Motor Speech Deficits Research Articles

Cortical Tracking of Speech Is Reduced in Adults Who Stutter When Listening for Speaking.

The purpose of this study was to investigate cortical tracking of speech (CTS) in adults who stutter (AWS) compared to typically fluent adults (TFAs) to test the involvement of the speech-motor network in tracking rhythmic speech information. Participants' electroencephalogram was recorded while they simply listened to sentences (listening only) or completed them by naming a picture (listening for speaking), thus manipulating the upcoming involvement of speech production. We analyzed speech-brain coherence and brain connectivity during listening. During the listening-for-speaking task, AWS exhibited reduced CTS in the 3- to 5-Hz range (theta), corresponding to the syllabic rhythm. The effect was localized in the left inferior parietal and right pre/supplementary motor regions. Connectivity analyses revealed that TFAs had stronger information transfer in the theta range in both tasks in fronto-temporo-parietal regions. When considering the whole sample of participants, increased connectivity from the right superior temporal cortex to the left sensorimotor cortex was correlated with faster naming times in the listening-for-speaking task. Atypical speech-motor functioning in stuttering impacts speech perception, especially in situations requiring articulatory alertness. The involvement of frontal and (pre)motor regions in CTS in TFAs is highlighted. Further investigation is needed into speech perception in individuals with speech-motor deficits, especially when smooth transitioning between listening and speaking is required, such as in real-life conversational settings. https://doi.org/10.23641/asha.27234885.

Alterations of synaptic plasticity in Angelman syndrome model mice are rescued by 5-HT7R stimulation.

Angelman syndrome (AS) is a severe neurodevelopmental disorder characterized by motor disfunction, seizures, intellectual disability, speech deficits, and autism-like behavior, showing high comorbidity with Autism Spectrum Disorders (ASD). It is known that stimulation of the serotonin receptor 7 (5-HT7R) can rescue some of the behavioral and neuroplasticity dysfunctions in animal models of Fragile X and Rett syndrome, two pathologies associated with ASD. In view of these observations, we hypothesised that alterations of 5-HT7R signalling might also be involved in AS. To test this hypothesis, we stimulated 5-HT7R with the selective agonist LP-211 to investigate its possible beneficial effects on synaptic dysfunctions and altered behavior in the AS mice model. In mutant mice, we observed impairment of the synaptic machinery of protein synthesis, which was reversed by 5-HT7R activation. Moreover, stimulation of 5-HT7R was able to: i) enhance dendritic spine density in hippocampal neurons, which was reduced in AS mice; ii) restore impaired long-term potentiation (LTP) in hippocampal slices of the AS mice; iii) improve cognitive performance of the mutant animals subjected to the fear conditioning paradigm. Altogether, our results, showing beneficial effects of 5-HT7R stimulation in restoring molecular and cognitive deficits associated with AS, suggest that targeting 5-HT7R could be a promising therapeutic approach for the pathology.

Dysfluency in primary progressive aphasia: Temporal speech parameters

ABSTRACT Analysing spontaneous speech in individuals experiencing fluency difficulties holds potential for diagnosing speech and language disorders, including Primary Progressive Aphasia (PPA). Dysfluency in the spontaneous speech of patients with PPA has mostly been described in terms of abnormal pausing behaviour, but the temporal features related to speech have drawn little attention. This study compares speech-related fluency parameters in the three main variants of PPA and in typical speech. Forty-three adults participated in this research, thirteen with the logopenic variant of PPA (lvPPA), ten with the non-fluent variant (nfvPPA), nine with the semantic variant (svPPA), and eleven who were healthy age-matched adults. Participants’ fluency was assessed through a picture description task from which 42 parameters were computed including syllable duration, speaking pace, the duration of speech chunks (i.e. interpausal units, IPU), and the number of linguistic units per IPU and per second. The results showed that each PPA variant exhibited abnormal speech characteristics reflecting various underlying factors, from motor speech deficits to higher-level issues. Out of the 42 parameters considered, 37 proved useful for characterising dysfluency in the three main PPA variants and 35 in distinguishing among them. Therefore, taking into account not only pausing behaviour but also temporal speech parameters can provide a fuller understanding of dysfluency in PPA. However, no single parameter by itself sufficed to distinguish one PPA group from the other two, further evidence that dysfluency is not dichotomous but rather multidimensional, and that complementary multiparametric analyses are needed.

Detection of Subclinical Motor Speech Deficits after Presumed Low-Grade Glioma Surgery.

Motor speech performance was compared before and after surgical resection of presumed low-grade gliomas. This pre- and post-surgery study was conducted on 15 patients (mean age = 41) with low-grade glioma classified based on anatomic features. Repetitions of /pa/, /ta/, /ka/, and /pataka/ recorded before and 3 months after surgery were analyzed regarding rate and regularity. A significant reduction (6 to 5.6 syllables/s) pre- vs. post-surgery was found in the rate for /ka/, which is comparable to the approximate average decline over 10-15 years of natural aging reported previously. For all other syllable types, rates were within normal age-adjusted ranges in both preoperative and postoperative sessions. The decline in /ka/ rate might reflect a subtle reduction in motor speech production, but the effects were not severe. All but one patient continued to perform within normal ranges post-surgery; one performed two standard deviations below age-appropriate norms pre- and post-surgery in all syllable tasks. The patient experienced motor speech difficulties, which may be related to the tumor's location in an area important for speech. Low-grade glioma may reduce maximum speech-motor performance in individual patients, but larger samples are needed to elucidate how often the effect occurs.

Listening to Yourself and Watching Your Tongue: Distinct Abilities and Brain Regions for Monitoring Semantic and Phonological Speech Errors.

Despite the many mistakes we make while speaking, people can effectively communicate because we monitor our speech errors. However, the cognitive abilities and brain structures that support speech error monitoring are unclear. There may be different abilities and brain regions that support monitoring phonological speech errors versus monitoring semantic speech errors. We investigated speech, language, and cognitive control abilities that relate to detecting phonological and semantic speech errors in 41 individuals with aphasia who underwent detailed cognitive testing. Then, we used support vector regression lesion symptom mapping to identify brain regions supporting detection of phonological versus semantic errors in a group of 76 individuals with aphasia. The results revealed that motor speech deficits as well as lesions to the ventral motor cortex were related to reduced detection of phonological errors relative to semantic errors. Detection of semantic errors selectively related to auditory word comprehension deficits. Across all error types, poor cognitive control related to reduced detection. We conclude that monitoring of phonological and semantic errors relies on distinct cognitive abilities and brain regions. Furthermore, we identified cognitive control as a shared cognitive basis for monitoring all types of speech errors. These findings refine and expand our understanding of the neurocognitive basis of speech error monitoring.

Patterns of hemispheric compromise in primary progressive apraxia of speech: From clinical profiling to differential diagnosis.

In this issue of European Journal of Neurology, Robinson et al. present a novel study on primary progressive apraxia of speech. The authors describe different clinicopathological profiles in patients with left-dominant, right-dominant, and bilateral atrophy of the supplementary motor area and lateral premotor cortex. This commentary discusses the importance of this evidence for understanding individual differences among these patients, distinguishing them from those with nonfluent variant primary progressive aphasia, and analyzing the relations between motor speech deficits and underlying pathology.

The efficacy of acoustic-based articulatory phenotyping for characterizing and classifying four divergent neurodegenerative diseases using sequential motion rates.

Despite the impacts of neurodegeneration on speech function, little is known about how to comprehensively characterize the resulting speech abnormalities using a set of objective measures. Quantitative phenotyping of speech motor impairments may have important implications for identifying clinical syndromes and their underlying etiologies, monitoring disease progression over time, and improving treatment efficacy. The goal of this research was to investigate the validity and classification accuracy of comprehensive acoustic-based articulatory phenotypes in speakers with distinct neurodegenerative diseases. Articulatory phenotypes were characterized based on acoustic features that were selected to represent five components of motor performance: Coordination, Consistency, Speed, Precision, and Rate. The phenotypes were first used to characterize the articulatory abnormalities across four progressive neurologic diseases known to have divergent speech motor deficits: amyotrophic lateral sclerosis (ALS), progressive ataxia (PA), Parkinson's disease (PD), and the nonfluent variant of primary progressive aphasia and progressive apraxia of speech (nfPPA + PAOS). We then examined the efficacy of articulatory phenotyping for disease classification. Acoustic analyses were conducted on audio recordings of 217 participants (i.e., 46 ALS, 52 PA, 60 PD, 20 nfPPA + PAOS, and 39 controls) during a sequential speech task. Results revealed evidence of distinct articulatory phenotypes for the four clinical groups and that the phenotypes demonstrated strong classification accuracy for all groups except ALS. Our results highlight the phenotypic variability present across neurodegenerative diseases, which, in turn, may inform (1) the differential diagnosis of neurological diseases and (2) the development of sensitive outcome measures for monitoring disease progression or assessing treatment efficacy.

Auditory Verb Generation Performance Patterns Dissociate Variants of Primary Progressive Aphasia.

Primary progressive aphasia (PPA) is a clinical syndrome in which patients progressively lose speech and language abilities. Three variants are recognized: logopenic (lvPPA), associated with phonology and/or short-term verbal memory deficits accompanied by left temporo-parietal atrophy; semantic (svPPA), associated with semantic deficits and anterior temporal lobe (ATL) atrophy; non-fluent (nfvPPA) associated with grammar and/or speech-motor deficits and inferior frontal gyrus (IFG) atrophy. Here, we set out to investigate whether the three variants of PPA can be dissociated based on error patterns in a single language task. We recruited 21 lvPPA, 28 svPPA, and 24 nfvPPA patients, together with 31 healthy controls, and analyzed their performance on an auditory noun-to-verb generation task, which requires auditory analysis of the input, access to and selection of relevant lexical and semantic knowledge, as well as preparation and execution of speech. Task accuracy differed across the three variants and controls, with lvPPA and nfvPPA having the lowest and highest accuracy, respectively. Critically, machine learning analysis of the different error types yielded above-chance classification of patients into their corresponding group. An analysis of the error types revealed clear variant-specific effects: lvPPA patients produced the highest percentage of “not-a-verb” responses and the highest number of semantically related nouns (production of baseball instead of throw to noun ball); in contrast, svPPA patients produced the highest percentage of “unrelated verb” responses and the highest number of light verbs (production of take instead of throw to noun ball). Taken together, our findings indicate that error patterns in an auditory verb generation task are associated with the breakdown of different neurocognitive mechanisms across PPA variants. Specifically, they corroborate the link between temporo-parietal regions with lexical processing, as well as ATL with semantic processes. These findings illustrate how the analysis of pattern of responses can help PPA phenotyping and heighten diagnostic sensitivity, while providing insights on the neural correlates of different components of language.

Sex differences in the symptom presentation of stroke: A systematic review and meta-analysis.

Early diagnosis through symptom recognition is vital in the management of acute stroke. However, women who experience stroke are more likely than men to be initially given a nonstroke diagnosis and it is unclear if potential sex differences in presenting symptoms increase the risk of delayed or missed stroke diagnosis. To quantify sex differences in the symptom presentation of stroke and assess whether these differences are associated with a delayed or missed diagnosis. PubMed, EMBASE, and the Cochrane Library were systematically searched up to January 2021. Studies were included if they reported presenting symptoms of adult women and men with diagnosed stroke (ischemic or hemorrhagic) or transient ischemic attack (TIA) and were published in English. Mean percentages with 95% confidence intervals (CIs) of each symptom were calculated for women and men. The crude relative risks (RRs) with 95% CI of symptoms being present in women, relative to men, were also calculated and pooled. Any data on the delayed or missed diagnosis of stroke for women compared to men based on symptom presentation were also extracted. Pooled results from 21 eligible articles showed that women and men presented with a similar mean percentage of motor deficit (56% in women vs 56% in men) and speech deficit (41% in women vs 40% in men). Despite this, women more commonly presented with nonfocal symptoms than men: generalized nonspecific weakness (49% vs 36%), mental status change (31% vs 21%), and confusion (37% vs 28%), whereas men more commonly presented with ataxia (44% vs 30%) and dysarthria (32% vs 27%). Women also had a higher risk of presenting with some nonfocal symptoms: generalized weakness (RR 1.49, 95% CI 1.09-2.03), mental status change (RR 1.44, 95% CI 1.22-1.71), fatigue (RR 1.42, 95% CI 1.05-1.92), and loss of consciousness (RR 1.30, 95% CI 1.12-1.51). In contrast, women had a lower risk of presenting with dysarthria (RR 0.89, 95% CI 0.82-0.95), dizziness (RR 0.87, 95% CI 0.80-0.95), gait disturbance (RR 0.79, 95% CI 0.65-0.97), and imbalance (RR 0.68, 95% CI 0.57-0.81). Only one study linking symptoms to definite stroke/TIA diagnosis found that pain and unilateral sensory loss are associated with lower odds of a definite diagnosis in women compared to men. Although women showed a higher prevalence of some nonfocal symptoms, the prevalence of focal neurological symptoms, such as motor weakness and speech deficit, was similar for both sexes. Awareness of sex differences in symptoms in acute stroke evaluation, careful consideration of the full constellation of presenting symptoms, and further studies linking symptoms to diagnostic outcomes can be helpful in improving early diagnosis and management in both sexes.

Consonantal Landmarks as Predictors of Dysarthria among English-Speaking Adults with Cerebral Palsy.

The current study explored the possibility that the consonantal landmarks served as predictors of dysarthric speech produced by English-speaking adults with cerebral palsy (CP). Additionally, the relationship between the perceptual severity of dysarthric speech and the consonantal landmarks was explored. The analyses included 210 sentences from the TORGO database produced by seven English-speaking CP speakers with dysarthria and seven typically developing controls matched in age and gender. The results indicated that the clinical group produced more total landmark features than did the control group. A binominal regression analysis revealed that the improper control of laryngeal vibration and the inability to tactically control the energy in a voiced segment would lead to the higher likelihood of dysarthric speech. A multinominal regression analysis revealed that producing too many +v and −v landmark features would lead to higher perceptual severity levels among the CP speakers. Together with literature, the current study proposed that the landmark-based acoustic analysis could quantify the differences in consonantal productions between dysarthric and non-dysarthric speech and reflect the underlying speech motor deficits of the population in concern.

Surgical Outcome of Encephaloduroarteriomyosynangiosis for Moyamoya Disease.

Indirect bypass surgeries for moyamoya disease have included modifications of procedures involving placement of the superficial temporal artery on the brain pial surface. We evaluate the functional and angiographic outcomes of patients treated with encephaloduroarteriomyosynangiosis (indirect) revascularization and examine the outcome in relation to demographic and radiological factors. Patients treated surgically for moyamoya disease over a 14-year period were identified. Demographics, clinical presentation, and radiology were analyzed to assign a stage for the disease (Suzuki staging) and the extent of revascularization (Matsushima grade) at the last follow-up. A modified Rankin score was used to assess the clinical status at presentation and the functional outcome at follow-up. There were 46 patients operated on by a single surgeon over a 14-year period. A higher incidence of motor deficits, seizures, and speech deficits was seen in the pediatric population. Age, sex, preoperative Suzuki disease stage, and hemispheric involvement had no bearing on angiographic outcome at last follow-up. Three of 46 patients (6.5%) developed immediate postoperative complications. Among 43 patients on follow-up, 39 had stable disease or showed improvement in clinical symptoms with 90% event-free status at last follow-up. Indirect revascularization procedures are an effective alternative to direct cerebral revascularizations in the early or advanced stages of moyamoya disease. This is effective in a predominant ischemic presentation as noted in our series.

Motor speech disorders in the nonfluent, semantic and logopenic variants of primary progressive aphasia

ObjectiveMotor speech disorders (MSDs) are characteristic for nonfluent primary progressive aphasia (nfvPPA). In primary progressive aphasia (PPA) of the semantic (svPPA) and of the logopenic type (lvPPA), speech motor function is considered typically intact. However, knowledge on the prevalence of MSDs in svPPA and lvPPA is mainly based on studies with a priori knowledge of PPA syndrome diagnosis. This fully blinded retrospective study aims to provide data on the prevalence of all types of MSDs in a large sample of German-speaking patients with different subtypes of PPA. MethodTwo raters, blinded for PPA subtype, independently evaluated connected speech samples for MSD syndrome and severity from 161 patients diagnosed with nfvPPA, svPPA or lvPPA in the database of the German Consortium of Frontotemporal Lobar Degeneration (FTLDc). In case of disagreement, a third experienced rater re-evaluated the speech samples, followed by a consensus procedure. Consensus was reached for 160 patients (74 nfvPPA, 49 svPPA, 37 lvPPA). Main resultsAcross all PPA syndromes, 43.8% of the patients showed MSDs. Patients with nfvPPA demonstrated the highest proportion of MSDs (62.2%), but MSDs were also identified in svPPA (26.5%) and lvPPA (29.7%), respectively. Overall, dysarthria was the most common class of MSDs, followed by apraxia of speech. In addition, we identified speech abnormalities presenting as “syllabic speech”, “dysfluent speech”, and “adynamic speech”. DiscussionOur study confirmed MSDs as frequently occurring in PPA. The study also confirmed MSDs to be most common in patients with nfvPPA. However, MSDs were also found in substantial proportions of patients with svPPA and lvPPA. Furthermore, our study identified speech motor deficits that have not received attention in previous studies on PPA. The results are discussed against the background of the existing literature on MSDs in PPA, including theoretical considerations of the neuroanatomical conditions described for each of the different subtypes of PPA.

One novel GRN null mutation, two different aphasia phenotypes

Progranulin gene (GRN) mutations are among the leading causes of frontotemporal lobar degeneration, a group of neurodegenerative diseases characterized by remarkable clinical heterogeneity. In this article, we report the new GRN 708+4A>T splicing mutation, identified in 2 siblings of a family with several members affected by cognitive, behavioral, and motor disorders. Plasma progranulin dosage and GRN expression analysis, together with in silico prediction studies, supported the pathogenicity of the mutation. Both the patients displayed a clinical syndrome in which language impairment was largely predominant. However, motor speech deficits were the major feature in one case, diagnosed as progressive nonfluent aphasia, whereas marked semantic alterations were present in the other, whose clinical phenotype was in favor of a mixed aphasia. The profile of neuroanatomical alterations from imaging studies was in line with the clinical phenotypes. Therefore, also this novel GRN mutation is associated with haploinsufficiency and phenotypic heterogeneity, which are both typical features of progranulinopathies.

Reading Outcomes for Individuals With Histories of Suspected Childhood Apraxia of Speech.

Purpose The primary aims of this study were to examine the speech-language correlates of decoding difficulties in children with histories of suspected childhood apraxia of speech (sCAS) and to identify predictors of low-proficiency reading levels. Method Participants were school-age children and adolescents, 7-18 years of age, diagnosed with sCAS (n = 40) or speech sound disorder but no sCAS (SSD-no sCAS; n = 119). The sCAS and SSD-no sCAS reading groups were compared on measures of performance IQ, oral language, phonological awareness, rapid automatic naming, diadochokinetic rates, single word articulation, and multisyllable and nonsense word repetition. Logistic regression analyses were employed to identify predictors of low-proficiency reading in the sCAS and SSD-no sCAS groups. Results Sixty-five percent of the participants with sCAS compared to 24% of those with SSD-no sCAS were classified as low-proficiency readers based on nonsense and single word decoding. Analysis failed to reveal significant differences in reading, oral language, or phonological awareness between low-proficiency readers with sCAS and low-proficiency readers with SSD-no sCAS. Oral language and phonological awareness skills were the best predictors of reading level for all participants, followed by performance on multisyllabic word repetition and diadochokinetic rate. Conclusions The language and phonological awareness deficits of children with sCAS are related to their risks for reading failure. To a lesser degree, motor speech deficits and speech sound production also increase risks for reading difficulties. The findings justify early intervention for this subset of children.

Differentiating Typical From Atypical Speech Production in 5-Year-Old Children With Cerebral Palsy: A Comparative Analysis.

Objective Early diagnosis of speech disorders in children with cerebral palsy (CP) is of critical importance. A key problem is differentiating those with borderline or mild speech motor deficits from those who are within an age-appropriate range of variability. We sought to quantify how well functional speech measures differentiated typically developing (TD) children from children with CP. Method We studied speech production in 45 children with CP (26 with clinical speech motor impairment [SMI] and 19 with no evidence of speech motor impairment [NSMI]) and in 29 TD children of the same age. Speech elicitation tasks were used. Intelligibility, speech rate, and intelligible words per minute were examined. Results All measures differentiated between all 3 groups of children with considerable precision based on area under the receiver operating characteristic curve (AUC) data. AUC was highest for overall intelligibility, which ranged from .88 to .99. Intelligible words per minute also yielded very strong AUCs, ranging from .81 to .99. In each of the receiver operating characteristic models, discrimination between groups was highest for children with speech motor impairment versus TD children. Data indicated that 90% of TD children had overall intelligibility above 87% at 5 years of age, but that no child was 100% intelligible. Furthermore, 90% children with SMI had intelligibility below 72%. Conclusion Findings suggest that functional speech measures differentiate very clearly between children with and without CP and that even children who do not show evidence of speech motor impairment have functional differences in their speech production ability relative to TD peers.

Effects of Concurrent Manual Task Performance on Connected Speech Acoustics in Individuals With Parkinson Disease.

Purpose Prior investigations suggest that simultaneous performance of more than 1 motor-oriented task may exacerbate speech motor deficits in individuals with Parkinson disease (PD). The purpose of the current investigation was to examine the extent to which performing a low-demand manual task affected the connected speech in individuals with and without PD. Method Individuals with PD and neurologically healthy controls performed speech tasks (reading and extemporaneous speech tasks) and an oscillatory manual task (a counterclockwise circle-drawing task) in isolation (single-task condition) and concurrently (dual-task condition). Results Relative to speech task performance, no changes in speech acoustics were observed for either group when the low-demand motor task was performed with the concurrent reading tasks. Speakers with PD exhibited a significant decrease in pause duration between the single-task (speech only) and dual-task conditions for the extemporaneous speech task, whereas control participants did not exhibit changes in any speech production variable between the single- and dual-task conditions. Conclusions Overall, there were little to no changes in speech production when a low-demand oscillatory motor task was performed with concurrent reading. For the extemporaneous task, however, individuals with PD exhibited significant changes when the speech and manual tasks were performed concurrently, a pattern that was not observed for control speakers. Supplemental Material https://doi.org/10.23641/asha.8637008.

Longitudinal Growth in Single Word Intelligibility Among Children With Cerebral Palsy From 24 to 96 Months of Age: Predicting Later Outcomes From Early Speech Production.

Purpose Children with cerebral palsy (CP) are at risk for significant communication problems. Reduced speech intelligibility is common, even for those who do not have speech motor deficits. Development of intelligibility has not been comprehensively quantified in children with CP; as a result, we are currently unable to predict later speech outcomes. Such information would advance treatment decision making. We sought to examine growth in speech intelligibility among children with CP using a prospective longitudinal design, with a focus on age of crossing target intelligibility thresholds, age of greatest intelligibility growth, and how well intelligibility at 36 months predicted intelligibility at 96 months. Method Sixty-nine children with CP were followed longitudinally between 24 and 96 months of age. A total of 566 time points were examined across children ( M = 8.2 time points per child, SD = 2.6). We fitted a nonlinear random effects model for longitudinal observations and then used the fitted model trajectories to generate descriptive analyses of growth. We used results of the model to generate a set of simulations, which we analyzed to determine how well 36-month intelligibility data predicted 96-month data. Results Half of children crossed 25% and 50% intelligibility thresholds at 36 and 49 months of age, respectively. Slightly more than half of children did not reach 75% intelligibility by 96 months of age. Age of crossing 25%, 50%, and 75% intelligibility thresholds was highly negatively correlated with intelligibly at 96 months. Children had the steepest intelligibility growth at 36 months, followed by 48 and 60 months. Intelligibility at 36 months was highly predictive of intelligibility at 96 months. Conclusions The developmental window from 3 to 5 years constitutes a time of rapid growth in speech intelligibility in children with CP. Children who cross intelligibility thresholds of 25%, 50%, and 75% at earlier ages have better outcomes when they are older; early performance is highly predictive of later speech intelligibility outcomes. Children with CP as a group have delayed speech intelligibility development but are still growing through 96 months of age.

Management of Self-injurious Behaviors in Children with Neurodevelopmental Disorders: A Pharmacotherapy Overview.

Neurodevelopmental disorders (NDDs), a group of disorders affecting ~1-2% of the general population, are caused by changes in brain development that result in behavioral and cognitive alterations, sensory and motor changes, and speech and language deficits. Neurodevelopmental disorders encompass a heterogeneous group of disorders including, but not limited to, Smith-Magenis syndrome, Lesch-Nyhan disease, cri du chat syndrome, Prader-Willi syndrome, pervasive developmental disorders, fragile X syndrome, Rett syndrome, Cornelia de Lange syndrome, and Down syndrome. Self-injurious behaviors (SIBs) are common in children with NDDs; depending on the specific NDD, the incidence of SIBs is nearly 100%. The management of SIBs in this population is complex, and little high-quality data exist to guide a consistent approach to therapy. However, managing SIBs is of the utmost importance for the child as well as the family and caregivers. Behavior therapies must be implemented as first-line therapy. If behavioral interventions alone fail, pharmacotherapy becomes an essential part of management plans. The limited available evidence for the use of common pharmacologic agents, such as second-generation antipsychotics, and less common agents, such as clonidine, n-acetylcysteine, riluzole, naltrexone, and topical anesthetics, is reviewed. Additional data from well-designed studies in children with NDDs are needed to gain a better understanding of this common and troublesome problem including efficacy and safety implications associated with pharmacotherapy. Until then, clinicians must rely on the limited available data, clinical expertise, and ongoing systematic monitoring when managing SIBs in children with NDDs.

Frontal Anatomical Correlates of Cognitive and Speech Motor Deficits in Amyotrophic Lateral Sclerosis.

The goal of this study was to identify neurostructural frontal lobe correlates of cognitive and speaking rate changes in amyotrophic lateral sclerosis (ALS). 17 patients diagnosed with ALS and 12 matched controls underwent clinical, bulbar, and neuropsychological assessment and structural neuroimaging. Neuropsychological testing was performed via a novel computerized frontal battery (ALS-CFB), based on a validated theoretical model of frontal lobe functions, and focused on testing energization, executive function, emotion processing, theory of mind, and behavioral inhibition via antisaccades. The measure of speaking rate represented bulbar motor changes. Neuroanatomical assessment was performed using volumetric analyses focused on frontal lobe regions, postcentral gyrus, and occipital lobes as controls. Partial least square regressions (PLS) were used to predict behavioral (cognitive and speech rate) outcomes using volumetric measures. The data supported the overall hypothesis that distinct behavioral changes in cognition and speaking rate in ALS were related to specific regional neurostructural brain changes. These changes did not support a notion of a general dysexecutive syndrome in ALS. The observed specificity of behavior-brain changes can begin to provide a framework for subtyping of ALS. The data also support a more integrative framework for clinical assessment of frontal lobe functioning in ALS, which requires both behavioral testing and neuroimaging.

Mind-Body Interventions, Psychological Stressors, and Quality of Life in Stroke Survivors.

Background and Purpose- Psychological stressors, including poststroke depression, poststroke anxiety, and posttraumatic stress disorder, are highly prevalent in stroke survivors. These symptoms exact a significant toll on stroke survivors. Clinical and research efforts in stroke recovery focus on motor disability, speech and language deficits, and cognitive dysfunction while largely neglecting psychological stressors. Evidence suggests mind-body interventions in other chronic illness populations decrease symptoms of depression, regulate immune responses, and promote resilience, yet similar studies are lacking in stroke populations. This review aims to synthesize evidence of the effects of mind-body interventions on psychological stressors, quality of life, and biological outcomes for stroke survivors. Methods- A systematic search of PubMed, PsycINFO, and CINAHL was conducted from database inception to November 2017. Results- Eight studies were included in the review, with a total of 292 participants. Mind-body interventions included yoga or tai chi. Of the 5 included randomized controlled trials, most were pilot or feasibility studies with small sample sizes. Psychological stressors, including poststroke depression and anxiety, along with the quality of life, improved over time, but statistically significant between-group differences were largely absent. The 3 included studies with a qualitative design reported themes reflecting improvement in psychological stressors and quality of life. No included studies reported biological outcomes. Conclusions- Studies of mind-body interventions suggest a possible benefit on psychological stressors and quality of life; however, rigorously designed, sufficiently powered randomized controlled trials with mixed-methods design are warranted to delineate specific treatment effects of these interventions. Studies with both biological and psychological stressors as outcomes would provide evidence about interaction effects of these factors on stroke-survivor responses to mind-body interventions.