → nonword).

Outcomes & Results: Patients with semantic dementia showed greater sensitivity to phonological attributes (e.g., phoneme length, wordlikeness) of the target items relative to semantic attributes (e.g., familiarity). Patients with PNFA showed the opposite pattern, marked by sensitivity to word frequency, age of acquisition, familiarity, and imageability.

Conclusions: We interpret these results in favour of a processing strategy such that in the context of a focal phonological impairment patients revert to an over-reliance on preserved semantic processing abilities. In contrast, a focal semantic impairment forces both reliance on and hypersensitivity to phonological attributes of target words. We relate this interpretation to previous hypotheses about the nature of verbal short-term memory in progressive aphasia.     

Quest for the best: effects of errorless and active encoding on word re-learning in semantic dementia

Semantic dementia is a neurocognitive disorder characterised by a steady and progressive loss of semantic knowledge in the presence of relatively preserved other cognitive skills. Recent treatment studies have proven that language rehabilitation aimed at anomia in semantic dementia can be successful. The objective of this study was to examine the separate and interactive effects of errorless vs. errorful and active vs. passive learning approaches to anomia and their effects on naming and comprehension of treated items, as well as maintenance and generalisation of treatment gains. Seven participants with semantic dementia re-learned two sets of words (one for which participants retained auditory comprehension, and one for which they did not) in each of four different treatment methods based on those approaches. Errorless learning proved more successful than errorful learning in restoring lexical representations in all but one participant while there was no interaction between effects of errorless and active approaches on treatment success. Maintenance of treatment gains showed an advantage for errorless learning at one but not three months post-treatment, although all overall gains were maintained to a significant degree at both time points. Effects of both treatment and maintenance were stronger for items for which participants showed preserved auditory comprehension. The results are discussed in a framework of progressive language disorders and applicability of errorless methods to language rehabilitation in semantic dementia.     

Differing neuropsychological and neuroanatomical correlates of abnormal reading in early-stage semantic dementia and dementia of the Alzheimer type

Individuals with semantic dementia (SD) were differentiated neuropsychologically from individuals with dementia of the Alzheimer type (DAT) at very mild-to-mild stages (clinical dementia rating 0.5 or 1). A picture naming and recognition memory experiment provided a particularly useful probe for early identification, with SD individuals showing preserved picture recognition memory and impaired naming, and DAT individuals tending to show the reverse dissociation. The identification of an early SD group provided the opportunity to inform models of reading by exploring the influence of isolated lexical semantic impairment on reading regular words. Results demonstrated prolonged latency in both SD and DAT group reading compared to a control group but exaggerated influence of frequency and length only for the SD group. The SD reading pattern was associated with focal atrophy of the left temporal pole. These cognitive-neuroanatomical findings suggest a role for the left temporal pole in lexical/semantic components of reading and demonstrate that cortical thickness differences in the left temporal pole correlate with prolonged latency associated with increased reliance on sublexical components of reading.     

Visual search patterns in semantic dementia show paradoxical facilitation of binding processes

While patients with Alzheimer's disease (AD) show deficits in attention, manifested by inefficient performance on visual search, new visual talents can emerge in patients with frontotemporal lobar degeneration (FTLD), suggesting that, at least in some of the patients, visual attention is spared, if not enhanced. To investigate the underlying mechanisms for visual talent in FTLD (behavioral variant FTD [bvFTD] and semantic dementia [SD]) patients, we measured performance on a visual search paradigm that includes both feature and conjunction search, while simultaneously monitoring saccadic eye movements. AD patients were impaired relative to healthy controls (NC) and FTLD patients on both feature and conjunction search. BvFTD patients showed less accurate performance only on the conjunction search task, but slower response times than NC on all three tasks. In contrast, SD patients were as accurate as controls and had faster response times when faced with the largest number of distracters in the conjunction search task. Measurement of saccades during visual search showed that AD patients explored more of the image, whereas SD patients explored less of the image before making a decision as to whether the target was present. Performance on the conjunction search task positively correlated with gray matter volume in the superior parietal lobe, precuneus, middle frontal gyrus and superior temporal gyrus. These data suggest that despite the presence of extensive temporal lobe degeneration, visual talent in SD may be facilitated by more efficient visual search under distracting conditions due to enhanced function in the dorsal frontoparietal attention network.     

Neuroelectric correlates of a neuropsychological model of word decoding and semantic processing in reading disabled children

The purpose of this study was to distinguish the characteristics and components of event related potentials (ERPs) correlated with word decoding and semantic processing in a subgroup of children with specific reading disabilities related to visual processing deficiencies. The results were compared with those obtained from a group of normal readers previously studied (Ostrosky et al. in press). Visual ERPs were recorded to four stimuli: three physically different but with the same semantic content: a frequently used noun written in capitals (COCHE), the same noun in handwriting, the pictorial representation of the noun (drawing), and a neutral stimulus consisting of a checkerboard. Four derivations were used: occipital (O1-O2) and parietal (P3-P4) with reference to linked mastoids. Data were analyzed using multivariate procedures. A Principal Component Analysis with Varimax rotation of the solution was applied. In the normal readers, we found some components in the occipital derivations which identified the words presented in different styles of handwriting and others which seemed to identify these words with the pictorial representation (and not with the neutral stimuli). The first "verbal" components were situated around the 156-256 ms latency range and the second "semantic" components were observed at over 380 ms. In the disabled readers, there was no "verbal" or "semantic" recognition grouping of these physically different stimuli. As opposed to the normal readers, interhemispheric responses were symmetrical. At the left parietal leads in the normal readers, the morphology of the verbal stimuli (capitals and handwriting) were very similar throughout the sweep and both were very different from the nonverbal stimuli. At 376 ms the four stimuli elicited a prominent negative peak in which the verbal stimuli elicited significantly higher amplitude than the nonverbal stimuli. In the disabled group, the morphologies of the four stimuli were very similar and no significant differences were observed. The results are discussed in accordance with a hypothetical model of the neural bases underlying reading. The model takes into account the complexity of the reading process and the various cognitive skills required for its adequate performance, and emphasizes a complex, dynamic interplay between occipital, parietal and temporal areas.     

Treating a semantic word production deficit in aphasia with verbal and gesture methods

Background: Classical aphasiology, based on the study of stroke sequelae, fuses speech fluency and grammatical ability. Nonfluent (Broca's) aphasia often is accompanied by agrammatism; whereas in the fluent aphasias grammatical deficits are not typical. The assumption that a similar relationship exists in primary progressive aphasia (PPA) has led to the dichotomisation of this syndrome into fluent and nonfluent subtypes.

Aims: This study compared elements of fluency and grammatical production in the narrative speech of individuals with PPA to determine if they can be dissociated from one another.

Methods &; Procedures: Speech samples from 37 individuals with PPA, clinically assigned to agrammatic (N?=?11), logopaenic (N?=?20), and semantic (N?=?6) subtypes, and 13 cognitively healthy control participants telling the “Cinderella Story” were analysed for fluency—i.e., words per minute (WPM) and mean length of utterance in words (MLU-W)—and grammaticality, i.e., the proportion of grammatically correct sentences, open-to-closed-class word ratio, noun-to-verb ratio, and correct production of verb inflection, noun morphology, and verb argument structure. Between-group differences were analysed for each variable. Correlational analyses examined the relation between WPM and each grammatical variable, and an off-line measure of sentence production.

Outcomes &; Results: Agrammatic and logopaenic groups both had lower scores on the fluency measures and produced significantly fewer grammatical sentences than did semantic and control groups. However, only the agrammatic group evinced significantly impaired production of verb inflection and verb argument structure. In addition some semantic participants showed abnormal open-to-closed and noun-to-verb ratios in narrative speech. When the sample was divided on the basis of fluency, all the agrammatic participants fell in the nonfluent category. The logopaenic participants varied in fluency but those with low fluency showed variable performance on measures of grammaticality. Correlational analyses and scatter plots comparing fluency and each grammatical variable revealed dissociations within PPA participants, with some nonfluent participants showing normal grammatical skill.

Conclusions: Grammatical production is a complex construct comprising correct usage of several language components, each of which can be selectively affected by disease. This study demonstrates that individuals with PPA show dissociations between fluency and grammatical production in narrative speech. Grammatical ability, and its relationship to fluency, varies from individual to individual, and from one variant of PPA to another, and can even be found in individuals with semantic PPA in whom a fluent aphasia is usually thought to accompany preserved ability to produce grammatical utterances.     

ERP effects of listening to speech: semantic ERP effects

In this study, event-related brain potential effects of speech processing are obtained and compared to similar effects in sentence reading. In two experiments spoken sentences were presented with semantic violations in sentence-final or mid-sentence positions. For these violations N400 effects were obtained that were very similar to N400 effects obtained in reading. However, the N400 effects in speech were preceded by an earlier negativity (N250). This negativity is not commonly observed with written input. The early effect is explained as a manifestation of a mismatch between the word forms expected on the basis of the context, and the actual cohort of activated word candidates that is generated on the basis of the speech signal.