Language Skills in Patients With Alexander Disease.

To examine interrater agreement and validity of the Functional Communication Classification System (FCCS) for young children with cerebral palsy (CP) aged 2 or 3 years. Speech-language pathologist (SLP) and parent FCCS ratings for 31 children with CP (aged 2y, n=16; aged 3y, n=15; 18 males, 13 females) were examined for interrater agreement using a weighted Cohen's kappa statistic. Relationships between FCCS (SLP) ratings and: (1) concurrent validity with the Language Use Inventory, a standardized pragmatic assessment for children aged 18 to 47 months, (2) gross motor and fine motor function, (3) associated impairments (visual and intellectual), and (4) primary expressive communication mode were examined using Spearman's correlation coefficients. Almost perfect interrater agreement between SLP and parent FCCS ratings were found (kw =0.94). Correlations with FCCS (SLP) were excellent for pragmatic function (rs =-0.83,p<0.001), intellectual function (rs =0.89, p<0.001), and primary expressive communication mode (rs =0.92,p<0.001). Correlations were good for gross motor function (rs =0.72,p<0.001) and visual impairment (rs =0.70,p<0.001) and fair for fine motor function (rs =0.53,p<0.002). Analysis was unwarranted for epilepsy (n=1 out of 31) and hearing-associated impairments (n=0 out of 31). The FCCS has excellent interrater agreement and validity for communication classification of children with CP aged 2 or 3 years and is highly suitable for surveillance and research purposes. What this paper adds The Functional Communication Classification System (FCCS) is a valid instrument for children with cerebral palsy (CP) aged 2 or 3 years. Excellent agreement exists between speech-language pathologist and parent FCCS ratings. The FCCS has excellent correlation with intelligence, pragmatic function, and primary expressive mode. Stronger correlations with the Gross Motor Function Classification System and vision exist for children aged 2 or 3 years. Weaker correlations with manual ability exist when compared to older children with CP.

Functional classifications for cerebral palsy: correlations between the gross motor function classification system (GMFCS), the manual ability classification system (MACS) and the communication function classification system (CFCS).

Purpose To examine novice inter-rater agreement and clinical utility perspectives for speech and communication classification of children with cerebral palsy (CP). Method Twenty-one clinicians (speech-language pathologists [SLPs] n = 11; physiotherapists [PTs] n = 5; occupational therapists [OTs] n = 5) novice to the Viking Speech Scale (VSS), Functional Communication Classification System (FCCS), and Communication Function Classification System (CFCS) rated eight unfamiliar children with CP (8–16 years) following classification orientation. Inter-rater agreement was examined between (a) novices, (b) novice SLPs vs. PTs and OTs, and (c) novice vs. expert (kappa statistics). Utility perceptions were scored regarding classification terminology, ease of use, assistive decision-making resources, and construct validity and were analysed using Kruskal–Wallis H-tests. Result Rating agreement between novices was substantial (VSS, k = 0.72, 95% CI [0.53–0.92]) to moderate (FCCS, k = 0.44, 95% CI [0.23–0.65]; CFCS, k = 0.45, 95% CI [0.18–0.71]), and almost perfect between novice and expert ratings (VSS, kw = 0.89, 95% CI [0.86–0.92]; FCCS, kw = 0.89, 95% CI [0.86–0.92]; CFCS, kw = 0.86, 95% CI [0.82–0.91]). Statistically significant differences, presented highest to lowest, were found for clinical utility: terminology (VSS, FCCS, CFCS; p = 0.02), assistive decision-making resources (FCCS, VSS, CFCS; p = 0.009), and construct validity (FCCS, CFCS, VSS; p < 0.001). Conclusion Novice raters achieved substantial agreement for speech classification, supporting utilisation in clinical, research, and CP register activities. Orientation to communication classification constructs, content, and instructions is recommended for novice raters.

The objective of this study was to examine the effects of prenatal cocaine and polydrug exposure on language development of preschool children using a prospective longitudinal model, controlling for confounders. Children who were exposed to cocaine in utero (n = 209) and nonexposed children (n = 189) were followed prospectively at birth and at 1, 2, 4, and 6 years of age and were compared on receptive, expressive, and total language scores across time using random coefficient models, controlling for confounders. A significant, stable effect of cocaine exposure on language development was observed over time for all language domains, with cocaine exposure related to poorer language performance. Cigarette exposure was related to lower receptive language scores. Environmental influences on language scores were also observed. Both the cocaine-exposed and nonexposed children declined in language performance over time. Prenatal cocaine exposure has a stable negative effect on language skills during the first 6 years of life. Both cocaine-exposed and nonexposed children showed decreased language growth over time; however, cocaine-exposed children demonstrated linguistic deficits compared with nonexposed peers and did not catch up. Cigarette and environmental influences were also noted.

Event Abstract Back to Event Language and communication in children with agenesis of the corpus callosum Vanessa Siffredi1, 2, 3*, Alissandra McIlroy1, 2, Vicki Anderson1, 2, 4, Richard Leventer1, 2, 4, Amanda Wood5 and Megan M. Spencer-Smith1, 6 1 Murdoch Childrens Research Institute, Australia 2 The University of Melbourne, Australia 3 The University of Geneva, Switzerland 4 The Royal Children's Hospital, Australia 5 University of Birmingham, United Kingdom 6 Swinburne University of Technology, Australia Background Expressive and receptive language are predominantly processed by the left hemisphere. Interhemispheric transfer is important for the integration of linguistic and communication related information. The corpus callosum, the largest white matter pathway connecting the two cerebral hemispheres, plays a crucial role in the transfer and integration of language and communication information across hemispheres. Developmental absence (agenesis) of the corpus callosum (AgCC) is a congenital brain malformation resulting from disruption of corpus callosum formation. This study aims to: 1) describe language and communication skills in children with AgCC; and 2) examine the role of general intellectual ability, brain structure (partial or complete AgCC; intactness of anterior and posterior commissures) and clinical factors (presence of seizure disorder, genetic condition) as predictors of language and communication abilities. Methods Fifteen children with AgCC (partial n=8, complete n=7) aged 8 to 15 years (M= 12.03, SD=2.16) were recruited. Language (receptive and expressive skills), general intellectual and communication abilities were estimated using standardised measures. Brain MRI was reviewed using a standardised coding system. Results Preliminary analysis showed that children with AgCC performed below the test mean on all measures: expressive (p=.001), receptive (p=.001), communication (p=.005). There was some variability in language and communication skills: communication ratings were higher than expressive language scores (p=.015), which were higher than receptive language scores (p=.016). A series of regressions showed that general intellectual ability predicted expressive language scores (p=.001), and approached significance for receptive language scores (p=.064). Intactness of the corpus callosum predicted communication ratings (p=.043), with complete AgCC associated with poorer outcomes. Clinical factors and intactness of anterior and posterior commissures were not significantly associated with language and communication abilities. Discussion Language and communication abilities are reduced in children with AgCC. Contrary to expectations, children with AgCC showed better communication than language abilities, with greatest difficulties in expressive language. General intellectual ability predicted language but not communication abilities. The intactness of the corpus callosum predicted communication but not language abilities. Acknowledgements This study was supported by the Victorian Government’s Operational Infrastructure Support Program; by the Murdoch Childrens Research Institute; and by the Swiss National Science Foundation (SNSF). Keywords: Agenesis of the Corpus callosum, Language, Communication, MRI, developmental neuropsychology Conference: ACNS-2013 Australasian Cognitive Neuroscience Society Conference, Clayton, Melbourne, Australia, 28 Nov - 1 Dec, 2013. Presentation Type: Oral Topic: Language Citation: Siffredi V, McIlroy A, Anderson V, Leventer R, Wood A and Spencer-Smith MM (2013). Language and communication in children with agenesis of the corpus callosum. Conference Abstract: ACNS-2013 Australasian Cognitive Neuroscience Society Conference. doi: 10.3389/conf.fnhum.2013.212.00151 Copyright: The abstracts in this collection have not been subject to any Frontiers peer review or checks, and are not endorsed by Frontiers. They are made available through the Frontiers publishing platform as a service to conference organizers and presenters. The copyright in the individual abstracts is owned by the author of each abstract or his/her employer unless otherwise stated. Each abstract, as well as the collection of abstracts, are published under a Creative Commons CC-BY 4.0 (attribution) licence (https://creativecommons.org/licenses/by/4.0/) and may thus be reproduced, translated, adapted and be the subject of derivative works provided the authors and Frontiers are attributed. For Frontiers’ terms and conditions please see https://www.frontiersin.org/legal/terms-and-conditions. Received: 15 Oct 2013; Published Online: 25 Nov 2013. * Correspondence: Ms. Vanessa Siffredi, Murdoch Childrens Research Institute, Melbourne, Australia, vanessa.siffredi@mcri.edu.au Login Required This action requires you to be registered with Frontiers and logged in. To register or login click here. Abstract Info Abstract The Authors in Frontiers Vanessa Siffredi Alissandra McIlroy Vicki Anderson Richard Leventer Amanda Wood Megan M Spencer-Smith Google Vanessa Siffredi Alissandra McIlroy Vicki Anderson Richard Leventer Amanda Wood Megan M Spencer-Smith Google Scholar Vanessa Siffredi Alissandra McIlroy Vicki Anderson Richard Leventer Amanda Wood Megan M Spencer-Smith PubMed Vanessa Siffredi Alissandra McIlroy Vicki Anderson Richard Leventer Amanda Wood Megan M Spencer-Smith Related Article in Frontiers Google Scholar PubMed Abstract Close Back to top Javascript is disabled. Please enable Javascript in your browser settings in order to see all the content on this page.

Alexander's disease, a leukodystrophy characterized by Rosenthal fibers (RFs) in the brain, is categorized into three subtypes: infantile, juvenile, and adult. Although most are sporadic, occasional familial Alexander's disease cases have been reported for each subtype. Hereditary adult-onset Alexander's disease shows progressive spastic paresis, bulbar or pseudobulbar palsy, palatal myoclonus symptomatologically, and prominent atrophy of the medulla oblongata and upper spinal cord on magnetic resonance imaging. Recent identification of GFAP gene mutations in the sporadic infantile- and juvenile-onset Alexander's disease prompted us to examine the GFAP gene in two Japanese hereditary adult-onset Alexander's disease brothers with autopsy in one case. Both had spastic paresis without palatal myoclonus, and magnetic resonance imaging showed marked atrophy of the medulla oblongata and cervicothoracic cord. The autopsy showed severely involved shrunken pyramids, but scarce Rosenthal fibers (RFs). Moderate numbers of Rosenthal fibers (RFs) were observed in the stratum subcallosum and hippocampal fimbria. In both cases, we found a novel missense mutation of a G-to-T transition at nucleotide 841 in the GFAP gene that results in the substitution of arginine for leucine at amino acid residue 276 (R276L). This is the first report of identification of the causative mutation of the GFAP gene for neuropathologically proven hereditary adult-onset Alexander's disease, suggesting a common molecular mechanism underlies the three Alexander's disease subtypes.

This study examined the extent to which children born preterm (< 37 weeks) and/or who have low birth weight (< 2,500 g) catch up with their full term peers in terms of their language abilities at early school age (≥ 5 to < 9 years). A systematic literature search identified empirical studies that fit the inclusion criteria. Data from the tests/questionnaires used for meta-analysis spanned the following language categories: total language score, expressive language, receptive language, pragmatics, phonological awareness, and grammar. The means (standard deviations) were extracted from the studies and were converted to mean difference and 95% confidence intervals to test for overall effect. Sixteen studies met the inclusionary criteria, for a total of 2,739 participants, of which 1,224 were born full term and 1,515 were born preterm. It is important to note that the preterm cohort represented very preterm infants who have a very low birth weight. The meta-analysis found that preterm infants scored significantly worse on total language (p < .001), receptive language (p < .001), expressive language (p < .001), phonological awareness (p < .001), and grammar (p = .03) than their full term peers. However, preterm infants did not score significantly worse than their peers on their pragmatics (p = .19). Children born VPT and who have VLBW perform worse than their peers on their total language, receptive language, expressive language, phonological awareness, and grammar abilities by early school age. This information is important for speech-language pathologists to consider as children born prematurely reach school age.

The relationship of high quality day care to middle-class 3-year-olds' language performance

Background: Play is very important as it helps cognitive, language, social, emotional and physical development.The value of play is increasingly recognized by researchers for children as the evidence mounts of its relationship with intellectual achievement, language development and emotional wellbeing. Aim of Study:To assess play skills in a group of children having cognitive delay (mild and moderate cognitive delay with or without Down's syndrome) and to study the correlation between play level, language skills and cognitive skills in these children.Subjects and Methods: 75 Egyptian preschool children aged between 2 and 5 years were divided into 3 groups; group of normal children, group of children with Cognitive Delay (CD) and group of Down Syndrome's (DS).They were subjected to the protocol of Arabic language assessment and a play observation checklist designed in this study.The checklist covered the following items; level of play, behavior of the child during play, receptive skills & language use during play, child interaction with peers & with adults as well as problem solving during play.Results: Normal children under study had higher play skills and language skills than children with CD & DS children.Cognitive level of children with CD and DS was correlated positively with some aspects of their play skills such as language use during play in CD and problem solving, receptive skills and language use during play in DS children.Total Language score was highly correlated with all play aspects in CD and most of play aspects in DS children. Conclusion:Cognitive level, development of play skills and language abilities were interrelated in groups under study (CD and DS).

The goal of this study was to examine structured language skills in children with perinatal strokes. Participants were 28 school-age children with early focal brain lesions (17 with left hemisphere [LH] damage, 11 with right hemisphere [RH] damage), and 57 controls. A standardized test of language (Clinical Evaluation of Language Fundamentals–Revised) was administered. Receptive, Expressive, and Total Language scores, as well as subtest scores, were analyzed. Control participants scored within the normal range, whereas the LH and RH groups scored significantly more poorly than did controls. There were no differences between the LH and RH groups on any of the language scores, and all scores were below the 14th percentile. Within the lesion group as a whole, scores were not related to lesion laterality, site, or severity. Results also were not accounted for by socioeconomic status or IQ. However, children who experienced seizures demonstrated significantly poorer performance than did children who did not experience seizures. Damage to either the LH or RH early in development adversely affects later language abilities, particularly on tasks with structured and complex linguistic demands. Although lesion side has little effect, the presence or absence of seizures is a major contributor to language outcome.

This study examined the relationship between parental self-efficacy in parents of young deaf and hard-of-hearing (DHH) children and children's spoken language skills. A retrospective within-subjects study design was used that included 24 mother-child dyads with DHH children. Parental self-efficacy was assessed using the Scale of Parental Involvement and Self-Efficacy-Revised. Children's language abilities were assessed using the Preschool Language Scale-5th edition. Our data revealed no significant associations between global measures of parental self-efficacy and children's auditory comprehension, expressive communication, and total language scores. However, positive correlations were found between child language skills and specific parents' beliefs about their ability to support their child's spoken language development, their ability to use strategies to help their child communicate, and their active involvement in intervention. Findings highlight the importance of examining discrete aspects of parental self-efficacy as it specifically relates to parents supporting their DHH child's spoken language development. Future directions and implications are provided.

The purpose of this study was to determine the significance and directions of the relationships among oral and manual fine motor skills and language abilities among Spanish-English bilingual children. If such relationships exist, this would support a shared biological influence on motor and language development. Participants included 56 bilingual children, 24 of whom met criteria for developmental language disorder (DLD), recruited based on teacher concern for language and/or reading comprehension abilities. Students participated in a battery of baseline tests to determine motor, language, and cognitive abilities. Correlations among all variables were examined for direction of relationships. Regression models explored the predictive power of motor skills with Spanish and English language ability as the outcome measure. Oral fine motor abilities (diadochokinetic rate productions of /pa/ and /pata/) predicted Spanish (but not English) oral language abilities in the expected direction (i.e., faster rates were associated with better language). Manual fine motor performance on computer tapping tasks was not related to performance in either language. Oral fine motor abilities are related to language abilities in bilingual children, but only for the native language. We did not find reliable differences in oral and manual fine motor skills between groups of bilingual children with and without DLD. These findings support a limited role of shared biological influences on motor and language development.

To determine the concentration of glial fibrillary acidic protein (GFAP) in cerebrospinal fluid (CSF) and plasma in Alexander disease (AxD) and whether GFAP levels are predictive of disease phenotypes. CSF and plasma were collected (longitudinally when available) from AxD participants and non-AxD controls. The concentration of GFAP was compared between groups using Wilcoxon rank sum test. The Kruskal-Wallis test was used to compare GFAP concentrations across multiple groups (clinical phenotype or common genetic variants occurring at p.Arg79, p.Arg88, p.Arg239). GFAP concentrations were significantly elevated at baseline in both AxD CSF (N = 44) compared to controls (N = 46, p < 0.0001) and AxD plasma (N = 96) compared to other leukodystrophy plasma controls (N = 67, p < 0.0001). CSF and plasma GFAP concentrations differed between AxD cerebral, intermediate, and bulbospinal phenotypes (Kruskal-Wallis test, CSF: p = 0.0235, plasma: p < 0.0001). The median GFAP change/year among patients sampled < 8 years of age at baseline was an increase of 91,100 [pg/mL]/year (IQR 136,050) in CSF and 2850 [pg/mL]/year (IQR 9500) in plasma compared to patients > 8 years whose medians decreased over time (-41,000 [pg/mL]/year [IQR 90,300], CSF; -843 [pg/mL]/year [IQR 3900], plasma). The fold change in GFAP from first to last sampling were significantly different before and after 8 years at baseline (Wilcoxon rank sum test, CSF: p = 0.0232, plasma: p = 0.0002). A significant association was not detected between GFAP variant and GFAP levels. GFAP concentrations are higher in the cerebral phenotype and increase over time in young children. These data can be used to formulate biomarker qualification and context-of-use in AxD.

Estimated effects of in utero cocaine exposure on language development through early adolescence

Despite accumulated evidence that language development depends on basic cognitive processes, the balance in contributions of verbal and non-verbal cognitive skills to language abilities is still underexplored. Little is known about which cognitive measures best predict the degree of severity in children with language disorder (LD). To examine the association between verbal and non-verbal cognitive abilities with language abilities in typically developing and language impaired 8-year-old children, as well as which cognitive abilities are most effective in distinguishing LD severity levels. Children (N = 509) from the Language-8 Study, which oversampled probable cases of children with LD from a population-based cohort in Norway, were assessed at 8 years. Language skills were assessed using the Norwegian Clinical Evaluation of Language Fundamentals-4 (CELF-4). Children's verbal and non-verbal cognitive abilities were assessed via standardized cognitive measures. An exploratory factor analysis (EFA) was first conducted to uncover the underlying factor structure of the cognitive variables. Using a hierarchical multiple regression analysis, we then examined to what extent the non-verbal cognition factor explained language abilities above and beyond verbal cognition factors. Lastly, multinomial logistic regression was used to examine which cognitive measures best predicted the degree of severity in the children with LD. The EFA resulted in three factors (Verbal Cognition, Processing Speed and Memory, and Non-Verbal Cognition). The hierarchical multiple regression analysis revealed that all three cognitive factors contributed significantly to individual variation in language abilities. Non-Verbal Cognition explained 5.4% variance in language abilities above and beyond that accounted for by Verbal Cognition and Processing Speed and Memory. Results from the multinomial logistic regression analysis indicated that cognitive subtests, including Familiar Sequences, WASI Vocabulary and WASI Similarities, not only distinguished LD from typically developing children, but were also efficient in distinguishing severity of LD symptoms. This study confirms concurrent links between language and non-verbal cognitive skills above and beyond the contribution of verbal cognitive skills. The results provide further evidence that children with LD experience both language and cognitive problems in mid-childhood. Our findings suggest implications for LD intervention and diagnosis. The findings support the importance of measuring both verbal and non-verbal cognitive skills when making an LD diagnosis, and point to the potential of targeting underlying cognitive skills as one strategy to support language abilities. What is already known on the subject Language development is dependent on basic cognitive processes. These include both verbal and non-verbal cognitive abilities. Children with LD often experience both language and cognitive problems. There is evidence that performance on cognitive tests may be associated with the degree of severity of LD. What this paper adds to existing knowledge The current results from a large population-based cohort establish that a number of verbal and non-verbal cognitive abilities are tightly linked to variation in language abilities and the degree of severity of LD. Our study confirms concurrent links between language and non-verbal cognitive abilities above and beyond the contribution of verbal cognitive abilities. We also identify specific verbal and non-verbal cognitive tests that distinguish between typical children and children with LD, as well as LD severity. What are the potential or actual clinical implications of this work? Our findings support the importance of measuring both verbal and non-verbal cognitive skills when making an LD diagnosis. Our findings also point to the potential of targeting underlying cognitive skills as one strategy to support language abilities. We suggest that future intervention studies focus on the impact of non-verbal cognitive skills on language development in children with LD.