Bilingualism in a Case of the Non-fluent/agrammatic Variant of Primary Progressive Aphasia

There is a growing body of research on language impairment in bilingual speakers with neurodegenerative diseases. Evidence as to which language is better preserved is rather inconclusive. Various factors seem to influence language performance, most notably age of acquisition, level of proficiency, immersion and degree of exposure to each language. The present study examined fluency, lexical, discourse and grammatical abilities of a Greek-French late bilingual man with the non-fluent/agrammatic variant of primary progressive aphasia (nfvPPA). Speech samples derived from three different narrative tasks in both languages were analyzed using quantitative production analysis (QPA) and fluency measures. The first aim of the study was to compare the participant's connected speech production to that of Greek-speaking normal controls. The second aim was to determine whether Greek (L1) and French (L2) were differentially impaired. To our knowledge, this is the first report of connected speech deficits in a Greek-speaking patient with PPA and the first study which uses QPA to compare L1 and L2 narratives in a bilingual speaker with PPA. Compared to neurologically healthy controls, our participant was impaired in lexical, discourse and grammatical productivity measures, but did not differ in measures of grammatical accuracy. The presence of dysfluencies, reduced speech rate and simplified syntax is consistent with the pattern of impairment reported for the nfvPPA. Results showed that narrative production measures did not differ significantly between languages. However, they suggest a slightly worse performance in his second, non-dominant, language despite a similar pattern of impairment in both languages. Lengthy exposure to L2 and regular activation of L2 through daily use may explain the preservation of discourse abilities in his non-dominant language. This study calls attention to factors such as language dominance, proficiency, patterns of use, and exposure to a language. These factors play a key role in assessing bilingual individuals with PPA and making clinical decisions

Profiling language and cognition in greek-speaking patients with primary progressive aphasia

Η Πρωτοπαθής Προοδευτική Αφασία (ΠΠΑ) είναι μία σπάνια μορφή Άνοιας η οποία χαρακτηρίζεται από μια αργά εξελισσόμενη γλωσσική διαταραχή. Τα άτομα με ΠΠΑ μπορούν να κατηγοριοποιηθούν σε τρεις κλινικές παραλλαγές (σημασιολογική, μη ρέουσα/αγραμματική και λογοπενική παραλλαγή) βάσει συγκεκριμένων γνωρισμάτων ομιλίας και λόγου. Σκοπός της παρούσας ερευνητικής μελέτης είναι η λεπτομερής καταγραφή των γλωσσικών χαρακτηριστικών της ΠΠΑ και των παραλλαγών της στην Ελληνική γλώσσα, καθώς και η συσχέτισή τους με συνυπάρχοντα γνωστικά ελλείμματα. Οι περισσότερες έρευνες στο χώρο της ΠΠΑ έχουν διεξαχθεί στην Αγγλική γλώσσα και μόνο ένας πολύ μικρός αριθμός μελετών εστιάζεται σε μη αγγλόφωνους ασθενείς. Για τον σκοπό αυτό εξετάστηκαν 13 άτομα με ΠΠΑ καθώς και 9 άτομα με νόσο Alzheimer (ΝΑ) με συμπτώματα ήπιας και μέτριας βαρύτητας. Δεκαπέντε υγιείς ενήλικες, εξισωμένοι ως προς το φύλο, την ηλικία και το επίπεδο εκπαίδευσης, αποτέλεσαν την ομάδα ελέγχου.Primary Progressive Aphasia (PPA) is a degenerative condition characterized by progressive loss of language function. Individuals with PPA are divided into three clinical variants based on distinct speech and language features and patterns of cognitive decline: the semantic variant of PPA (svPPA), the non-fluent/agrammatic variant of PPA (nfvPPA) and the logopenic variant of PPA (lvPPA). The most common types of neurodegeneration in PPA are frontotemporal lobar degeneration (FTD) and Alzheimer’s disease (AD). The main aim of the research was to describe the clinical presentation of PPA and provide a detailed cognitive-linguistic profile of PPA for the Greek-language. The vast majority of studies in PPA involve participants whose native language is English. Detailed reports of PPA in other languages are scarce.Chair of the committee: Andreas Anayiotos, Professor External examiner: Spyridoula Varlokosta, ProfessorComplete

Frontotemporal dementia: a comparative case study of Greek-speaking individuals with the non-fluent and semantic variants of Primary Progressive Aphasia

Presented in 20th International Science of Aphasia Conference, 2019, 23-26 September, Rome. ItalyFrontotemporal Dementia (FTD) is an umbrella term that encompasses degenerative disorders of the frontal and anterior temporal lobes that affect behavior and language. FTD overlaps clinically and pathologically with Primary Progressive Aphasia (PPA). PPA is a degenerative syndrome characterized by progressive loss of language function. The consensus criteria for PPA recognize three variants: the non-fluent/agrammatic variant of PPA (nfvPPA), the semantic variant of PPA (svPPA) and the logopenic variant of PPA (lvPPA) (Gorno-Tempini et al., 2011). Each PPA variant has a specific profile of language impairment, different distribution of atrophy on neuroimaging and different likelihood of underlying molecular pathology. Typically, nfvPPA is associated with fronto-insular atrophy, svPPA with atrophy of the anterior and inferior temporal lobe and lvPPA with atrophy of temporo-parietal regions. The most common types of neurodegeneration in PPA are frontotemporal lobar degeneration and Alzheimer’s disease (Spinelli et al., 2017). FTD includes two out of three PPA variants, the nfvPPA and svPPA, as the most typical pathology of these variants is frontotemporal lobar degeneration. PPA is characterized by a more partial and progressive pattern of damage than stroke-induced aphasia and targets areas such as the anterior temporal lobe that are rarely affected by stroke (Mesulam, 2016). Clinical and neuroimaging research on PPA has advanced our understanding of the language network. It has shown, for example, that the left anterior temporal lobe plays a critical role in single word comprehension and object naming and that the traditional ‘Wernicke’s area’ is important for language repetition and sentence comprehension but not single word comprehension (Mesulam et al., 2019). The aim of this study is to compare the clinical presentation of the language variants of FTD, nfvPPA and svPPA, in two Greek-speaking individuals with PPA. Greek is an underrepresented language in the literature on PPA research. It is a highly inflected and stem-based language. To this end, a comprehensive battery of neuropsychological tests and narrative analysis was employed

Το γνωστικό και γλωσσικό προφίλ ελληνόφωνων ατόμων με πρωτοπαθή προοδευτική αφασία

Primary Progressive Aphasia (PPA) is a degenerative condition characterized by progressive loss of language function. Individuals with PPA are divided into three clinical variants based on distinct speech and language features and patterns of cognitive decline: the semantic variant of PPA (svPPA), the non-fluent/agrammatic variant of PPA (nfvPPA) and the logopenic variant of PPA (lvPPA). The most common types of neurodegeneration in PPA are frontotemporal lobar degeneration (FTD) and Alzheimer’s disease (AD).The main aim of the research was to describe the clinical presentation of PPA and provide a detailed cognitive-linguistic profile of PPA for the Greek-language. The vast majority of studies in PPA involve participants whose native language is English. Detailed reports of PPA in other languages are scarce. To that end, 13 individuals with PPA, at the early and moderate stages of the disease, were evaluated. Nine demographically matched adults with AD have also completed the cognitive-linguistic battery. Fifteen neurotypical adults, matched for gender, age, and education have served as controls. The assessment battery included neuropsychological tools for the evaluation of speech, language, other cognitive domains (attention, memory, executive and visuospatial functions) and mood. Linguistic assessment targeted auditory comprehension, motor speech, narrative production, naming, repetition, reading and writing. In addition, information about the level of functioning and the presence of neuropsychiatric symptoms was collected by each participant’s primary caregiver. Differences were documented in neuropsychological testing and connected speech production between Greek-speaking individuals with AD and PPA. PPA participants were less impaired than AD participants in the delay conditions of episodic memory measures. However, they too were impaired in executive tasks, especially for working memory and phonemic verbal fluency. Naming, single word comprehension, auditory comprehension of complex material, repetition, reading and writing were all affected. The most informative measures in differentiating svPPA and lvPPA from AD participants were repetition of long frequent sentences, frequency of phonological errors, mean sentence length and sentence elaboration index in connected speech. Regarding narrative production, differences between a picture description and a story retell task were found for fluency, lexical selection, discourse and sentence productivity but not for grammatical accuracy measures. For the PPA group, measures of fluency, lexical selection, discourse and sentence productivity were correlated with executive control, short-term memory and to a lesser degree with working memory. Fewer differences between the tasks were documented for the AD group. Both tasks were able to capture connected speech deficits in PPA and AD and in that sense, both methods can be used interchangeably. However, story retell seems to be more sensitive in identifying deficits at the syntactic level of language production and may assist in the differential diagnosis between PPA and AD. Inspection of individual profiles in individuals with PPA revealed heterogeneity in cognitive function, linguistic and narrative discourse abilities. Participants with svPPA presented with more typical phenotypes in comparison to the participants with lvPPA. Non-language cognitive deficits were common in lvPPA. Neuropsychiatric symptoms were reported for lvPPA participants, but to a lesser extent than for FTD participants. Participants with a prominent movement disorder manifested impairment in other areas, including speech, language and cognition. Differences were also documented for 4 participants in cognitive, linguistic abilities and discourse production over time. The pattern of differences in performance of each participant was different. Despite, similar cognitive status at initial assessment, participants with lvPPA have shown greater decline than a participant with svPPA. All three were further affected in memory, writing and lexical retrieval. The lvPPA participants exhibited further difficulty with sentence repetition. One participant presented with a naming impairment. Naming was further affected, and a mild semantic deficit was documented in his second assessment. Further studies with large PPA cohorts and balanced representation of each PPA variant, combining neuropsychological, linguistic and neuroimaging testing could better explore PPA subtyping.Η Πρωτοπαθής Προοδευτική Αφασία (ΠΠΑ) είναι μία σπάνια μορφή Άνοιας η οποία χαρακτηρίζεται από μια αργά εξελισσόμενη γλωσσική διαταραχή. Τα άτομα με ΠΠΑ μπορούν να κατηγοριοποιηθούν σε τρεις κλινικές παραλλαγές (σημασιολογική, μη ρέουσα/αγραμματική και λογοπενική παραλλαγή) βάσει συγκεκριμένων γνωρισμάτων ομιλίας και λόγου. Σκοπός της παρούσας ερευνητικής μελέτης είναι η λεπτομερής καταγραφή των γλωσσικών χαρακτηριστικών της ΠΠΑ και των παραλλαγών της στην Ελληνική γλώσσα, καθώς και η συσχέτισή τους με συνυπάρχοντα γνωστικά ελλείμματα. Οι περισσότερες έρευνες στο χώρο της ΠΠΑ έχουν διεξαχθεί στην Αγγλική γλώσσα και μόνο ένας πολύ μικρός αριθμός μελετών εστιάζεται σε μη αγγλόφωνους ασθενείς. Για τον σκοπό αυτό εξετάστηκαν 13 άτομα με ΠΠΑ καθώς και 9 άτομα με νόσο Alzheimer (ΝΑ) με συμπτώματα ήπιας και μέτριας βαρύτητας. Δεκαπέντε υγιείς ενήλικες, εξισωμένοι ως προς το φύλο, την ηλικία και το επίπεδο εκπαίδευσης, αποτέλεσαν την ομάδα ελέγχου. Η αξιολογητική διαδικασία περιλάμβανε νευροψυχολογικές δοκιμασίες για την εκτίμηση των γλωσσικών, των γνωστικών ικανοτήτων και της διάθεσης των συμμετεχόντων. Η γνωστική αξιολόγηση εστιάστηκε στους τομείς της προσοχής, της μνήμης, των επιτελικών και των οπτικοχωρικών ικανοτήτων. Επιπλέον, αξιολογήθηκε η λεκτική κατανόηση, ο κινητικός μηχανισμός της ομιλίας, ο αφηγηματικός λόγος, η ικανότητα κατονομασίας, επανάληψης, ανάγνωσης και γραφής. Πληροφορίες σχετικά με τη λειτουργικότητα και πιθανά συνοδά ψυχιατρικά συμπτώματα συλλέχθηκαν από τους φροντιστές τους με τη χρήση ερωτηματολογίων. Καταγράφηκαν διαφορές στις γνωστικές και γλωσσικές λειτουργίες μεταξύ των συμμετεχόντων με ΠΠΑ και ΝΑ. Οι δοκιμασίες που βρέθηκε ότι μπορεί να βοηθήσουν στη διαφοροδιάγνωση είναι η επανάληψη προτάσεων με μεγάλο μήκος και αυξημένη συχνότητα εμφάνισης, ο αριθμός των φωνολογικών παραφασιών, το μέσο μήκος πρότασης και ο δείκτης ανάπτυξης πρότασης. Συγκρίνοντας την παραγωγή αφηγηματικού λόγου κατά την περιγραφή μιας εικόνας και την αναδιήγηση μιας ιστορίας, βρέθηκε ότι και οι δύο δοκιμασίες μπορούν να χρησιμοποιηθούν για την καταγραφή ελλειμμάτων. Η αναδιήγηση μιας ιστορίας φαίνεται ότι επιτρέπει επιπρόσθετα την διαφοροδιάγνωση των ατόμων με ΠΠΑ και ΝΑ. Το γνωστικό και γλωσσικό προφίλ κάθε συμμετέχοντα συζητήθηκε σε σχέση με τα ισχύοντα κλινικά κριτήρια. Αναλύοντας τις γνωστικές και γλωσσικές τους δεξιότητες, διαπιστώθηκε ποικιλομορφία, ιδιαίτερα για τους συμμετέχοντες με τη λογοπενική παραλλαγή της νόσου. Από τη μελέτη τεσσάρων περιπτώσεων που αξιολογήθηκαν ξανά μετά από διάστημα ενός έτους, βρέθηκε ότι οι δύο συμμετέχοντες με τη λογοπενική παραλλαγή παρουσίασαν γρηγορότερη έκπτωση, καθώς και έκπτωση σε περισσότερους τομείς, σε σχέση με τους άλλους δύο συμμετέχοντες. Προοπτικές μελέτες με μεγαλύτερο αριθμό συμμετεχόντων και ισορροπημένη αντιπροσώπευση των τριών παραλλαγών της ΠΠΑ είναι απαραίτητες προκειμένου να διερευνηθούν περαιτέρω και να διευρυνθούν τα συμπεράσματα αυτής της μελέτης

Comparing two cases of the non-fluent and semantic variants of primary progressive aphasia using neuropsychological, narrative and acoustic measures

Introduction. PPA is a degenerative syndrome characterized by progressive, relatively isolated, loss of language function. The current consensus criteria for PPA identify three variants: the non-fluent/agrammatic variant of PPA (nfvPPA), the semantic variant of PPA (svPPA) and the logopenic variant of PPA (lvPPA) (Gorno-Tempini et al., 2011), each with a distinct profile of language impairment, distribution of atrophy and underlying pathology. The aim of this study is to compare the clinical presentation of the nfPPA and svPPA, in two Greek-speaking individuals with PPA, using a battery of neuropsychological tests, narrative analysis and acoustic measures. Greek is a highly inflected and stem-based language, underrepresented in the literature on PPA research. Participants. The first participant with the nfvPPA is a 61-year-old man with 6 years of formal education. The second participant with the semantic variant of PPA is a 73-year-old man with 9 years of education. Both participants were assessed five years post-onset. The first participant had a sum of boxes score of 9 on the FTLD-modified Clinical Dementia Rating, whereas the second one a score of 6. The control group consisted of 12 neurologically healthy adults, native Greek speakers, with a mean age of 68.08 (SD = 5.52) years and a mean of 13 (SD = 3.19) years of education. Procedure. Participants were evaluated using a comprehensive battery of neuropsychological tests. Quantitative production analysis (QPA) (Saffran, Berndt, & Schwartz, 1989) was used for the narrative analysis of a picture description and a story retell task. Acoustic analysis was performed in order to calculate temporal measures of participants’ speech. Statistical analysis. Performance of each subject was compared to that of the control sample using the Crawford and Howell’s method (Crawford, Garthwaite, & Porter, 2010). T values were also calculated to compare the scores of the two subjects with reference to the control sample (Crawford, Garthwaite, & Wood, 2010). Results. The participant with the nfvPPA performed worse than the participant with the svPPA on tasks of working memory (p = 0.025), syntactic comprehension (p = 0.014) and reading fluency for words (p < 0.001). Furthermore, he was slower in temporal measures of speech production (Table 1). The participant with the svPPA was more impaired in confrontation naming (p < 0.001), single word comprehension (p < 0.001) and object semantics (p = 0.001). The narrative production measures that differed significantly between the two participants were speech rate (slower for the nfvPPA participant, p = 0.007), average pause duration (longer for the nfvPPA participant, p < 0.001), false starts per min (more for the nfvPPA participant, p = 0.045), proportion of nouns (lower for the svPPA participant, p = 0.012) and closed class words (lower for the nfvPPA participant, p = 0.016). Discussion. The results confirm the distinctive features of both PPA variants: motor speech and syntactic processing difficulties in the case of the participant with the nfvPPA, anomia and a single word comprehension deficit in the case of the participant with the svPPA. Neuropsychological testing combined with narrative and acoustic analysis have enabled the documentation of speech and language deficits present in these cases of PPA and the comparison of the two participants

Comparing Individuals With PPA to Individuals With AD: Cognitive and Linguistic Profiles

Primary Progressive Aphasia (PPA) is a degenerative condition characterized by the progressive loss of language function. In PPA, aphasia is the most prominent deficit at onset. On the other hand, memory deficits are the hallmark of Alzheimer's disease (AD). The first aim of the study was to establish differences on neuropsychological testing and connected speech production between Greek-speaking individuals with AD and PPA. The second aim was to investigate the executive deficit involvement in the two conditions. Ten individuals with PPA and 9 individuals with AD took part in a comprehensive cognitive-linguistic evaluation. Fifteen demographically matched neurologically healthy adults served as controls. Participants were evaluated using a battery of neuropsychological measures. Quantitative production analysis and acoustic analysis were performed to calculate narrative and temporal measures of the participants' speech. Participants with PPA differed significantly from participants with AD on linguistic measures. They performed worse on the long frequent sentences' subtest of the Sentence Repetition Test and they produced fewer narrative and unique words in picture description. They also produced shorter, less elaborated sentences, and made more phonological errors. The two groups did not differ significantly on memory, executive, visuospatial and semantic composite measures. Compared to neurotypical adults, participants with AD were impaired in memory, and executive function. They also exhibited lexical retrieval difficulties, as well as difficulties in linguistic tasks with an increased processing load. Participants with PPA performed within normal limits on the delay conditions of episodic memory measures. However, they too were impaired in executive tasks, especially for short-term memory and verbal fluency. The production of phonological errors, difficulty in repeating long frequent sentences, and the production of simple and short sentences has differentiated participants PPA not only from neurotypical controls but also from participants with AD. No single measure could differentiate the AD group from the other two groups. These findings should be interpreted with caution considering the small sample size

Quantitative connected speech analysis in a case of non-fluent/agrammatic primary progressive aphasia

Presented in 1st Panhellenic Congress on Neuropsychology, 27-29 April 2018, Athens, Greece.OBJECTIVE: Primary progressive aphasia (PPA) is a neurodegenerative syndrome characterized by a selective loss of lan-guage functions. In the nonfluent/agrammatic variant (nfvPPA), speech is slow and hesitant. Utterances are shorter, less complex and contain grammatical errors. Single word production deficits in PPA have been extensively examined. How-ever, connected speech analysis has only recently begun to be systematically studied. The aim of the present study was to investigate connected speech deficits in a Greek-speaking person with nfvPPA.MATERIAL - METHOD: Participant LJ is a 60-year-old right-handed man, with 6 years of formal education. At the time of the study, he had a FTLD-modified CDR score of 9 (MMSE=17/30). A narrative sample was collected using the “cookie theft” picture from BDAE and analyzed following the procedures described by Saffran et al. (1989) for quantitative pro-duction analysis (QPA). QPA summary measures, percentages of dysfluent variables and counts of errors were computed. LJ’s scores were compared to a healthy control group included in a study by Varkanitsa (2012). T-values were calculated using the Crawford and Howell’s method (Crawford and Garthwaite, 2012).RESULTS: Speech rate was 40.37 words per minute. Dysfluencies included silent pauses, filled pauses, false starts, sound distortions and repetitions (23%, 20%, 3%, 2% and 1% of total words produced). LJ produced less nouns (p<.05) and adverbs (p<.025), but more pronouns (p<.0005) and verbs (p<.05) compared to controls. He used less narrative words (p<.05) and more single word utterances (p<.0005).CONCLUSIONS: This case study reports differences between an individual with nfvPPA and healthy controls in lexical selection and discourse productivity measures. It serves as an example of how connected speech analysis may be used for the evaluation of multiple linguistic levels not captured by traditional aphasia tests

Naming intervention in a case of semantic variant of primary progressive aphasia: A 2 year follow up study

Background: The semantic variant of Primary Progressive Aphasia (svPPA), a subtype of Frontotemporal dementia, is a neurodegenerative condition which primarily affects language. Evidence suggests that treatment can improve naming in svPPA and slow the progression of anomia. Aim/s: The purpose of the study was to evaluate a naming intervention which relies on independent practice and to monitor maintenance over two years in a case of svPPA. Method: The participant was diagnosed with mild svPPA (MMSE=28/30, CDR=1). Naming performance was established for the Rossion & Pourtois object set. Three lists (experimental I, experimental II and control) were formed from items which were not named by the participant, matched for relevant psycholinguistic variables. Intervention focused on the semantic features of words. Training lasted for two weeks and consisted of therapistled and daily home practice. Independent practice of the first set continued for another 4 weeks and included copying of the list and confrontation naming of picture cards with the written word behind the picture to assist errorless learning. Follow-up assessments were undertaken at 6 months, one year and two years postintervention. Results: Improvement in naming was still evident at the 2 years follow-up assessment (p=0.008). The difference between the first experimental and control list was significant at 2 years post-intervention (U=761, p<0.05). No difference between the second experimental and control list was found beyond 6 months. Conclusion: Results are consistent with previous research, confirming improvement in naming and deterioration after a period with no practice. This case study suggests that daily independent practice may be used as a means of maintaining treatment gains in svPPA