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.

Localizing apraxia in corticobasal syndrome: a morphometric MRI study.

Apraxia localization has relied on voxel-based, lesion-symptom mapping studies in left hemisphere stroke patients. Studies on the neural substrates of different manifestations of apraxia in neurodegenerative disorders are scarce. The primary aim of this study was to look into the neural substrates of different manifestations of apraxia in a cohort of corticobasal syndrome patients (CBS) by use of cortical thickness. Twenty-six CBS patients were included in this cross-sectional study. The Goldenberg apraxia test (GAT) was applied. 3D-T1-weighted images were analyzed via the automated recon-all Freesurfer version 6.0 pipeline. Vertex-based multivariate General Linear Model analysis was applied to correlate GAT scores with cortical thickness. Deficits in imitation of meaningless gestures correlated with bilateral superior parietal atrophy, extending to the angular and supramarginal gyri, particularly on the left. Finger imitation relied predominantly on superior parietal lobes, whereas the left angular and supramarginal gyri, in addition to superior parietal lobes, were critical for hand imitation. The widespread bilateral clusters of atrophy in CBS related to apraxia indicate different pathophysiological mechanisms mediating praxis in neurodegenerative disorders compared to vascular lesions, with implications both for our understanding of praxis and for the rehabilitation approaches of patients with apraxia.

Apraxia Patterns for the Differentiation between Alzheimer's Disease and Frontotemporal Dementia Variants.

Background and Objectives: Despite the increasing use of biomarkers, differentiation between Alzheimer's disease (AD), behavioral variant Frontotemporal Dementia (bvFTD), and Primary Progressive Aphasia (PPA) remains a challenge. Apraxia is a supportive feature for diagnosing AD but is underrepresented in other dementia types. Herein, we investigated the presence and characteristic profiles of limb, verbal, and non-verbal apraxia in three major dementia types. Materials and Methods: Test for Upper Limb Apraxia (TULIA) and Apraxia Battery for Adults-2 (ABA-2) were administered in patients with AD (n = 22), bvFTD (n = 41), and PPA (n = 22), with 20 individuals serving as healthy controls (HC). Composite and subdomain scores were compared between each patient group and the HC. Praxis profiles indicative of each dementia type and a possible predictive value were sought. Results: Apraxia provided high diagnostic accuracy for detecting dementia compared with HC (sensitivity: 63.6-100%, specificity: 79.2-100%). Patients with AD performed worse when imitating intransitive gestures as well as pantomiming transitive gestures (mean differences: 2.10 and 3.12, respectively), compared with bvFTD. PPA patients, compared with bvFTD, had comparable results in limb, verbal, and non-verbal praxis assessments, despite the greater deterioration in the outcome. Compared with patients with AD, PPA had increased pathological outcomes in verbal (86.4% vs. 40.9%) and non-verbal apraxia (31.8% vs. 0%), while bvFTD had increased pathological outcomes in verbal apraxia (85.4% vs. 44.5%). Finally, apraxia is correlated with cognitive decline. Conclusions: Apraxia profile evaluation could contribute to the differentiation between AD and Frontotemporal Dementia (FTD). Both TULIA and ABA-2 are reliable tools that can be performed as bed-side tests in clinical practice.

Dynamic Temporal and Tactile Cueing in Young Children With Childhood Apraxia of Speech: A Multiple Single-Case Design.

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.

Beyond 'speech delay': Expanding the phenotype of BRPF1-related disorder.

Pathogenic variants in BRPF1 cause intellectual disability, ptosis and facial dysmorphism. Speech and language deficits have been identified as a manifestation of BRPF1-related disorder but have not been systematically characterized. We provide a comprehensive delineation of speech and language abilities in BRPF1-related disorder and expand the phenotype. Speech and language, and health and medical history were assessed in 15 participants (male = 10, median age = 7 years 4 months) with 14 BRPF1 variants. Language disorders were common (11/12), and most had mild to moderate deficits across receptive, expressive, written, and social-pragmatic domains. Speech disorders were frequent (7/9), including phonological delay (6/9) and disorder (3/9), and childhood apraxia of speech (3/9). All those tested for cognitive abilities had a FSIQ ≥70 (4/4). Participants had vision impairment (13/15), fine (8/15) and gross motor delay (10/15) which often resolved in later childhood, infant feeding impairment (8/15), and infant hypotonia (9/15). We have implicated BRPF1-related disorder as causative for speech and language disorder, including childhood apraxia of speech. Adaptive behavior and cognition were strengths when compared to other monogenic neurodevelopmental chromatin-related disorders. The universal involvement of speech and language impairment is noteable, relative to the high degree of phenotypic variability in BRPF1-related disorder.

[Early indicators of childhood apraxia of speech in late talkers: general guidelines to intervention]. / Indicadores tempranos de dispraxia verbal en el inicio tardío: orientaciones para la intervención Inmaculada.

INTRODUCTION: The population of children with slow emergence of language development varies widely, both in their initial profile and in their response to intervention. In this sense, there is a group of late talkers who continue to show persistent language difficulties, in some cases exhibiting signs compatible with verbal dyspraxia. METHOD: In this paper we present the different response to intervention of two profiles of late talkers. Specifically, the Target Word© program (Hanen Centre) was implemented, which is addressed to latetalking children and their families. It combines the technique of focused stimulation with guidance to parents on strategies that stimulate global language development. RESULTS: Much of the symptomatology shown in one case of poor progress coincides with retrospective descriptions of children subsequently diagnosed with dyspraxia and can be considered early indicators of the disorder: unintelligibility, reduced consonant inventory or difficulties in word repetition. DISCUSSION: The different response to intervention contributes to diagnostic decision making and the early implementation of specific strategies directed to improve speech learning skills by incorporating motor learning principles. The few studies of intervention in suspected verbal dyspraxia in early childhood offer promising results on a variety of speech assessment indicators, and provide practitioners with valuable information with which to support the intervention in this population.

Introducción: La población de niños que comienzan con lentitud el desarrollo del lenguaje varía ampliamente, tanto en su perfil inicial como en la respuesta a la intervención. En este sentido, existe un grupo de niños, denominados hablantes tardíos, que continúan mostrando dificultades persistentes en el lenguaje. Algunos de estos niños muestran signos compatibles con la dispraxia verbal, y que se ponen de manifiesto a lo largo de la intervención. Método: En este trabajo presentamos la diferente respuesta a la intervención de dos perfiles de hablante tardío. Concretamente, se aplicó el programa Target Word©, del centro Hanen, que conjuga la técnica de la estimulación focalizada con la orientación a padres sobre estrategias que promueven el desarrollo del lenguaje. Resultados: Gran parte de la sintomatología mostrada en uno de los dos casos, que experimentó un progreso pobre, coincide con las descripciones retrospectivas de niños posteriormente diagnosticados con dispraxia y pueden considerarse indicadores tempranos del trastorno: ininteligibilidad, inventario consonántico reducido o dificultades en la repetición de palabras. Discusión: La diferente respuesta a la intervención contribuye a la toma de decisiones diagnósticas y a la aplicación temprana de estrategias específicas para la mejora de las habilidades de aprendizaje del habla mediante la incorporación de los principios del aprendizaje motor. Los escasos estudios de intervención en casos de sospecha de dispraxia verbal en la infancia temprana ofrecen resultados prometedores en diversos indicadores de evaluación del habla, y proporcionan a los profesionales una información valiosa en la que fundamentar la intervención en esta población.

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.

Characterizing Speech Errors Across Primary Progressive Apraxia of Speech Subtypes.

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 subtypes 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.

Characteristics of Lexical Stress in Hebrew-Speaking Children With Childhood Apraxia of Speech.

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.

What works, how and in which contexts when supporting parents to implement intensive speech and language therapy at home for children with speech sound disorder? A protocol for a realist review.

INTRODUCTION: Speech and language therapists (SLTs) worldwide report challenges with providing recommended, evidence-based intervention intensity for children with speech sound disorder (SSD). Challenges such as service constraints and/or family contexts impact on access to optimal therapy intensity. Existing research indicates that empowering and training parents to deliver intervention at home, alongside SLT support, offers one possible solution to increasing the intensity of intervention children with SSD receive. Digital health could increase accessibility to intensive home practice and help sustain engagement with therapy activities. Further exploration is needed around what makes parent-implemented interventions for children with SSD effective, for who and in which situations. This paper outlines the protocol for a realist review which aims to explore the active ingredients and contextual factors of effective digital parent-led interventions. METHODS AND ANALYSIS: A realist review will explore the research question, following six stages. The scope of the review will be determined, and initial programme theories will be developed about what works in digital parent-implemented interventions for SSD, for whom, how, why and in what circumstances. Relevant secondary data, identified through a formal search strategy, will be selected, appraised, analysed and synthesised using realist principles to test and further refine the initial programme theories. This process will develop refined underpinning explanatory theories which capture the interaction between contexts, mechanisms and outcomes of the intervention. An expert steering group will provide insight to inform explanatory theories, searches, and dissemination. ETHICS AND DISSEMINATION: Ethical approval is not required for this review. The refined programme theories from the review will inform the next stages of a wider study. A subsequent realist evaluation will test and further refine theories with key stakeholders. Following this, the underpinning programme theory will be used to coproduce a digital tool, to support parents to deliver home intervention alongside SLT support.

Navigation abilities and spatial anxiety in individuals with and without Developmental Coordination Disorder (DCD/Dyspraxia).

BACKGROUND: Navigation skills are essential for independent living as they allow us to explore our environment; find our way to new locations, refine pathways to familiar locations and retrace our route home. Alongside motor coordination difficulties, there is evidence that individuals with Developmental Coordination Disorder (DCD/Dyspraxia) experience spatial processing difficulties, which are known to negatively affect navigation abilities. However, although self-reports indicate that adults with DCD have difficulties with sense of direction and navigation, no known studies have measured navigation abilities and strategies in adults with DCD. Furthermore, given evidence that individuals with DCD report higher levels of anxiety, we will additionally investigate associations between anxiety and navigation in this group. AIMS: This study compares navigation abilities, navigation strategies and spatial anxiety in adults with and without DCD. METHODS: Participants include 226 Adults aged 18-55 years, across two groups 1) DCD (N = 138, 111 F:25 M; 2:Other) 2); Typically Developing (N = 88, 77 F: 11 M). In this cross-sectional study, participants completed a series of tasks on the online Qualtrics platform. This included the Adult Developmental Coordination Disorder Checklist, the State-Trait Anxiety Inventory, the Wayfinding Anxiety Measure, the Wayfinding Questionnaire, the Wayfinding Strategy Questionnaire, and a navigation task. RESULTS: Our analysis shows that 1) compared to those with typical development, individuals with DCD have similar navigation performance but lower navigation and orientation scores, and distance estimation scores. 2) Movement co-ordination difficulties were only a significant predictor of landmark recognition and egocentric path route knowledge, and played no role for other aspects of navigation performance. 3) For wayfinding strategy use the DCD group used orientation strategies significantly less often than those with typical development, however there was no group difference in the use of route strategies. 4) The DCD group had significantly higher spatial anxiety scores across navigation, manipulation and imagery spatial sub-domains, even after controlling for general anxiety. 5) Spatial navigation anxiety was a significant predictor of navigational skill for all three wayfinding measures (navigation & orientation, distance estimation and spatial anxiety). CONCLUSIONS: The findings establish benchmarks of navigational skills in DCD and highlight spatial anxiety and route strategies as factors that may inhibit navigation success and could help specify suitable intervention targets.

Predicting Disability in Progressive Supranuclear Palsy Using Bedside Frontal-Lobe Signs.

BACKGROUND: Frontal lobe signs in progressive supranuclear palsy (PSP) are prevalent and occur early in the disease. Although they are recognized in clinical practice, studies are needed to systematically investigate them for an in-depth understanding of the neurological substrate and their potential prognostic implications in the disease. OBJECTIVES: To study the predictive role of frontal lobe signs in PSP, as well as to describe their neuropsychological and anatomical correlations. METHODS: Nine recognized signs of frontal lobe dysfunction were assessed in 61 patients with PSP. Those signs able to predict PSP Rating Scale (PSPRS) score at baseline were selected, a survival analysis was performed and associations with neuropsychological tests and cortical thickness parameters in brain MRI were studied. RESULTS: Grasping, anosognosia and orobuccal apraxia predicted the PSPRS score independently of age, gender, clinical subtype and disease duration. The occurrence of groping in the first 4 years could be a predictor of survival. Grasping and anosognosia were associated with frontal cognitive dysfunction, whereas orobuccal apraxia and groping were related to a more widespread cognitive impairment, involving temporal-parietal areas. Presence of groping showed an extensive cortical atrophy, with predominant prefrontal, temporal and superior parietal cortical thinning. CONCLUSIONS: Grasping, groping, anosognosia and orobuccal apraxia are easily evaluable bedside clinical signs that reflect distinct stages of disease progression. Grasping, anosognosia and orobuccal apraxia predict disease disability in patients with PSP, and early onset groping could be a survival predictor.

Atypical Cogan's syndrome: A case report.

Cogan's syndrome is a rare autoimmune inflammatory disease, characterized by interstitial keratitis and audio-vestibular signs. The syndrome was first described in 1945 by David G. Cogan. Then, it was only in 1980 when Haynes et al. proposed diagnostic criteria for patients with other symptoms and was qualified as atypical form of Cogan's syndrome. Herein, we report a case of a 28-year-old woman with atypical Cogan's syndrome. The patient was treated with corticosteroids and received a cochlear implant.

The impact for DCD - USA study: The current state of Developmental Coordination Disorder (DCD) in the United States of America.

BACKGROUND: Developmental Coordination Disorder (DCD) is among the most under-recognized and under-supported disorders worldwide. AIMS: To present a preliminary national study that evaluated the unmet needs of children with DCD in the USA using the Impact for DCD survey. METHODS AND PROCEDURES: 232 parents of individuals aged 5-18 years provided responses from 36 items in five domains (diagnosis, activity/participation, education, therapy, and social/emotional health). OUTCOMES AND RESULTS: Most children (81.9%) had a formal diagnosis for movement difficulties, and 91.6% of parents reported that receiving a diagnosis was helpful, but most had not heard of the diagnosis before. The most common co-occurring diagnoses were childhood apraxia of speech and other speech-language disorders (24.6%), ADHD (23.1%), and anxiety (18.8%). Most parents reported that their children withdrew from or avoided movement-related activities (53%), and nearly all (94.8%) were concerned about the impact of motor difficulties on their children's social and emotional health. Only 37% of parents reported feeling that their child received sufficient therapy. CONCLUSIONS AND IMPLICATIONS: Generally, parents reported feeling frustrated with others' understanding and awareness of the condition and with therapy services. The results shown here provide timely data that can support efforts for increased awareness, improved diagnosis, and increased availability of services for DCD in the USA.

Diffusion tensor imaging-based multi-fiber tracking reconstructions can regionally differentiate phonetic versus prosodic subtypes of progressive apraxia of speech.

Two subtypes of progressive apraxia of speech (PAOS) have been recognized: phonetic PAOS (PAOS_ph) where speech output is dominated by distorted sound substitutions and prosodic PAOS (PAOS_pr) which is dominated by segmented speech. We investigate whether these PAOS subtypes have different white matter microstructural abnormalities measured by diffusion tensor tractography. Thirty-three patients with PAOS (21 PAOS_ph and 12 PAOS_pr) and 19 healthy controls were recruited by the Neurodegenerative Research Group (NRG) and underwent diffusion MRI. Using a whole-brain tractography approach, fractional anisotropy (FA) and mean diffusivity (MD) were extracted for cortico-cortical, cortico-subcortical, cortical-projection, and cerebello-cortical white matter tracts. A hierarchical linear model was applied to assess tract-level FA and MD across groups. Both PAOS_ph and PAOS_pr showed degeneration of cortico-cortical, cortico-subcortical, cortical-projection, and cerebello-cortical white matter tracts compared to controls. However, degeneration of the body of corpus callosum, superior thalamic radiation, and superior cerebellar peduncle was greater in PAOS_pr compared to PAOS_ph, and degeneration of the inferior segment of the superior longitudinal fasciculus (SLF) was greater in PAOS_ph compared to PAOS_pr. Worse parkinsonism correlated with greater degeneration of cortico-cortical and cortico-subcortical tracts in PAOS_ph. Apraxia of speech articulatory error score correlated with degeneration of the superior cerebellar peduncle tracts in PAOS_pr. Phonetic and prosodic PAOS involve the compromise of a similar network of tracts, although there are connectivity differences between types. Whereas clinical parameters are the current gold standard to distinguish PAOS subtypes, our results allege the use of DTI-based tractography as a supplementary method to investigate such variants.

Pitch Variation Skills in Cantonese Speakers With Apraxia of Speech After Stroke: Preliminary Findings of Acoustic Analyses.

PURPOSE: Literature on apraxia of speech (AOS) in Chinese speakers is sparse compared to the English literature. This study aims to examine the pitch variation skills of Cantonese adults with AOS poststroke in terms of perceptual tone accuracy, acoustic fundamental frequency (fo) changes, and repetition durations on items with different syllable structures, lexical status, and tone syllables in various positions in a sequencing context. METHOD: Six Cantonese adults with AOS poststroke (AOS group), six adults without AOS poststroke (nAOS group), and six healthy controls (HC group) performed the tone sequencing task (TST), which was adapted from oral diadochokinetic tasks, with three different tone syllables. Tone accuracy, fo values across 10 time points, and acoustic repetition durations were compared within and between the groups. RESULTS: The AOS group produced significantly lower tone accuracy and different fo changes on the three Cantonese tone syllables compared with the control groups and significantly longer repetition durations than the HC group. The AOS group showed more difficulty with the tone syllables with the consonant-vowel structure, while a priming effect was observed on the T2 (high-rising) syllables with lexical meanings. A unique lowering of fo in the final syllable of the trisyllabic items was observed only in the AOS group. CONCLUSIONS: The AOS group showed degraded pitch variation skills. The effects of the three linguistic elements were discussed. Future investigations are called for to adapt the TST in other tonal languages to determine if degraded pitch variation skills are present in other tonal language speakers with AOS.

[Behçet's and Cogan's syndromes - The Variable Vessel Vasculitides]. / Behçet- und Cogan-Syndrom.

Behçet's syndrome and Cogan's syndrome constitute the group of variable vessel vasculitides in the Chapel-Hill Nomenclature. They involve arteries and veins of all sizes. As reflected in the name "syndrome", both diseases can manifest with different individual symptoms. Both formally are rare diseases, but the Cogan syndrome is much rarer than Behçet`s. For the latter, there are diagnosis and classification criteria as well as European (EULAR, European Alliance of Associations for Rheumatology) treatment recommendations. The symptomatology, diagnostic measures and treatment as well as some considerations about pathogenesis will be discussed in this article.

The Role of Dual-Phase 18 F-FP-CIT PET to Early Diagnosis of Corticobasal Syndrome.

BACKGROUND: Corticobasal syndrome (CBS) is a neurodegeneration characterized by asymmetric parkinsonism, dystonia, myoclonus, and apraxia. In the early stage, CBS presents with asymmetric parkinsonism and cortical symptoms (apraxia and alien hand), and neuroimaging finding is often vague, making early clinical differentiation from idiopathic Parkinson disease (IPD) challenging. This study was performed to delineate the specific patterns of cortical hypoperfusion, dopamine transporter (DAT) uptake using dual-phase FP-CIT PET in discriminating between CBS and IPD at early stage. PATIENTS AND METHODS: The study enrolled clinically diagnosed CBS (n = 11) and IPD (n = 22) patients (age and sex matched). All participants underwent dual-phase 18 F-FP-CIT PET, and regional SUV ratio (SUVR) was obtained by semiquantitative analysis. The early perfusion imaging and DAT imaging were compared between groups. RESULTS: The regional SUVRs (early phase) of the frontal lobe, thalamus, cingulate, and caudate were significantly lower in patients with CBS, whereas the SUVR of occipital lobe was lower in the IPD group. The CBS group exhibited more prominent asymmetry than the IPD group, particularly in the perirolandic area, superior frontal gyrus, and anterior parietal lobe in early phase PET. Striatal DAT uptake (delayed phase) revealed that the caudate showed lower SUVR and prominent asymmetry in the CBS group, and the caudate-to-putamen ratio (CP ratio) was significantly lower in CBS patients ( P < 0.001). Among the parameters (early and delayed), the CP ratio in DAT exhibited the most powerful discriminative power from receiver operating characteristic curve comparison (area under curve = 0.983). CONCLUSIONS: This study demonstrated that the dual-phase FP-CIT PET is useful in differentiating CBS and IPD in the early stage of the disease, and a lower CP ratio of DAT imaging is highly informative for distinguishing between corticobasal degeneration and IPD.

Clinical and MRI characterization of apraxic syndrome in corticobasal degeneration: A single-case study.

Objective: To identify the cortical and subcortical distribution of atrophy and the disorganization of white matter bundles underlying the apraxic disorders in a patient with corticobasal degeneration (CBD). Method: Patient underwent appropriate neuropsychological tasks aimed at identifying the nature of the apraxic disorder and morphometric structural MRI with whole-brain voxel-wise analysis. Results: Progressive limbkinetic apraxia (LKA) with onset in the right upper limb with subsequent extension to the limbs, trunk, orofacial district, and eye movements was documented, associated with element of ideomotor apraxia (IMA). The MRI study showed grey matter atrophy extending to much of the frontal cortex bilaterally, including the precentral cortex, and into the inferior parietal regions. Caudate and putamen were involved on the left. Significant clusters of white matter atrophy were found in the bilateral superior longitudinal fasciculus (SLF), inferior longitudinal fasciculus (ILF) and corpus callosum (CC). Sensory evoked potentials (SEPs) and motor evoked potentials (MEPs) were normal. Conclusion: Previous observations in CBD indicate lack of inhibitory control from the sensory to the primary motor cortex with dysfunctional frontoparietal and cortico-motoneuron projections. Our neuroimaging data are partially consistent with these observations suggesting that the apraxic disorder in our patient might be produced by the disconnection of the primary motor cortex from the parietal areas that prevents selection and control of muscle movements, in the presence of preserved cortico-motoneuron as demonstrated by normal PEM. Apraxic disorders in CBD are high-level deficits of movement control that spare the motoneuron.