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Hispanics are the fastest growing ethnicity in the United States, yet there are limited well-validated neuropsychological tools in Spanish, and an even greater paucity of normative standards representing this population. The Spanish NIH Toolbox Cognition Battery (NIHTB-CB) is a novel neurocognitive screener; however, the original norms were developed combining Spanish- and English-versions of the battery. We developed normative standards for the Spanish NIHTB-CB, fully adjusting for demographic variables and based entirely on a Spanish-speaking sample. A total of 408 Spanish-speaking neurologically healthy adults (ages 18–85 years) and 496 children (ages 3–7 years) completed the NIH Toolbox norming project. We developed three types of scores: uncorrected based on the entire Spanish-speaking cohort, age-corrected, and fully demographically corrected (age, education, sex) scores for each of the seven NIHTB-CB tests and three composites (Fluid, Crystallized, Total Composites). Corrected scores were developed using polynomial regression models. Demographic factors demonstrated medium-to-large effects on uncorrected NIHTB-CB scores in a pattern that differed from that observed on the English NIHTB-CB. For example, in Spanish-speaking adults, education was more strongly associated with Fluid scores, but showed the strongest association with Crystallized scores among English-speaking adults. Demographic factors were no longer associated with fully corrected scores. The original norms were not successful in eliminating demographic effects, overestimating children’s performances, and underestimating adults’ performances on the Spanish NIHTB-CB. The disparate pattern of demographic associations on the Spanish versus English NIHTB-CB supports the need for distinct normative standards developed separately for each population. Fully adjusted scores presented here will aid in more accurately characterizing acquired brain dysfunction among U.S. Spanish-speakers. (JINS, 2016, 21, 364–374)
Demographic factors impact neuropsychological test performances and accounting for them may help to better elucidate current brain functioning. The NIH Toolbox Cognition Battery (NIHTB-CB) is a novel neuropsychological tool, yet the original norms developed for the battery did not adequately account for important demographic/cultural factors known to impact test performances. We developed norms fully adjusting for all demographic variables within each language group (English and Spanish) separately. The current study describes the standards for individuals tested in English. Neurologically healthy adults (n=1038) and children (n=2917) who completed the NIH Toolbox norming project in English were included. We created uncorrected scores weighted to the 2010 Census demographics, and applied polynomial regression models to develop age-corrected and fully demographically adjusted (age, education, sex, race/ethnicity) scores for each NIHTB-CB test and composite (i.e., Fluid, Crystallized, and Total Composites). On uncorrected NIHTB-CB scores, age and education demonstrated significant, medium-to-large associations, while sex showed smaller, but statistically significant effects. In terms of race/ethnicity, a significant stair-step effect on uncorrected NIHTB-CB scores was observed (African American<Hispanic<White). After applying normative corrections, NIHTB-CB no longer demonstrated any significant associations with demographic factors. The previously developed norms still maintained significant associations with demographic factors, and demonstrated more variable impairment rates in segments of the healthy normative sample. Similar to other neuropsychological tests, demographic factors demonstrated significant associations with unadjusted NIHTB-CB scores. Application of fully corrected scores will help account for unwanted variance that is associated with non-clinical factors to more accurately reflect effects of disease-related changes in brain function. (JINS, 2015, 21, 378–391)
Biomedical measures are critical in the initial patient-screening and -selection phases of a clinical trial in autism and related disorders. These measures can also play an important role in the assessment and characterization of response and can provide an opportunity to study underlying etiologic and pathophysiologic processes. Thus, biomedical measures, including clinical laboratory analyses, metabolic screening, and chromosomal analysis, are used to screen for potential safety-related problems, to decrease biological and genetic heterogeneity, and to define subgroups. Neurobiological measures can be examined as possible predictors, modifiers or surrogates of therapeutic response, and adverse effects. Neurobiological research measures can also be used to study mechanisms and extent of drug action and to perform baseline and longitudinal investigations of possible pathophysiologic alterations. The potential utility and desirability of specific measures are considered and the general approach to choosing measures for incorporation is discussed.
This study describes psychometric properties of the NIH Toolbox Cognition Battery (NIHTB-CB) Composite Scores in an adult sample. The NIHTB-CB was designed for use in epidemiologic studies and clinical trials for ages 3 to 85. A total of 268 self-described healthy adults were recruited at four university-based sites, using stratified sampling guidelines to target demographic variability for age (20–85 years), gender, education, and ethnicity. The NIHTB-CB contains seven computer-based instruments assessing five cognitive sub-domains: Language, Executive Function, Episodic Memory, Processing Speed, and Working Memory. Participants completed the NIHTB-CB, corresponding gold standard validation measures selected to tap the same cognitive abilities, and sociodemographic questionnaires. Three Composite Scores were derived for both the NIHTB-CB and gold standard batteries: “Crystallized Cognition Composite,” “Fluid Cognition Composite,” and “Total Cognition Composite” scores. NIHTB Composite Scores showed acceptable internal consistency (Cronbach’s alphas=0.84 Crystallized, 0.83 Fluid, 0.77 Total), excellent test–retest reliability (r: 0.86–0.92), strong convergent (r: 0.78–0.90) and discriminant (r: 0.19–0.39) validities versus gold standard composites, and expected age effects (r=0.18 crystallized, r=−0.68 fluid, r=−0.26 total). Significant relationships with self-reported prior school difficulties and current health status, employment, and presence of a disability provided evidence of external validity. The NIH Toolbox Cognition Battery Composite Scores have excellent reliability and validity, suggesting they can be used effectively in epidemiologic and clinical studies. (JINS, 2014, 20, 1–11)
This study sought to determine what factors contribute to normal developmental changes in performance on the block design task. The target models were systematically varied to emphasize global, intermediate, and local pattern structures. One hundred children between 4.5 and 9 years of age were tested in the first experiment. Correct performance and error types differed significantly as a function of age and pattern type. Broken configuration errors were particularly common for the global patterns. In the second experiment, 48 children between 4.5 and 8 years of age were tested using designs with a superimposed grid (cued condition). Error rates were lower in the cued condition and broken configuration errors were less frequent. These results suggest that children have more difficulty parsing more cohesive patterns, but they can modify their strategies when the square matrix is provided by the pattern structure. (JINS, 1996, 2, 392–402.)
Autism was first described by Leo Kanner in 1943. He described 11 children with “extreme autistic aloneness” p. 242, failure to use language in a communicative fashion, and an obsessive desire for the maintenance of sameness. In the 60 years since this classic paper, there have been numerous studies on every aspect of autism, with the pace of research accelerating greatly in the last 15 years. Kanner's original description has held up remarkably well, and forms the basis for the three domains of diagnostic criteria found in DSM-IV-TR (American Psychiatric Association, 2000).
Impairment in social relationships, the first domain, has four behavioral markers: (a) impaired nonverbal communication, including eye contact and gesture, (b) poor peer relationships, including lack of interest in peers when young, and odd, one-sided relationships later on, (c) lack of joint attention (pointing to indicate interest, bringing items to show others, following a point), and (d) lack of emotional reciprocity, such as failure to notice or share another's distress.
The second domain is impairment in language and symbolic capacity, and includes: (a) language delay, (b) impaired ability to carry on a two-way conversation (when there is sufficient language), (c) perseverative and repetitive language, such as repeating what others say or what the child has heard in commercials or videos, or repeating favorite phrases over and over, and (d) absent, delayed, or repetitive pretend play.
Children with specific language impairment (LI) have deficits on some
nonverbal tasks, but it is not clear if these are related to specific
visuospatial deficits or to more general deficits in processing
strategies. Children with LI were given two visuospatial tasks that we
have shown to be sensitive to strategy use as well as specific processing
deficits. In Study 1, children with LI (N = 29, ages 6 to 12
years) performed significantly worse than typically developing children
(N = 26) on the Hierarchical Forms Memory task. In Study 2,
children with LI (N = 15; ages 9 to 12 years) performed
significantly worse than typically developing children (N = 40)
on the Rey-Osterrieth Complex Figure task. Children with LI were less
accurate and tended to use a fairly piecemeal (immature) strategy when
copying the figure and were less likely to draw the core rectangle in a
more integrated fashion during the immediate memory condition. These
results suggest children with LI have subtle deficits on visuospatial
tasks that may be more indicative of limitations associated with
processing load and planning than of specific visuospatial processing
deficits (JINS, 2006, 12, 465–474.)
The current study presents both longitudinal behavioral data
and functional activation data documenting the effects of early
focal brain injury on the development of spatial analytic
processing in two children, one with prenatal left hemisphere
(LH) injury and one with right hemisphere (RH) injury. A
substantial body of evidence has shown that adults and children
with early, lateralized brain injury show evidence of spatial
analytic deficits. LH injury compromises the ability to encode
the parts of a spatial pattern, while RH injury impairs pattern
integration. The two children described in this report show
patterns of deficit consistent with the site of their injury.
In the current study, their longitudinal behavioral data spanning
the age range from preschool to adolescence are presented in
conjunction with data from a functional magnetic resonance imaging
(fMRI) study of spatial processing. The activation results provide
evidence that alternative profiles of neural organization can
arise following early focal brain injury, and document
where in the brain spatial functions are carried out
when regions that normally mediate them are damaged. In addition,
the coupling of the activation with the behavioral data allows
us to go beyond the simple mapping of functional sites, to ask
questions about how those sites may have come to mediate the
spatial functions. (JINS, 2003, 9, 604–622.)
Autism is a neurobiological disorder that is diagnosed through careful behavioral assessment in early childhood. In this paper, we review recent studies that have attempted to reveal the underlying causes of autism using a variety of techniques. Particular emphasis is placed on techniques that have been used by a number of different laboratories, including structural magnetic resonance imaging and postmortem studies of neuroanatomy. Neurobiological and neuropsychological data from individuals across a wide age range are examined from a neurodevelopmental perspective. We discuss how these recent advances have led us to develop a growth dysregulation hypothesis of autism. Finally, we discuss how this hypothesis may lead to new innovations in autism research.
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