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A) Boston Diagnostic Aphasia Examination Profiles for nfvPPA and stroke groups. Normal values illustrated as broken black line. Colour coding of individual profiles based on Aphasia Severity Rating Scale;
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Patients with non-fluent aphasias display impairments of expressive and receptive grammar. This has been attributed to deficits in processing configurational and hierarchical sequencing relationships. This hypothesis had not been formally tested. It was also controversial whether impairments are specific to language, or reflect domain general defic...
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... sentence comprehension task on the 'verb and sentences test' (VAST) ( Bastiaanse et al., 2003) (mean 87.5%, range 70-100%). Similarly, the patients varied in their degree of expressive agrammatism, but none was completely unimpaired. Samples of speech from the participants are available in supplementary materials, and speech profiles are shown in Fig. 1A. BDAE profiles were independently rated by authors TEC, HR and KP. Inter-rater reliability for grammatical form was high, with pairwise Pearson correlations of 0.87, 0.85 and 0.85. Areas of significant grey or white matter loss are shown in Fig. 1B, upper panel. It is widely recognised that patients with nfvPPA report difficulties with ...
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... from the participants are available in supplementary materials, and speech profiles are shown in Fig. 1A. BDAE profiles were independently rated by authors TEC, HR and KP. Inter-rater reliability for grammatical form was high, with pairwise Pearson correlations of 0.87, 0.85 and 0.85. Areas of significant grey or white matter loss are shown in Fig. 1B, upper panel. It is widely recognised that patients with nfvPPA report difficulties with understanding speech ( Goll et al., 2010;Cope et al., 2014;Grube et al., 2016). We asked the patients in this study to complete visual analogue scales assessing their difficulty with 'Understanding speech in a quiet room', 'Telling the direction a ...
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... asked the patients in this study to complete visual analogue scales assessing their difficulty with 'Understanding speech in a quiet room', 'Telling the direction a sound is coming from', 'Understanding speech in a noisy restaurant', 'Hearing announcements at a bus or rail station' as well as 'How loud do people tell you your TV is?' Compared to a matched group of controls, patients differed only in reporting more difficulty with 'Understanding speech in a quiet room' (p=0.02). Of the Boston Diagnostic Aphasia Examination (BDAE) subscores, this difficulty was strongly correlated only with 'Grammatical form' (r 2 =0.778, p < 0.001, Supplementary Figure 1). ...
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... were: a single stroke of at least six months chronicity resulting in at least one month of non-fluent aphasia, with MRI evidence of involvement of either left inferior trigone or operculum. Samples of speech from the participants are available in supplementary materials, and speech profiles (triple marked by authors TEC, HR and KP) are shown in Fig. 1A. On the whole, the stroke group had more severe language impairments than the nfvPPA group. All had some degree of impairment of grammatical form in free speech, and all but two had impairment of receptive grammar on the VAST (mean 70%, range 40-100%). Lesion overlap maps are shown in Fig. 1B, lower ...
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... (triple marked by authors TEC, HR and KP) are shown in Fig. 1A. On the whole, the stroke group had more severe language impairments than the nfvPPA group. All had some degree of impairment of grammatical form in free speech, and all but two had impairment of receptive grammar on the VAST (mean 70%, range 40-100%). Lesion overlap maps are shown in Fig. 1B, lower ...
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... and nine nearest-neighbour controls were used to create a DARTEL template. This was then applied to the remaining 27 controls. Resultant images were normalised to MNI space in SPM12 with an 8 mm smoothing kernel, and separate statistical comparisons were performed for grey and white matter, with total intracranial volume and age as covariates (Fig. ...
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... performance for each individual sequence is shown in Fig. 3A, and the results of the signal detection theory analysis are illustrated in Fig. 3B. The hierarchical cluster analysis is illustrated in Fig. 4. Performance did not differ between sequences heard during exposure and test phases (Fig. 3A sequences 1-3) and those that were novel during the test phase (Fig. 3A sequences 4-6), so these were collapsed. Results are presented for the non-parametric discrimination measure A′, but the same pattern of findings was present for the parametric equivalent d′ (Supplementary Tables 2 and ...
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... There are a number of possible reasons for this. It might be a consequence of the tone language being more affected by the basic auditory sequence processing deficits previously demonstrated in nfvPPA ( Goll et al., 2010;Grube et al., 2016). Alternatively, it might reflect involvement of the right IFC, which was spared in the stroke group (Fig. 1B), and is posited to have a role in prosodic and tonality based judgments. Nonetheless, patients with nfvPPA maintained the same pattern of learning as the other groups (Fig. 4), demonstrating that this deficit is a specific difficulty with processing tonal input rather than a breakdown of the separation of phonological vs tonal ...
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... between the two are vastly different. Nor can the results be trivially explained by floor effects in processing the more complex relationships for the following reasons: 1) the nfvPPA group show strikingly parallel behaviour in relation to the control group, consistent with a proportional impairment even on the linear sequencing operation (Fig. 3B, panel 1); 2) the stroke patients may well have reached a floor in performance on the complex sequences in the nonsense word task, but even excluding this group did not cause the group by complexity interaction to approach significance; 3) all groups improved over the three testing runs, and performance improvement was parallel in relation to ...
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... these findings provide a theoretical basis upon which an exposure-based speech therapy for grammar could be built with the aim of improving subjective difficulty with speech comprehension (Supplementary Figure 1). The results imply that there is potential for improvement from an intensive paradigm based on repeated exposuretest cycles. ...
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... The finding that learning did not generalise across modalities implies that such a therapy would need to use linguistic material, as it would be unlikely to be so well learnt with non-linguistic material or to transfer across domains. In contrast, the finding that patients were able to generalise perfectly from sequences heard during exposure (Fig. 3A, sequences 1-3), to those that were novel during the test phase (Fig. 3A, sequences 4-6), to the extent that performance did not differ, suggests that such a therapy might not need to be comprehensive with regards to specific sentence structures. Instead, it is envisaged that a graded programme could be designed, such that training focusses initially ...
Citations
... The learner may receive reinforcement when that salient stimulus is paired to a relevant response, but do not otherwise receive explicit feedback. Individuals with aphasia and traumatic brain injury also evidence this type of learning in implicit learning paradigms (Cope et al., 2017;Peñaloza et al., 2015Peñaloza et al., , 2017Schuchard et al., 2017;Schuchard & Thompson, 2017;Vallila-Rohter & Kiran, 2013a, 2013b, suggesting that it relies on brain systems that differ from those underlying the language impairment. However, there is not consensus on the brain regions or networks associated with implicit learning of language-like stimuli. ...
Language rehabilitation centers on modifying its use through experience-based neuroplasticity. Implicit statistical learning of language is essential to its acquisition and likely its rehabilitation following brain injury, but its corresponding brain networks remain elusive. Coordinate-based meta-analyses were conducted to identify common and distinct brain activity across 25 studies coded for meta-data and experimental contrasts (grammatical or non-grammatical). The resultant brain regions served as seeds for profiling functional connectivity in large task-independent and task-dependent data sets. Hierarchical clustering of these profiles grouped brain regions into three subnetworks associated with grammatical/non-grammatical processes. Functional decoding clarified the mental operations associated with those subnetworks. Results support a left-dominant language sub-network and two cognitive control networks as scaffolds for grammar rule identification, maintenance, and application in healthy adults. These data suggest that cognitive control is necessary to track regularities across stimuli and imperative for rule identification and application of grammar. Future empirical investigation of these brain networks in language learning in individuals with brain injury will clarify their prognostic role in language recovery.
... The features and corresponding response (the measured amount of litter in kg km −1 ) are used to fit a random forest regression are highly correlated will be assigned to clusters. We use hierarchical Ward-linkage clustering for this, based on Spearman rank-order correlations (McCann et al., 2019;Cope et al., 2017). This way, the total set of features is reduced to 59 feature clusters. ...
... Correlated features are put into clusters using hierarchical Ward-linkage clustering (McCann et al., 2019;Cope et al., 2017). ...
Coastlines potentially harbor a large part of litter entering the oceans such as plastic waste. The relative importance of the physical processes that influence the beaching of litter is still relatively unknown. Here, we investigate the beaching of litter by analyzing a data set of litter gathered along the Dutch North Sea coast during extensive beach cleanup efforts between the years 2014–2019. This data set is unique in the sense that data is gathered consistently over various years by many volunteers (a total of 14,000), on beaches which are quite similar in substrate (sandy). This makes the data set valuable to identify what environmental variables might play an important role in the beaching process, and to explore the variability of beach litter. We investigate this by fitting a random forest machine learning regression model to the observed litter concentrations. We find that especially tides play an important role, where an increasing tidal variability and tidal height lead to less litter found on beaches. Relatively straight and exposed coastlines appear to accumulate more litter. The regression model indicates that transport of litter through the marine environment is also important in explaining beach litter variability. By understanding what processes cause the accumulation of litter on the coast, recommendations can be given for more effective removal of litter from the marine environment. We estimate that 16,000–31,400 kilograms (95 % confidence interval) of litter are located on the 365 kilometers of Dutch North Sea coastline.
... [1] They found, however, that 3 of 6 individuals with nfaPPA presented with word comprehension deficits and 2 presented with object recognition difficulty, consistent with mixed PPA. Cope et al [16] found that individuals with nfaPPA performed more poorly than those with stroke aphasia in processing a tone-based language and attributed this finding to the auditory processing deficit in nfaPPA reported by Grube et al. [15] In a related case report, Utianski et al [17] described the evolution of disproportionate difficulty with comprehension of spoken compared to written language, and difficulty perceiving environmental sounds and common musical tunes, in a 65-yearold woman with PPA and apraxia of speech whose clinical presentation became characteristic of nfaPPA. ...
The premise of this study is that spoken word recognition and object knowledge are impaired in semantic variant primary progressive aphasia (PPA) (svPPA) and are spared in logopenic variant (lvPPA) and nonfluent agrammatic primary progressive aphasia (nfaPPA) at disease onset. Over time, however, there may be heterogeneity in these abilities in lvPPA and nfaPPA. We hypothesized that individuals with svPPA would demonstrate poorer performance on baseline spoken word recognition and object knowledge than those with lvPPA and nfaPPA) as documented in the literature, but that rates of decline over time on spoken word recognition and object knowledge would be similar in all 3 PPA variants because these become less distinguishable with disease progression.
The aim of this study was to investigate longitudinal patterns of decline in spoken word recognition and object knowledge across PPA variants.
Ninety-five individuals with PPA completed the Semantic Word Picture Matching and Semantic Associates tests at baseline to establish expected performance in these areas. Thirty-five individuals completed follow-up testing.
The distributions of trichotomized mean rates of decline in object knowledge were similar for lvPPA and svPPA (P = .05). There were weak negative correlations between symptom duration and baseline scores on Semantic Word Picture Matching (r[37] = −0.399, P = .01), and baseline scores on Semantic Associates (r[37] = −0.394, P = .01) in lvPPA.
Degradation of spoken word recognition and object knowledge occurs over time in lvPPA. Further investigation of the receptive language deficits in PPA is warranted to characterize language changes that lessen the distinctions between PPA variants with disease progression.
... People with PPA may benefit from physiologically informed cognitive rehabilitation strategies, akin to strategies designed to enhance neuroplasticity after stroke aphasia [92,93]: recent work suggests that people with all major forms of PPA have retained capacity for perceptual learning of degraded speech [52••], and that patients with nfvPPA show preserved faculty for artificial grammar learning [94], suggesting the need for future trials focused on exposure-based approaches to rehabilitation of agrammatism and degraded speech perception. In svPPA, right-lateralised brain regions show elevated activity in magnetoencephalography when listening to spoken words, whilst dorsal regions appear to compensate for damaged ventral regions when patients read irregular words, together suggesting a degree of functional plasticity in brain networks with relatively preserved integrity [63,64]. ...
Purpose of Review
The term primary progressive aphasia (PPA) refers to a diverse group of dementias that present with prominent and early problems with speech and language. They present considerable challenges to clinicians and researchers.
Recent Findings
Here, we review critical issues around diagnosis of the three major PPA variants (semantic variant PPA, nonfluent/agrammatic variant PPA, logopenic variant PPA), as well as considering ‘fragmentary’ syndromes. We next consider issues around assessing disease stage, before discussing physiological phenotyping of proteinopathies across the PPA spectrum. We also review evidence for core central auditory impairments in PPA, outline critical challenges associated with treatment, discuss pathophysiological features of each major PPA variant, and conclude with thoughts on key challenges that remain to be addressed.
Summary
New findings elucidating the pathophysiology of PPA represent a major step forward in our understanding of these diseases, with implications for diagnosis, care, management, and therapies.
... These tasks are well suited for the separate investigation of the learning of linguistic regularities (e.g., non-random sequences of sounds) and nonverbal sequences (e.g., repetitive patterns of visual forms or hand movements). Artificial grammar and verbal sequence learning may tap similar underlying cognitive constructs, especially the acquisition of phonemic sequences and structures (Conway & Pisoni, 2008;Cope et al., 2017), which may be a central feature of glossolalia (Samarin, 1973). Consider the sequence learning task presented in Fig. 1b. ...
... In the phoneme sequence condition, the sequence of the auditorily presented letters, but not their visual-spatial locations, had a repeating pattern. phonological sequences/artificial grammar and non-linguistic sequences is independent (Cope et al., 2017). ...
... Based on the assumption of Samarin (1973), which highlighted the importance of implicitly acquired phonetic rules in glossolalia, the main hypothesis of the present study was that glossolalia is associated with an enhancement in statistical learning abilities for language regularities, including both artificial grammar and phonemic sequencing learning. In contrast, we hypothesized similar performances in glossolalists and non-glossolalists on the nonverbal response sequence condition, which is not directly related to language acquisition (Cope et al., 2017;Goschke et al., 2001). Finally, we tested the hypothesis that more intensive glossolalia activities would be associated with better verbal statistical learning. ...
Glossolalia (“speaking in tongues”) is a rhythmic utterance of word‐like strings of sounds, regularly occurring in religious mass gatherings or various forms of private religious practices (e.g., prayer and meditation). Although specific verbal learning capacities may characterize glossolalists, empirical evidence is lacking. We administered three statistical learning tasks (artificial grammar, phoneme sequence, and visual‐response sequence) to 30 glossolalists and 30 matched control volunteers. In artificial grammar, participants decide whether pseudowords and sentences follow previously acquired implicit rules or not. In sequence learning, they gradually draw out rules from repeating regularities in sequences of speech sounds or motor responses. Results revealed enhanced artificial grammar and phoneme sequence learning performances in glossolalists compared to control volunteers. There were significant positive correlations between daily glossolalia activity and artificial grammar learning. These results indicate that glossolalists exhibit enhanced abilities to extract the statistical regularities of verbal information, which may be related to their unusual language abilities.
... Although we did not test the SD patients in the current study on their knowledge of the stimulus words, we know from substantial previous research and clinical experience in SD that the patients would easily repeat PLAY or PLAYED or PLATE, but would not necessarily know the words' identities in the full sense of understanding their meanings. There is evidence from non-human primates that right sided frontoetemporal interactions support structured sequence learning (Wilson et al., 2013;Wilson, Marslen-Wilson, & Petkov, 2017), and that similar analysis strategies are employed to learn artificial grammars in healthy (Wilson, Smith, & Petkov, 2015) and aphasic (Cope et al., 2017b) human listeners. However, these paradigms were explicitly designed to be independent of semantics, and hence represent a very different cognitive task to that described here. ...
In the healthy human brain, the processing of language is strongly lateralised, usually to the left hemisphere, while the processing of complex non-linguistic sounds recruits brain regions bilaterally. Here we asked whether the anterior temporal lobes, strongly implicated in semantic processing, are critical to this special treatment of spoken words. Nine patients with semantic dementia (SD) and fourteen age-matched controls underwent magnetoencephalography and structural MRI. Voxel based morphometry demonstrated the stereotypical pattern of SD: severe grey matter loss restricted to the anterior temporal lobes, with the left side more affected. During magnetoencephalography, participants listened to word sets in which identity and meaning were ambiguous until word completion, for example PLAYED versus PLATE. Whereas left-hemispheric responses were similar across groups, patients demonstrated increased right hemisphere activity 174-294 msec after stimulus disambiguation. Source reconstructions confirmed recruitment of right-sided analogues of language regions in SD: atrophy of anterior temporal lobes was associated with increased activity in right temporal pole, middle temporal gyrus, inferior frontal gyrus and supramarginal gyrus. Overall, the results indicate that anterior temporal lobes are necessary for normal and efficient lateralised processing of word identity by the language network.
... In cases where tests and language structures were similar over different test sessions or conditions (e.g., Cope et al., 2017;Goranskaya et al., ;Mueller et al., 2010), we combined the means and SDs from each of the multiple test sessions, and computed the one sample difference from chance. The pooled mean was simply computed as the arithmetic mean across the sessions, weighted by number of participants in the session. ...
Artificial grammar learning (AGL) has become an important tool used to understand aspects of human language learning and whether the abilities underlying learning may be unique to humans or found in other species. Successful learning is typically assumed when human or animal participants are able to distinguish stimuli generated by the grammar from those that are not at a level better than chance. However, the question remains as to what subjects actually learn in these experiments. Previous studies of AGL have frequently introduced multiple potential contributors to performance in the training and testing stimuli, but meta‐analysis techniques now enable us to consider these multiple information sources for their contribution to learning—enabling intended and unintended structures to be assessed simultaneously. We present a blueprint for meta‐analysis approaches to appraise the effect of learning in human and other animal studies for a series of artificial grammar learning experiments, focusing on studies that examine auditory and visual modalities. We identify a series of variables that differ across these studies, focusing on both structural and surface properties of the grammar, and characteristics of training and test regimes, and provide a first step in assessing the relative contribution of these design features of artificial grammars as well as species‐specific effects for learning.
... Emerging evidence suggests that generic disorders of nonverbal auditory information processing may underpin the canonical PPA syndromes [40,[94][95][96] and that these syndromes may further be stratified by profiles of autonomic reactivity to emotional and other salient stimuli [61,97,98]. This evidence dovetails with the recent finding that implicit auditory sequence learning is retained in nfvPPA [99]. Such observations could motivate novel biomarkers and treatment strategies that do not depend on specific language capacities (a practical advantage in mounting large-scale, international trials in PPA). ...
The primary progressive aphasias are a heterogeneous group of focal ‘language-led’ dementias that pose substantial challenges for diagnosis and management. Here we present a clinical approach to the progressive aphasias, based on our experience of these disorders and directed at non-specialists. We first outline a framework for assessing language, tailored to the common presentations of progressive aphasia. We then consider the defining features of the canonical progressive nonfluent, semantic and logopenic aphasic syndromes, including ‘clinical pearls’ that we have found diagnostically useful and neuroanatomical and other key associations of each syndrome. We review potential diagnostic pitfalls and problematic presentations not well captured by conventional classifications and propose a diagnostic ‘roadmap’. After outlining principles of management, we conclude with a prospect for future progress in these diseases, emphasising generic information processing deficits and novel pathophysiological biomarkers.
Electronic supplementary material
The online version of this article (10.1007/s00415-018-8762-6) contains supplementary material, which is available to authorized users.
INTRODUCCIÓN:
Las demencias son un conjunto de trastornos neurocognitivos, en personas con edad menor a 65 años sobresale la demencia frontotemporal, síndrome neurodegenerativo heterogéneo que tiene dos grandes variantes: conductual y afasia primaria progresiva. En esta última se describen tres variantes: no fluente, semántica y logopénica, que exigen en la práctica conocimientos actualizados para su diferenciación y comprensión. El objetivo de este escrito es hacer una revisión narrativa sobre las tres variantes clínicas de la afasia primaria progresiva, profundizando en diagnóstico, evolución, características imagenológicas y manejo.
MATERIALES Y MÉTODOS:
Artículo de revisión narrativa a partir del estado del arte en literatura biomédica sobre demencia frontotemporal, afasia primaria progresiva y sus variantes.
RESULTADOS:
El compromiso del lenguaje y de otras funciones cognitivas, así como los hallazgos imagenológicos, son heterogéneos en las tres variantes. Semiológicamente, la afasia primaria progresiva no fluente se caracteriza por apraxia del habla, la variante logopénica por fallas en la nominación y la variante semántica por fallas en el significado del mensaje. El compromiso imagenológico en la afasia primaria progresiva no fluente es más frontoinsular y corticosubcortical; en la variante semántica es habitualmente temporal del lado dominante; y en la variante logopénica priman alteraciones temporoparietales. No hay tratamiento específico, pero se puede vincular algunas opciones farmacológicas con procesos/técnicas de rehabilitación del lenguaje.
CONCLUSIÓN:
Si bien se trata de una forma heterogénea de demencia, tiene características clínicas (síntomas, signos y evolución) e imagenológicas importantes a la hora de su detección y diagnóstico en ambientes clínicos.
