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Laurie E Cutting - One of the best experts on this subject based on the ideXlab platform.
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Cognitive Profile of Children with Neurofibromatosis and Reading Disabilities
Child Neuropsychology, 2010Co-Authors: Laurie E Cutting, Terry M LevineAbstract:A large percentage of children with Neurofibromatosis Type 1(NF-1) have learning disabilities, often in the realm of reading. Previous studies have indicated that children with NF-1 show a neuropsychological Profile similar to idiopathic reading disabilities (IRD); however, studies typically have not subdivided children with NF-1 into those who do and do not have RD (NF+RD and NFnoRD, respectively). The current study examined the Cognitive Profile of children with NF-1 with and without RD and compared them to children with IRD as well as to typically developing readers (Controls). Findings showed that children with NF+RD performed similarly to children with IRD on phonological, rapid naming, and reading comprehension measures; however, children with NF+RD displayed pronounced visual-spatial deficits as compared to IRD and Control groups. In addition, when comparing the NF-1 groups to each other as well as to the control and IRD groups, the current study reported that there were no oral language difference...
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Cognitive Profile of neurofibromatosis type 1
Seminars in Pediatric Neurology, 2006Co-Authors: Terry M Levine, April Materek, Jessica Abel, Madeline Odonnell, Laurie E CuttingAbstract:General consensus has yet to be reached with regard to the exact Cognitive Profile of children with neurofibromatosis type 1 (NF1). The current overview seeks to provide a more comprehensive review of the literature by examining studies that have specifically compared individuals with NF1 to siblings, controls, and/or norms. We also examined results of studies that compared individuals with NF1 with various manifestations to each other. Consistent with previous reviews, no clear Cognitive Profile emerged; however, greater insight into patterns was obtained. Additionally, future directions for research on NF1 were suggested.
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Cognitive Profile of Neurofibromatosis Type 1: Rethinking Nonverbal Learning Disabilities
Learning Disabilities Research and Practice, 2004Co-Authors: Laurie E Cutting, Amy M. Clements, Andrea D. Lightman, Pamula D. Yerby-hammack, Martha B. DencklaAbstract:The Cognitive Profiles of children with Neurofibromatosis Type 1 (NF-1) have many similarities to those observed in learning disabilities in the general school population, as well as some distinct features. Approximately 30–65 percent of children with NF-1 have learning disabilities; most commonly, they have language and reading disabilities, along with motor, visuomotor, and visuospatial deficits. Similar to learning disabilities in the general population, approximately 50 percent of children with NF-1 have Attention Deficit Hyperactivity Disorder (ADHD). In this article, the genetic and physical characteristics, the diagnostic criteria of NF-1, and the Cognitive Profile of children with NF-1 (including relationships between neurological abnormalities and cognition) are reviewed. Additionally, the types of educational interventions that would be indicated for children with NF-1 are discussed.
Joost Raaphorst - One of the best experts on this subject based on the ideXlab platform.
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the Cognitive Profile of behavioural variant ftd and its similarities with als a systematic review and meta analysis
Journal of Neurology Neurosurgery and Psychiatry, 2018Co-Authors: Emma Beeldman, Joost Raaphorst, Michelle Klein Twennaar, Rosanne Govaarts, Yolande A L Pijnenburg, Rob J De Haan, Marianne De Visser, Ben SchmandAbstract:Approximately 30% of patients with amyotrophic lateral sclerosis (ALS) have Cognitive impairment and 8%–14% fulfil the criteria for behavioural variant frontotemporal dementia (bv-FTD). The Cognitive Profiles of ALS and bv-FTD have been reported to be comparable, but this has never been systematically investigated. We aimed to determine the Cognitive Profile of bv-FTD and examine its similarities with that of ALS, to provide evidence for the existence of a Cognitive disease continuum encompassing bv-FTD and ALS. We therefore systematically reviewed neuropsychological studies on bv-FTD patients and healthy volunteers. Neuropsychological tests were divided in 10 Cognitive domains and effect sizes were calculated for all domains and compared with the Cognitive Profile of ALS by means of a visual comparison and a Pearson’s r correlation coefficient. We included 120 studies, totalling 2425 bv-FTD patients and 2798 healthy controls. All Cognitive domains showed substantial effect sizes, indicating Cognitive impairment in bv-FTD patients compared to healthy controls. The Cognitive domains with the largest effect sizes were social cognition, verbal memory and fluency (1.77–1.53). The Cognitive Profiles of bv-FTD and ALS (10 Cognitive domains, 1287 patients) showed similarities on visual comparison and a moderate correlation 0.58 (p=0.13). When social cognition, verbal memory, fluency, executive functions, language and visuoperception were considered, i.e. the Cognitive Profile of ALS, Pearson’s r was 0.73 (p=0.09), which raised to 0.92 (p=0.03), when language was excluded in this systematic analysis of patients with a non-language subtype of FTD. The Cognitive Profile of bv-FTD consists of deficits in social cognition, verbal memory, fluency and executive functions and shows similarities with the Cognitive Profile of ALS. These findings support a Cognitive continuum encompassing ALS and bv-FTD.
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the Cognitive Profile of myotonic dystrophy type 1 a systematic review and meta analysis
Cortex, 2017Co-Authors: Kees Okkersen, Roy P. C. Kessels, Melanie Buskes, J M M Groenewoud, Hans Knoop, Baziel G M Van Engelen, Joost RaaphorstAbstract:Abstract Objective To examine the Cognitive Profile of patients with myotonic dystrophy type 1 (DM1) on the basis of a systematic review and meta-analysis of the literature. Methods Embase, Medline and PsycInfo were searched for studies reporting ≥1 neuropsychological test in both DM1 patients and healthy controls. Search, data extraction and risk of bias analysis were independently performed by two authors to minimize error. Neuropsychological tests were categorized into 12 Cognitive domains and effect sizes (Hedges' g) were calculated for each domain and for tests administered in ≥5 studies. Results DM1 participants demonstrated a significantly worse performance compared to controls in all Cognitive domains. Effect sizes ranged from −.33 (small) for verbal memory to −1.01 (large) for visuospatial perception. Except for the domains global cognition, intelligence and social cognition, wide confidence intervals (CIs) were associated with moderate to marked statistical heterogeneity that necessitates careful interpretation of results. Out of the individual tests, the Rey–Osterrieth complex figure-copy (both non-verbal memory and visuoconstruction) showed consistent impairment with acceptable heterogeneity. Conclusion In DM1 patients, Cognitive deficits may include a variable combination of global Cognitive impairment with involvement across different domains, including social cognition, memory and visuospatial functioning. Although DM1 is a heterogeneous disorder, our study shows that meta-analysis is feasible, contributes to the understanding of brain involvement and may direct bedside testing. The protocol for this study has been registered in PROSPERO (International prospective register of systematic reviews) under ID: 42016037415.
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the Cognitive Profile of als a systematic review and meta analysis update
Journal of Neurology Neurosurgery and Psychiatry, 2016Co-Authors: Emma Beeldman, Joost Raaphorst, Michelle Klein Twennaar, Marianne De Visser, Ben Schmand, Rob J De HaanAbstract:Cognitive impairment is present in approximately 30% of patients with amyotrophic lateral sclerosis (ALS) and, especially when severe, has a negative impact on survival and caregiver burden. Our 2010 meta-analysis of the Cognitive Profile of ALS showed impairment of fluency, executive function, language and memory. However, the limited number of studies resulted in large confidence intervals. To obtain a more valid assessment, we updated the meta-analysis and included methodological improvements (controlled data extraction, risk of bias analysis and effect size calculation of individual neuropsychological tests). Embase, Medline and PsycInfo were searched for neuropsychological studies of non-demented patients with ALS and age-matched and education-matched healthy controls. Neuropsychological tests were categorised in 13 Cognitive domains and effect sizes (Hedges' g) were calculated for each domain and for individual tests administered in ≥5 studies. Subgroup analyses were performed to assess the influence of clinical and demographic variables. Forty-four studies were included comprising 1287 patients and 1130 healthy controls. All Cognitive domains, except visuoperceptive functions, showed significant effect sizes compared to controls. Cognitive domains without bias due to motor impairment showed medium effect sizes (95% CI): fluency (0.56 (0.43 to 0.70)), language (0.56 (0.40 to 0.72)), social cognition (0.55 (0.34 to 0.76)), or small effect sizes: delayed verbal memory 0.47 (0.27 to 0.68)) and executive functions (0.41 (0.27 to 0.55)). Individual neuropsychological tests showed diverging effect sizes, which could be explained by bias due to motor impairment. Subgroup analyses showed no influence of bulbar disease onset and depression and anxiety on the Cognitive outcomes. The Cognitive Profile of ALS consists of deficits in fluency, language, social cognition, executive functions and verbal memory. Social cognition is a new Cognitive domain with a relatively large effect size, highlighting the overlap between ALS and frontotemporal dementia. The diverging effect sizes for individual neuropsychological tests show the importance of correction for motor impairment in patients with ALS. These findings have implications for bedside testing, the design of Cognitive screening measures and full neuropsychological examinations.
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the Cognitive Profile of amyotrophic lateral sclerosis a meta analysis
Amyotrophic Lateral Sclerosis, 2009Co-Authors: Joost Raaphorst, Rob J De Haan, Marianne De Visser, W H J P Linssen, Ben SchmandAbstract:We aimed to clarify the Profile of Cognitive impairment in ALS, by meta-analysis of published studies. Criteria for inclusion were: ALS diagnosed according to El Escorial criteria; control group matched for age and education; correction for bias due to motor impairment or dysarthria; no dementia in patients and controls. Effect sizes reflecting a difference in neuropsychological performance between ALS patients and controls were calculated for 12 Cognitive domains. The effect of demographic and clinical variables (age, disease duration, site of onset) on cognition was assessed in a moderator analysis. Of 48 eligible articles, 16 studies encompassing 554 ALS patients were included. Significant effect sizes were found for the Mini Mental State Examination (d=0.8), immediate verbal memory (d=0.5), visual memory (d=0.4), fluency (d=0.5), psychomotor speed (d=0.7), language (d=0.5) and executive functioning (d=0.3). The results of the latter three domains are less reliable due to the possibility of publication bias. Psychomotor speed, and to a lesser extent fluency, may have been influenced by motor impairment, despite attempts to correct for motor slowness. In conclusion, the diversity of Cognitive problems in ALS seems greater than was previously thought. ALS patients may suffer from Cognitive impairment in multiple domains, including memory dysfunction.
Allan L Reiss - One of the best experts on this subject based on the ideXlab platform.
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the Cognitive developmental Profile associated with fragile x syndrome a longitudinal investigation of Cognitive strengths and weaknesses through childhood and adolescence
Development and Psychopathology, 2016Co-Authors: Evemarie Quintin, Booil Jo, Scott S Hall, Jennifer L Bruno, Lindsay C Chromik, Mira Raman, Amy A Lightbody, Arianna Martin, Allan L ReissAbstract:Few studies have investigated developmental strengths and weaknesses within the Cognitive Profile of children and adolescents with fragile X syndrome (FXS), a single-gene cause of inherited intellectual impairment. With a prospective longitudinal design and using normalized raw scores ( Z scores) to circumvent floor effects, we measured Cognitive functioning of 184 children and adolescents with FXS (ages 6 to 16) using the Wechsler Scale of Intelligence for Children on one to three occasions for each participant. Participants with FXS received lower raw scores relative to the Wechsler Scale of Intelligence for Children normative sample across the developmental period. Verbal comprehension, perceptual organization, and processing speed Z scores were marked by a widening gap from the normative sample, while freedom from distractibility Z scores showed a narrowing gap. Key findings include a relative strength for verbal skills in comparison with visuospatial–constructive skills arising in adolescence and a discrepancy between working memory (weakness) and processing speed (strength) in childhood that diminishes in adolescence. Results suggest that the Cognitive Profile associated with FXS develops dynamically from childhood to adolescence. Findings are discussed within the context of aberrant brain morphology in childhood and maturation in adolescence. We argue that assessing disorder-specific Cognitive developmental Profiles will benefit future disorder-specific treatment research.
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impact of Cognitive Profile on social functioning in prepubescent females with turner syndrome
Child Neuropsychology, 2013Co-Authors: Jeanfrancois Lepage, David S. Hong, Bria Dunkin, Allan L ReissAbstract:Social deficits are prevalent in Turner syndrome (TS); however, the extent to which these difficulties are secondary to characteristic Cognitive impairments is not well known. Here, we sought to establish the relative contribution of executive functions, visuospatial abilities, and IQ to social difficulties in young girls with TS. Forty TS girls and 19 typically developing (TD) children were assessed with the Social Responsiveness Scale (SRS), the Motor-Free Visual Spatial Test (MVPT-3), the Behavior Rating Inventory of Executive Function (BRIEF), and an IQ test. Hierarchical multiple regression analyses were conducted with the SRS subscales as outcome variables. In a first step, the Cognitive factors were entered (verbal IQ, BRIEF global score, MVPT-3, and age), followed by the group variable in a second step. In comparison to TD, TS participants were significantly impaired on all main measures. All six regression models with the SRS subscales were significant and revealed that global executive functions...
Isabelle Soulieres - One of the best experts on this subject based on the ideXlab platform.
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assessing intelligence at autism diagnosis mission impossible testability and Cognitive Profile of autistic preschoolers
Journal of Autism and Developmental Disorders, 2019Co-Authors: Valerie Courchesne, Claudine Jacques, Dominique Girard, Isabelle SoulieresAbstract:Intelligence in minimally verbal children on the autism spectrum (AS) is at risk of being underestimated. The present study investigated testability and Cognitive Profile of preschool autistic children using conventional tools and strength-informed tools. Fifty-two AS children and fifty-four typical children matched on age (31–77 months) were assessed. Testability increased with age in both groups, was generally lower in AS children, but not related to their test performance. Typical children performed significantly better than AS children on conventional tools, but performance of both groups was similar on strength-informed tools. Differences of performance across tests were much greater in the AS group. These results emphasize the heterogenous, yet characteristic, Cognitive Profile in preschool children, and introduce the usefulness of flexible testing.
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discrepancy between wisc iii and wisc iv Cognitive Profile in autism spectrum what does it reveal about autistic cognition
PLOS ONE, 2015Co-Authors: Annemarie Nader, Patricia Jelenic, Isabelle SoulieresAbstract:The Cognitive Profile and measured intellectual level vary according to assessment tools in children on the autism spectrum, much more so than in typically developing children. The recent inclusion of intellectual functioning in the diagnostic process for autism spectrum disorders leads to the crucial question on how to assess intelligence in autism, especially as some tests and subtests seem more sensitive to certain neurodevelopmental conditions. Our first aim was to examine the Cognitive Profile on the current version of the most widely used test, the Wechsler Intelligence Scales for Children (WISC-IV), for a homogenous subgroup of children on the autism spectrum, i.e. corresponding to DSM-IV diagnosis of “autism”. The second aim was to compare Cognitive Profiles obtained on the third edition versus 4th edition of WISC, in order to verify whether the WISC-IV yields a more distinctive Cognitive Profile in autistic children. The third aim was to examine the impact of the WISC-IV on the Cognitive Profile of another subgroup, children with Asperger’s Syndrome. 51 autistic, 15 Asperger and 42 typically developing children completed the WISC-IV and were individually matched to children who completed the WISC-III. Divergent WISC-IV Profiles were observed despite no significant intelligence quotient difference between groups. Autistic children scored significantly higher on the Perceptual Reasoning Index than on the Verbal Comprehension Index, a discrepancy that nearly tripled in comparison to WISC-III results. Asperger children scored higher on the VCI than on other indexes, with the lowest score found on the Processing Speed Index. WISC-IV Cognitive Profiles were consistent with, but more pronounced than WISC-III Profiles. Cognitive Profiles are a valuable diagnostic tool for differential diagnosis, keeping in mind that children on the autism spectrum might be more sensitive to the choice of subtests used to assess intelligence.
Roy P. C. Kessels - One of the best experts on this subject based on the ideXlab platform.
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the Cognitive Profile of myotonic dystrophy type 1 a systematic review and meta analysis
Cortex, 2017Co-Authors: Kees Okkersen, Roy P. C. Kessels, Melanie Buskes, J M M Groenewoud, Hans Knoop, Baziel G M Van Engelen, Joost RaaphorstAbstract:Abstract Objective To examine the Cognitive Profile of patients with myotonic dystrophy type 1 (DM1) on the basis of a systematic review and meta-analysis of the literature. Methods Embase, Medline and PsycInfo were searched for studies reporting ≥1 neuropsychological test in both DM1 patients and healthy controls. Search, data extraction and risk of bias analysis were independently performed by two authors to minimize error. Neuropsychological tests were categorized into 12 Cognitive domains and effect sizes (Hedges' g) were calculated for each domain and for tests administered in ≥5 studies. Results DM1 participants demonstrated a significantly worse performance compared to controls in all Cognitive domains. Effect sizes ranged from −.33 (small) for verbal memory to −1.01 (large) for visuospatial perception. Except for the domains global cognition, intelligence and social cognition, wide confidence intervals (CIs) were associated with moderate to marked statistical heterogeneity that necessitates careful interpretation of results. Out of the individual tests, the Rey–Osterrieth complex figure-copy (both non-verbal memory and visuoconstruction) showed consistent impairment with acceptable heterogeneity. Conclusion In DM1 patients, Cognitive deficits may include a variable combination of global Cognitive impairment with involvement across different domains, including social cognition, memory and visuospatial functioning. Although DM1 is a heterogeneous disorder, our study shows that meta-analysis is feasible, contributes to the understanding of brain involvement and may direct bedside testing. The protocol for this study has been registered in PROSPERO (International prospective register of systematic reviews) under ID: 42016037415.
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a 4 year follow up study of Cognitive functioning in patients with type 2 diabetes mellitus
Diabetologia, 2010Co-Authors: E. Van Den Berg, Yael D Reijmer, L. Jaap Kappelle, J. H. F. M. Wijdenes-de Bresser, Roy P. C. Kessels, Geert Jan BiesselsAbstract:Aims/hypothesis Type 2 diabetes mellitus is associated with moderate decrements in Cognitive functioning, mainly in verbal memory, information-processing speed and executive functions. How this Cognitive Profile evolves over time is uncertain. The present study aims to provide detailed information on the evolution of Cognitive decrements in type 2 diabetes over time.
