Severe Language Impairment Research Articles

Psychiatric symptoms in autoimmune encephalitis. A case report

IntroductionEarly stages of autoimmune encefalitis (AE) often present cognitive and neuropsychiatric symptoms such as personality change, irritability, axiety, depression, behavioral disorders, hallucinations, disorientation, sleep-wake cycle reversals, …). Thus often these cases are first treated as psychiatric disorders.ObjectivesA literature review throughout a case report presentation.MethodsWe present the case of a 25-year old female with a medical history of iron-deficiency anemia who arrives at the emergency service. She presents the following one week of evolution clinical picture: complex auditive hallucinations, behavioral disturbances, sleep disorder and short term memory impairment. Neurological examination, LP and craneal CT are all normal. CSF analysis has no abnormalities. Thus she entered the psychiatric ward. There she was treated with neuroleptics with no improvement of symptoms presenting a severe psychomotor agitation and language impairment. After neurology interconsultation AE is suspected.ResultsShe was performed an EEG (left temporal epileptiform activity), CSF (inflammatory pattern), MRI (bilateral temporal lobe hyperintensity). Suspecting limbic encephalitis the presence of anti-NMDAR antibodies was tested , which turned out to be positive. First she was treated with corticotherapy with mild results. Then she was treated with intravenous immunoglobulin improving significantly.ConclusionsAnti-NMDAR encephalitis is usually a multistage illness. Early in the course of disease psychiatric manifestations are not rare. Therefore the proper diagnosis and approach of AE may requiere a highly organized assessment, starting with detailed history and physical examination and an appropriate testing to exclude other possible relevant pathologies.DisclosureNo significant relationships.

The GAIRS Checklist: a useful global assessment tool in patients with Rett syndrome

BackgroundRett Syndrome is a severe, neurodevelopmental disorder mainly caused by mutations in the MECP2 gene, affecting around 1 in 10,000 female births. Severe physical, language, and social impairments impose a wide range of limitations in the assessment of the abilities of Rett patients. This study proposes an analysis and first validation of a Global Assessment and Intervention in Rett syndrome (GAIRS) Checklist for assessing behavioral, intellectual, academic, neuropsychological and psychosocial manifestations in patients with Rett Syndrome. We administered the GAIRS Checklist to 113 Italian patients with Rett Syndrome aged 4–42.Aims of this studyTo examine the psychometric characteristics of the GAIRS Checklist. Moreover, the aim is also to examine the validity of GAIRS with test–retest correlation, convergent validity with similar functional measurements, such as the Vineland scales, and divergent validity with severity of disease scale, such as the RARS scale and severity of neuropsychiatric evaluations.ResultsAll 10 subscales of GAIRS were positively and significantly related to each other and to the total GAIRS score, and the subscales showed high levels of Cronbach’s alpha values (from .77 to .95). Principal axis factoring suggested two factors that explain 60% of the variance. Test–retest reliability is 0.82. This means that psychometric properties are reliable. Correlation for Concurrent validity with Vineland score was high and Divergent Validity with RARS was also high.ConclusionThe GAIRS Checklist used for Rett syndrome is acceptable and feasible to complete assessment in a clinical setting. Moreover, it can detect the complexity of this disease and may suggest the next step in terms of specific training in Rett syndrome.

Autism severity aggregates with family psychiatric history in a community-based autism sample.

The purpose of this study was to examine family psychiatric history in individuals with autism spectrum disorder (ASD) and its association with clinical presentation. Participants were 798 individuals with a clinical diagnosis of ASD, confirmed by the Autism Diagnostic Observation Schedule, Second Edition (ADOS-2), enrolled in Rhode Island Consortium for Autism Research and Treatment, a statewide research registry. Prior research suggests a specific behavioral phenotype in individuals with ASD who have family members with psychiatric diagnoses, including higher IQ and less severe language impairment. However, studies have not specifically investigated autism severity. We hypothesized that increased psychiatric family history would be associated with increased autism severity symptoms. Results show a strong association of increased burden of first-degree family psychiatric history with higher autism symptom severity as measured by Social Responsiveness Scale, Second Edition (SRS-2), but not with ADOS-2 severity scores, IQ, or adaptive functioning. These findings support the importance of investigating the contribution of psychiatric family history toward clinical ASD presentation. LAY SUMMARY: This study explored how family psychiatric history is related to clinical presentation of Autism Spectrum Disorder (ASD). Higher amounts of first-degree family psychiatric history was associated with higher autism symptom severity as measured by the Social Responsiveness Scale, Second Edition (SRS-2). The contribution of psychiatric family history requires ongoing investigation.

Collaborative referencing using hand gestures in Wernicke’s aphasia: Discourse analysis of a case study

ABSTRACT Background: Collaborative referencing tasks have evolved from historically being used as experiments to study human interactions in various disciplines, to being adapted as a clinical intervention for individuals with cognitive-communication disorders such as aphasia. For both neurotypical adults and adults with cognitive-communication disorders, evidence on successful referencing is heavily based on spoken language data. The patterns of collaborative referencing in individuals with severe spoken language impairments who extensively use hand gestures for communication remain unclear. Aims: We aimed to investigate the patterns of collaborative referencing between Clyde, an individual with severe Wernicke’s aphasia using co-speech hand gestures, and a clinician-partner during the collaborative referencing intervention (CRI). Methods & Procedures: This case study included 15 CRI sessions between Clyde and the clinician-partner. Each session consisted of a photo-matching game in which the pair completed six trials with alternating turns to describe and match treatment cards over a low barrier. Data analysis included (a) measuring the mean card placement accuracy; (b) measuring the mean length of CRI trials; and (c) analysing the frequency and type of hand gestures used in no common ground and common ground conditions, and then examining if the multimodal references (i.e., labels for treatment cards produced using verbal expressions and hand gestures) simplified across CRI sessions. Outcomes & Results: Largely, the results were consistent with previous evidence on CRI: the pair scored 99.63% mean card placement accuracy and completed each trial in shorter time periods, indicating reduced collaborative effort. During the no common ground condition, the pair used a high frequency of hand gestures and a variety of gesture-type combinations to reference each card, while they used a low frequency of hand gestures dominated by iconic gestures in the common ground condition. The multimodal references were found to be simplified across sessions by the shift from using indefinite to definite verbal references, and reduced frequency and specific (iconic) types of hand gestures. Conclusions: Despite severe spoken language impairments, speakers and partners can develop common ground for successful collaborative referencing by effectively using hand gestures. Clinical implications including the role of clinicians in being a skilful and multimodal communication partner and the rich communicative environment offered by CRI will be discussed.

Describing Vocalizations in Young Children: A Big Data Approach Through Citizen Science Annotation.

Purpose Recording young children's vocalizations through wearables is a promising method to assess language development. However, accurately and rapidly annotating these files remains challenging. Online crowdsourcing with the collaboration of citizen scientists could be a feasible solution. In this article, we assess the extent to which citizen scientists' annotations align with those gathered in the lab for recordings collected from young children. Method Segments identified by Language ENvironment Analysis as produced by the key child were extracted from one daylong recording for each of 20 participants: 10 low-risk control children and 10 children diagnosed with Angelman syndrome, a neurogenetic syndrome characterized by severe language impairments. Speech samples were annotated by trained annotators in the laboratory as well as by citizen scientists on Zooniverse. All annotators assigned one of five labels to each sample: Canonical, Noncanonical, Crying, Laughing, and Junk. This allowed the derivation of two child-level vocalization metrics: the Linguistic Proportion and the Canonical Proportion. Results At the segment level, Zooniverse classifications had moderate precision and recall. More importantly, the Linguistic Proportion and the Canonical Proportion derived from Zooniverse annotations were highly correlated with those derived from laboratory annotations. Conclusions Annotations obtained through a citizen science platform can help us overcome challenges posed by the process of annotating daylong speech recordings. Particularly when used in composites or derived metrics, such annotations can be used to investigate early markers of language delays.

A paradigmatic autistic phenotype associated with loss of PCDH11Y and NLGN4Y genes

BackgroundMost studies relative to Y chromosome abnormalities are focused on the sexual developmental disorders. Recently, a few studies suggest that some genes located on Y chromosome may be related to different neurodevelopment disorders.Case presentationWe report a child with sexual developmental disorder associated with a peculiar phenotype characterized by severe language impairment and autistic behaviour associated with a mosaicism [45,X(11)/46,XY(89)] and a partial deletion of the short and long arm of Y chromosome (del Yp11.31q11.23) that also involves the loss of both PCDH11Y and NLGN4Y genes. To our knowledge no study has ever reported the occurrence of the lack of both PCDH11Y and NLGN4Y located in the Y chromosome in the same patient.ConclusionsWe hypothesized a functional complementary role of PCDH11Y and NLGN4Y within formation/maturation of the cerebral cortex. The impairment of early language development may be mainly related to the lack of PCDH11Y that underlies the early language network development and the later appearance of the autistic behaviour may be mainly related to deficit of inhibitory glicinergic neurotransmission NLGN4Y-linked.

Alternating Hemiplegia of Childhood: Genotype-Phenotype Correlations in a Cohort of 39 Italian Patients.

Alternating hemiplegia of childhood is a rare neurological disease characterized by paroxysmal movement disorders and chronic neurological disturbances, with onset before 18 months of age. Mutations in the ATP1A3 gene have been identified in up to 80% of patients. Thirty-nine patients [20 females, 19 males, mean age 25.32 years (7.52–49.34)] have been recruited through the Italian Biobank and Clinical Registry for Alternating Hemiplegia of Childhood. Demographic data, genotype, paroxysmal movement disorders, chronic neurological features, and response to flunarizine have been analyzed. ATP1A3 gene mutations have been detected in 92.3% of patients. Patients have been divided into three groups—p.Asp801Asn mutation patients (26%), p.Glu815Lys cases (23%), and patients with other ATP1A3 mutations—and statistically compared. The Italian cohort has a higher percentage of ATP1A3 gene mutation than reported in literature (92.3%). Our data confirm a more severe phenotype in patients with p.Glu815Lys mutation, with an earlier age of onset of plegic (p = 0.02 in the correlation with other mutations) and tonic attacks. P.Glu815Lys patients most frequently present altered muscle tone, inability to walk (p = 0.01 comparing p.Glu815Lys and p.Asp801Asn mutations), epilepsy, and a more severe grade of dystonia (p < 0.05 comparing p.Glu815Lys and p.Asp801Asn mutations). They have moderate/severe intellectual disability and severe language impairment (p < 0.05). Interestingly, flunarizine seems to be more efficacious in patients with p.Glu815Lys mutation than p.Asp801Asn. In conclusion, our research suggests a genotype–phenotype correlation and provides information on this disorder's features, clinical course, and treatment.

De novo variants of DEAF1 cause intellectual disability in six Chinese patients

BackgroundIt has been reported that de novo heterozygous variants of DEAF1 can cause DEAF1-associated neurodevelopmental disorder. The purpose of this article is to explore the clinical and genetic characteristics of Chinese patients harboring de novo DEAF1 variants. MethodsWe assembled a cohort of six unrelated patients with de novo variants in DEAF1. Clinical and genetic features of these patients were summarized. ResultsEach child showed intellectual disability (ID)/ global developmental delay (GDD). Severe language impairment was prominent. Behavior problems, seizures, sleep disturbance, and a high pain threshold were common features. DEAF1-related seizures were reported to be difficult to treat or intractable. Seizures in our cohort were almost all treatable. Valproic acid was the most commonly used drug. Five heterozygous missense mutations of DEAF1 gene were identified, three of which (p.W234C, p.L203P, p.H275Q) were not published in literature before. ConclusionMutations of DEAF1 gene should be considered in ID/GDD patients with a nonspecific phenotype, comprising intellectual disability, prominent speech delay, abnormal behaviors, especially autism. In our study, DEAF1-related epilepsy is completely treatable in Eastern-Asian individuals when compared to patients in other regions, and valproic acid can be used as a first choice. The knowledge of DEAF1-related neurodevelopmental disorder and the de novo variant database of DEAF1 were expanded.

Long-term course after inpatient speech therapy: educational and language development of children with severe specific language impairment (SLI)

Studies from primarily English-speaking countries have shown that specific language impairments can lead to disadvantages in educational and professional development. Corresponding studies for Germany have not been published. This study surveys the educational and language outcomes of adolescents and young adults who were treated in an inpatient setting during childhood. A total of 193young adults who had received inpatient treatment between 1998 and 2005 at the Department of Communication Disorders of the Department of Otorhinolaryngology, Head, and Neck Surgery (ENT) of the Mainz University Medical Center were assessed. The cohort was contacted by telephone and interviewed about aspects of their educational and language development using aspecially developed questionnaire. It was possible to include 70participants in the study. Almost half (48.6%; n = 34) of the participants had attended aregular elementary school and 50% (n = 35) attended aspecial school with afocus on speech-language development (others: 1.4%, n = 1). Regarding school-leaving qualifications, 31.5% (n = 22) finished school with an Abitur/Fachabitur (high-school-level certificate), 33% (n = 23) with aRealschulabschluss (secondary school certificate), 30% (n = 21) with aHauptschulabschluss (lower secondary certificate), and 4% (n = 3) with aspecial school certificate. Only one participant left school without aqualification. Of the interviewed participants, 71% (n = 50) do not feel any speech language limitations anymore. The results indicate apositive educational and language development of children with SLI after inpatient treatment in Germany. Over 90% of the participants finished school with aregular certification and most of them do not feel any speech and language limitations anymore.

Detailed Clinical and Psychological Phenotype of the X-linked HNRNPH2-Related Neurodevelopmental Disorder.

ObjectiveTo expand the clinical phenotype of the X-linked HNRNPH2-related neurodevelopmental disorder in 33 individuals.MethodsParticipants were diagnosed with pathogenic or likely pathogenic variants in HNRNPH2 using American College of Medical Genetics and Genomics/Association of Molecular Pathology criteria, largely identified via clinical exome sequencing. Genetic reports were reviewed. Clinical data were collected by retrospective chart review and caregiver report including standardized parent report measures.ResultsWe expand our clinical characterization of HNRNPH2-related disorders to include 33 individuals, aged 2–38 years, both females and males, with 11 different de novo missense variants, most within the nuclear localization signal. The major features of the phenotype include developmental delay/intellectual disability, severe language impairment, motor problems, growth, and musculoskeletal disturbances. Minor features include dysmorphic features, epilepsy, neuropsychiatric diagnoses such as autism spectrum disorder, and cortical visual impairment. Although rare, we report early stroke and premature death with this condition.ConclusionsThe spectrum of X-linked HNRNPH2-related disorders continues to expand as the allelic spectrum and identification of affected males increases.

The relationship between developmental language disorder and dyslexia in European Portuguese school-aged children

ABSTRACT Developmental Language Disorder (DLD) [Also referred to as Specific Language Impairment (SLI)] and dyslexia are neurodevelopmental disorders which show similar behavioral manifestations. In this study, between-group comparisons and frequency analysis were combined to investigate the relationship between DLD and dyslexia. European Portuguese children aged 7–10 years, with DLD (N = 7) or dyslexia (N = 11) were recruited and compared to age-matched typically developing (TD) children (N = 21) on phonological processing, language andf literacy measures. The between-group comparison revealed that for phonological processing, the clinical groups scored significantly below TD children on most tasks, yet the DLD group performed similarly to TD children for RAN speed and digit span. The clinical groups did not statistically differ in their phonological processing abilities. For language abilities, children with dyslexia did not differ from TD children, whilst children with DLD performed significantly below TD children on all measures and significantly below children with dyslexia for vocabulary. Finally, for literacy measures, there were no statistical differences between clinical groups which underperformed on all measures when compared to TD children. The frequency analysis showed that children with DLD exhibited a lower prevalence of RAN difficulties when compared to children with dyslexia, whilst children with DLD tended to show more frequent nonword repetition and phoneme deletion deficits. Additionally, whilst children with DLD consistently showed more prevalent language impairments, both clinical groups demonstrated similar prevalence rates of literacy deficits compared to TD children. These findings lend support to the additional deficit model as children with DLD show more severe and prevalent language impairments than those with dyslexia, despite similar phonological and literacy difficulties.

Electroencephalographic abnormalities in preschool children with language delay

Background: Most children seem to be slow in the initial days of learning language but some children continue to have problems. Studies show that speech and language development affects 5–10% of preschool children. A high proportion of electroencephalogram (EEG) abnormalities and epileptic syndromes has been found in children with severe language impairments in western studies. Aims and Objectives: This study aims to look at the rate of occurrence of EEG abnormalities in preschool children with language disorders in the local population. Materials and Methods: Fifty two preschool children (

Évaluation clinique des pratiques intégratives dans les troubles du spectre autistique (EPIGRAM) : méthodologie, population à l’inclusion et satisfaction des familles à 12 mois

BackgroundIntegrative care practices (ICPs) in the management of autism spectrum disorders (ASDs) do not currently receive sufficient rigorous evaluation. Moreover, they lack formalisation despite their important place in clinical practice, particularly in France, where they are implemented in day care hospital structures. We present here the methodology of the EPIGRAM study financed by the French General Directorate of Healthcare Organisations (PREPS 2013), the profile of the inclusive population (M0), and the experience of families after 12 months of care. MethodIn order to homogenise practices, a manual has been formalised following the methodology of the clinical pathway and the recommendations of the High Authority of health. We then conducted an open, multi-centre, observational study in a natural environment in routine care evaluating ICPs in ASDs for children aged 3 to 6 years. The main inclusion criteria were the diagnosis (typical or atypical autism, according to the ICD10) and the duration of care (2 to 4 half-days per week) during the first year of care. The children were clinically assessed using several instruments including the PEP-3 (Psychoeducational Profile-3) and the CARS (Children Autism Rating Scale). In order to maintain a blind assessment for the main variable, randomly assigned videos of the PEP-3 were rated by two independent psychologists. A questionnaire, completed separately by the families and the professionals at inclusion and at one year, complemented the study. ResultsWe included 89 children demonstrated to have severe autism through the CARS (mean=44; SD=6.51) and moderate autism through he PEP-3 maladaptive behaviour category (CAT MB) (mean=30; SD=29.89). The children were all diagnosed before the age of 3 years 6 months. Most of the children had severe language impairments. With PEP-3, the impairment is more pronounced in gross motor skills and characteristic verbal behaviours. Thirty percent of the families had a low socioeconomic status and 56 % were first generation immigrants. Seventy-seven percent of the children were enrolled in kindergarten with school life support. Children of immigrant parents have more severe autism (CARS : mean=45.8 (±6.51) vs. 41.2 (±5.58), t-test, P=0.001) in addition to a more marked cognitive deficit (CVP : median=24.5 [min–max: 9.75–45.5] vs. 52 [min–max: 26–76], Wilcoxon, P=0.001). At entry, the collection of the signs at the structured clinical observation met the signs noted by the families as being the object of their concern. With standardised tools, these observations constituted an efficient functional and multidimensional analysis for the integrative care project. At 12 months, the families’ level of satisfaction was high and consistent with that of the practitioners. ConclusionOur results show that the day units take care of a population of children with predominantly severe and complex ASDs, for the most part, from families with low socioeconomic status and first-generation immigration. The relevance of the multidimensional observation and the care project of the ISPs were confirmed by the families at entry and after 12 months of care.

Language Differentiation Using Augmentative and Alternative Communication: An Investigation of Spanish-English Bilingual Children With and Without Language Impairments.

Purpose Children with severe speech and language impairments growing up in dual language environments may communicate in more than one language using augmentative and alternative communication (AAC). This study investigated predictors of bilingual children's ability to differentiate between Spanish and English using an AAC iPad app during a cued language-switching task and examined whether switching between languages using AAC incurred a cognitive cost. Method Participants were 58 Spanish-English bilingual children ages 4;0-6;11 (years;months; 23 with language impairments). Children received standardized language and cognitive assessments and completed an experimental language-switching task in which they were asked to differentiate between languages using an AAC iPad app containing English and Spanish vocabulary layouts paired with voice output. Results Results of a binary logistic regression indicated that, when controlling for age, processing speed significantly predicted whether children were classified as high or low performers on the experimental task. Nonparametric tests indicated that switching between languages did not incur a cognitive cost as evidenced by similar response times on trials where participants were required to switch between languages compared to trials where they did not switch. Conclusion This study contributes to the understanding of how young bilingual children with and without language impairments conceptualize and discriminate between languages represented in a visual-graphic modality paired with speech output. Supplemental Material https://doi.org/10.23641/asha.13289330.

Case report: Pitt-Hopkins like syndrome with CNTNAP2 mutation

BackgroundPitt-Hopkins syndrome (PHS) is a rare cause of severe intellectual disability, seizures, language impairment, and peculiar facial dysmorphism. It is caused by a mutation in transcription factor 4 (TCF4). Through molecular karyotyping and mutational analysis, a study identified recessive defects in two genes, contactin associated protein like 2 (CNTNAP2) and Neurexin I (NRXN1), in patients with similar presentations of Pitt-Hopkins syndrome and called Pitt-Hopkins-like syndrome (Zweier et al., J Med Genet 80: 994-1001, 2007).We present the first case report of a child in Iraq with Pitt-Hopkins-like syndrome that was referred to the Welfare Children’s Hospital/Medical City of Baghdad because of her intellectual disability.Case presentationThe patient was 4-year-old female child who presented with psychomotor delay and language impairment. She had frequent attacks of the respiratory tract and eye infections. Ophthalmologic examination revealed left-sided esotropia and severe myopia. Routine hematologic, serologic, and chemistry tests were within normal ranges. EEG revealed diffuse theta slowing and diffuse beta activity. The audiological test was normal. NCS and EMG showed normal results. Echo study, chest X-ray, and abdominal/pelvic ultrasound revealed normal findings. Brain MRI showed mild bilateral frontal-temporal atrophy. Whole-exome sequencing (WES) revealed a homozygous stop mutation in CNTNAP2 with a heterozygous state in both parents.ConclusionIntellectual disability may result from different types of abnormal cellular processes and with widening the use of molecular gene analysis in cases of intellectual disability, underdiagnosed cases of Pitt-Hopkins and Pitt-Hopkins-like syndromes may be uncovered.

The Applicability of the Patient-Specific Functional Scale (PSFS) in Rehabilitation for Patients with Acquired Brain Injury (ABI) - A Cohort Study.

AimThe primary aim of this study was to investigate the applicability of the Patient-Specific Functional Scale (PSFS) in patients with acquired brain injury (ABI) admitted to a specialized rehabilitation unit in a regional hospital. A secondary aim was to identify patient characteristics and functioning that predicted changes in the PSFS.Patients and MethodsIn a cohort study, 59 patients with ABI were assessed for the ability to complete the PSFS. A trained multidisciplinary team applied the PSFS as part of a collaborative development of rehabilitation goals. The modified Rankin Scale (mRS), the Functional Ambulation Categories (FAC), the Rivermead Behavioural Memory Test (RBMT), the Norwegian Basic Aphasia Assessment (NBAA) and the Loewenstein Occupational Therapy Cognitive Assessment (LOTCA) were used to identify characteristics of the sample. Multivariate regression analyses were performed to investigate associations between changes in the PSFS score from admission to discharge and a selected set of participant baseline characteristics and functioning.ResultsFifty-four patients (92%) of the patients with ABI were able to complete the PSFS. The five (8%) who were unable to complete the PSFS had severe cognitive or language impairment. The PSFS score improved by a mean of 2.6 (SD 2.0) points from admission to discharge. The LOTCA score made the strongest unique contribution to explain the change in the PSFS score (beta = 0.477, p= 0.020).ConclusionIn the present study, most patients with ABI (92%) were able to complete the PSFS. Cognitive function on admission was a predictor of improved functioning on the PSFS.

Neural Evidence for Speech Processing Deficits During a Cocktail Party Scenario in Minimally and Low Verbal Adolescents and Young Adults with Autism.

As demonstrated by the Cocktail Party Effect, a person's attention is grabbed when they hear their name in a multispeaker setting. However, individuals with autism (ASD) are commonly challenged in multispeaker settings and often do not respond to salient speech, including one's own name (OON). It is unknown whether neural responses during this Cocktail Party scenario differ in those with ASD and whether such differences are associated with expressive language or auditory filtering abilities. We measured neural responses to hearing OON in quiet and multispeaker settings using electroencephalography in 20 minimally or low verbal ASD (ASD-MLV), 27 verbally fluent ASD (ASD-V), and 27 neurotypical (TD) participants, ages 13-22. First, we determined whether TD's neural responses to OON relative to other names could be quantified with early frontal mismatch responses (MMRs) and late, slow shift parietal and frontal responses (LPPs/FNs). Second, we compared the strength of MMRs and LPPs/FNs across the three groups. Third, we tested whether participants with poorer auditory filtering abilities exhibited particularly weak neural responses to OON heard in a multispeaker setting. Our primary finding was that TDs and ASD-Vs, but not ASD-MLVs, had significant MMRs to OON in a multispeaker setting, and strength of LPPs positively correlated with auditory filtering abilities in those with ASD. These findings reveal electrophysiological correlates of auditory filtering disruption within a clinical population that has severe language and communication impairments and offer a novel neuroimaging approach to studying the Cocktail Party effect in neurotypical and clinical populations. Autism Res 2020, 13: 1828-1842. © 2020 International Society for Autism Research and Wiley Periodicals LLC. LAY SUMMARY: We found that minimally and low verbal adolescents and young adults with autism exhibit decreased neural responses to one's own name when heard in a multispeaker setting. In addition, decreased strength of neural responses in those with autism correlated with decreased auditory filtering abilities. We propose that these neural deficits may reflect the ineffective processing of salient speech in noisy settings and contribute to language and communication deficits observed in autism.

The role of microstructural integrity of major language pathways in narrative speech in the first year after stroke

Background and PurposeLeft hemisphere stroke often results in a variety of language deficits due to varying patterns of damage to language networks. The Cookie Theft picture description task, a classic, quick bedside assessment, has been shown to quantify narrative speech reliably. In this study, we utilized diffusion tensor imaging (DTI) to assess language network white matter tract correlates of lexical-semantic and syntactic impairments longitudinally. MethodsTwenty-eight patients with mild to severe language impairments after left hemispheric lobar and/or subcortical ischemic stroke underwent the Cookie Theft picture description test and DTI up to three different time points: within the first three months, six months and twelve months after stroke. Dorsal and ventral stream language pathways were segmented to obtain DTI integrity metrics of both hemispheres. Multivariable regression models and partial correlation analyses adjusted for age, education, and lesion load were conducted to evaluate the temporal DTI profile of the white matter microstructural integrity of the language tracts as neural correlates of narrative speech within the first year after stroke. ResultsAmong all the major language white matter pathways, the integrity of the left arcuate (AF), inferior fronto-occipital, and inferior longitudinal fasciculi (ILF) were related to picture description performance. After FDR correction, left ILF fractional anisotropy correlated with syntactic cohesiveness (r=0.85,p=0.00087) within the first three months after stroke, whereas at one year post-stroke, the strongest correlations were found between lexical-semantic performance and left AF radial diffusivity (r = −0.71, p = 0.00065). ConclusionOur study provides a temporal profile of associations between the integrity of the main language pathways and lexical semantics and syntactic impairments in left hemispheric strokes.

The association between communication impairments and acquired alexithymia in chronic stroke patients

ABSTRACT Introduction Language dysfunction has recently been suggested to be one route to alexithymia, an impairment in recognizing and communicating one’s own emotions. Neuropsychological evidence is needed to investigate the possibility that acquired language problems could underlie acquired alexithymia. Method This project examined data from a large group of chronic stroke patients (N = 118) to test whether self-reported or behavioral measures of language and communication problems were associated with alexithymia. We also examined the impact of hemisphere of damage on alexithymia. Results We found no differences in alexithymia levels for patients with observed language impairments on brief tests of picture naming, comprehension, and reading vs unimpaired patients. However, self-reported communication difficulties were found to be associated with higher scores of alexithymia, even after controlling for depression and anxiety. Patients with left- versus right-hemisphere damage did not differ in their alexithymia scores. Conclusions We found partial support for the language hypothesis of alexithymia. We discuss potential reasons for the discrepant findings between the self-report and objective language measures and suggest that self-report measures may be more sensitive to milder, more pragmatic language impairments, as opposed to the severe structural language impairments measured by the cognitive screening tests.

Glial fibrillary acidic protein (GFAP)-antibody in children with focal seizures of undetermined cause.

Anti-neuronal antibodies that are related with autoimmune encephalitis syndromes may also be found in children with new onset seizures or chronic epilepsy. To unravel the significance of autoimmune astrocytopathy in epilepsy, we investigated serum antibody to glial fibrillary acidic protein (GFAP), another autoantigen described in autoimmune encephalitis with seizures, in 38 children with focal seizures of undetermined cause. GFAP antibody was screened with cell based assay and indirect immunohistochemistry and was found in two boys with normal brain MRI and unrevealing medical history prior to seizures. The 2-year-old boy had chronic treatment-resistant frontal lobe epilepsy. The 2.5-year-old boy had a single episode of focal seizures and remained seizure free thereafter in a follow-up period of 4years. Nevertheless, he showed severe cognitive and language impairment. These results suggest that autoimmune astrocytopathy may be present in some epilepsy patients. Whether this immune response is a bystander effect generated by seizure-induced astrocytosis or directly involved in epileptogenesis needs to be further studied.