More than a piece of cake: Noun classifier processing in primary progressive aphasia.

INTRODUCTION: Clinical understanding of primary progressive aphasia (PPA) has been primarily derived from Indo-European languages. Generalizing certain linguistic findings across languages is unfitting due to contrasting linguistic structures. While PPA patients showed noun classes impairments, Chinese languages lack noun classes. Instead, Chinese languages are classifier language, and how PPA patients manipulate classifiers is unknown. METHODS: We included 74 native Chinese speakers (22 controls, 52 PPA). For classifier production task, participants were asked to produce the classifiers of high-frequency items. In a classifier recognition task, participants were asked to choose the correct classifier. RESULTS: Both semantic variant (sv) PPA and logopenic variant (lv) PPA scored significantly lower in classifier production task. In classifier recognition task, lvPPA patients outperformed svPPA patients. The classifier production scores were correlated to cortical volume over left temporal and visual association cortices. DISCUSSION: This study highlights noun classifiers as linguistic markers to discriminate PPA syndromes in Chinese speakers. HIGHLIGHTS: Noun classifier processing varies in the different primary progressive aphasia (PPA) variants. Specifically, semantic variant PPA (svPPA) and logopenic variant PPA (lvPPA) patients showed significantly lower ability in producing specific classifiers. Compared to lvPPA, svPPA patients were less able to choose the accurate classifiers when presented with choices. In svPPA, classifier production score was positively correlated with gray matter volume over bilateral temporal and left visual association cortices in svPPA. Conversely, classifier production performance was correlated with volumetric changes over left ventral temporal and bilateral frontal regions in lvPPA. Comparable performance of mass and count classifier were noted in Chinese PPA patients, suggesting a common cognitive process between mass and count classifiers in Chinese languages.

Primary progressive aphasia: six questions in search of an answer.

Here, we review recent progress in the diagnosis and management of primary progressive aphasia-the language-led dementias. We pose six key unanswered questions that challenge current assumptions and highlight the unresolved difficulties that surround these diseases. How many syndromes of primary progressive aphasia are there-and is syndromic diagnosis even useful? Are these truly 'language-led' dementias? How can we diagnose (and track) primary progressive aphasia better? Can brain pathology be predicted in these diseases? What is their core pathophysiology? In addition, how can primary progressive aphasia best be treated? We propose that pathophysiological mechanisms linking proteinopathies to phenotypes may help resolve the clinical complexity of primary progressive aphasia, and may suggest novel diagnostic tools and markers and guide the deployment of effective therapies.

Symptom-led staging for semantic and non-fluent/agrammatic variants of primary progressive aphasia.

INTRODUCTION: Here we set out to create a symptom-led staging system for the canonical semantic and non-fluent/agrammatic variants of primary progressive aphasia (PPA), which present unique diagnostic and management challenges not well captured by functional scales developed for Alzheimer's disease and other dementias. METHODS: An international PPA caregiver cohort was surveyed on symptom development under six provisional clinical stages and feedback was analyzed using a mixed-methods sequential explanatory design. RESULTS: Both PPA syndromes were characterized by initial communication dysfunction and non-verbal behavioral changes, with increasing syndromic convergence and functional dependency at later stages. Milestone symptoms were distilled to create a prototypical progression and severity scale of functional impairment: the PPA Progression Planning Aid ("PPA-Squared"). DISCUSSION: This work introduces a symptom-led staging scheme and functional scale for semantic and non-fluent/agrammatic variants of PPA. Our findings have implications for diagnostic and care pathway guidelines, trial design, and personalized prognosis and treatment for PPA. HIGHLIGHTS: We introduce new symptom-led perspectives on primary progressive aphasia (PPA). The focus is on non-fluent/agrammatic (nfvPPA) and semantic (svPPA) variants. Foregrounding of early and non-verbal features of PPA and clinical trajectories is featured. We introduce a symptom-led staging scheme for PPA. We propose a prototype for a functional impairment scale, the PPA Progression Planning Aid.

Bilingual Primary Progressive Aphasia: A Scoping Review of Assessment and Treatment Practices.

BACKGROUND: Primary progressive aphasia (PPA) is a neurodegenerative syndrome characterized by speech and/or language impairment with relatively spared cognition. Research investigating behavioral speech-language intervention and methods for cognitive-linguistic assessment in PPA has predominantly centered around monolingual speakers. This gap hinders the widespread adoption of evidence-based approaches and exacerbates the inequities faced by culturally and linguistically diverse populations living with PPA. OBJECTIVE: This scoping review synthesizes the current evidence for assessment and treatment practices in bilingual PPA as well as the operationalization of bilingualism in PPA. METHODS: Arksey & O'Malley's scoping review methodology was utilized. Information was extracted from each study and entered into a data-charting template designed to capture information regarding operationalization of bilingualism in PPA and assessment and treatment practices. RESULTS: Of the 16 identified studies, 14 reported the results of assessments conducted in both languages. Three studies reported positive naming treatment outcomes. Thirteen studies included English-speaking participants, revealing linguistic bias. Most studies reported age of acquisition, proficiency, and patterns of language use rather than providing an operational definition for bilingualism. CONCLUSIONS: Neither formal assessment measures nor clear guidelines for assessment of bilingual PPA currently exist; however, language-specific measures are emerging. Speech-language intervention in bilingual PPA has been relatively unexplored, representing a significant gap in the literature. In order to improve diagnostic and treatment options for bilingual PPA, targeted efforts to increase representation of bilinguals from various sociocultural contexts, as well as those who speak a variety of language pairs, is necessary.

"They are dealing with people's lives ": Diagnostic and post-diagnostic healthcare experiences in primary progressive aphasia.

PURPOSE: The healthcare experience is a multifaceted and varied process, particularly for people living with complex conditions such as primary progressive aphasia (PPA). Different experiences influence pathways through the health system, impacting client outcomes. To our knowledge, no previous studies have directly explored the healthcare experiences of people with PPA and their families. This study aimed to explore the experiences of people living with PPA from the perspective of both the person with PPA and their families during diagnostic and post-diagnostic phases, and to identify factors influencing service access and perceptions of quality of care. METHOD: The study followed an Interpretive Phenomenological Analysis (IPA) approach. In-depth, semi-structured interviews were completed with three people with PPA and their primary care partner, and two further care partners of people with PPA. RESULT: Five superordinate themes were identified: characterising the assessment experience, getting a diagnosis, moving beyond the diagnosis, participant interactions with clinicians, and overall service provision. The five superordinate themes comprised 14 subthemes. CONCLUSION: The study provides preliminary insights into the complexity of the PPA healthcare journey, and the need for increased accessibility of information and supports following diagnosis. The findings inform recommendations for improving quality of care and the development of a PPA service framework or care pathway.

Speech Motor Profiles in Primary Progressive Aphasia.

PURPOSE: Previous research on motor speech disorders (MSDs) in primary progressive aphasia (PPA) has largely focused on patients with the nonfluent/agrammatic variant of PPA (nfvPPA), with few systematic descriptions of MSDs in variants other than nfvPPA. There has also been an emphasis on studying apraxia of speech, whereas less is known about dysarthria or other forms of MSDs. This study aimed to examine the qualitative and quantitative characteristics of MSDs in a prospective sample of individuals with PPA independent of subtype. METHOD: We included 38 participants with a root diagnosis of PPA according to current consensus criteria, including one case with primary progressive apraxia of speech. Speech tasks comprised various speech modalities and levels of complexity. Expert raters used a novel protocol for auditory speech analyses covering all major dimensions of speech. RESULTS: Of the participants, 47.4% presented with some form of MSD. Individual speech motor profiles varied widely with respect to the different speech dimensions. Besides apraxia of speech, we observed different dysarthria syndromes, special forms of MSDs (e.g., neurogenic stuttering), and mixed forms. Degrees of severity ranged from mild to severe. We also observed MSDs in patients whose speech and language profiles were incompatible with nfvPPA. CONCLUSIONS: The results confirm that MSDs are common in PPA and can manifest in different syndromes. The findings emphasize that future studies of MSDs in PPA should be extended to all clinical variants and should take into account the qualitative characteristics of motor speech dysfunction across speech dimensions. SUPPLEMENTAL MATERIAL: https://doi.org/10.23641/asha.22555534.

Evaluating the Association Between Genetically Proxied Neurodevelopmental Language Phenotypes and the Risk of Primary Progressive Aphasia.

BACKGROUND AND OBJECTIVES: Primary progressive aphasia (PPA) is a neurodegenerative syndrome of progressive language decline. PPA has 3 main subtypes: logopenic, semantic, and agrammatic. Observational studies suggested an association between language-related neurodevelopmental phenotypes and an increased risk of PPA. We sought to assess such relationships through Mendelian randomization (MR) approach, which can suggest potentially causal associations. METHODS: Genome-wide significant single-nucleotide polymorphisms (SNPs) associated with dyslexia (42 SNPs), developmental speech disorders (29 SNPs), and left-handedness (41 SNPs) were used as genetic proxies for the exposures. Eighteen of 41 SNPs of left-handedness were associated with structural asymmetry of the cerebral cortex. Genome-wide association study summary statistics were obtained from publicly available databases for semantic (308 cases/616 controls) and agrammatic PPA (269 cases/538 controls). The logopenic PPA (324 cases/3,444 controls) was approximated by proxy through the rubric of clinically diagnosed Alzheimer disease with salient language impairment. Inverse-weighted variance MR was performed as the main analysis for testing the relationship between the exposures and outcomes. Sensitivity analyses were completed to test the robustness of the results. RESULTS: Dyslexia, developmental speech disorders, and left-handedness were not associated with any PPA subtype (p > 0.05). The genetic proxy of cortical asymmetry in left-handedness was significantly associated with agrammatic PPA (ß = 4.3, p = 0.007), but not with other PPA subtypes. This association was driven by microtubule-related genes, primarily by a variant that is in complete linkage disequilibrium with MAPT gene. Sensitivity analyses were overall consistent with the primary analyses. DISCUSSION: Our results do not support a causal association between dyslexia, developmental speech disorders, and handedness with any of the PPA subtypes. Our data suggest a complex association between cortical asymmetry genes and agrammatic PPA. Whether the additional association with left-handedness is necessary remains to be determined but is unlikely, given the absence of association between left-handedness and PPA. Genetic proxy of brain asymmetry (regardless of handedness) was not tested as an exposure due to lack of suitable genetic proxy. Furthermore, the genes related to cortical asymmetry associated with agrammatic PPA are implicated in microtubule-related proteins (TUBA1B, TUBB, and MAPT), which is keeping with the association of tau-related neurodegeneration in this PPA variant.

Patterns of hemispheric compromise in primary progressive apraxia of speech: From clinical profiling to differential diagnosis.

In this issue of European Journal of Neurology, Robinson et al. present a novel study on primary progressive apraxia of speech. The authors describe different clinicopathological profiles in patients with left-dominant, right-dominant, and bilateral atrophy of the supplementary motor area and lateral premotor cortex. This commentary discusses the importance of this evidence for understanding individual differences among these patients, distinguishing them from those with nonfluent variant primary progressive aphasia, and analyzing the relations between motor speech deficits and underlying pathology.

The ScreeLing: Detecting Semantic, Phonological, and Syntactic Deficits in the Clinical Subtypes of Frontotemporal and Alzheimer's Dementia.

The ScreeLing is a screening instrument developed to assess post-stroke aphasia, via the linguistic levels Syntax, Phonology, and Semantics. It could also be a useful test for the clinical subtypes of frontotemporal dementia (FTD) and Alzheimer's dementia (AD), as specific and often selective disorders are expected. Its ability to differentiate between the clinical subtypes of FTD and AD is, however, still unknown. We investigated differences in ScreeLing total and subscores, linguistic-level disorders' relationship with disease severity, and classification abilities, in patients with behavioral variant FTD (bvFTD; n = 46), patients with primary progressive aphasia (PPA; n = 105) (semantic variant primary progressive aphasia [svPPA], non-fluent variant primary progressive aphasia [nfvPPA], and logopenic variant primary progressive aphasia [lvPPA], AD [n = 20] and controls [n = 35]). We examined group differences in ScreeLing total and subscores, and one-, two- or three-level linguistic disorders using one-way analyses of covariance (ANCOVAs) or Quade's rank ANCOVA. We used frequency analyses to obtain the occurrence of the linguistic-level disorders. We determined sensitivity and specificity by the area under the curve by receiver-operating characteristics analyses to investigate classification abilities. The total score was lower in patients (bvFTD: 63.8 ± 8.5, svPPA: 58.8 ± 11.3, nfvPPA: 63.5 ± 8.4, lvPPA: 61.7 ± 6.6, AD: 63.8 ± 5.5) than controls (71.3 ± 1.0) (p < .001). Syntax subscores were lower in svPPA (19.4 ± 4.6; p < .001) and lvPPA (20.3 ± 3.2; p = .002) than controls (23.8 ± 0.4). Phonology subscores were lower in lvPPA (19.8 ± 2.6) than bvFTD (21.7 ± 2.8) (p = .010). Semantics subscores were lowest in svPPA (17.8 ± 5.0; p < .002). A selective phonological disorder was most prevalent in lvPPA (34.9%). The higher the disease severity, the more linguistic-level disorders. The optimal cutoff for the total score was 70, and 23 for all three subscores. Good classification abilities were found for the Semantics (svPPA vs. bvFTD), Phonology (lvPPA vs. svPPA), and Syntax (nfvPPA vs. lvPPA) subscores. This easy to administer test gives information about language processing with the potential to improve differential diagnosis in memory clinics and in the future potentially also clinical trial planning.

"Please Don't Assess Him to Destruction": The R.A.I.S.E. Assessment Framework for Primary Progressive Aphasia.

PURPOSE: Speech-language pathologists (SLPs) assess people with primary progressive aphasia (PPA) through measurements of speech, language, communication, and well-being, with the aims of identifying challenges and strengths, monitoring change, and informing treatment directions and supports. The purpose of this clinical focus article is to highlight the necessity for person-centered assessment specific to PPA and to conceptualize a framework that acknowledges the multifaceted nature of assessment for this population. In this framework, the unique challenges posed by a diagnosis of PPA are addressed with the aim to provide practical guidance for clinicians and to support reflection on current practices. METHOD: In clinical and research practice, assessment of people with PPA requires an ever-evolving approach that is centered on the client. In this clinical focus article, a discussion-based consensus process was used to synthesize authentic longitudinal experiences of people with PPA to explore assessment approaches, tools, and philosophies. RESULTS: This analysis of person-centered assessment identifies seven essential components of assessment in PPA that set the foundation for the five steps of the R.A.I.S.E. Assessment framework. These components each contribute to a clear definition of assessment that reveals clients' competencies with a strengths-based focus; prioritizes the reciprocity of benefits; promotes dynamic assessment; and recognizes the complexity, evolution of assessment over time, and advocacy. CONCLUSIONS: This clinical focus article takes a novel look at assessment in PPA by stepping away from assessment practices that focus on revealing deficits and decline and, instead, provides practical recommendations through the conceptualization of a PPA-specific assessment framework. The R.A.I.S.E. Assessment framework is grounded in principles of uplifting clients through person-centered assessment, keeps pace with best practice in PPA intervention, and contributes to a supportive experience for clients and families in the face of a progressive diagnosis over time.

Access, referral, service provision and management of individuals with primary progressive aphasia: A survey of speech-language therapists in Italy.

BACKGROUND AND OBJECTIVES: In Italy, approximately 650 individuals receive a diagnosis of primary progressive aphasia (PPA) every year. Unfortunately, the frequency with which patients are referred to speech-language services is suboptimal, likely due to skepticism regarding the value of speech-language therapy in the context of neurodegeneration. MATERIALS AND METHODS: We conducted a virtual survey of speech and language therapists (SLTs) across Italy, to collect information about the assessment, intervention and management of patients with PPA. To ensure that as many SLTs as possible received the survey, the Italian Federation of SLTs (Federazione Logopedisti Italiani, FLI) aided in disseminating the survey. RESULTS: In total, 336 respondents participated in the online survey, 140 of whom had previous experience with PPA patients. Respondents indicated having seen a total of 428 PPA patients in the previous 24 months (three patients on average, range: 0-40). SLTs who reported never working with PPA identified underdiagnoses, low referral rates and the rarity of the clinical syndrome as major reasons for their lack of experience with PPA. SLTs with experience working with PPA indicated that patients may not have accessed services because of service dysfunction and geographical barriers. Respondents reported using informal interviews during assessments and tests developed for post-stroke aphasia, while impairment-based/restitutive interventions were utilised most often. CONCLUSION: Findings may serve to inform health policy organisations regarding the current shortcomings and needed recommendations for improving the care of individuals with PPA in Italy. Improving awareness of the utility of rehabilitation among SLTs and other clinical service providers may serve to facilitate access to intervention, which in turn will serve to better support individuals living with PPA. WHAT THIS PAPER ADDS: What is already known on the subject Speech and language therapists (SLTs) play a crucial role in the assessment, diagnosis and treatment of people with primary progressive aphasia (PPA). However, the frequency with which individuals with PPA are referred for speech and language services is suboptimal due to skepticism regarding the value of speech and language therapy in the context of neurodegeneration, the scarcity of SLTs with expertise in the treatment of PPA and the lack of awareness of the SLT role amongst referrers. What this paper adds to existing knowledge In recognition of the lack of published information on the provision of speech and language therapy services and clinicians' approaches to the assessment and treatment of individuals with PPA in Italy, we conducted an online survey to evaluate the current referral patterns for speech and language therapy services and to examine the current barriers to access these services for individuals with PPA in Italy. What are the potential or actual clinical implications of this work? The data presented here support that SLTs view treatment as useful for individuals with PPA and other professional figures and may serve to improve access to intervention, which in turn will serve to better support individuals living with PPA. The results highlight the need to inform health policy organisations about current gaps and aid in developing recommendations for improving the care of individuals with PPA, in order to understand how SLTs can best support individuals with PPA and their families.

Utility of a Spanish version of Three Words-Three Shapes Test to detect memory impairment in primary progressive aphasia.

INTRODUCTION: Three Words-Three Shapes (3W3S) is a bedside test that assesses verbal and non-verbal memory and has proven useful in staging memory decline in amnestic disorders and primary progressive aphasia. Given its simple structure, the 3W3S can be easily adapted to other languages maintaining the original shapes and only modifying the words. We aim to validate a Spanish version of the 3W3S test and establish whether memory loss patterns present in amnesic disorders associated with Alzheimer's etiology and PPA were correctly characterized. METHOD: The translation and adaptation of the 3W3S were performed according to standardized guidelines and applied to a cohort of patients with Dementia of Alzheimer's type (DAT = 20), mild cognitive impairment (aMCI= 20), primary progressive aphasia (PPA = 20), and healthy controls (HC = 20). RESULTS: In verbal memory performance, PPA patients' score was lower than that of MCI and HC and similar to DAT's in the effortless encoding (p < 0.001), delayed recall (p < 0.001), and recognition (p < 0.012). For non-verbal performance, PPA patients performed better than DAT and similar to HC and MCI subjects (p < 0.001). CONCLUSIONS: Results show good applicability of 3W3S to determine memory function in PPA patients, independently from language ability. Visual and verbal components of memory are dissociated in PPA.

My experience of living with nonfluent/agrammatic variant primary progressive aphasia: Challenges, compensatory strategies and adaptations.

BACKGROUND: Primary progressive aphasia (PPA) is a rare neurodegenerative brain disorder characterized by declining language ability. There is currently no way to reverse or slow the course of the progressive brain degeneration, nor is there a cure for PPA. Throughout the course of the disease, any treatment must therefore be palliative in nature and should be designed to manage symptoms and improve the quality of life of the affected person. There is little information in the medical literature about strategies to make meaningful improvements to the quality of life of people with PPA written from the perspective of those living with this condition. AIMS: I have a clinical diagnosis of the nonfluent/agrammatic variant of PPA (nfvPPA), supported by imaging. In this report I discuss my experience of the progressive loss of language and communication skills, and detail the challenges I have been facing. I also describe how my quality of life has been enhanced by the early initiation of treatment focusing on communication strategies targeted to my specific impairments and designed to support my individual interests and goals. METHODS & PROCEDURES: I was fortunate to obtain an early diagnosis from a cognitive neurologist experienced with PPA. From the onset of my language difficulties, I have received excellent personalized care from a multidisciplinary medical team including speech-language pathologists, a cognitive neurologist and other doctors. MAIN CONTRIBUTIONS: My life during the early stage of nfvPPA has been enriched by personalized care focused on supporting the particular activities, interests and goals that are most important and meaningful to me. As my disease has progressed, I have benefited from an evolving range of strategies and adaptations targeted to the specific deficits in the areas of speaking, writing and reading that I have been facing at any given time. In addition, I have adopted methods to enhance the benefit of these language-directed strategies. And I have been employing evidence-based approaches that improve general brain health and thereby indirectly support my language. CONCLUSIONS & IMPLICATIONS: My experience represents a model for the personalized care of people in the early stage of nfvPPA. WHAT THIS PAPER ADDS: What is already known on the subject There is minimal information in the medical literature describing the subjective experience of a person with PPA. There is little information in the medical literature about strategies to make meaningful improvements to the quality of life of people in the early stage of PPA. What this paper adds to existing knowledge I have a clinical diagnosis of nfvPPA, supported by imaging. In this paper I give a first-person account of my experience of the progressive loss of language and communication skills, and I detail the challenges I have been facing. I describe how my quality of life during the early stage of nfvPPA has been enhanced by an evolving range of strategies and adaptations tailored to my speech and language deficits as they have arisen. These compensatory strategies have focused on supporting the particular activities, interests and goals that are most important and meaningful to me. What are the potential or actual clinical implications of this work? The description of my subjective experience of the progressive loss of language and communication skills offers insight for speech-language pathologists, neurologists and other professionals involved in the clinical care of people in the early stage of nfvPPA. My experience represents a model for the personalized clinical care of people in the early stage of this disorder.

'Like going into a chocolate shop, blindfolded': What do people with primary progressive aphasia want from speech and language therapy?

BACKGROUND: Primary progressive aphasia (PPA) describes a group of language-led dementias. PPAs are complex, diverse and difficult to diagnose, and therefore conventional models of aphasia and dementia treatment do not meet their needs. The research evidence on intervention for PPA is developing, but to date there are only a few case studies exploring the experiences of people with PPA (PwPPA) themselves. AIMS: To explore the experiences and opinions of PwPPA and their communication partners (CPs) to understand how speech and language therapy (SLT) services can better meet their needs. METHODS & PROCEDURES: A qualitative research approach was used whereby PwPPA and their friends or family members were recruited to participate in focus groups, via advertisements in the Rare Dementia Support PPA group newsletters. Consenting participants were allocated to attend one of four focus groups hosted on an online video conferencing platform. Participants were asked about their communication difficulties, and how SLT could address these needs. All meetings were transcribed, and data were examined using reflexive thematic analysis. OUTCOMES & RESULTS: Six PwPPA and 14 CPs representing all three PPA variants and mixed PPA participated in the focus groups. Four main themes were identified during the analysis of the focus group discussions: (1) CPs' burden, (2) adjusting to the diagnosis, (3) communication abilities and difficulties and (4) beyond language. A further 10 subthemes were identified. CONCLUSIONS & IMPLICATIONS: This study provides a greater understanding of the experiences and needs of PwPPA and their families in relation to SLT. This work underlines the importance of a person-centred approach that considers the broader needs of both the PwPPA and the people around them. This will enable service providers to deliver SLT that meets the needs of PwPPA and their families and will also inform future research in this field. WHAT THIS PAPER ADDS: What is already known on this subject We know that PwPPA can maintain or even make improvements in word retrieval and speech fluency with SLT exercises. There is also developing evidence of the benefits of interventions such as CP training, communication aid support and other functional interventions. What this paper adds to existing knowledge This study provides an understanding of the experiences and opinions of people living with PPA and their families in relation to SLT. Results demonstrate that PwPPA and their families have to navigate a complex journey, identifying strategies to support communication but also the influence of personality and other cognitive symptoms. SLT was useful, but not always available. What are the potential or actual clinical implications of this work? This study will enable service providers to better plan, justify funding for and delivery of SLT that will meet the needs of PwPPA and their families. Most importantly this work underlines the importance of a person-centred approach, incorporating the broader needs of the person with PPA and those around them.

Use and Perceived Effectiveness of Communication Modes Reported by Persons With Primary Progressive Aphasia.

PURPOSE: Primary progressive aphasia (PPA) is a clinical neurodegenerative dementia syndrome characterized by early, selective, and progressive language impairment. PPA onset is gradual, providing time to potentially identify additional or alternative expressive communication modes; however, reports of communication mode use and effectiveness by persons with PPA have not been described. This study characterized the use, frequency, and perceived effectiveness of communication modes reported by individuals with PPA. METHOD: Forty-one participants with mild-to-moderate PPA completed a structured interview detailing the type, frequency, and perceived effectiveness of 12 potential communication modes, categorized by technology required (no-tech, low-tech, and high-tech). The ratio of modes used was compared across technology categories with a repeated-measures generalized linear model assuming a binomial distribution with an overall Wald chi-square statistic, followed by pairwise post hoc t-test comparisons. RESULTS: Of the 12 communication modes assessed, participants reported using a median of eight (range: 5-10). All participants affirmed using speech, facial expressions, and talking on the phone. Frequency and perceived effectiveness ratings for these three modes were endorsed at the "some/most of the time" level for more than 80% of the participants. No-tech mode use was significantly higher than reported high-tech and low-tech modes (p = .004 and p < .0001, respectively). Even so, while some high-tech modes (apps) and some low-tech modes (nonelectronic augmentative and alternative communication) had fewer users, effectiveness ratings were moderate to high for all but one user. CONCLUSIONS: Persons with mild-to-moderate language impairment due to PPA report using a range of communication modes with moderate-to-high frequency and perceived effectiveness. These outcomes provide practical information when considering mode refinement or expansion during intervention to maximize communication participation. Barriers to modality use may include low awareness or access, which could be queried by future studies and supported by speech and language interventions. SUPPLEMENTAL MATERIAL: https://doi.org/10.23641/asha.21614262.

Neuropsychiatric outcomes and caregiver distress in primary progressive aphasia.

BACKGROUND: In this study, we aimed to outline the neuropsychiatric consequences of primary progressive aphasia (PPA) and to understand how neuropsychiatric symptomatology affects distress in caregivers. METHODS: The Neuropsychiatric Inventory (NPI) including the distress index (NPI-Distress) was used. Additional information about the caregiver burden was obtained using Zarit Burden Interview (ZBI). NPI, NPI-Distress, and ZBI data from 17 patients with a clinical diagnosis of PPA were compared with 10 stroke aphasia patients. Neuropsychiatric symptomatology was investigated based on three clusters; Mood, Frontal/Comportmental, and Psychotic/Disruptive. Additionally, the Activities of Daily Living Questionnaire (ADLQ) was used to outline the functional impairment. Twelve healthy controls were included to compare the neurocognitive test scores with PPA and stroke aphasia groups. RESULTS: A greater number of neuropsychiatric symptoms were observed in the PPA group compared to the stroke aphasia group. The number of symptoms in Mood, and Frontal/Comportmental clusters were greater than the number of symptoms in Psychotic/Disruptive clusters in the PPA group, whereas no significant relationship between the number of symptoms and symptom clusters was found in the stroke aphasia group. In the PPA group, a strong correlation was found between the NPI-Frequency × Severity scores and the NPI-Distress scores. Moreover, the NPI-Distress scores in the PPA group strongly correlated with the ZBI scores. Scores for anxiety, irritability/lability, and apathy had a stronger correlation with the NPI-Distress scores compared to the other NPI symptoms. The Communication subscale was the most impaired domain in the PPA group. Travel, and Employment and Recreation subscales showed greater functional impairment in the stroke aphasia group compared to the PPA group. CONCLUSIONS: Neuropsychiatric symptoms in PPA in our study were more frequent than previously reported. Furthermore, the distress index of the NPI was not only correlated with the severity of the neuropsychiatric symptoms but also reflected the overall burden on the caregivers in the PPA group.

Primary progressive aphasia. / Primær progressiv afasi.

Neurodegenerative dementia may, in rare cases, initially manifest as isolated language impairments in the absence of other cognitive symptoms. These impairments are often somewhat imprecisely referred to as difficulties with 'word finding'. There are several variants of this form of dementia, each caused by different underlying neuropathologies. Occasionally problems with speech rather than language predominate. Patients may have exclusively language or speech-related symptoms for several years, but eventually all will progress to generalised dementia. This clinical review describes primary progressive aphasia: a collective term for forms of dementia that begin with language impairments.

Silent Pauses and Speech Indices as Biomarkers for Primary Progressive Aphasia.

Background and Objectives: Recent studies highlight the importance of investigating biomarkers for diagnosing and classifying patients with primary progressive aphasia (PPA). Even though there is ongoing research on pathophysiological indices in this field, the use of behavioral variables, and especially speech-derived factors, has drawn little attention in the relevant literature. The present study aims to investigate the possible utility of speech-derived indices, particularly silent pauses, as biomarkers for primary progressive aphasia (PPA). Materials and Methods: We recruited 22 PPA patients and 17 healthy controls, from whom we obtained speech samples based on two elicitation tasks, i.e., cookie theft picture description (CTP) and the patients' personal narration of the disease onset and course. Results: Four main indices were derived from these speech samples: speech rate, articulation rate, pause frequency, and pause duration. In order to investigate whether these indices could be used to discriminate between the four groups of participants (healthy individuals and the three patient subgroups corresponding to the three variants of PPA), we conducted three sets of analyses: a series of ANOVAs, two principal component analyses (PCAs), and two hierarchical cluster analyses (HCAs). The ANOVAs revealed significant differences between the four subgroups for all four variables, with the CTP results being more robust. The subsequent PCAs and HCAs were in accordance with the initial statistical comparisons, revealing that the speech-derived indices for CTP provided a clearer classification and were especially useful for distinguishing the non-fluent variant from healthy participants as well as from the two other PPA taxonomic categories. Conclusions: In sum, we argue that speech-derived indices, and especially silent pauses, could be used as complementary biomarkers to efficiently discriminate between PPA and healthy speakers, as well as between the three variants of the disease.

Does a rabbit have feathers or fur? Development of a 42-item semantic memory test (SMT-42).

OBJECTIVE: We present the preliminary study of the 42-item Semantic Memory Test (SMT-42), a test developed to distinguish semantic variant primary progressive aphasia (svPPA) from the other variants: logopenic (lPPA) and nonfluent/agrammatic (naPPA). The test requires the patient to retrieve the conceptual features of items belonging to different lexical categories. METHODS: In the first study, we administered the French version of the SMT-42 to a population of healthy subjects and to patients with svPPA matched to a subgroup of the healthy subjects. In the second study, we administered the SMT-42 to four groups of patients (with svPPA, lPPA, naPPA and Alzheimer's disease [AD], respectively) to study its capacity to differentiate patients suffering from svPPA from the other patients. RESULTS: In the first study, 109 healthy subjects were included, 15 of whom were paired with 15 subjects presenting with svPPA. In the second study, designed to compare groups presenting a primary progressive aphasia variant and AD, 12 subjects with svPPA, 6 with naPPA and 9 with lPPA were included, along with 21 subjects with AD. The subjects presenting a semantic deficit were clearly distinguished from the others by their results on the SMT-42 (svPPA: mean = 30.0 (5.9); lPPA: mean = 37.8 (3.3), d = 1.5, p = 0.002; naPPA: mean = 39.8 (1.9), d = 1.89, p = 0.001; AD: mean = 38.5 (2.4), d = 1.63, p < 0.001); (svPPA: median = 31; lPPA: median = 38, U = 9, p = 0.002; naPPA: median = 40.5, U = 1.5, p = 0.001; AD: median = 39, U = 13.5, p < 0.001). CONCLUSION: The SMT-42 is simple, rapidly administered (3 minutes on average), easily scored and has good sensitivity, and it appears to be an effective tool for semantic screening in routine clinical practice.

Characterization of the logopenic variant of Primary Progressive Aphasia: A systematic review and meta-analysis.

The linguistic and anatomical variability of the logopenic variant of Primary Progressive Aphasia (lv-PPA) as defined by current diagnostic criteria has been the topic of an intense debate. The present review and meta-analysis aims at characterizing the profile of lv-PPA, by a comprehensive analysis of the available literature on the neuropsychological, neuroimaging, electrophysiological, pathological, and genetic features of lv-PPA. We conducted a systematic bibliographic search, leading to the inclusion of 207 papers. Of them, 12 were used for the Anatomical Likelihood Estimation meta-analysis on grey matter revealed by magnetic resonance imaging data. The results suggest that the current guidelines outline a relatively consistent syndrome, characterized by a core set of linguistic and, to a lesser extent, non-linguistic deficits, mirroring the involvement of left temporal and parietal regions typically affected by Alzheimer Disease pathology. Variations of the lv-PPA profile are discussed in terms of heterogeneity of the neuropsychological instruments and the diagnostic criteria adopted.