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Biomedical subjects

C B Mervis

Publications and source records attributed to C B Mervis.

At least 19 recordsLinked to original sources
PubMed2000

Williams syndrome: cognition, personality, and adaptive behavior.

Williams syndrome is caused by a microdeletion of at least 16 genes on chromosome 7q11.23. The syndrome results in mild to moderate mental retardation or learning disability. The behavioral phenotype for Williams syndrome is characterized by a distinctive cognitive profile and an unusual personality profile. Relative to overall level of intellectual ability, individuals with Williams syndrome typically show a clear strength in auditory rote memory, a strength in language, and an extreme weakness in visuospatial construction. The personality of individuals with Williams syndrome involves high sociability, overfriendliness, and empathy, with an undercurrent of anxiety related to social situations. The adaptive behavior profile for Williams syndrome involves clear strength in socialization skills (especially interpersonal skills related to initiating social interaction), strength in communication, and clear weakness in daily living skills and motor skills, relative to overall level of adaptive behavior functioning. Literature relevant to each of the components of the Williams syndrome behavioral phenotype is reviewed, including operationalizations of the Williams syndrome cognitive profile and the Williams syndrome personality profile. The sensitivity and specificity of these profiles for Williams syndrome, relative to individuals with other syndromes or mental retardation or borderline normal intelligence of unknown etiology, is considered. The adaptive behavior profile is discussed in relation to the cognitive and personality profiles. The importance of operationalizations of crucial components of the behavioral phenotype for the study of genotype/phenotype correlations in Williams syndrome is stressed. MRDD Research Reviews 2000;6:148-158.

Adaptation, Psychological↗
PubMed2000

The Williams syndrome cognitive profile.

Williams syndrome is a rare neurodevelopmental disorder caused by a hemizygous deletion of approximately 1.5 megabases on chromosome 7q11.23. In this article, we outline a Williams Syndrome Cognitive Profile (WSCP) that operationalizes the cognitive characteristics of the syndrome using measures of absolute and relative performance on subtests of the Differential Abilities Scales (Elliot, 1990a). Testing confirmed excellent sensitivity and specificity scores for the WSCP. Seventy-four of 84 individuals with Williams syndrome fit the WSCP while only 4 participants in a contrast group met all of the WSCP criteria. It was also found that the WSCP does not vary greatly with chronological age or overall level of cognitive ability for individuals with Williams syndrome. Possible applications for the WSCP include psychoeducational evaluation and empirical research such as the search for genotype/phenotype relations in this genetically based syndrome.

Adolescent↗
PubMed2000

Expressive vocabulary ability of toddlers with Williams syndrome or Down syndrome: a comparison.

School-aged children and adults with Williams syndrome have repeatedly been found to evidence an expressive vocabulary advantage relative to same-aged individuals with Down syndrome. However, Singer Harris, Bellugi, Bates, Jones, and Rossen (1997) argued that this advantage is reversed during the initial period of language acquisition; during this time, children with Down syndrome have larger expressive vocabularies than children with Williams syndrome. This result may have been due to methodological problems, however. This study uses a different design to reconsider the question of whether toddlers with Williams syndrome show an expressive vocabulary advantage over same-aged toddlers with Down syndrome. Parents of twenty-four 2-year-olds with Williams syndrome and twenty-eight 2-year-olds with Down syndrome completed the vocabulary checklist from the MacArthur Communicative Development Inventory: Words and Sentences. The 2 groups were carefully matched for chronological age (CA). Results indicated that the toddlers with Williams syndrome had substantially and significantly larger expressive vocabulary sizes than did the CA-matched children with Down syndrome. Additional analyses of children for whom data were available between the ages of 2 years 0 months and 2 years 3 months indicated that the expressive vocabulary advantage for children with Williams syndrome was present even at this very young age when none of the children had begun to produce word combinations. The Discussion section that follows addresses the discrepancy between these findings and those of Singer Harris et al. and considers the variability present within both the Williams syndrome and Down syndrome samples. Also discussed is the continuity across the lifespan in both the expressive vocabulary advantage shown by individuals with Williams syndrome relative to same-aged individuals with Down syndrome and the expressive vocabulary variability within each syndrome.

Age Factors↗
PubMed1999

Comparing productive vocabulary measures from the CDI and a systematic diary study.

Expressive vocabulary data gathered during a systematic diary study of one male child's early language development are compared to data that would have resulted from longitudinal administration of the MacArthur Communicative Development Inventories spoken vocabulary checklist (CDI). Comparisons are made for (1) the number of words at monthly intervals (9; 10.15 to 2; 0.15), (2) proportion of words by lexical class (i.e. noun, predicate, closed class, 'other'), (3) growth curves. The CDI underestimates the number of words in the diary study, with the underestimation increasing as vocabulary size increases. The proportion of diary study words appearing on the CDI differed as a function of lexical class. Finally, despite the differences in vocabulary size, logistic curves proved to be the best fitting model to characterize vocabulary development as measured by both the diary study and the CDI. Implications for the longitudinal use of the CDI are discussed.

Child Language↗
PubMed1999

Methodological issues in cross-syndrome comparisons: matching procedures, sensitivity (Se), and specificity (Sp).

We are impressed with the magnitude and potential importance of the studies presented by Sigman and Ruskin in this monograph. The within-syndrome findings for the children with autism concerning relations between early joint attention and a range of cognitive abilities a full 9 years later provide the strongest evidence so far that early nonverbal communication skills play an important role in the later development of language, intelligence, and social relations with peers. The purpose of the monograph was not limited to within-syndrome research questions, however. Sigman and Ruskin state that a major goal of the research reported in the monograph was to identify specific, unique, and universal deficits for autism and Down syndrome. They base their method of identifying such syndrome characteristics on the group-matching procedure. Given that this procedure is fraught with difficulties, we are concerned that many of Sigman and Ruskin's cross-syndrome comparisons may be incorrect. We do not mean to single out Sigman and Ruskin. The group-matching method is frequently used in special populations research, with the null hypothesis of no differences on the control variable being accepted at dangerously low p values. Our concerns with the group-matching problem extend to much of the extant research that attempts to identify characteristics of individuals based on the performance of their syndrome group relative to a control group. The profiling procedure we outlined seems more fruitful and conceptually satisfying than the traditional matching method. When profiling is not possible, however, it is important to consider the impact of CA confounds and statistical decision procedures used to ensure matching on the control variable, when interpreting syndrome differences on variables of interest.

Adolescent↗
PubMed1998

Disentangling early language development: modeling lexical and grammatical acquisition using an extension of case-study methodology.

The early lexical and grammatical development of 1 male child is examined with growth curves and dynamic-systems modeling procedures. Lexical-development described a pattern of logistic growth (R2 = .98). Lexical and plural development shared the following characteristics: Plural growth began only after a threshold was reached in vocabulary size; lexical growth slowed as plural growth increased. As plural use reached full mastery, lexical growth began again to increase. It was hypothesized that a precursor model (P. van Geert, 1991) would fit these data. Subsequent testing indicated that the precursor model, modified to incorporate brief yet intensive plural growth, provided a suitable fit. The value of the modified precursor model for the explication of processes implicated in language development is discussed.

Child, Preschool↗
PubMed1998

Impact of intuitive theories on feature recruitment throughout the continuum of expertise.

Expertise in object domains involves both the perceptual learning of the differentiating and higher order features that are indicative of concepts and the elaboration of intuitive theories. Triad-similarity judgments, feature-salience ratings, and verbal protocols were used to investigate the effects of theories on the recruitment of features across different categorization contexts, as well as the degree to which expert categorization skills transferred to less familiar domains. Whereas novices considered features that indicated overall similarity to be more perceptually salient than were modified parts that indicated taxonomic relations, experts found them equally salient. Experts' theories were instrumental in directing feature recruitment in contexts involving identification, image generation, and similarity decisions. Experts' theories also supported the transfer of categorization skills to related, less familiar domains. The relation of mutual dependence between perceptual learning and theory development throughout the continuum of expertise is considered.

Adult↗
PubMed1997

Development of children's understanding of basic-subordinate inclusion relations.

Four studies focused on developmental differences in the representation of basic-subordinate inclusion relations. Tests of comprehension and production of category names, induction, and responses to direct questions pertaining to inclusion revealed a marked developmental gap between the production of subordinate category names and complete understanding of basic-subordinate inclusion relations. However, even 3-year-olds showed rudimentary knowledge of the asymmetry of inclusion. Discrepancies between children's performance on categorization tasks involving familiar and unfamiliar subordinate categories suggest that understanding of the logical nature of inclusion relations is constructed through the integration of categorical knowledge from familiar domains. Interactions among the knowledge base, pragmatic sensitivity, and information processing efficiency in the development of inclusion are discussed.

Age Factors↗
PubMed1997

Effects of varying levels of expertise on the basic level of categorization.

Six experiments were conducted on the effects of expertise on basic-level categorization. Individuals with varying levels of knowledge about songbirds generated lists of attributes, named objects, identified and discriminated among object silhouettes, verified category membership at 4 hierarchical levels, and visually identified songbirds primed either by species-specific, related, or unrelated birdsong. Results indicated that the original basic level never lost its privileged status. Expertise increased access to categorical information at the subordinate level for intermediate exports and at both the subordinate and sub-subordinate levels for advanced experts, causing these sublevels to function as basic. Throughout the continuum of expertise, conceptual knowledge interacted with perception. Accordingly, experts attended to different and more subtle perceptual features than novices.

Animals↗
PubMed1996

LIM-kinase1 hemizygosity implicated in impaired visuospatial constructive cognition.

To identify genes important for human cognitive development, we studied Williams syndrome (WS), a developmental disorder that includes poor visuospatial constructive cognition. Here we describe two families with a partial WS phenotype; affected members have the specific WS cognitive profile and vascular disease, but lack other WS features. Submicroscopic chromosome 7q11.23 deletions cosegregate with this phenotype in both families. DNA sequence analyses of the region affected by the smallest deletion (83.6 kb) revealed two genes, elastin (ELN) and LIM-kinase1 (LIMK1). The latter encodes a novel protein kinase with LIM domains and is strongly expressed in the brain. Because ELN mutations cause vascular disease but not cognitive abnormalities, these data implicate LIMK1 hemizygosity in imparied visuospatial constructive cognition.

Base Sequence↗
PubMed1996

Mapping the meanings of novel visual symbols by youth with moderate or severe mental retardation.

The word-learning ability of 12 school-age subjects with moderate or severe mental retardation was assessed. Subjects had little or no functional speech and used the System for Augmenting Language with visual-graphic symbols for communication. Their ability to fast map novel symbols revealed whether they possessed the novel name-nameless category (N3C) lexical operating principle. On first exposure, 7 subjects were able to map symbol meanings for novel objects. Follow-up assessments indicated that mappers retained comprehension of some of the novel words for up to delays of 15 days and generalized their knowledge to production. Ability to fast map reliably was related to symbol achievement status. Implications for understanding vocabulary acquisition by youth with mental retardation were discussed.

Adolescent↗
PubMed1995

Early lexical acquisition and the vocabulary spurt: a response to Goldfield & Reznick.

Vocabulary development of three children (aged 1;6-1;8 at the start of the study) who had not begun to evidence a vocabulary spurt even though their productive vocabularies already included well over 50 words was followed to determine if these children eventually would have a vocabulary spurt. All three children evidenced a spurt, beginning at a mean productive vocabulary size of 112 words. The vocabulary spurt occurred at about the same time as the children first demonstrated the ability to sort objects exhaustively and to fast map new object names. Results are discussed in the context of Goldfield & Reznick's (1990; Reznick & Goldfield, 1992) argument that a substantial proportion of children never evidence a vocabulary spurt.

Child Language↗
PubMed1995

Acquisition of the novel name-nameless category (N3C) principle by young children who have Down syndrome.

Acquisition of the novel name-nameless category (N3C) principle by 22 children with Down syndrome between the ages of 2.42 and 3.33 years was examined to investigate the generalizability of a new approach to early lexical development: the developmental lexical principles framework. Results indicated that, as predicted, the N3C principle (operationally defined as the ability to fast map a new word to a [basic level] category), is not available at the start of lexical acquisition. The predicted link between ability to use the N3C principle and ability to perform exhaustive categorization of objects was supported. Children who used the principle had significantly larger productive vocabularies than did those who did not and, according to maternal report, had begun to acquire new words rapidly.

Child Language↗
PubMed1994

Early object labels: the case for a developmental lexical principles framework.

Universally, object names make up the largest proportion of any word type found in children's early lexicons. Here we present and critically evaluate a set of six lexical principles (some previously proposed and some new) for making object label learning a manageable task. Overall, the principles have the effect of reducing the amount of information that language-learning children must consider for what a new word might mean. These principles are constructed by children in a two-tiered developmental sequence, as a function of their sensitivity to linguistic input, contextual information, and social-interactional cues. Thus, the process of lexical acquisition changes as a result of the particular principles a given child has at his or her disposal. For children who have only the principles of the first tier (reference, extendibility, and object scope), word learning has a deliberate and laborious look. The principles of the second tier (categorical scope, novel name-nameless category' or N3C, and conventionality) enable the child to acquire many new labels rapidly. The present unified account is argued to have a number of advantages over treating such principles separately and non-developmentally. Further, the explicit recognition that the acquisition and operation of these principles is influenced by the child's interpretation of both linguistic and non-linguistic input is seen as an advance.

Child Language↗
PubMed1994

Acquisition of the novel name--nameless category (N3C) principle.

Toddlers' acquisition of the Novel Name-Nameless Category (N3C) principle was examined to investigate the developmental lexical principles framework and the applicability of the specificity hypothesis to relations involving lexical principles. In Study 1, we assessed the ability of 32 children between the ages of 16 and 20 months to use the N3C principle (operationally defined as the ability to fast map). As predicted, only some of the children could fast map. This finding provided evidence for a crucial tenet of the developmental lexical principles framework: Some lexical principles are not available at the start of language acquisition. Children who had acquired the N3C principle also had significantly larger vocabularies and were significantly more likely to demonstrate 2-category exhaustive sorting abilities than children who had not acquired the principle. The 2 groups of children did not differ in either age or object permanence abilities. The 16 children who could not fast map were followed longitudinally until they attained a vocabulary spurt; at that time, their ability to fast map was retested (Study 2). Results provided a longitudinal replication of the findings of Study 1. Implications of these findings for both the developmental lexical principles framework and the specificity hypothesis are discussed.

Child Development↗
PubMed1994

Two-year-olds readily learn multiple labels for the same basic-level category.

2 basic frameworks for lexical principles have been proposed (Golinkoff, Mervis, & Hirsh-Pasek; Markman). In many types of situations, these frameworks make the same predictions regarding 2-year-olds' interpretation of novel terms. However, the predictions diverge in some cases. 3 experiments were conducted to address 1 such situation: the case in which the child hears a novel term but already knows a label for each of the objects present. Results of all 3 studies converged on the same outcome: The new term was most likely to be treated as a second basic-level name for the category to which the object belonged. Analyses of individual patterns of responding revealed that, of the 58 subjects, 50 made more basic-level responses than part responses, 1 made equal numbers of basic-level and part responses, and 7 made more part responses than basic-level responses. Implications of these findings for models of early lexical development are discussed.

Age Factors↗