Link between post-stroke psychopathology and scope-of-action awareness.

Background: Epidemiological research has failed to confirm laterality of lesion site as a neurobiological source of post-stroke psychopathology. However, acquired communication disorders have proved to be a key risk factor for depression, apart from established parameters such as pre-stroke psychopathology and physical immobility. Objectives: The present work examines a new predictor of post-stroke psychopathology: psychological flexibility. This concept describes an accepting attitude toward irreversible loss following stroke while using remaining agency. Design: Overall, 70 individuals engaged in a cross-sectional study conducted in the subacute stage after an ischemic or hemorrhagic event, a period with elevated prevalence of mental-health problems (2 weeks to 6 months after stroke). Methods: Outcomes included standardized self-report and clinician-rated measures of depression, anxiety disorders, and general psychopathology (Beck Depression Inventory; Hospital Anxiety and Depression Scale; ICD-10 Symptom Rating; Hamilton Depression Rating Scale) alongside lack of psychological flexibility (Acceptance and Action Questionnaire II). The study design controlled for pre-stroke psychopathology and physical immobility (Barthel Index). Results: Partial correlation analyses revealed a significant medium-to-large association between the entire set of clinical outcomes and lack of psychological flexibility (r ⩽ 0.62, p < 0.001). In moderator analyses, the magnitude of this association did not vary significantly with diagnosis of acquired communication disorders (i.e., aphasia, apraxia of speech or dysarthria; separately or combined). Conclusion: The current results demonstrate a substantial link between post-stroke psychopathology and psychological flexibility. This finding opens new avenues for research on depression and other mental-health problems in stroke survivors with and without acquired communication disorders. Registration: www.drks.de; identifier: DRKS00031204.

Sense of agency may predict mental-health problems after stroke The present work examines a possible key to mental-health problems after stroke: psychological flexibility. By definition, this term describes an accepting attitude toward irreversible loss following stroke while using remaining agency. Showing a link between psychiatric syndromes and psychological flexibility, the current results motivate new behavioral forms of treatment beyond pharmacotherapy to alleviate mental-health problems after stroke.

A Japanese Patient With Progressive Word Deafness and Logoclonia.

Primary progressive aphasia (PPA) is classified into several types. However, syndromes that do not belong to these typical types of PPA have been reported, such as cases where only progressive apraxia of speech (AOS) is present in the early stages of the disease. Moreover, there have been a few case reports of patients with progressive word deafness complicated with logoclonia as a speech disorder. Herein, we report a case of degenerative disease with phonological paraphasia, AOS, impaired speech sound cognition, and logoclonia with disease progression. The written language comprehension of the patient was superior to that of spoken language, and she could communicate in writing. She was neurologically normal; had no apraxia, agnosia, or memory impairment; and was independent in activities of daily living. Although she had moderate hearing loss, her speech-related hearing loss was disproportionately high. In analyses of brain imaging, dilation of the left Sylvian fissure, atrophy, and hypoperfusion of the superior temporal gyrus were prominent, and hypoperfusion of the left lateral frontal lobe was observed. Cerebrospinal fluid examination suggested that Alzheimer's disease (AD) pathology was unlikely, and decreased uptake was observed on a dopamine-active transporter scan. Word deafness was thought to be the most likely cause of the dissociation between spoken and written language in our patient. Our case and previous studies suggested that there is a syndrome presenting word deafness and speech movement disorders among patients with progressive communication disorders as a different entity from PPA. Although patients with this syndrome may experience agrammatism, inner language disorders are generally not as severe, and disorders in areas closer to the primary area, such as the motor and auditory areas, as well as cortical and subcortical disorders, are involved in this syndrome.

Animal-assisted services for adults with acquired neurogenic communication disorders: A scoping review.

BACKGROUND: There is increasing interest in the incorporation of animal-assisted services (AAS) in therapy for adults with acquired neurogenic communication disorders. AAS have the potential to enhance speech and language therapy engagement and outcomes. However, a greater understanding of the nature and potential benefits of these interventions is needed. AIMS: To describe the existing evidence for the incorporation of AAS in therapy with adults with acquired neurogenic communication disorders and to identify areas for future research. METHODS & PROCEDURES: A scoping review was conducted and reported in alignment with the Preferred Reporting Items for Systematic Reviews and Meta-analyses checklist extension for scoping reviews (PRISMA-ScR). Seven databases (PubMed, Embase, CINAHL, PsycINFO, Cochrane, Scopus, Web of Science) and grey literature (Google) were searched. Two reviewers independently screened titles, abstracts and full texts against eligibility criteria using Covidence software. The Template for Intervention Description and Replication (TIDieR) checklist guided extraction of intervention data. MAIN CONTRIBUTIONS: A total of 17 studies with adults with aphasia, apraxia of speech and cognitive-communication disorders were included. While terminology varied, most interventions met the definition of animal-assisted therapy or animal-assisted activity and used therapy dogs. Across studies, a range of outcomes were targeted, and positive benefits were reported for participant mood, emotions, motivation and satisfaction. There were mixed, but mostly positive, benefits on social behaviour, communication and participation. CONCLUSIONS & IMPLICATIONS: AAS has been incorporated in therapy for adults with acquired neurogenic communication disorders, predominately with people with dementia. Across studies, communication impairments and AAS interventions were insufficiently or inconsistently described. Improved reporting would assist understanding of the potential benefits of AAS as an adjunct therapy. A quality appraisal of existing studies, and meta-analysis of findings, is needed to draw conclusions about the effectiveness of AAS as a complementary therapy for people with acquired neurogenic communication disorders. WHAT THIS PAPER ADDS: What is already known on this subject There is increasing interest and research in AAS as an adjunct to traditional speech and language therapy. Several clinical populations have been shown to benefit from the incorporation of AAS as a complementary therapy approach, including adults with acquired neurogenic communication disorders. To date there has not been a comprehensive review of literature in the area. What this paper adds to the existing knowledge This review aimed to describe what is known about AAS as an adjunct intervention for adults with acquired neurogenic communication disorders. A total of 17 studies were identified, the majority conducted with people living with dementia. Overall, consistent positive benefits of AAS were reported on participant mood, positive emotions, motivation, and satisfaction. There were mixed, but mostly positive, benefits of AAS on social behaviour, including both verbal and non-verbal communication outcomes, and participation. What are the practical and clinical implications of this work? These results suggest a potential therapeutic benefit for the inclusion of AAS into traditional modalities used by speech-language therapists with people with acquired neurogenic communication disorders. However, clinicians should view these findings with caution. Studies were often insufficiently or inconsistently reported, and a quality appraisal and meta-analysis of existing studies would be needed to draw clear conclusions on the effectiveness of AAS as a complementary approach for people with acquired neurogenic communication disorders.

Expanding the molecular landscape of childhood apraxia of speech: evidence from a single-center experience.

Background: Childhood apraxia of speech (CAS) is a genetically heterogeneous pediatric motor speech disorder. The advent of whole exome sequencing (WES) and whole genome sequencing techniques has led to increased identification of pathogenic variants in CAS genes. In an as yet uncharacterized Italian cohort, we aimed both to identify new pathogenic gene variants associated with CAS, and to confirm the disease-related role of genes already reported by others. We also set out to refine the clinical and neurodevelopmental characterization of affected children, with the aim of identifying specific, gene-related phenotypes. Methods: In a single-center study aiming to explore the genetic etiology of CAS in a cohort of 69 Italian children, WES was performed in the families of the 34 children found to have no copy number variants. Each of these families had only one child affected by CAS. Results: High-confidence (HC) gene variants were identified in 7/34 probands, in two of whom they affected KAT6A and CREBBP, thus confirming the involvement of these genes in speech impairment. The other probands carried variants in low-confidence (LC) genes, and 20 of these variants occurred in genes not previously reported as associated with CAS. UBA6, ZFHX4, and KAT6A genes were found to be more enriched in the CAS cohort compared to control individuals. Our results also showed that most HC genes are involved in epigenetic mechanisms and are expressed in brain regions linked to language acquisition processes. Conclusion: Our findings confirm a relatively high diagnostic yield in Italian patients.

Considerations for identifying subtypes of speech sound disorder.

BACKGROUND: 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. AIMS: To study the distinct underlying processes that differentiate phonological processing, phonological planning or motor planning deficits. METHOD: The literature on the nature of SSDs is reviewed to reveal diagnostic signs at the level of distal causes, proximal factors and surface characteristics. MAIN CONTRIBUTION: 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. CONCLUSIONS & IMPLICATIONS: 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. WHAT THIS PAPER ADDS: What is already known on the subject 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). What this paper adds to the existing knowledge 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. What are the potential or actual clinical implications of this work? 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.

MED13L-related disorder characterized by severe motor speech impairment.

Background: MED13L-related disorder is associated with intellectual disability, motor delay, and speech deficits. Previous studies have focused on broad clinical descriptions of individuals, but limited information regarding specific speech diagnoses and results of direct testing has been published to date. We conducted deep phenotyping to characterize the speech, language, motor, cognitive, and adaptive phenotypes of individuals with MED13L-related disorder. Methods: In this cross-sectional study, we administered standardized articulation, language, motor, and cognitive testing to 17 children and adolescents (mean age 9y 9m; SD 4y 5m; range 4y 2m to 19y 7m). In-person testing was supplemented with broad developmental, medical, and behavioral information collected virtually from a cohort of 67 individuals. Results: All individuals who completed in-person articulation testing met diagnostic criteria for speech apraxia, dysarthria, or both. Language impairment was present in all of the in-person cohort and almost all (97%) of the virtual cohort. Those who were able to complete motor testing demonstrated significant deficits in visual motor integration (mean 57.08, SD 9.26). Full scale IQs fell in the borderline to intellectual disability range, consistent with reported cognitive impairment in 97% of the virtual cohort. Notable medical features included hypotonia (83%), vision problems (72%), recurrent otitis media (58%), gastrointestinal problems (57%), and seizures (31%). Conclusions: MED13L-related disorder is characterized by a high rate of motor speech disorders that occur in the context of globally impaired motor, language, and cognitive skills. Children would benefit from intensive, individualized speech therapy and the early adoption of augmentative communication strategies.

Caregiver and child perspectives of wellbeing in school-age children with childhood apraxia of speech.

PURPOSE: Childhood apraxia of speech (CAS) is a communication deficit that impacts a child's overall quality of life, including their academic and social interactions, yet few studies have investigated the impact from the child's perspective. Using The World Health Organization's International Classification of Functioning, Disability, and Health (ICF) framework, we examined the children's and caregivers' perspectives on the impact of CAS on their overall wellbeing. METHOD: Survey data were collected from 32 child-caregiver dyads for children ages 9-17 years with CAS. Items elicited responses along a 5-point scale to examine the perceived impact of children's speech impairments using the framework, including impact on communication, interpersonal interactions/relationships, and major life events. RESULT: Results from non-parametric statistical analyses revealed: (a) caregivers reported a significantly greater impact than children across individual ICF domains; (b) child responses were not significantly correlated with their caregiver's responses or with the child's age, gender, or severity indicators; and (c) both caregivers and children indicated the domain of communication was impacted more than other domains. CONCLUSION: Results suggest a potential disconnect in the perceived impact of CAS between the caregiver and child. Recommendations include facilitating a dialogue between the child-caregiver dyad and considering treatment goals that address wellbeing.

A systematic scoping review of the literacy skills of children with childhood apraxia of speech: Recommendations for best practice and further research.

PURPOSE: 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. METHOD: Nine databases were searched to identify relevant articles. Included articles were categorised by study design, quality, and confidence of CAS diagnosis. RESULT: 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. CONCLUSIONS: 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.

A targeted review of prosodic production in agrammatic aphasia.

It is unclear whether individuals with agrammatic aphasia have particularly disrupted prosody, or in fact have relatively preserved prosody they can use in a compensatory way. A targeted literature review was undertaken to examine the evidence regarding the capacity of speakers with agrammatic aphasia to produce prosody. The aim was to answer the question, how much prosody can a speaker "do" with limited syntax? The literature was systematically searched for articles examining the production of grammatical prosody in people with agrammatism, and yielded 16 studies that were ultimately included in this review. Participant inclusion criteria, spoken language tasks, and analysis procedures vary widely across studies. The evidence indicates that timing aspects of prosody are disrupted in people with agrammatic aphasia, while the use of pitch and amplitude cues is more likely to be preserved in this population. Some, but not all, of these timing differences may be attributable to motor speech programming deficits (AOS) rather than aphasia, as these conditions frequently co-occur. Many of the included studies do not address AOS and its possible role in any observed effects. Finally, the available evidence indicates that even speakers with severe aphasia show a degree of preserved prosody in functional communication.

Childhood Apraxia of Speech: A Descriptive and Prescriptive Model of Assessment and Diagnosis.

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.

The yes-no reversal phenomenon in patients with primary progressive apraxia of speech.

Patients who have a yes-no reversal respond "yes" when they mean no and vice versa. The unintentional response can be made both verbally and with gestures (e.g., head shake or nod, thumbs up or down). Preliminary reports associate this phenomenon with 4-repeat tauopathies including primary progressive apraxia of speech (PPAOS), nonfluent/agrammatic primary progressive aphasia, and corticobasal syndrome; however, the significance and timing of this symptom relative to others are not well understood. Whereas some accounts associate yes-no reversals with other binary reversals (e.g., up/down, hot/cold) and attribute the reversals to disturbances of selection within the language system, others implicate more general inhibitory control processes. Here, we compared clinical and neuroimaging findings across 30 patients with PPAOS (apraxia of speech in the absence of aphasia), 15 of whom had a yes-no reversal complaint and 15 who did not. The two groups did not differ on any of the language or motor speech measures; however, patients who had the yes-no reversal received lower scores on the Frontal Assessment Battery and motor assessments. They also had greater hypometabolism in the left supplementary motor area and bilateral caudate nuclei on [18F]-fluorodeoxyglucose PET, but only the right caudate nucleus cluster survived correction for multiple comparisons. We interpret these results to suggest that the yes-no reversal phenomenon is associated with cognitive abilities that are supported by the frontostriatal network; more specifically, impaired response inhibition.

Multimodal cross-examination of progressive apraxia of speech by diffusion tensor imaging-based tractography and Tau-PET scans.

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.

Efficacy of LSVT LOUD® on Phonatory Control and Voice Quality in Patients with Primary Progressive Apraxia of Speech: Case Studies.

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.

The encoding of speech modes in motor speech disorders: whispered versus normal speech in apraxia of speech and hypokinetic dysarthria.

Speakers with motor speech disorders (MSD) present challenges in speech production, one of them being the difficulty to adapt their speech to different modes. However, it is unclear whether different types of MSD are similarly affected when it comes to adapting their speech to various communication contexts. This study investigates the encoding of speech modes in individuals with AoS following focal brain damage and in individuals with hypokinetic dysarthria (HD) secondary to Parkinson's disease. Participants with mild-to-moderate MSD and their age-matched controls performed a delayed production task of pseudo-words in two speech modes: normal and whispered speech. While overall accuracy did not differ significantly across speech modes, participants with AoS exhibited longer response latencies for whispered speech, reflecting difficulties in the initiation of utterances requiring an unvoiced production. In contrast, participants with HD showed faster response latencies for whispered speech, indicating that this speech mode is easier to encode/control for this population. Acoustic durations followed these same trends, with participants with AoS showing greater lengthening for whispered speech as compared to controls and to participants with HD, while participants with HD exhibited milder lengthening. Contrary to the predictions of speech production models, suggesting that speech mode changes might be particularly difficult in dysarthria, the present results suggest that speech mode adaptation rather seems particularly costly for participants with AoS.

Differential spectral characteristics of the Spanish fricative /s/ in the articulation of individuals with dysarthria and apraxia of speech.

PURPOSE: This study examines whether there are differences in the speech of speakers with dysarthria, speakers with apraxia and healthy speakers in spectral acoustic measures during production of the central-peninsular Spanish alveolar sibilant fricative /s/. METHOD: To this end, production of the sibilant was analyzed in 20 subjects with dysarthria, 8 with apraxia of speech and 28 healthy speakers. Participants produced 12 sV(C) words. The variables compared across groups were the fricative's spectral amplitude difference (AmpD) and spectral moments in the temporal midpoint of fricative execution. RESULTS: The results indicate that individuals with dysarthria can be distinguished from healthy speakers in terms of the spectral characteristics AmpD, standard deviation (SD), center of gravity (CoG) and skewness, the last two in context with unrounded vowel, while no differences in kurtosis were detected. Participants with AoS group differ significantly from healthy speaker group in AmpD, SD and CoG and Kurtosis, the first one followed unrounded vowel and the latter two followed by rounded vowels. In addition, speakers with apraxia of speech group returned significant differences with respect to speakers with dysarthria group in AmpD, CoG and skewness. CONCLUSIONS: The differences found between the groups in the measures studied as a function of the type of vowel context could provide insights into the distinctive manifestations of motor speech disorders, contributing to the differential diagnosis between apraxia and dysarthria in motor control processes.

Histologic tau lesions and magnetic resonance imaging biomarkers differ across two progressive supranuclear palsy variants.

Progressive supranuclear palsy is a neurodegenerative disease characterized by the deposition of four-repeat tau in neuronal and glial lesions in the brainstem, cerebellar, subcortical and cortical brain regions. There are varying clinical presentations of progressive supranuclear palsy with different neuroimaging signatures, presumed to be due to different topographical distributions and burden of tau. The classic Richardson syndrome presentation is considered a subcortical variant, whilst progressive supranuclear palsy with predominant speech and language impairment is considered a cortical variant, although the pathological underpinnings of these variants are unclear. In this case-control study, we aimed to determine whether patterns of regional tau pathology differed between these variants and whether tau burden correlated with neuroimaging. Thirty-three neuropathologically confirmed progressive supranuclear palsy patients with either the Richardson syndrome (n = 17) or speech/language (n = 16) variant and ante-mortem magnetic resonance imaging were included. Tau lesion burden was semi-quantitatively graded in cerebellar, brainstem, subcortical and cortical regions and combined to form neuronal and glial tau scores. Regional magnetic resonance imaging volumes were converted to Z-scores using 33 age- and sex-matched controls. Diffusion tensor imaging metrics, including fractional anisotropy and mean diffusivity, were calculated. Tau burden and neuroimaging metrics were compared between groups and correlated using linear regression models. Neuronal and glial tau burden were higher in motor and superior frontal cortices in the speech/language variant. In the subcortical and brainstem regions, only the glial tau burden differed, with a higher burden in globus pallidus, subthalamic nucleus, substantia nigra and red nucleus in Richardson's syndrome. No differences were observed in the cerebellar dentate and striatum. Greater volume loss was observed in the motor cortex in the speech/language variant and in the subthalamic nucleus, red nucleus and midbrain in Richardson's syndrome. Fractional anisotropy was lower in the midbrain and superior cerebellar peduncle in Richardson's syndrome. Mean diffusivity was greater in the superior frontal cortex in the speech/language variant and midbrain in Richardson's syndrome. Neuronal tau burden showed associations with volume loss, lower fractional anisotropy and higher mean diffusivity in the superior frontal cortex, although these findings did not survive correction for multiple comparisons. Results suggest that a shift in the distribution of tau, particularly neuronal tau, within the progressive supranuclear palsy network of regions is driving different clinical presentations in progressive supranuclear palsy. The possibility of different disease epicentres in these clinical variants has potential implications for the use of imaging biomarkers in progressive supranuclear palsy.

The Effects of Yoga Breathing Before Motor Speech Practice in Acquired Apraxia of Speech: An n-of-1 Trial.

A previous study discovered that two speakers with moderate apraxia of speech increased their sequential motion rates after unilateral forced-nostril breathing (UFNB) practiced as an adjunct to speech-language therapy in an AB repeated-measures design. The current study sought to: (1) delineate possible UFNB plus practice effects from practice effects alone in motor speech skills; (2) examine the relationships between UFNB integrity, participant-reported stress levels, and motor speech performance; and (3) sample a participant-led UFNB training schedule to contribute to the literature's growing understanding of UFNB dosage. A single-subject (n-of-1 trial), ABAB reversal design was used across four motor speech behaviors. A 60-year-old female with chronic, severe apraxia of speech participated. The researchers developed a breathing app to assess UFNB practice integrity and administer the Simple Aphasia Stress Scale after each UFNB session. The participant improved from overall severe to moderate apraxia of speech on the Apraxia Battery for Adults. Visual inspection of graphs confirmed robust motor speech practice effects for all variables. Articulatory-kinematic variables demonstrated sensitivity to the UFNB-plus-practice condition and correlated to stress scale scores but not UFNB integrity scores. The participant achieved 20-minute UFNB sessions 4 times per week. Removal of UFNB during A2 (UFNB withdrawal) and after a 10-day break during B2 (UFNB full dosage) revealed UFNB practice effects on stress scale scores. UFNB with motor speech practice may benefit articulatory-kinematic skills compared to motor speech practice alone. Regular, cumulative UFNB practice appeared to lower self-perceived stress levels. These findings, along with prior work, provide a foundation to further explore yoga breathing and its use with speakers who have apraxia of speech.

Progression to corticobasal syndrome: a longitudinal study of patients with nonfluent primary progressive aphasia and primary progressive apraxia of speech.

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.

Combined assessment of progressive apraxia of speech brain microstructure by diffusion tensor imaging tractography and multishell neurite orientation dispersion and density imaging.

BACKGROUND: Progressive apraxia of speech (PAOS) is characterized by difficulties with motor speech programming and planning. PAOS targets gray matter (GM) and white matter (WM) microstructure that can be assessed using diffusion tensor imaging (DTI) and multishell applications, such as neurite orientation dispersion and density imaging (NODDI). In this study, we aimed to apply DTI and NODDI to add further insight into PAOS tissue microstructure. METHODS: Twenty-two PAOS patients and 26 age- and sex-matched controls, recruited by the Neurodegenerative Research Group (NRG) at Mayo Clinic, underwent diffusion MRI on 3T MRI. Brain maps of fractional anisotropy (FA) and mean diffusivity (MD) from DTI and intracellular volume fraction (ICVF) and isotropic volume fraction (IsoVF) from NODDI were generated. Global WM and GM, and specific WM tracts were identified using tractography and lobar GM regions. RESULTS: Global WM differences between PAOS and controls were greatest for ICVF, and global GM differences were greatest for MD and IsoVF. Abnormalities in key WM tracts involved in PAOS, including the body of the corpus callosum and frontal aslant tract, were identified with FA, MD, and ICVF, with excellent differentiation of PAOS from controls (area under the receiver operating characteristic curves >.90). MD and ICVF identified abnormalities in arcuate fasciculus, thalamic radiations, and corticostriatal tracts. Significant correlations were identified between an index of articulatory errors and DTI and NODDI metrics from the arcuate fasciculus, frontal aslant tract, and inferior longitudinal fasciculus. CONCLUSIONS: DTI and NODDI represent different aspects of brain tissue microstructure, increasing the number of potential biomarkers for PAOS.