Focus Group Findings to Support the Preliminary Development of the Augmented Reality Education Experience (AREduX).

Dementia is considered a global health priority with projections of the disease set to increase dramatically across the world. Current support for persons living with dementia (PLWD) relies on long-term care and local service centers to provide education and support. Augmented reality-based programs continue to gain momentum across health sectors, becoming an innovative approach that provides an opportunity to have a visceral experience, which can deepen understanding and provide an embodied perspective of other groups within a relatively short time frame. There is increasing interest in developing approaches to aid patient care outcomes for PLWD and their caregivers. Hence, healthcare providers (HCPs) who are appropriately trained and equipped to provide quality care to PLWD are essential and of international concern. The purpose of this research program is to develop an augmented reality (AR) education experience (AREduX), a proof of concept prototype in the form of a digital resource that uses AR to simulate the physical and cognitive symptoms that PLWD experience. The findings from a stakeholder focus group will allow for the preliminary development of the AREduX.

Description of connected speech across different elicitation tasks in the logopenic variant of primary progressive aphasia.

BACKGROUND: Despite its importance, in-depth analysis of connected speech is often neglected in the diagnosis of primary progressive aphasia (PPA) - especially for the logopenic variant (lvPPA) for which unreliable differential diagnosis has been documented. Only a few studies have been conducted on this topic in lvPPA. AIMS: The aim of this study was to describe and compare lexico-semantic and morphosyntactic features of connected speech in participants with lvPPA, in comparison with healthy controls, using three different elicitation tasks (i.e., picture description, story narration and semi-structured interviews). In addition to a number of discourse features, we were particularly interested in the presence or absence of syntactic deficits in this PPA variant in line with recent findings. METHODS & PROCEDURES: A prospective group study was conducted to compare lvPPA participants (n = 13) to age- and education-matched healthy controls (n = 13). For each individual, connected speech was obtained using three tasks: (1) The Cookie Theft picture description; (2) Cinderella Story; (3) Topic-directed interview. Production on each task was recorded, transcribed and analysed according to the Quantitative Production Analysis (QPA) protocol, a tool developed by Berndt et al. (2000) for the analysis of sentence production in aphasia. Differences between lvPPA and healthy controls and among elicitation tasks were analysed using repeated measures multilevel mixed-effects regression, separately for each outcome. OUTCOMES & RESULTS: Four measures were significantly different between lvPPA participants and healthy controls across all elicitation tasks. Specifically, lvPPA participants produced a reduced proportion of open-class words, a higher proportion of verbs, a higher proportion of pronouns and fewer well-formed sentences. For these measures, the difference between lvPPA and healthy controls was consistent among elicitation tasks, except for the proportion of well-formed sentences, where the difference between the two groups was significantly greater in the story narration task than in the other tasks. CONCLUSIONS & IMPLICATIONS: Across elicitation tasks that used the same analysis protocol (i.e., QPA), a similar pattern of deficits in connected speech emerged in lvPPA patients. Importantly, the findings replicate previous studies, which used different elicitation tasks and analysis protocols. Especially in relation to the documented syntactic deficits, these findings provide implications for differential diagnosis in PPA. WHAT THIS PAPER ADDS: What is already known on the subject Connected speech analysis can provide an important contribution to the language assessment for the logopenic variant of primary progressive aphasia (lvPPA). However, only a few studies have been conducted with this population. What this paper adds to existing knowledge This study highlights differences between patients with lvPPA and healthy controls regarding the proportion of open-class words, nouns, verbs and well-formed sentences. What are the potential or actual clinical implications of this work? Importantly, our results highlight syntactic deficits in the same group of individuals with lvPPA, using the same analysis protocol and across various elicitation tasks, which has implications for differential diagnosis.

Episodic memory decline is associated with deficits in coherence of discourse.

This study investigates coherence of discourse in the production of autobiographical narratives by individuals with aMCI. Autobiographical interviews were analyzed to determine whether reduced episodic recall was related to deficits in discourse coherence. A coherence rating scale was used to evaluate relatedness of the autobiographical details produced by participants to the topic of discourse. Interviews were transcribed, segmented into details, and divided into sets of episodic, semantic, or supplementary information, which were subsequently analysed with the coherence rating scale. We predicted that the known episodic deficits observed in aMCI could also affect the retrieval of coherent episodic information. The results revealed deficits in coherence could be found in both episodic and semantic information in the aMCI group. These results suggest that the cognitive deficits experienced by individuals with aMCI may go beyond their known difficulty in recalling episodic details, as they also affect the controlled retrieval of both episodic and semantic information.

Impaired coherence for semantic but not episodic autobiographical memory in semantic variant primary progressive aphasia.

Language deficits, including word-finding difficulties and impaired single-word comprehension, have been found in patients with semantic variant primary progressive aphasia (svPPA). These deficits characterize the linguistic abilities of patients with svPPA on a micro-linguistic level (word and sentence level). On a macro-linguistic level (discourse level), svPPA patients' discourse has been described as "empty". Few studies have considered the contribution of a linguistic impairment to the difficulty of producing autobiographical narratives. In the present study, we assessed svPPA patients' discourse coherence during autobiographical narratives in order to characterize the nature of their speech on a macro-linguistic level and to investigate the relationship between discourse production and memory in a naturalistic context. We collected samples of discourse in which svPPA patients and healthy controls (matched in age, education, sex and handedness) reported autobiographical events. Their narratives were assessed with a rating scale to evaluate global coherence of discourse. The protocols were also analysed using the Autobiographical Interview method (Levine, Svoboda, Hay, Winocur, & Moscovitch, 2002) and categorized as episodic (information about events at a specific time and place), semantic (general knowledge), or supplementary details (metacognitive statements, repeated information, editorializing). Where possible, patients were assessed longitudinally at three time points over two years. Patients with svPPA produced a reduced number of episodic details, while the number of semantic details produced was comparable to controls. However, the episodic information produced by patients was coherent with the topic of discourse, while semantic information was not. These results suggest that svPPA patients produce semantic information comparable to controls in quantity but not quality, whereas the opposite is the case for episodic information.

White Matter Disruption and Connected Speech in Non-Fluent and Semantic Variants of Primary Progressive Aphasia.

Differential patterns of white matter disruption have recently been reported in the non-fluent (nfvPPA) and semantic (svPPA) variants of primary progressive aphasia (PPA). No single measure is sufficient to distinguish between the PPA variants, but connected speech allows for the quantification of multiple measures. The aim of the present study was to further investigate the white matter correlates associated with connected speech features in PPA. We examined the relationship between white matter metrics and connected speech deficits using an automated analysis of transcriptions of connected speech and diffusion tensor imaging in language-related tracts. Syntactic, lexical, and semantic features were automatically extracted from transcriptions of topic-directed interviews conducted with groups of individuals with nfvPPA or svPPA as well as with a group of healthy controls. A principal component analysis was performed in order to reduce the number of language measures and yielded a five-factor solution. The results indicated that nfvPPA patients differed from healthy controls on a syntactic factor, and svPPA patients differed from controls on two semantic factors. However, the patient groups did not differ on any factor. Moreover, a correlational analysis revealed that the lexical richness factor was significantly correlated with radial diffusivity in the left inferior longitudinal fasciculus, which suggests that semantic deficits in connected speech reflect a disruption of this ventral pathway, and which is largely consistent with the results of previous studies. Using an automated approach for the analysis of connected speech combined with probabilistic tractography, the present findings demonstrate that nfvPPA patients are impaired relative to healthy controls on syntactic measures and have increased radial diffusivity in the left superior longitudinal fasciculus, whereas the svPPA group was impaired on lexico-semantic measures relative to controls and showed increased radial diffusivity in the uncinate and inferior longitudinal fasciculus bilaterally.

Lack of Frank Agrammatism in the Nonfluent Agrammatic Variant of Primary Progressive Aphasia.

BACKGROUND/AIMS: Frank agrammatism, defined as the omission and/or substitution of grammatical morphemes with associated grammatical errors, is variably reported in patients with nonfluent variant primary progressive aphasia (nfPPA). This study addressed whether frank agrammatism is typical in agrammatic nfPPA patients when this feature is not required for diagnosis. METHOD: We assessed grammatical production in 9 patients who satisfied current diagnostic criteria. Although the focus was agrammatism, motor speech skills were also evaluated to determine whether dysfluency arose primarily from apraxia of speech (AOS), instead of, or in addition to, agrammatism. Volumetric MRI analyses provided impartial imaging-supported diagnosis. RESULTS: The majority of cases exhibited neither frank agrammatism nor AOS. CONCLUSION: There are nfPPA patients with imaging-supported diagnosis and preserved motor speech skills who do not exhibit frank agrammatism, and this may persist beyond the earliest stages of the illness. Because absence of frank agrammatism is a subsidiary diagnostic feature in the logopenic variant of PPA, this result has implications for differentiation of the nonfluent and logopenic variants, and indicates that PPA patients with nonfluent speech in the absence of frank agrammatism or AOS do not necessarily have the logopenic variant.

Wh- questions and passive sentences in non-fluent variant PPA and semantic variant PPA: Longitudinal findings of an anagram production task.

Using an anagram task, we investigated longitudinal syntactic production by individuals with semantic variant primary progressive aphasia (svPPA) and non-fluent variant PPA (nfvPPA), compared to controls. The accuracy of the production of active and passive, and reversible and non-reversible sentences, as well as of wh- questions was compared across the three groups. Results showed a different pattern of syntactic impairment across the two patient groups. The nfvPPA group showed difficulty with wh- questions at Time 1 and impairment with the passive structure approximately one year later, at Time 2. Surprisingly, the svPPA group also showed difficulty with wh- questions from Time 1, but still no difficulty on passive sentences, two years later, at Time 3. Neither group had difficulty with reversibility. The results of the nfvPPA patients were as expected, and the results of the svPPA patients suggest that this group may exhibit a greater syntactic impairment than is typically recognized.

Profiling Speech and Pausing in Amyotrophic Lateral Sclerosis (ALS) and Frontotemporal Dementia (FTD).

OBJECTIVE: This study examines reading aloud in patients with amyotrophic lateral sclerosis (ALS) and those with frontotemporal dementia (FTD) in order to determine whether differences in patterns of speaking and pausing exist between patients with primary motor vs. primary cognitive-linguistic deficits, and in contrast to healthy controls. DESIGN: 136 participants were included in the study: 33 controls, 85 patients with ALS, and 18 patients with either the behavioural variant of FTD (FTD-BV) or progressive nonfluent aphasia (FTD-PNFA). Participants with ALS were further divided into 4 non-overlapping subgroups--mild, respiratory, bulbar (with oral-motor deficit) and bulbar-respiratory--based on the presence and severity of motor bulbar or respiratory signs. All participants read a passage aloud. Custom-made software was used to perform speech and pause analyses, and this provided measures of speaking and articulatory rates, duration of speech, and number and duration of pauses. These measures were statistically compared in different subgroups of patients. RESULTS: The results revealed clear differences between patient groups and healthy controls on the passage reading task. A speech-based motor function measure (i.e., articulatory rate) was able to distinguish patients with bulbar ALS or FTD-PNFA from those with respiratory ALS or FTD-BV. Distinguishing the disordered groups proved challenging based on the pausing measures. CONCLUSIONS AND RELEVANCE: This study demonstrated the use of speech measures in the identification of those with an oral-motor deficit, and showed the usefulness of performing a relatively simple reading test to assess speech versus pause behaviors across the ALS-FTD disease continuum. The findings also suggest that motor speech assessment should be performed as part of the diagnostic workup for patients with FTD.

Verb production in the nonfluent and semantic variants of primary progressive aphasia: the influence of lexical and semantic factors.

Differential patterns of impairment with respect to noun and verb production have been observed in the nonfluent and semantic variants of primary progressive aphasia. However, the factors influencing this discrepancy remain unclear. The present study evaluates verb retrieval in primary progressive aphasia using a naming task and a story completion task. Findings indicate that patients with the semantic variant are influenced by familiarity, frequency, and age of acquisition in both object and action naming, whereas patients with the nonfluent variant are not. Surprisingly, there were no differences in either group between object and action naming, presumably because the lists were well matched on pertinent variables. In the story completion task, greater impairment in semantically heavier than in semantically lighter verbs was observed for the semantic variant, and grammaticality and verb tense agreement was significantly lower in the nonfluent variant. The present findings suggest that lexicosemantic attributes affect verb production in the semantic variant, whereas both lexicosemantic and syntactic attributes affect verb production in the nonfluent variant.

Automated classification of primary progressive aphasia subtypes from narrative speech transcripts.

In the early stages of neurodegenerative disorders, individuals may exhibit a decline in language abilities that is difficult to quantify with standardized tests. Careful analysis of connected speech can provide valuable information about a patient's language capacities. To date, this type of analysis has been limited by its time-consuming nature. In this study, we present a method for evaluating and classifying connected speech in primary progressive aphasia using computational techniques. Syntactic and semantic features were automatically extracted from transcriptions of narrative speech for three groups: semantic dementia (SD), progressive nonfluent aphasia (PNFA), and healthy controls. Features that varied significantly between the groups were used to train machine learning classifiers, which were then tested on held-out data. We achieved accuracies well above baseline on the three binary classification tasks. An analysis of the influential features showed that in contrast with controls, both patient groups tended to use words which were higher in frequency (especially nouns for SD, and verbs for PNFA). The SD patients also tended to use words (especially nouns) that were higher in familiarity, and they produced fewer nouns, but more demonstratives and adverbs, than controls. The speech of the PNFA group tended to be slower and incorporate shorter words than controls. The patient groups were distinguished from each other by the SD patients' relatively increased use of words which are high in frequency and/or familiarity.

Whole-brain white matter disruption in semantic and nonfluent variants of primary progressive aphasia.

Semantic (svPPA) and nonfluent (nfPPA) variants of primary progressive aphasia are associated with distinct patterns of cortical atrophy and underlying pathology. Little is known, however, about their contrasting spread of white matter disruption and how this relates to grey matter (GM) loss. We undertook a structural MRI study to investigate this relationship. We used diffusion tensor imaging, tract-based spatial statistics, and voxel-based morphometry to examine fractional anisotropy (FA) and directional diffusivities in nine patients with svPPA and nine patients with nfPPA, and compared them to 16 matched controls after accounting for global GM atrophy. Significant differences in topography of white matter changes were found, with more ventral involvement in svPPA patients and more widespread frontal involvement in nfPPA individuals. However, each group had both ventral and dorsal tract changes, and both showed spread of diffusion abnormalities beyond sites of local atrophy. There was a clear dissociation in sensitivity of diffusion tensor imaging measures between groups. SvPPA patients showed widespread changes in FA and radial diffusivity, whereas changes in axial diffusivity were more restricted and proximal to sites of GM atrophy. NfPPA patients showed isolated changes in FA, but widespread axial and radial diffusivity changes. These findings reveal the extent of white matter disruption in these variants of PPA after accounting for GM loss. Further, they suggest that differences in the relative sensitivity of diffusion metrics may reflect differences in the nature of underlying white matter pathology in these two subtypes.

When more yields less: speaking and writing deficits in nonfluent progressive aphasia.

Fourteen patients with nonfluent progressive aphasia (NFPA) performed a picture description task in both spoken- and written-output conditions, as well as tests of confrontation naming, spelling to dictation and reading aloud of single words and text. Relative to controls, the patients' spoken and written picture descriptions were reduced in length, speed and amount of information. Of particular interest, and accounting for the first part of the article's title, was a pervasive pattern of poorer spoken and written output associated with the requirement to produce more; this was true when 'more' meant either (a) longer vs. shorter single words or (b) connected language vs. single words. Deficits in spoken and written naming were largely parallel and modality-specific output impairments (such as dysarthria in speech or letter-formation problems in writing) seemed to account for the minority of cases who exhibited a discrepancy. Most patients showed no evidence of agrammatism or reduced verb production in their speech, which typically had normal proportions of content and function words as well as nouns and verbs. By contrast, some degree of telegraphic output was observed in the written narratives of a number of patients. Our results argue against several candidates for the main functional locus of impairment in NFPA, but it is likely that deficits in grammatical processing, working memory, planning/executive skills, speech motor abilities and phonological processing all play a role.

Corticobasal degeneration as a cognitive disorder.

The presence of cognitive impairment in corticobasal degeneration (CBD) is now widely recognised. Our review of the literature reveals that, although the pattern and severity of neuropsychological impairments can be highly variable across patients, several general trends can be identified. The most characteristic impairments are limb apraxia (usually ideomotor), constructional and visuospatial difficulties, acalculia, frontal dysfunction, and nonfluent aphasia. The limb apraxia is associated with deficits in drawing, copying, and handwriting, but there is emerging evidence that the problems with handwriting are not due exclusively to the apraxia. The findings with respect to episodic memory are more variable, but when there is impairment in this area, it tends to be milder than that seen in Alzheimer's disease. Semantic memory functioning appears relatively preserved but has been poorly studied. Problems with speech are common, and may be due to dysarthria or buccofacial apraxia. Aphasia, although initially considered rare, is in fact a common accompaniment of CBD, may be the presenting feature, and is typically nonfluent in type. More systematic investigation of the clinical and neuropathological overlap between progressive nonfluent aphasia (generally considered to be a form of frontotemporal dementia) and CBD is needed.

Language function and dysfunction in corticobasal degeneration.

OBJECTIVE: S: The authors assessed language functioning in corticobasal degeneration (CBD), an area that has received little systematic study. Aphasia has been reported occasionally, and the authors hypothesized that appropriate assessments would reveal at least mild language impairment, particularly affecting phonologic (sound-based) processing, even in cases without frank aphasia. METHODS: A series of 10 unselected patients with CBD (one with pathologic confirmation) were administered neuropsychological tests assessing the following aspects of cognitive functioning: verbal fluency, naming, reading, oral spelling, auditory-verbal short-term memory, phoneme blending and segmentation, visuospatial skills, and semantic memory. RESULTS: Phonologic and spelling impairments were prevalent, even in nonaphasic patients. The prevalence of visuospatial, constructional, and frontal impairments, demonstrated in previous research, was also replicated. A minority of patients had deficits in semantic memory, naming, and reading, but the impairments were usually mild. CONCLUSIONS: The authors found phonologic impairment to be a typical feature of CBD. There is substantial overlap between progressive nonfluent aphasia and CBD, and the linguistic impairment can be thought of as a continuum, with mild phonologic impairment at one end and severe aphasia at the other.

Progressive non-fluent aphasia is associated with hypometabolism centred on the left anterior insula.

Progressive non-fluent aphasia (PNFA) is a syndrome in which patients lose the ability to communicate fluently in the context of relative preservation of single word comprehension and non-linguistic cognitive abilities. Neuroimaging in case studies with PNFA has failed to identify a consistent neural substrate for the language disorder. In this study of a group of patients (n=10) whose presenting complaint was progressive dysfluency, resting cerebral metabolism was measured using [18F]fluorodeoxyglucose-PET and analysed with the technique of statistical parametric mapping (SPM). Regional atrophy was assessed with voxel-based morphometry (VBM). Seven patients had a 'pure' PNFA syndrome, while the remaining three had additional features of a more pervasive dementia. Compared with controls, the patients showed hypometabolism in several regions that, most notably, included the left anterior insula/frontal opercular region. The VBM analysis revealed only one small area of atrophy in the left peri-Sylvian region. Analysis of the pure PNFA cases (n=7) relative to controls yielded qualitatively similar results to those of the whole group, suggesting that these cases were also at risk of a more generalized dementia, a finding borne out in subsequent follow-up of two cases to date. The PNFA group was then compared with a group with Alzheimer's disease (n=10) whose clinical profile did not include non-fluent aphasic features. In this analysis, the only persisting hypometabolic region was that centred over the left anterior insula. VBM did not identify any regional differences in atrophy between PNFA and Alzheimer's disease. In the light of current theories of fluent language production, the findings offer anatomical evidence that the breakdown in fluency is due to a motor articulatory planning deficit (speech apraxia) combined with a variable degree of agrammatism.