Category and letter fluency in semantic dementia, primary progressive aphasia, and Alzheimer's disease

This study examined the impact of various degenerative dementias on access to semantic knowledge and the status of semantic representations. Patients with semantic dementia, primary progressive aphasia, and Alzheimer's disease were compared with elderly controls on tasks of category and letter fluency, with number of words generated, mean lexical frequency and errors recorded. The findings are consistent with the view that category and letter fluency rely on both common and unique cognitive processes. Fluency tasks, with the richness of data obtained, are valuable in distinguishing different dementia syndromes from one another.     

The neural basis of syntactic deficits in primary progressive aphasia

Patients with primary progressive aphasia (PPA) vary considerably in terms of which brain regions are impacted, as well as in the extent to which syntactic processing is impaired. Here we review the literature on the neural basis of syntactic deficits in PPA. Structural and functional imaging studies have most consistently associated syntactic deficits with damage to left inferior frontal cortex. Posterior perisylvian regions have been implicated in some studies. Damage to the superior longitudinal fasciculus, including its arcuate component, has been linked with syntactic deficits, even after gray matter atrophy is taken into account. These findings suggest that syntactic processing depends on left frontal and posterior perisylvian regions, as well as intact connectivity between them. In contrast, anterior temporal regions, and the ventral tracts that link frontal and temporal language regions, appear to be less important for syntax, since they are damaged in many PPA patients with spared syntactic processing.     

Semantic interference during object naming in agrammatic and logopenic primary progressive aphasia (PPA)

This study examined the time course of object naming in 21 individuals with primary progressive aphasia (PPA) (8 agrammatic (PPA-G); 13 logopenic (PPA-L)) and healthy age-matched speakers (n = 17) using a semantic interference paradigm with related and unrelated interfering stimuli presented at stimulus onset asynchronies (SOAs) of −1000, −500, −100 and 0 ms. Results showed semantic interference (SI) (i.e. significantly slower RTs in related compared to unrelated conditions) for all groups at −500, −100 and 0 ms, indicating timely spreading activation to semantic competitors. However, both PPA groups showed a greater magnitude of SI than normal across SOAs. The PPA-L group and six PPA-G participants also evinced SI at −1000 ms, suggesting an abnormal time course of semantic interference resolution, and concomitant left hemisphere cortical atrophy in brain regions associated with semantic processing. These subtle semantic mapping impairments in non-semantic variants of PPA may contribute to the anomia of these patients.     

Effect of lexical cues on the production of active and passive sentences in Broca's and Wernicke's aphasia

This study compared the sentence production abilities of individuals with Broca's and Wernicke's aphasia in an attempt to explore the extent to which impaired lexical retrieval impedes sentence production. The ability to produce active and passive reversible and non-reversible sentences was examined when varying amounts of lexical information was provided. The results showed that both Wernicke's and Broca's aphasic individuals were impaired in passive sentence production and that these difficulties were not overcome when lexical cues (the relevant nouns and uninflected verb) were provided. However when auxiliary and past tense morphemes were provided along with the verb stem, production of passive sentences improved drastically for both groups. Analysis of error patterns, however, revealed differences between the two groups, suggesting that Broca's aphasic subjects may find passive sentences difficult due to problems with retrieving the relevant grammatical morphemes. Subjects with Wernicke's aphasia may have been unable to automatically access the passive sentence structure.     

Self-awareness and self-monitoring of cognitive and behavioral deficits in behavioral variant frontotemporal dementia, primary progressive aphasia and probable Alzheimer's disease

Lack of insight is a core diagnostic criterion for behavioral variant frontotemporal dementia (bvFTD), and is believed to be intact in the early stages of primary progressive aphasia (PPA). In other neurological conditions, symptom-specific insight has been noted, with behavioral symptoms appearing especially vulnerable to reduced insight. Different components of insight, self-awareness and self-monitoring, are also often considered separate phenomena. The current study compared insight in patients with PPA, bvFTD, and probable Alzheimer's disease (PrAD) and a group of cognitively intact control subjects. Additionally, differences in insight for the domains primarily affected by the three types of dementia, namely, Behavior, Naming, and Memory, were assessed, and self-awareness and self-monitoring were compared. A total of 55 participants were enrolled. Participants were asked to complete self-estimate scales demonstrating their perceived ability immediately prior to, and immediately following a test in each domain of interest. Results indicated that PPA and normal control groups performed very similarly on control (Weight and Eyesight) and cognitive domains, whereas bvFTD and PrAD patients were unable to accurately assess Memory. All three diagnostic groups failed to accurately assess their behavioral symptoms, suggesting that this domain is vulnerable to loss of insight across diagnoses. Naming ability, in contrast, was either accurately assessed or underestimated in all groups. Finally, there were no notable differences between self-awareness and self-monitoring, potential explanations for this are examined.     

Semantic and syntactic aspects of the mass/count distinction: a case study of semantic dementia

Semantic and syntactic contributions to processing of mass and count nouns were assessed by examining the performance of a patient suffering from a pure semantic deficit. Semantic and syntactic processing was evaluated on grammaticality judgement and sentence-picture matching tasks, respectively, where each task involved mass and count readings of metonymic nouns. While the patient did not show impaired performance on the grammaticality judgment task, he manifested difficulties in making mass/count distinctions in the sentence-picture matching task. It is thus argued that while distributionally the mass/count distinction may be established on a purely syntactic basis, cognitive processing of mass/count information requires both intact syntactic and semantic knowledge.     

Deregulated semantic cognition contributes to object‐use deficits in Alzheimer's disease: A comparison with semantic aphasia and semantic dementia

Executive control is impaired from the early stages of Alzheimer's Disease (AD) and this produces deregulated semantic cognition (Corbett, Jefferies, Burns, & Lambon Ralph, 2012 ; Perry, Watson, & Hodges, 2000 ). While control deficits should affect semantic retrieval across all modalities, previous studies have typically focused on verbal semantic tasks. Even when non‐verbal semantic tasks have been used, these have typically employed simple picture‐matching tasks, which may be influenced by abnormalities in covert naming. Therefore, in the present study, we examined 10 patients with AD on a battery of object‐use tasks, in order to advance our understanding of the origins of non‐verbal semantic deficits in this population. The AD patients’ deficits were contrasted with previously published performance on the same tasks within two additional groups of patients, displaying either semantic degradation (semantic dementia) or deregulation of semantic retrieval (semantic aphasia; Corbett, Jefferies, Ehsan, & Lambon Ralph, 2009 ). While overall accuracy was comparable to the scores in both other groups, the AD patients’ object‐use impairment most closely resembled that observed in SA; they exhibited poorer performance on comprehension tasks that placed strong demands on executive control. A similar pattern was observed in the expressive domain: the AD and SA groups were relatively good at straightforward object use compared to executively demanding, mechanical puzzles. Error types also differed: while all patients omitted essential actions, the SA and AD groups’ demonstrations also featured unrelated intrusions. An association between AD patients’ object use and their scores on standard executive measures suggested that control deficits contributed to their non‐verbal semantic deficits. Moreover, in a task specifically designed to manipulate executive demand, patients with AD (and SA) exhibited difficulty in thinking flexibly about the non‐canonical uses of everyday objects, especially when distracted by semantically related objects. This study provides converging evidence for the notion that a failure of regulatory control contributes to multimodal semantic impairment in AD and uniquely demonstrates this pattern for the highly non‐verbal domain of object use.