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Background Aims Method Main Contribution Conclusions & Implications WHAT THIS PAPER ADDS What is already known on the subject What this paper adds to the existing knowledge What are the potential or actual clinical implications of this work? Speech sound disorders (SSDs) in children are heterogeneous. Differentiating children with SSDs into distinct subtypes is important so that each child receives a treatment approach well suited to the particular difficulties they are experiencing.To study the distinct underlying processes that differentiate phonological processing, phonological planning or motor planning deficits.The literature on the nature of SSDs is reviewed to reveal diagnostic signs at the level of distal causes, proximal factors and surface characteristics.Subtypes of SSDs may be identified by linking the surface characteristics of the children's speech to underlying explanatory proximal factors. The proximal factors may be revealed by measures of speech perception skills, phonological memory and speech–motor control. The evidence suggests that consistent phonological disorder (CPD) can be identified by predictable patterns of speech error associated with speech perception errors. Inconsistent phonological disorder (IPD) is associated with a deficit in the selection and sequencing of phonemes, that is, revealed as within‐word inconsistency and poor phonological memory. The motor planning deficit that is specific to childhood apraxia of speech (CAS) is revealed by transcoding errors on the syllable repetition task and an inability to produce [pətəkə] accurately and rapidly.Children with SSDs form a heterogeneous population. Surface characteristics overlap considerably among those with severe disorders, but certain signs are unique to particular subtypes. Careful attention to underlying causal factors will support the accurate diagnosis and selection of personalized treatment options. SSD in children are heterogenous, with numerous subtypes of primary SSD proposed. Diagnosing the specific subtype of SSD is important in order to assign the most efficacious treatment approach for each child. Identifying the distinct subtype for each child is difficult because the surface characteristics of certain subtypes overlap among categories (e.g., CPD or IPD; CAS).SSD in children are heterogenous, with numerous subtypes of primary SSD proposed. Diagnosing the specific subtype of SSD is important in order to assign the most efficacious treatment approach for each child. Identifying the distinct subtype for each child is difficult because the surface characteristics of certain subtypes overlap among categories (e.g., CPD or IPD; CAS). The diagnostic challenge might be eased by systematic attention to explanatory factors in relation to the surface characteristics, using specific tests for this purpose. Word identification tasks tap speech perception skills; repetition of short versus long strings of nonsense syllables permits observation of phonological memory and phonological planning skills; and standard maximum performance tests provide considerable information about speech motor control.The diagnostic challenge might be eased by systematic attention to explanatory factors in relation to the surface characteristics, using specific tests for this purpose. Word identification tasks tap speech perception skills; repetition of short versus long strings of nonsense syllables permits observation of phonological memory and phonological planning skills; and standard maximum performance tests provide considerable information about speech motor control. Children with SSDs should receive comprehensive assessments of their phonological processing, phonological planning and motor planning skills frequently, alongside examinations of their error patterns in connected speech. Such assessments will serve to identify the child's primary challenges currently and as they change over developmental time.Children with SSDs should receive comprehensive assessments of their phonological processing, phonological planning and motor planning skills frequently, alongside examinations of their error patterns in connected speech. Such assessments will serve to identify the child's primary challenges currently and as they change over developmental time. [ABSTRACT FROM AUTHOR]

We encountered two acute stroke cases with apraxia of speech (AOS) due to left internal border‐zone infarction. Neither case had a lower precentral gyrus lesion in the left frontal lobe. The findings obtained confirmed the relationship between AOS with improvement and damage to the left frontal aslant tract (FAT) in stroke. FAT integrity has potential as a prognostic imaging biomarker of AOS. It is important to visualize the fiber connections of white matter using diffusion tensor tractography, which will predict the outcome of AOS. [ABSTRACT FROM AUTHOR]

The cerebellum is traditionally known to subserve motor functions. However, for several decades, the concept of the "cerebellar cognitive affective syndrome" has evolved. Studies in healthy participants and patients have confirmed the cerebellar role in language. The exact involvement of the cerebellum regarding cerebellar aphasia remains uncertain. We included 43 cerebellar stroke patients who were tested at 3 months post-onset with the Boston Naming Test (BNT), the Token Test (TT), and the Diagnostic Instrument for Mild Aphasia (DIMA). Lesion side (left/right) and volume (cm3) were investigated. Patients significantly deviated on the following: BNT (p<0.001), TT (p<0.05), DIMA subtests: sentences repetition (p=0.001), semantic odd-picture-out (p<0.05), sentence completion (p<0.05) without an effect of lesion location (left/right) or volume (cm3) (p>0.05). Our clinical study confirms a non-lateralized cerebellar aphasia post-stroke, characterized by impairments in word retrieval, phonology, semantics, and syntax resembling cerebral-induced aphasia. The integral cerebellum appears to interact with eloquent cortico-subcortical language areas. [ABSTRACT FROM AUTHOR]

Ataxia-ocular apraxia 2 (AOA2) is a rare neurodegenerative autosomal recessive disorder with no effective treatment. In this study, we present the case of a patient diagnosed with AOA2, who experienced walking instability and uncoordinated movement. The patient underwent transcranial alternating current stimulation (tACS) treatment for 4 weeks with follow-up after 1 month. The effectiveness of the treatment was evaluated using the International Cooperative Ataxia Rating Scale (ICARS), the Scale for the Assessment and Rating of Ataxia (SARA), the 9-Hole Peg Test (9HPT), and functional near-infrared spectroscopy (fNIRS). Following treatment, the patient's ataxia symptoms showed significant improvement and continued to be alleviated during the follow-up period, suggesting a lasting effect of tACS treatment. Our findings from this case study provide compelling evidence for the potential of tACS as a treatment option for AOA2. [ABSTRACT FROM AUTHOR]

Speech is the most common means of communication in humans. Any defect in accurate speech production ability results in the development of speech sound disorder (SSD), a condition that can significantly impair an individual's academic performance, social interactions, and relationships with peers and adults. This study investigated the genetic basis of SSD in three Pakistani families. We performed family-based genome-wide parametric linkage analysis and homozygosity mapping in three consanguineous families with SSD from the Punjab province of Pakistan. The Test for Assessment of Articulation and Phonology in Urdu (TAAPU) was used to analyze the speech articulation data and determine the Percentage Correct Consonants (PCC) score. The PCC score defined the affected and unaffected individuals in each family. Parametric linkage analysis revealed a linkage to chromosome 5 (5q21.3-5q23.1) with a significant logarithm of the odds (LOD) score of 3.13 in a Pakistani family with specific language impairment-97 (PKSLI-97) under an autosomal recessive mode of inheritance. The other two families showed a suggestive linkage at 6p22.1, 14q12, and 16q12.1 under the recessive mode of inheritance. Interestingly, homozygosity mapping showed a loss of heterozygosity in the linkage region at 5q15-5q23.1, shared among seven affected (mostly in the younger generation) and one unaffected individual of PKSLI-97. Our analysis identified the 6p22 locus previously implicated in dyslexia, childhood apraxia of speech (CAS), and language impairment, confirming the role of KIAA0319 and DCDC2 in this locus. These findings provide statistical evidence for the genomic regions associated with articulation disorder and offer future opportunities to further the role of genes in speech production. [ABSTRACT FROM AUTHOR]

AbstractOverviewMethodResultConclusionNon-word repetition (NWR) is one of the most effective predictors of language impairments in children as it has been found to correlate with various language measures and the association between NWR and vocabulary is well documented in typically developing (TD) studies. However, there is a dire need for investigations of language skills in Kuwaiti Arabic individuals with Down Syndrome, and this study set out to fill a gap in this field.In this paper, we compare the vocabulary and NWR skills of a group of 48 individuals with DS aged 6–20 years to a group of 44 TD children aged 3–10 years matched on nonverbal IQ. Furthermore, we investigate the correlations among these language measures in the two groups and examine whether NWR can predict receptive and expressive vocabulary in these two groups.Results found DS participants performed significantly less than the TD group on the three language measures (receptive vocabulary t(90)= −3.17, p < .01, expressive vocabulary t(90)= −3.27, p < .01, and NWR t(90)= −8.32, p < .01). Moreover, there were strong correlations between NWR and vocabulary (receptive and expressive) in the TD group but not the DS group.Findings supported the working memory model and the phonological processing account for the TD group. On the other hand, the poor association between NWR and vocabulary in the DS group might be due to poor phonological discrimination difficulties and speech discrimination difficulties. [ABSTRACT FROM AUTHOR]

This study compared language samples from typically developing 4-year-olds who spoke African American English (AAE), Jamaican English (JE), or Mainstream American English (MAE) to assess the value of using language sample analysis (LSA) measures for characterising language use across dialects of English. Specific LSA metrics included mean length of utterance (MLU) in morphemes and in words, the Index of Productive Syntax (IPSyn), Developmental Sentence Scoring (DSS) and measures of lexical diversity. Children demonstrated diverse linguistic patterns across dialects, but a Kruskal–Wallis H test did not reveal significant differences in scores obtained through LSA measures. Notably, the IPSyn captured morphosyntactic structures in each category across dialects where prior research has highlighted limitations. This preliminary study uses a case-based approach to illustrate the applicability of LSAs in describing linguistic variations across children who speak different dialects of English. Moreover, the findings from this study underscore the potential use of LSAs in describing linguistic patterns to support the characterisation of communication profiles for culturally and linguistically diverse children. [ABSTRACT FROM AUTHOR]

BackgroundAimsMethods & ProceduresOutcomes & ResultsConclusionsSelf-advocacy scripts are brief, scripted narratives that people with aphasia (PWA) use to self-disclose their aphasia, explain what it is, and request supportive communicative behaviors from communication partners (CPs). In previous research, PWA have reported that self-advocacy statements, including self-advocacy scripts, facilitate their interactions with unfamiliar CPs. To understand the benefits of self-advocacy scripts, it is important to directly investigate how unfamiliar CPs respond to them.The aim of this study was to characterize the thoughts and emotions reported by unfamiliar adults after listening to self-advocacy scripts produced by speakers with Broca’s aphasia and apraxia of speech.This experimental study included 238 adult research participants without aphasia. Each participant was randomly assigned to listen to one script produced by a speaker with Broca’s aphasia and apraxia of speech: either a self-advocacy script or a control script about the weather that does not mention the speaker’s aphasia. Then, they answered one open-ended question (analyzed using a codebook approach) and eight rating scale questions (analyzed using Bayesian linear regression) about the thoughts and emotions they experienced while listening to the speaker.Compared to the control script, the self-advocacy script improved listeners’ knowledge about the speakers’ condition, their perception of the speakers’ intelligence, and their patience and empathy for the speakers.Aphasia self-advocacy scripts can have powerful, positive effects on unfamiliar listeners. The present results highlight the importance of providing support in clinical practice for collaborative development of self-advocacy scripts between people with aphasia and their clinicians. [ABSTRACT FROM AUTHOR]

Background: Dementia is a general term for several progressive neurodegenerative disorders including Alzheimer's disease. Timely and accurate detection is crucial for early intervention. Advancements in artificial intelligence present significant potential for using machine learning to aid in early detection. Objective: Summarize the state-of-the-art machine learning-based approaches for dementia prediction, focusing on non-invasive methods, as the burden on the patients is lower. Specifically, the analysis of gait and speech performance can offer insights into cognitive health through clinically cost-effective screening methods. Methods: A systematic literature review was conducted following the PRISMA protocol (Preferred Reporting Items for Systematic Reviews and Meta-Analyses). The search was performed on three electronic databases (Scopus, Web of Science, and PubMed) to identify the relevant studies published between 2017 to 2022. A total of 40 papers were selected for review. Results: The most common machine learning methods employed were support vector machine followed by deep learning. Studies suggested the use of multimodal approaches as they can provide comprehensive and better prediction performance. Deep learning application in gait studies is still in the early stages as few studies have applied it. Moreover, including features of whole body movement contribute to better classification accuracy. Regarding speech studies, the combination of different parameters (acoustic, linguistic, cognitive testing) produced better results. Conclusions: The review highlights the potential of machine learning, particularly non-invasive approaches, in the early prediction of dementia. The comparable prediction accuracies of manual and automatic speech analysis indicate an imminent fully automated approach for dementia detection. [ABSTRACT FROM AUTHOR]

Background and purpose: Logopenic variant primary progressive aphasia (lvPPA) is a major variant presentation of Alzheimer's disease (AD) that signals the importance of communication dysfunction across AD phenotypes. A clinical staging system is lacking for the evolution of AD‐associated communication difficulties that could guide diagnosis and care planning. Our aim was to create a symptom‐based staging scheme for lvPPA, identifying functional milestones relevant to the broader AD spectrum. Methods: An international lvPPA caregiver cohort was surveyed on symptom development under an 'exploratory' survey (34 UK caregivers). Feedback from this survey informed the development of a 'consolidation' survey (27 UK, 10 Australian caregivers) in which caregivers were presented with six provisional clinical stages and feedback was analysed using a mixed‐methods approach. Results: Six clinical stages were endorsed. Early symptoms included word‐finding difficulty, with loss of message comprehension and speech intelligibility signalling later‐stage progression. Additionally, problems with hearing in noise, memory and route‐finding were prominent early non‐verbal symptoms. 'Milestone' symptoms were identified that anticipate daily‐life functional transitions and care needs. Conclusions: This work introduces a new symptom‐based staging scheme for lvPPA, and highlights milestone symptoms that could inform future clinical scales for anticipating and managing communication dysfunction across the AD spectrum. [ABSTRACT FROM AUTHOR]

Background and objectives: Nonfluent variant primary progressive aphasia (nfvPPA) and primary progressive apraxia of speech (PPAOS) can be precursors to corticobasal syndrome (CBS). Details on their progression remain unclear. We aimed to examine the clinical and neuroimaging evolution of nfvPPA and PPAOS into CBS. Methods: We conducted a retrospective longitudinal study in 140 nfvPPA or PPAOS patients and applied the consensus criteria for possible and probable CBS for every visit, evaluating limb rigidity, akinesia, limb dystonia, myoclonus, ideomotor apraxia, alien limb phenomenon, and nonverbal oral apraxia (NVOA). Given the association of NVOA with AOS, we also modified the CBS criteria by excluding NVOA and assigned every patient to either a progressors or non-progressors group. We evaluated the frequency of every CBS feature by year from disease onset, and assessed gray and white matter volume loss using SPM12. Results: Asymmetric akinesia, NVOA, and limb apraxia were the most common CBS features that developed; while limb dystonia, myoclonus, and alien limb were rare. Eighty-two patients progressed to possible CBS; only four to probable CBS. nfvPPA and PPAOS had a similar proportion of progressors, although nfvPPA progressed to CBS earlier (p-value = 0.046), driven by an early appearance of limb apraxia (p-value = 0.0041). The non-progressors and progressors both showed premotor/motor cortex involvement at baseline, with spread into prefrontal cortex over time. Discussion: An important proportion of patients with nfvPPA and PPAOS progress to possible CBS, while they rarely develop features of probable CBS even after long follow-up. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Creutzfeldt–Jakob disease is a neurodegenerative disorder caused by brain accumulation of a misfolded form of the cellular prion protein, whose diagnosis is challenging, particularly in early stages, due to the variability and nonspecificity of the clinical and radiological features. 18F‐fluorodeoxyglucose positron‐emitted tomography has the potential to be considered a crucial investigation in these patients, revealing metabolic abnormalities earlier than the conventional neuroimaging analysis. A 59‐year‐old man, the military officer, was referred to our Units for the onset of neurological symptoms rapidly evolving within a month, characterized by akinetic mutism, constructional apraxia, and disorders of spatial orientation. Brain 18F‐fluorodeoxyglucose (18F‐FDG) positron‐emitted tomography (PET)/CT depicted an asymmetric hypometabolism in the left fronto‐temporo‐parietal cortex, as well as in the left thalamus and the right cerebellar hemisphere, while the glucose metabolism appears to be preserved in the somatosensory cortex and the basal ganglia. Laboratory routine analyses, cerebrospinal fluid routine, infective tests, electroencephalography (EEG), and brain magnetic resonance (MR) were all unremarkable. A positive RT‐QuIC result on cerebro‐spinal fluid (CSF) was subsequently shown, without any pathogenic gene mutations and, therefore, the result was consistent with a diagnosis of sporadic Creutzfeld–Jacob disease. The clinical evolution was quickly unfavorable, and the patient died about 4 months after hospital admission. FDG PET/computed tomography (CT) has the potential to be considered a crucial investigation in these patients, documenting metabolic changes long time before other diagnostic investigations such as CSF, EEG, brain CT, and brain MR, thus suggesting a greater sensitivity of glucose metabolic evaluation in the early stage of the disease in question. [ABSTRACT FROM AUTHOR]

Purpose: There are multiple frameworks for goal writing that are applicable to the practice of speech-language pathology. Motor-based speech disorders are a subset of speech sound disorders that are thought to require specific elements of intervention that are typically not addressed in the traditional frameworks used in the clinical setting. The purpose of this tutorial is to review general approaches of goal writing and suggest additional elements that may be used to improve the efficiency and effectiveness of treatment for childhood motor speech disorders, specifically childhood apraxia of speech (CAS). Method: Existing models of goal writing were reviewed to ascertain elements common to most of these models. A basic framework was chosen and modified to include behaviors, conditions, and approaches to goal measurement tailored to the clinical needs of children with CAS. A resource for clinical decision making for children with CAS was developed to inform goal writing at the onset of treatment and adaptations that occur over the course of treatment. Case studies are presented to demonstrate how the presented framework can be applied to writing goals for motor-based treatment for two different children with CAS. Discussion: Children with CAS require a specialized approach to intervention, which requires goals to reflect the unique clinical needs of this population. This tutorial offers resources that use the best available research evidence and current understanding of effective treatment practices for CAS to guide clinical decision making for motor-based intervention and goal writing. This tutorial is intended to guide treatment planning across varied settings to facilitate progress and optimize treatment outcomes for children with CAS. [ABSTRACT FROM AUTHOR]

Background: Perceptual measures such as speech intelligibility are known to be biased, variant and subjective, to which an automatic approach has been seen as a more reliable alternative. On the other hand, automatic approaches tend to lack explainability, an aspect that can prevent the widespread usage of these technologies clinically. Aims: In the present work, we aim to study the relationship between four perceptual parameters and speech intelligibility by automatically modelling the behaviour of six perceptual judges, in the context of head and neck cancer. From this evaluation we want to assess the different levels of relevance of each parameter as well as the different judge profiles that arise, both perceptually and automatically. Methods and Procedures: Based on a passage reading task from the Carcinologic Speech Severity Index (C2SI) corpus, six expert listeners assessed the voice quality, resonance, prosody and phonemic distortions, as well as the speech intelligibility of patients treated for oral or oropharyngeal cancer. A statistical analysis and an ensemble of automatic systems, one per judge, were devised, where speech intelligibility is predicted as a function of the four aforementioned perceptual parameters of voice quality, resonance, prosody and phonemic distortions. Outcomes and Results: The results suggest that we can automatically predict speech intelligibility as a function of the four aforementioned perceptual parameters, achieving a high correlation of 0.775 (Spearman's ρ). Furthermore, different judge profiles were found perceptually that were successfully modelled automatically. Conclusions and Implications: The four investigated perceptual parameters influence the global rating of speech intelligibility, showing that different judge profiles emerge. The proposed automatic approach displayed a more uniform profile across all judges, displaying a more reliable, unbiased and objective prediction. The system also adds an extra layer of interpretability, since speech intelligibility is regressed as a direct function of the individual prediction of the four perceptual parameters, an improvement over more black box approaches. WHAT THIS PAPER ADDS: What is already known on this subject: Speech intelligibility is a clinical measure typically used in the post‐treatment assessment of speech affecting disorders, such as head and neck cancer. Their perceptual assessment is currently the main method of evaluation; however, it is known to be quite subjective since intelligibility can be seen as a combination of other perceptual parameters (voice quality, resonance, etc.). Given this, automatic approaches have been seen as a more viable alternative to the traditionally used perceptual assessments. What this study adds to existing knowledge: The present work introduces a study based on the relationship between four perceptual parameters (voice quality, resonance, prosody and phonemic distortions) and speech intelligibility, by automatically modelling the behaviour of six perceptual judges. The results suggest that different judge profiles arise, both in the perceptual case as well as in the automatic models. These different profiles found showcase the different schools of thought that perceptual judges have, in comparison to the automatic judges, that display more uniform levels of relevance across all the four perceptual parameters. This aspect shows that an automatic approach promotes unbiased, reliable and more objective predictions. What are the clinical implications of this work?: The automatic prediction of speech intelligibility, using a combination of four perceptual parameters, show that these approaches can achieve high correlations with the reference scores while maintaining a certain degree of explainability. The more uniform judge profiles found on the automatic case also display less biased results towards the four perceptual parameters. This aspect facilitates the clinical implementation of this class of systems, as opposed to the more subjective and harder to reproduce perceptual assessments. [ABSTRACT FROM AUTHOR]

GLUT1 deficiency syndrome (GLUT1DS) is a rare genetic disorder caused by a mutation in the SLC2A1 gene that limits the transport of glucose across the blood-brain barrier. Speech disorders and dysarthria are typical findings in patients with GLUT1DS, but have never been deeply phenotyped. The aim of the present study was to characterize speech abilities in a sample of patients with GLUT1DS. 30 patients with GLUT1DS were recruited. We reported impairments in different speech and oromotor domains: the speech was characterized by dysarthria, inaccurate articulation of consonants, abnormal nasal resonance, errors in intonation and prosody and low intelligibility. We observed difficulties in motor planning and programming. Moreover, we observed a significant difference between the dysarthric level of impairment with genotype groups. The presence of a speech disorder in patients with GLUT1DS represents a core feature of the syndrome. Our findings suggest that patients with GLUT1DS would benefit from a comprehensive neurocognitive assessment to detect strengths and weaknesses of the speech profile. Understanding the speech and language phenotype in GLUT1DS is critical for planning early intervention to positively influence the global development of patients with GLUT1DS. • The purpose of this study is to analyze and characterize the speech profile of a sample of patients with GLUT1DS. • The majority of the patients manifested dysarthria with different levels of severity. • A relationship between genotype and dysartric level of impairment was found. • The introduction of the ketogenic diet therapy might be a protective factor for cognitive outcomes in patients with GLUT1DS. [ABSTRACT FROM AUTHOR]

Background Aim Methods & Procedures Outcomes & Results Conclusions & Implications WHAT THIS PAPER ADDS What is already known on this subject What this paper adds to existing knowledge What are the potential or actual clinical implications of this work? Over 50% of individuals with aphasia face ongoing word‐finding issues. Studies have found phonologically oriented therapy helpful for English speakers, but this has not yet been studied in French. It is essential to assess the effectiveness of such a therapy in French, considering the distinct linguistic typologies between both languages, which may impact the outcomes of phonologically oriented interventions.This paper evaluates the effectiveness of French Phonological Component Analysis (Fr‐PCA) on communication skills of individuals with chronic aphasia and the impact of individual factors on treatment success.Eighteen individuals with chronic aphasia received 15 h of Fr‐PCA therapy over 5 weeks. Naming accuracy for treated and untreated words was measured before and after therapy, as well as at 3‐ and 6‐month follow‐ups. Secondary outcome measures included standardized tests measuring within‐level generalization (object and action naming) and across‐level generalization (repetition, verbal fluency, oral comprehension, communication effectiveness reported by a frequent communication partner).Fr‐PCA led to improved accuracy for treated (17 participants out of 18) and untreated words (9 participants out of 18), with gains maintained at 6‐month follow‐up (7 participants out of 10 for treated and 6 participants out of 10 for untreated), and generalization to communication effectiveness reported by a frequent communication partner (11 participants out of 16). Age, apraxia of speech severity and initial anomia severity impacted therapy gains.Though more research is needed, results suggest Fr‐PCA benefits French individuals living with aphasia. Identifying individual factors influencing therapy gains could enable clinicians to improve therapy tailoring. Phonological Component Analysis (PCA) improves naming of treated and untreated items in individuals living with aphasia. There is also evidence supporting long‐lasting benefits following PCA. However, PCA has never been studied in French, a language presenting with a different linguistic typology than English, and we know little as to which individual factors can influence PCA therapy benefits. Through group‐level analyses on both personalized sets and standardized tests, this study shows that PCA constitutes an effective therapy protocol for francophone individuals living with aphasia. The gains are measured on treated and untreated items and generalized to levels other than naming, such as communication effectiveness. Individual factors such as age, initial anomia severity and apraxia of speech severity influence therapy outcomes. There is now evidence supporting PCA in French, a language with strong morphological‐phonological interactions. Furthermore, when working with individuals living with aphasia and severe anomia or apraxia of speech, gains can still be made, but might be longer to attain. [ABSTRACT FROM AUTHOR]

Childhood apraxia of speech (CAS) represents a significant diagnostic and therapeutic challenge within the field of clinical neuropsychology, characterized by its nuanced presentation and multifactorial nature. The aim of this study was to distil and synthesize the broad spectrum of research into a coherent model for the assessment and diagnosis of CAS. Through a mixed-method design, the quantitative phase analyzed 290 studies, unveiling 10 clusters: developmental apraxia, tabby talk, intellectual disabilities, underlying speech processes, breakpoint localization, speech characteristics, functional characteristics, clinical practice, and treatment outcome. The qualitative phase conducted a thematic analysis on the most cited and recent literature, identifying 10 categories: neurobiological markers, speech motor control, perceptual speech features, auditory processing, prosody and stress patterns, parent- and self-report measures, intervention response, motor learning and generalization, comorbidity analysis, and cultural and linguistic considerations. Integrating these findings, a descriptive and prescriptive model was developed, encapsulating the complexities of CAS and providing a structured approach for clinicians. This model advances the understanding of CAS and supports the development of targeted interventions. This study concludes with a call for evidence-based personalized treatment plans that account for the diverse neurobiological and cultural backgrounds of children with CAS. Its implications for practice include the integration of cutting-edge assessment tools that embrace the heterogeneity of CAS presentations, ensuring that interventions are as unique as the children they aim to support. [ABSTRACT FROM AUTHOR]

Progressive apraxia of speech (PAOS) is a 4R tauopathy characterized by difficulties with motor speech planning. Neurodegeneration in PAOS targets the premotor cortex, particularly the supplementary motor area (SMA), with degeneration of white matter (WM) tracts connecting premotor and motor cortices and Broca's area observed on diffusion tensor imaging (DTI). We aimed to assess flortaucipir uptake across speech‐language‐related WM tracts identified using DTI tractography in PAOS. Twenty‐two patients with PAOS and 26 matched healthy controls were recruited by the Neurodegenerative Research Group (NRG) and underwent MRI and flortaucipir‐PET. The patient population included patients with primary progressive apraxia of speech (PPAOS) and non‐fluent variant/agrammatic primary progressive aphasia (agPPA). Flortaucipir PET scans and DTI were coregistered using rigid registration with a mutual information cost function in subject space. Alignments between DTI and flortaucipir PET were inspected in all cases. Whole‐brain tractography was calculated using deterministic algorithms by a tractography reconstruction tool (DSI‐studio) and specific tracts were identified using an automatic fiber tracking atlas‐based method. Fractional anisotropy (FA) and flortaucipir standardized uptake value ratios (SUVRs) were averaged across the frontal aslant tract, arcuate fasciculi, inferior frontal‐occipital fasciculus, inferior and middle longitudinal fasciculi, as well as the SMA commissural fibers. Reduced FA (p <.0001) and elevated flortaucipir SUVR (p =.0012) were observed in PAOS cases compared to controls across all combined WM tracts. For flortaucipir SUVR, the greatest differentiation of PAOS from controls was achieved with the SMA commissural fibers (area under the receiver operator characteristic curve [AUROC] = 0.83), followed by the left arcuate fasciculus (AUROC = 0.75) and left frontal aslant tract (AUROC = 0.71). Our findings demonstrate that flortaucipir uptake is increased across WM tracts related to speech/language difficulties in PAOS. [ABSTRACT FROM AUTHOR]

Background and purpose: The aim was to test the specificity of phenomenological criteria for functional tic‐like behaviours (FTLBs). The European Society for the Study of Tourette Syndrome (ESSTS) criteria for the diagnosis of FTLBs include three major criteria: age at symptom onset ≥12 years, rapid evolution of symptoms and specific phenomenology. Methods: Children and adolescents with primary tic disorders have been included in a Registry in Calgary, Canada, since 2017. Using the Yale Global Tic Severity Scale, the proportion of youth with primary tic disorders who met specific phenomenological criteria for FTLBs at first visit was assessed: (1) having ≥1 specific complex motor tic commonly seen in FTLBs, including complex arm/hand movements, self‐injurious behaviour, blocking, copropraxia; (2) having ≥1 specific complex phonic tic commonly seen in FTLBs, including saying words, phrases, disinhibited speech, coprolalia; (3) having a greater number of complex tics than simple tics. Children seen for the first time between 2017 and 2019 and between 2021 and 2023 were analysed separately. Results: Of 156 participants included between 2017 and 2019, high specificity (94.2%) of the age at onset criterion (≥12 years) and of having at least two complex motor behaviours and one complex phonic behaviour at first visit (96.2%) was observed. Some of the complex motor tics had lower specificity. The specificity of the FTLB diagnostic criterion of having more complex tics than simple tics was 89.7%. There was no significant difference in specificity of the criteria for children seen for the first time between 2017 and 2019 and between 2021 and 2023 (n = 149). Conclusion: This information supports the use of the ESSTS criteria for FTLBs in clinical practice. [ABSTRACT FROM AUTHOR]

Purpose: Pitch variations (tone productions) have been reported as a measure to differentiate Cantonese-speaking children with and without childhood apraxia of speech (CAS). This study aims to examine fundamental frequency (F0) changes within syllables and the effects of syllable structure, lexical status, and syllable positions on F0 in Cantonese-speaking preschool children with and without CAS. Method: Six children with CAS, six children with non-CAS speech sound disorder plus language disorder (S&LD), 22 children with speech sound disorder only (SSD), and 63 children with typical speech-language development (TD) performed the tone sequencing task (TST). Growth curve analysis was employed to analyze and compare the F0 values within syllables with three Cantonese tones (high level, high rising, and low falling). The analysis considered the effects of syllable structure (vowel and consonant–vowel), lexical status (word and nonword), and syllable position (initial, medial, and final) on F0, as well as comparisons within and between groups. Results: Within each group, the effects of syllable structure and position on F0 values were found with different patterns. Between-group comparisons showed that the CAS group had reduced F0 contrasts. The CAS group could be differentiated from the control groups based on interactions of F0 with syllable structure and position, but not lexical status. The dissimilarity of F0 values detected between the CAS and SSD/TD groups was more prominent than that observed between the CAS and S&LD groups. Conclusions: This study demonstrated that Cantonese-speaking children with CAS had difficulty in varying F0 within syllables as compared to those without CAS, suggesting pitch variation difficulty and language-specific impairment profiles in CAS. Future investigations of objective measures for identifying Cantonese speakers with CAS and cross-linguistic investigations using growth curve analysis and the TST are suggested. [ABSTRACT FROM AUTHOR]

This letter, published in the Journal of Neurology, discusses a case of aphasia caused by cerebellar hemorrhage. The cerebellum is known to be involved in cognitive and behavioral functions, and impairments in these areas are referred to as cerebellar cognitive affective syndrome (CCAS). While language impairments in CCAS are typically subtle, this case highlights the disruption of cerebellar compensation as a potential cause of aphasia. The patient initially experienced aphasia due to left parietal lobe infarction, which improved but recurred after cerebellar hemorrhage. The letter provides detailed information about the patient's symptoms and suggests that the disruption of cerebellar compensation may be responsible for the aphasia. [Extracted from the article]

Key Clinical Message: We present a case of a single left hemisphere temporal–parietal stroke with subacute global aphasia and severe verbal apraxia and moderate dysphagia. The patient underwent a combined transcranial direct current stimulation (tDCS) over the left dorsolateral prefrontal cortex (DLPFC) and language stimulation with Virtual Reality Rehabilitation System (VRRS). Patient was treated in a 1‐h session, for 5 days a week, for 4 consecutive weeks. After treatment, evident improvements in the comprehension of oral and written language, swallowing abilities, and caregiver burden were detected. Power spectrum analysis of EEG data revealed significant enhancements of θ, α, and β waves from baseline to follow‐up. These preliminary results seem to confirm the reliability of the tDCS translational application in conjunction with computer‐based cognitive treatment for language disorders in a patient with stroke‐induced aphasia. [ABSTRACT FROM AUTHOR]

Disagreements persist regarding the neural basis of syntactic processing, which has been linked both to inferior frontal and posterior temporal regions of the brain. One focal point of the debate concerns the role of inferior frontal areas in receptive syntactic ability, which is mostly assessed using sentence comprehension involving complex syntactic structures, a task that is potentially confounded with working memory. Syntactic acceptability judgments may provide a better measure of receptive syntax by reducing the need to use high working memory load and complex sentences and by enabling assessment of various types of syntactic violations. We therefore tested the perception of grammatical violations by people with poststroke aphasia (n = 25), along with matched controls (n = 16), using English sentences involving errors in word order, agreement, or subcategorization. Lesion data were also collected. Control participants performed near ceiling in accuracy with higher discriminability of agreement and subcategorization violations than word order; aphasia participants were less able to discriminate violations, but, on average, paralleled control participants discriminability of types of violations. Lesion-symptom mapping showed a correlation between discriminability and posterior temporal regions, but not inferior frontal regions. We argue that these results diverge from models holding that frontal areas are amodal core regions in syntactic structure building and favor models that posit a core hierarchical system in posterior temporal regions. [ABSTRACT FROM AUTHOR]

Background: Spinal muscular atrophy (SMA) is an autosomal recessive neuromuscular disease characterized by progressive muscle weakness and atrophy. Clinical trial data suggest early diagnosis and treatment are critical. The purpose of this study was to evaluate neurology appointment wait times for newborn screening identified infants, pediatric cases mirroring SMA symptomatology, and cases in which SMA is suspected by the referring physician. Approaches for triaging and expediting referrals in the US were also explored. Methods: Cure SMA surveyed healthcare professionals from two cohorts: (1) providers affiliated with SMA care centers and (2) other neurologists, pediatric neurologists, and neuromuscular specialists. Surveys were distributed directly and via Medscape Education, respectively, between July 9, 2020, and August 31, 2020. Results: Three hundred five total responses were obtained (9% from SMA care centers and 91% from the general recruitment sample). Diagnostic journeys were shorter for infants eventually diagnosed with SMA Type 1 if they were referred to SMA care centers versus general sample practices. Appointment wait times for infants exhibiting "hypotonia and motor delays" were significantly shorter at SMA care centers compared to general recruitment practices (p = 0.004). Furthermore, infants with SMA identified through newborn screening were also more likely to be seen sooner if referred to a SMA care center versus a general recruitment site. Lastly, the majority of both cohorts triaged incoming referrals. The average wait time for infants presenting at SMA care centers with "hypotonia and motor delay" was significantly shorter when initial referrals were triaged using a set of "key emergency words" (p = 0.036). Conclusions: Infants directly referred to a SMA care center versus a general sample practice were more likely to experience shorter SMA diagnostic journeys and appointment wait times. Triage guidelines for referrals specific to "hypotonia and motor delay" including use of "key emergency words" may shorten wait times and support early diagnosis and treatment of SMA. [ABSTRACT FROM AUTHOR]

Introduction Letters of recommendation are considered an important factor in the holistic ranking of orthopaedic residency applications. The standardized letter of recommendation (SLOR) was introduced in 2017 in response to the inherent bias and limited comparative value of traditional LORs. It includes standardized questions about an applicant’s orthopaedic qualifications, designed to enable fair comparisons among candidates. However, disparate and inconsistent findings have made it difficult to draw meaningful conclusions from individual studies on the SLOR and narrative letter of recommendation. Question/purposes In this systematic review we asked: (1) What is the distribution of applicant SLOR rating among nine domains and summative scores? (2) Are there applicant characteristics associated with SLOR ratings? (3) Is there gender and racial bias in the SLOR and narrative letter of recommendation? Methods PubMed, EBSCO, and Google Scholar electronic databases were queried on March 20, 2023, to identify all studies evaluating SLOR and narrative letter of recommendations between January 1, 2017, and March 20, 2023. Articles that evaluated orthopaedic SLOR or narrative LORs were included. Systematic reviews, case reports, duplicate studies between databases, and grey literature such as abstracts and articles on preprint servers were excluded. Eight studies including 6179 applicants and 18,987 letters of recommendation were included in the final analysis. The applicant classes ranged from years 2014 to 2020. Two reviewers independently evaluated the quality of the included studies using the Joanna Briggs Institute (JBI) tool for cross-sectional studies. The mean JBI score of included studies was 7.4 out of a maximum of 8, with higher scores indicating better quality. The primary outcome was to determine the distribution of applicant SLOR rankings in their summative score. Summative scores were separated into ranked to match, top one-third, middle one-third, lower one-third, and not a fit. Four studies reported the summative SLOR scores of applicants. Our secondary study goal was to assess applicant characteristics associated with SLOR summative scores and assess any bias present in the SLOR or narrative recommendation. Five studies compared SLOR ratings across applicant characteristics including gender, race, USMLE Step 1 score, USMLE Step 2 score, Alpha Omega Alpha (AVA) membership, clerkship grades, and publications. Gender and racial bias were also assessed across five studies. Four studies utilized a linguistic analysis software to search letters of recommendation for differences in word category use by gender and race. Results Studies consistently found that a higher percentage of candidates were identified in the top percentile blocks than is mathematically possible. For example, the two studies that tallied the proportion of candidates ranking in the top 10% of applicants found that 36% and 42% were rated as being in the top 10%. Similarly, articles found more than 87% of applicants scored in the top one-third. Studies had mixed results, but in general, they suggested that AVA status, higher Step 1 scores, and more research publications have a slightly positive association with increased SLOR scores. We found no evidence of gender bias against women, and in fact, most studies evaluating word choices found word choices for women candidates generally were stronger. Similarly, no consistent disparities were identified in word categories utilized in SLORs based on applicant race. Conclusion Most notably, a mathematically impossible proportion of applicants were counted in the top percentiles in letters of recommendation. This compromises readers’ abilities to differentiate candidates. Factors like AOA status and research publications displayed a modest positive correlation with SLOR scores. Gender bias against women or candidates from racial minority groups was not evident. Clinical Relevance We suggest that group SLOR authorship, with a consensus assessment by clerkship or residency directors, should be considered. We also recommend the incorporation of mean and SD scores for each letter writer (or group) alongside their letters. Furthermore, studies indicate that submitting all four SLOR letters can substantially improve an applicant’s chances of success. We advise students to choose strategically and submit the maximum allowable number of SLORs. [ABSTRACT FROM AUTHOR]

Introduction: As a hallmark feature of amyotrophic lateral sclerosis (ALS), bulbar involvement leads to progressive declines of speech and swallowing functions, significantly impacting social, emotional, and physical health, and quality of life. Standard clinical tools for bulbar assessment focus primarily on clinical symptoms and functional outcomes. However, ALS is known to have a long, clinically silent prodromal stage characterized by complex subclinical changes at various levels of the bulbar motor system. These changes accumulate over time and eventually culminate in clinical symptoms and functional declines. Detection of these subclinical changes is critical, both for mechanistic understanding of bulbar neuromuscular pathology and for optimal clinical management of bulbar dysfunction in ALS. To this end, we developed a novel multimodal measurement tool based on two clinically readily available, noninvasive instruments--facial surface electromyography (sEMG) and acoustic techniques--to hierarchically assess seven constructs of bulbar/speech motor control at the neuromuscular and acoustic levels. These constructs, including prosody, pause, functional connectivity, amplitude, rhythm, complexity, and regularity, are both mechanically and clinically relevant to bulbar involvement. Methods: Using a custom-developed, fully automated data analytic algorithm, a variety of features were extracted from the sEMG and acoustic recordings of a speech task performed by 13 individuals with ALS and 10 neurologically healthy controls. These features were then factorized into 10 composite outcome measures using confirmatory factor analysis. Statistical and machine learning techniques were applied to these composite outcome measures to evaluate their reliability (internal consistency), validity (concurrent and construct), and efficacy for early detection and progress monitoring of bulbar involvement in ALS. Results: The composite outcome measures were demonstrated to (1) be internally consistent and structurally valid in measuring the targeted constructs; (2) hold concurrent validity with the existing clinical and functional criteria for bulbar assessment; and (3) outperform the outcome measures obtained from each constituent modality in differentiating individuals with ALS from healthy controls. Moreover, the composite outcome measures combined demonstrated high efficacy for detecting subclinical changes in the targeted constructs, both during the prodromal stage and during the transition from prodromal to symptomatic stages. Discussion: The findings provided compelling initial evidence for the utility of the multimodal measurement tool for improving early detection and progress monitoring of bulbar involvement in ALS, which have important implications in facilitating timely access to and delivery of optimal clinical care of bulbar dysfunction. [ABSTRACT FROM AUTHOR]

Purpose: People with aphasia have been shown to experience higher rates of depression and mental health issues than stroke survivors without aphasia. Narrative-based counseling approaches to therapy in communication disorders have shown promise in several areas, such as stuttering. Little is known regarding how these approaches can be implemented with individuals with aphasia. This study sought to uncover what therapeutic processes could be utilized to overcome barriers to utilizing this approach with people with aphasia. Method: A qualitative case study design was utilized. The primary data source included audio-recorded intervention sessions of a narrative-based counseling approach. Secondary data sources included reflection journals produced by the clinician and artifacts created by the client, including letter written as a part of the therapeutic process and other documents co-constructed by the clinician and client that detailed the client's journey. Data were analyzed inductively, in which transcripts were coded by the researchers and compared across the data set until patterns were apparent in the data that were later defined as themes. Results: Analysis of data suggests that adapting narrative counseling techniques for success with people with aphasia consists of allowing adequate time for client response, building a therapeutic alliance, reiteration and saturation of solution-focused language and narratives, and maintaining a topic of conversation centered around aphasia. Conclusion: This study argues for further consideration of how narrative-based counseling techniques can be adapted and flexibly used in aphasia intervention. [ABSTRACT FROM AUTHOR]

Little is known about the literacy skills of children with childhood apraxia of speech (CAS) or effective methods for teaching them to read. This systematic scoping review aimed to synthesise what is known about this issue. Nine databases were searched to identify relevant articles. Included articles were categorised by study design, quality, and confidence of CAS diagnosis. Twenty-three articles were included, 17 described literacy skills of children with CAS and six trialled literacy interventions. Children with CAS had early skills deficits that manifest as literacy difficulties in the later school years and beyond. They frequently had poorer outcomes compared with both typical readers and children with other speech disorders. Both the extent of literacy impairment and responsiveness to intervention appear to be related to the severity of speech impairment. Four literacy interventions for children with CAS were identified. Children with CAS are at high risk of literacy difficulty and may require early literacy intervention to help them attain academic success. Further research is warranted to determine the longer-term literacy outcomes of children with CAS, appropriate means of assessment, and whether a systematic synthetic phonics approach is an effective form of literacy instruction for this population. [ABSTRACT FROM AUTHOR]

Background: Areca Nut (AN) is the fourth most commonly abused drug after nicotine, ethanol, and caffeine, due to its psychoactive properties provided by bioactive substances. Although previous studies have demonstrated AN's anxiolytic-like activity and potential benefits in ameliorating symptoms of depression and schizophrenia, there remains limited awareness regarding its association with brief psychotic disorder. Case presentation: This case report presents the clinical profile of a 30-year-old male patient with a history of betel nut chewing for the past 2 years, who exhibited sudden onset delusions, hallucinations, and disorganized speech and behavior upon increasing the dosage of betel nut consumption. The patient displayed a positive response to antipsychotic treatment, and symptoms resolved upon discontinuation of betel nut consumption. However, one month after discharge, the patient experienced a recurrence of auditory hallucinations upon resuming betel nut chewing. Through counseling and support, the importance of abstaining from betel nut use and maintaining medication compliance was emphasized, resulting in no recurrence of psychotic symptoms during the six-month follow-up. Conclusions: This case report highlights the related role of betel nut with brief psychotic disorder, especially when the chewing dosage is abruptly increased. It underscores the importance of considering betel nut as a potential precipitant related to acute psychiatric disorders in clinical settings. [ABSTRACT FROM AUTHOR]

Primary progressive apraxia of speech (PPAOS) is a neurodegenerative syndrome characterized by the progressive and initially isolated or predominant onset of difficulties in the planning/programming of movements necessary for speech production and can be accompanied by dysarthria. To date, no study has used an evidence-based treatment to address phonation control in patients with PPAOS. The aim of this study was to evaluate the feasibility and efficacy of LSVT LOUD® as a treatment for phonatory control in speakers with PPAOS. Three speakers with PPAOS received LSVT LOUD® therapy, and changes in phonatory control, voice quality and prosody were measured immediately, and one, four and eight weeks after the end of the treatment. Overall, the results suggest that the treatment is feasible and could improve voice quality, intensity, and control in some patients with PPAOS. The generalization of the results is also discussed. [ABSTRACT FROM AUTHOR]

Progressive apraxia of speech (PAOS) is a neurodegenerative motor-speech disorder that most commonly arises from a four-repeat tauopathy. Recent studies have established that progressive apraxia of speech is not a homogenous disease but rather there are distinct subtypes: the phonetic subtype is characterized by distorted sound substitutions, the prosodic subtype by slow and segmented speech and the mixed subtype by a combination of both but lack of predominance of either. There is some evidence that cross-sectional patterns of neurodegeneration differ across subtypes, although it is unknown whether longitudinal patterns of neurodegeneration differ. We examined longitudinal patterns of atrophy on MRI, hypometabolism on 18F-fluorodeoxyglucose-PET and tau uptake on flortaucipir-PET in a large cohort of subjects with PAOS that had been followed for many years. Ninety-one subjects with PAOS (51 phonetic, 40 prosodic) were recruited by the Neurodegenerative Research Group. Of these, 54 (27 phonetic, 27 prosodic) returned for annual follow-up, with up to seven longitudinal visits (total visits analysed = 217). Volumes, metabolism and flortaucipir uptake were measured for subcortical and cortical regions, for all scans. Bayesian hierarchical models were used to model longitudinal change across imaging modalities with PAOS subtypes being compared at baseline, 4 years from baseline, and in terms of rates of change. The phonetic group showed smaller volumes and worse metabolism in Broca's area and the striatum at baseline and after 4 years, and faster rates of change in these regions, compared with the prosodic group. There was also evidence of faster spread of hypometabolism and flortaucipir uptake into the temporal and parietal lobes in the phonetic group. In contrast, the prosodic group showed smaller cerebellar dentate, midbrain, substantia nigra and thalamus volumes at baseline and after 4 years, as well as faster rates of atrophy, than the phonetic group. Greater hypometabolism and flortaucipir uptake were also observed in the cerebellar dentate and substantia nigra in the prosodic group. Mixed findings were observed in the supplementary motor area and precentral cortex, with no clear differences observed across phonetic and prosodic groups. These findings support different patterns of disease spread in PAOS subtypes, with corticostriatal patterns in the phonetic subtype and brainstem and thalamic patterns in the prosodic subtype, providing insight into the pathophysiology and heterogeneity of PAOS. [ABSTRACT FROM AUTHOR]

Aphasiology has a long and rich tradition of contributing to understanding how culture, language, and social environment contribute to brain development and function. Recent breakthroughs in AI can transform the role of aphasiology in the digital age by leveraging speech data in all languages to model how damage to specific brain regions impacts linguistic universals such as grammar. These tools, including generative AI (ChatGPT) and natural language processing (NLP) models, could also inform practitioners working with clinical populations in the assessment and treatment of aphasia using AI-based interventions such as personalized therapy and adaptive platforms. Although these possibilities have generated enthusiasm in aphasiology, a rigorous interrogation of their limitations is necessary before AI is integrated into practice. We explain the history and first principles of reciprocity between AI and aphasiology, highlighting how lesioning neural networks opened the black box of cognitive neurolinguistic processing. We then argue that when more data from aphasia across languages become digitized and available online, deep learning will reveal hitherto unreported patterns of language processing of theoretical interest for aphasiologists. We also anticipate some problems using AI, including language biases, cultural, ethical, and scientific limitations, a misrepresentation of marginalized languages, and a lack of rigorous validation of tools. However, as these challenges are met with better governance, AI could have an equitable impact. [ABSTRACT FROM AUTHOR]

Cognitive interventions are helpful in the non-pharmacological management of Primary progressive aphasia (PPA) and other neurodegenerative disorders of cognition, by helping patients to compensate for their cognitive deficits and improve their functional independence. In this study, we examined the effectiveness of cognitive rehabilitation based on the use of mobile device technology in PPA. The aim of this research study was to determine if BL, a patient with semantic variant PPA (svPPA) and severe anomia, was able to learn using specific smartphone functions and an application to reduce her word finding difficulties. She was trained during the intervention sessions on a list of target pictures to measure changes in picture naming performance. Errorless learning was applied during learning. BL quickly learned to use smartphone functions and the application over the course of the intervention. She significantly improved her anomia for trained pictures, and to a lesser extent for untrained semantically related pictures. Picture naming performance was maintained six months after the intervention, and she continued to use her smartphone regularly to communicate with family members and friends. This study confirms that smartphone use can be learned in PPA, which can help reduce the symptoms of anomia and improve communication skills. [ABSTRACT FROM AUTHOR]

It is debated whether primary progressive apraxia of speech (PPAOS) and progressive agrammatic aphasia (PAA) belong to the same clinical spectrum, traditionally termed non-fluent/agrammatic variant primary progressive aphasia (nfvPPA), or exist as two completely distinct syndromic entities with specific pathologic/prognostic correlates. We analysed speech, language and disease severity features in a comprehensive cohort of patients with progressive motor speech impairment and/or agrammatism to ascertain evidence of naturally occurring, clinically meaningful non-overlapping syndromic entities (e.g. PPAOS and PAA) in our data. We also assessed if data-driven latent clinical dimensions with aetiologic/prognostic value could be identified. We included 98 participants, 43 of whom had an autopsy-confirmed neuropathological diagnosis. Speech pathologists assessed motor speech features indicative of dysarthria and apraxia of speech (AOS). Quantitative expressive/receptive agrammatism measures were obtained and compared with healthy controls. Baseline and longitudinal disease severity was evaluated using the Clinical Dementia Rating Sum of Boxes (CDR-SB). We investigated the data's clustering tendency and cluster stability to form robust symptom clusters and employed principal component analysis to extract data-driven latent clinical dimensions (LCD). The longitudinal CDR-SB change was estimated using linear mixed-effects models. Of the participants included in this study, 93 conformed to previously reported clinical profiles (75 with AOS and agrammatism, 12 PPAOS and six PAA). The remaining five participants were characterized by non-fluent speech, executive dysfunction and dysarthria without apraxia of speech or frank agrammatism. No baseline clinical features differentiated between frontotemporal lobar degeneration neuropathological subgroups. The Hopkins statistic demonstrated a low cluster tendency in the entire sample (0.45 with values near 0.5 indicating random data). Cluster stability analyses showed that only two robust subgroups (differing in agrammatism, executive dysfunction and overall disease severity) could be identified. Three data-driven components accounted for 71% of the variance [(i) severity-agrammatism; (ii) prominent AOS; and (iii) prominent dysarthria]. None of these data-driven LCDs allowed an accurate prediction of neuropathology. The severity-agrammatism component was an independent predictor of a faster CDR-SB increase in all the participants. Higher dysarthria severity, reduced words per minute and expressive and receptive agrammatism severity at baseline independently predicted accelerated disease progression. Our findings indicate that PPAOS and PAA, rather than exist as completely distinct syndromic entities, constitute a clinical continuum. In our cohort, splitting the nfvPPA spectrum into separate clinical phenotypes did not improve clinical-pathological correlations, stressing the need for new biological markers and consensus regarding updated terminology and clinical classification. [ABSTRACT FROM AUTHOR]

Purpose: Childhood apraxia of speech (CAS) is a multivariate motor speech disorder that requires a motor-based intervention approach. There is limited treatment research on young children with CAS, reflecting a critical gap in the literature given that features of CAS are often in full expression early in development. Dynamic Temporal and Tactile Cueing (DTTC) is a treatment approach designed for children with severe CAS, yet the use of DTTC with children younger than 3 years of age has not been examined. Method: A multiple single-case design was employed to examine the use of DTTC in seven children with CAS (aged 2.5–5 years) over the course of 6 weeks of intervention. Changes in word accuracy were measured in treated words from baseline to posttreatment and from baseline to maintenance (6 weeks posttreatment). Generalization of word accuracy changes to matched untreated words was also examined. A linear mixed-effects model was used to estimate the change in word accuracy for treated and untreated words across all children from baseline to posttreatment and to maintenance. A quasi-Poisson regression model was used to estimate mean change and calculate effect sizes for treated and untreated words. Results: Group-level analyses revealed significant changes in word accuracy for treated and untreated words at posttreatment and maintenance. At the child level, six of seven children displayed medium-to-large effect sizes where word accuracy increased in an average of 3.4/5 words across all children. Each child displayed some degree of generalization to untreated targets, specifically for words with the same syllable shape as the treated words. Conclusions: These results demonstrate that DTTC can yield positive change in some young children with CAS. Key differences in each child’s performance are highlighted. [ABSTRACT FROM AUTHOR]

Childhood Apraxia of Speech (CAS) is a disorder which affects not only speech-motor planning but also language ability more broadly. The present study attempts to explore the morphosyntactic, socio-cognitive, and prosody comprehension skills of children with CAS. Twenty preschool children with CAS and 20 typically developing children listened to a story, while viewing a wordless PowerPoint, picture presentation on a computer screen. Then, they were instructed to retell the story. Each participant listened to two stories, one with "lively" and one with "flat" prosody. The results revealed poor socio-cognitive abilities which were also evident in the use of verb-complement clauses, and poor prosody comprehension skills in the group with CAS. These findings affirm that morphosyntactic deficits are not the core characteristic of CAS, and suggest a connection between the production and comprehension of prosody, and between prosodic and (socio)-cognitive skills. [ABSTRACT FROM AUTHOR]

INTRODUCTION: Patients with Alzheimer's disease present with difficulty in lexical retrieval and reversal of the concreteness effect in nouns. Little is known about the phenomena before the onset of symptoms. We anticipate early linguistic signs in the speech of people who suffer from amnestic mild cognitive impairment (MCI). Here, we report the results of a corpus‐linguistic approach to the early detection of cognitive impairment. METHODS: One hundred forty‐eight English‐speaking Singaporeans provided natural speech data, on topics of their choice; 74 were diagnosed with single‐domain MCI (38 amnestic, 36 non‐amnestic), 74 cognitively healthy. The recordings yield 267,310 words, which are tagged for parts of speech. We calculate the per‐minute word counts and concreteness scores of all tagged words, nouns, and verbs in the dataset. RESULTS: Compared to controls, subjects with amnestic MCI produce fewer but more abstract nouns. Verbs are not affected. DISCUSSION: Slower retrieval of nouns and the reversal of the concreteness effect in nouns are manifested in natural speech and can be detected early through corpus‐based analysis. Highlights: Reversal of the concreteness effect is manifested in patients with Alzheimer's disease (AD) and semantic dementia.The paper reports a corpus‐based analysis of natural speech by people with amnestic and non‐amnestic mild cognitive impairment (MCI) and cognitively healthy controls.People with amnestic MCI produce fewer and more abstract nouns than people with non‐amnestic MCI and healthy controls. Verbs appear to be unaffected.The imageability problem can be detected in natural everyday speech by people with amnestic MCI, which carries a higher risk of conversion to AD. [ABSTRACT FROM AUTHOR]

The purpose of this study was to examine whether differences in motor speech features are related to presentations of dysphagia in progressive supranuclear palsy (PSP) given the sparsity of data examining this relationship. Motor speech disorder (MSD) type and severity along with specific swallowing variables were analysed to obtain insights among these relationships in 73 participants with PSP. Results revealed that most participants (93%) had dysarthria, with 19% having co-occurring apraxia of speech (AOS). Greater MSD severity was related to more severe pharyngeal phase impairments (95% CI [−0.917, −0.146], p = 0.008). While certain motor speech and swallowing scores varied minimally across participants, incremental changes in these functions were more likely to occur when specific MSD features were present. A trend for participants with spastic dysarthria and/or AOS to exhibit more severe dysphagia was observed. This study points to the need for thorough neurological evaluation, with inclusion of speech-language pathology consultation, in the standard of care for PSP. Comprehensive assessment of both motor speech and swallowing functions can inform differential diagnosis and assist patients/families facing decisions regarding modalities for communication and nutrition in the setting of neurodegenerative disease. Additional research may yield greater insights about relevant assessment and intervention considerations in PSP. [ABSTRACT FROM AUTHOR]

A Phase I study was conducted to examine the treatment effectiveness of the Kaufman Speech to Language Protocol using a research-operationalised protocol. It was hypothesised that articulatory accuracy would improve as a result of the treatment and that these improvements would be maintained after treatment was discontinued. A single case experimental design was used to evaluate the effectiveness of the Kaufman Speech to Language Protocol. Four children with a confirmed childhood apraxia of speech diagnosis were included in this study. Each child received 12 individual 1 hr treatment sessions that each consisted of an approximation setting phase and a practice phase. Probe data was collected during treatment and at post-treatment time points to measure treatment effectiveness and to measure changes in the untreated words. Untreated (control) sounds were included to test whether recorded improvements in articulatory accuracy could be attributed to the Kaufman Speech to Language Protocol. Two of the four children demonstrated a response to the intervention and maintenance of these changes, while the two remaining children demonstrated some generalisation in the absence of improved target (treatment) words. No specific child factors were clearly associated with positive treatment outcomes. This study replicated the findings of an earlier pilot study and found that the operationalised protocol for the Kaufman Speech to Language Protocol is effective in improving articulatory accuracy for some children with childhood apraxia of speech. Additional replication with a further refined treatment protocol and a larger sample size is needed to support a recommendation of clinical use of the Kaufman Speech to Language Protocol. [ABSTRACT FROM AUTHOR]

Background. Although patients can present with progressive pseudobulbar palsy due to neurodegenerative diseases, detection of the precise location of radiological abnormalities can be difficult. 18F-THK5351 was initially developed as a tau positron emission tomography (PET) tracer. Later, it was found to sensitively detect astrogliosis associated with neurodegeneration. Therefore, it has been used in diagnosis of various diseases. However, its utility in progressive pseudobulbar palsy was unknown. Methods. 18F-THK5351 PET results of two patients presenting with progressive pseudobulbar palsy are reported. Results. Patient 1 was a 77-year-old man with a two-year history, and Patient 2 was a 61-year-old woman with a 1-year history. Both patients presented with gradually progressive spastic dysarthria, suggesting pseudobulbar palsy without clinical lower motor neuron signs. Facial asymmetry was detected in both patients, while left-dominant pyramidal signs in the extremities were detected only in Patient 2. Brain magnetic resonance imaging did not show signal abnormality explaining pseudobulbar palsy. However, 18F-THK5351 PET clearly visualized bilateral increased uptake in limited areas of the posterior portion of the precentral gyrus, corresponding to the midportion of the primary motor cortex. Laterality of increased 18F-THK5351 uptake corresponded to the symptom laterality and was higher on the left and right side in patients 1 and 2, respectively. After one year, Patient 1 was unable to vocalize and could only produce grunts; concomitant apraxia of speech was suspected. Conclusions. 18F-THK5351 PET is a useful method to detect bilateral primary motor cortex involvement in patients presenting with progressive pseudobulbar palsy, likely by imaging astrogliosis. [ABSTRACT FROM AUTHOR]

Background: Primary progressive aphasia (PPA) diagnostic criteria underestimate the complex presentation of semantic (sv) and logopenic (lv) variants, in which symptoms partially overlap, and mixed clinical presentation (mixed-PPA) and heterogenous profile (lvPPA +) are frequent. Conceptualization of similarities and differences of these clinical conditions is still scarce. Methods: Lexical, semantic, phonological, and working memory errors from nine language tasks of sixty-seven PPA were analyzed using Profile Analysis based on Multidimensional Scaling, which allowed us to create a distributed representation of patients' linguistic performance in a shared space. Patients had been studied with [18F] FDG-PET. Correlations were performed between metabolic and behavioral data. Results: Patients' profiles were distributed across a continuum. All PPA, but two, presented a lexical retrieval impairment, in terms of reduced production of verbs and nouns. svPPA patients occupied a fairly clumped space along the continuum, showing a preponderant semantic deficit, which correlated to fusiform gyrus hypometabolism, while only few presented working memory deficits. Adjacently, lvPPA + presented a semantic impairment combined with phonological deficits, which correlated with metabolism in the anterior fusiform gyrus and posterior middle temporal gyrus. Starting from the shared phonological deficit side, a large portion of the space was occupied by all lvPPA, showing a combination of phonological, lexical, and working memory deficits, with the latter correlating with posterior temporo-parietal hypometabolism. Mixed PPA did not show unique profile, distributing across the space. Discussion: Different clinical PPA entities exist but overlaps are frequent. Identifying shared and unique clinical markers is critical for research and clinical practice. Further research is needed to identify the role of genetic and pathological factors in such distribution, including also higher sample size of less represented groups. [ABSTRACT FROM AUTHOR]

Despite most studies on the neurobiology of language demonstrating the central part of the perisylvian network involved in language and speech function, this review attempts to complement this view by focusing on the role of the orbitofrontal cortex (OFC). This region is primarily involved in goal-directed adaptive behavior. Recently, there has been increasing evidence that the OFC is involved in language and speech tasks. This review demonstrates that not only the linguistic tasks that involve the processing of socially, pragmatically and emotionally relevant information engage OFC and its neurobiological mechanisms, but also specific receptive and expressive language performances rely on specific neurophysiological properties of this region (e.g., the gray matter volume and the functional activation of OFC and the uncinate fasciculus that connects OFC), which in many cases, demand executive functions. These findings highlight: (1) The OFC plays a relevant role in the adaptive neurobiological function of language; (2) the neurobiological mechanisms beyond linguistic and speech processes complement and interplay with the language-unique processes to achieve successful comprehension and production in the changing communicative contexts. [ABSTRACT FROM AUTHOR]

This study investigated the relationship between early within-therapy probe naming performance and anomia therapy outcomes in individuals with aphasia. Thirty-four adults with chronic, post-stroke aphasia participated in the Aphasia Language Impairment and Functioning Therapy (Aphasia LIFT) programme, comprised of 48 h of comprehensive aphasia therapy. Sets of 30 treated and 30 untreated items identified at baseline were probed during impairment therapy which targeted word retrieval using a combined semantic feature analysis and phonological component analysis approach. Multiple regression models were computed to determine the relationship between baseline language and demographic variables, early within-therapy probe naming performance (measured after 3 h of impairment therapy) and anomia treatment outcomes. Early within-therapy probe naming performance emerged as the strongest predictor of anomia therapy gains at post-therapy and at 1-month follow-up. These findings have important clinical implications, as they suggest that an individual's performance after a brief period of anomia therapy may predict response to intervention. As such, early within-therapy probe naming may provide a quick and accessible tool for clinicians to identify potential response to anomia treatment. [ABSTRACT FROM AUTHOR]

Verbal and oral apraxia are two possible consequences of stroke. It seems that there are not sufficient studies regarding the frequency of these disorders. This study aimed to evaluate the frequency of Verbal and oral apraxia. In addition, the relationship between apraxia and some variables such as age, gender, and education, as well as the relationship between types of apraxia with each other, and damaged areas of the brain in apraxia of the oral system in Persian-speaking patients with stroke were studied. In this descriptive-analytical study, 42 patients participated using the convenient sampling method. Verbal and oral apraxia were assessed using the oral and verbal apraxia tasks for adults test. Data were analyzed using independent t-test, Chi-square, and Fisher's exact test. The frequency of patients with oral apraxia was 35.7%, those with verbal apraxia was 2.3%, and the combination of both verbal and oral apraxia was 4.7%. People with apraxia were significantly older than those without apraxia. There was not any significant relationship between apraxia and gender, apraxia and education, and oral apraxia with verbal apraxia (p < 0.05). The present study's findings showed the high frequency of post-stroke apraxia and the high rate of its incidence with age. [ABSTRACT FROM AUTHOR]

Purpose: Apraxia of speech (AOS) is a motor speech disorder affecting articulatory planning and speech programming. When AOS is the sole manifestation of neurodegeneration, it is termed primary progressive apraxia of speech (PPAOS). Recent work has shown that there are distinct PPAOS subtypes: phonetic, prosodic, and those that do not clearly align with either (mixed). PPAOS subtypes differ with respect to the predominating motor speech difficulties, as well as disease progression and underlying pathology. Because past studies have determined PPAOS subtype based on clinical impression, the goal of the present study was to quantitatively determine the distribution of speech error types across PPAOS sub-types in a word repetition task and to investigate how word complexity affects the type and number of speech errors across PPAOS subtypes. Method: Forty-five patients with PPAOS (13 phonetic, 23 prosodic, and nine mixed) and 45 healthy controls produced multiple repetitions of words that varied in phonetic complexity. Sound additions, deletions, and substitutions/distortions (phonetic errors) and within-word segmentations (prosodic errors) were calculated. Results: All three PPAOS groups produced significantly more errors than controls, but the total number of errors was comparable among subtypes. The phonetic group produced more phonetic-type errors compared to the prosodic group but comparable to the mixed group. The prosodic group produced more segmentations compared to the phonetic and mixed PPAOS groups. As word complexity increased, the total number of errors increased for PPAOS patients. The phonetic and prosodic groups were more likely to produce phonetic- and prosodic-type errors, respectively, as word complexity increased. Conclusions: This study provides novel quantitative data showing that PPAOS subtype can be supported by the type and distribution of speech errors in a word repetition task. This may facilitate earlier, more reliable differential diagnosis and aid in disease prognosis, as PPAOS subtypes have distinct disease trajectories. [ABSTRACT FROM AUTHOR]

Purpose: The purpose of the present study was to explore the ability of Hebrew-speaking children with childhood apraxia of speech (CAS) to produce lexical stress. Method: A total of 36 children aged between 4 and 7 years, 18 children with CAS, and 18 typically developing (TD) children participated in the study. All children completed language and speech assessments. The children imitated 20 weak--strong and strong--weak target words within short sentences and in isolation. Acoustic analysis of the vowels in the stressed and unstressed syllables of the target words and perceptual judgment of the words by six speech-language pathologists were conducted. Results: The acoustic analysis showed significant differences in duration, fundamental frequency, and amplitude between stressed and unstressed vowels in weak--strong and strong--weak words and in both groups of children. The total duration for both the stressed and weak syllables was longer in children with CAS compared to TD children. Rated on a Likert scale of 1-5, where 5 indicates correct production, the productions of lexical stress in the CAS group were judged as above 4 on average but were significantly worse compared to the TD group. The target productions of children with CAS were judged as including excessive, equal, or misplaced stress in 10.8% of the productions, whereas 5.8% of the target productions of the TD children were judged as having inappropriate stress. Conclusions: The present findings suggest that Hebrew-speaking children with CAS produce the acoustic characteristics of lexical stress similarly to their peers, and their productions are perceived as having relatively good lexical stress (above 4), although not as good as TD children. [ABSTRACT FROM AUTHOR]

Depression is one of the most frequent neuropsychiatric symptoms in corticobasal degeneration (CBD), a rare, sporadic, and late-onset progressive neurodegenerative disorder of unknown etiology. It is clinically characterized by a levodopa-poorly responsible akinetic-rigid syndrome, apraxia, limb dystonia, cognitive, mood, behavioral, and language disorders. This 4-repeat (4R) tauopathy is morphologically featured by asymmetric frontoparietal atrophy, neuronal loss, and gliosis in cortex and subcortex including substantia nigra, ballooned/achromatic neurons with filamentous 4R tau aggregates in cortex and striatum, widespread thread-like structures, pathognomonic "astroglial plaques", "tufted astrocytes", and numerous "coiled bodies" (in astrocytes and oligodendroglia) in cerebral white matter. CBD is non-specific, as pathologically proven cases include several clinical phenotypes. Pubmed and Google Scholar were systematically analyzed until October 2023, with focus on the prevalence, clinical manifestation, neuroimaging data, and treatment options of depression in CBD. Its prevalence is about 30–40% which is more frequent than in most other atypical parkinsonian syndromes. Depression usually does not correlate with motor and other clinical parameters, suggesting different pathophysiological mechanisms. Asymmetric atrophy and hypometabolism of frontoparietal cortical areas are associated with disruption of fronto-subcortical circuits, nigrostriatal dopaminergic, and cholinergic deficiency. Since no specific neuroimaging, neuropathological, or biomarker studies of depression in CBD are available, its pathobiological mechanisms and pathogenesis are poorly understood. Antidepressive therapy may be useful, but is often poorly tolerated. Depression in CBD, like in other parkinsonian syndromes, may be related to multi-regional patterns of cerebral disturbances and complex pathogenic mechanisms that deserve further elucidation as a basis for early diagnosis and adequate treatment to improve the quality of life in this fatal disease. [ABSTRACT FROM AUTHOR]