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Auteur Randi J. HAGERMAN |
Documents disponibles écrits par cet auteur (36)
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Sertraline May Improve Language Developmental Trajectory in Young Children with Fragile X Syndrome: A Retrospective Chart Review / Tri Indah WINARNI in Autism Research and Treatment, (March 2012)
[article]
Titre : Sertraline May Improve Language Developmental Trajectory in Young Children with Fragile X Syndrome: A Retrospective Chart Review Type de document : Texte imprimé et/ou numérique Auteurs : Tri Indah WINARNI, Auteur ; Weerasak CHONCHAIYA, Auteur ; Evan ADAMS, Auteur ; Jacky W. AU, Auteur ; Yi MU, Auteur ; Susan M. RIVERA, Auteur ; Danh V. NGUYEN, Auteur ; Randi J. HAGERMAN, Auteur Année de publication : 2012 Article en page(s) : 8 p. Langues : Anglais (eng) Index. décimale : PER Périodiques Résumé : Young children with fragile X syndrome (FXS) often experience anxiety, irritability, and hyperactivity related to sensory hyperarousal. However, there are no medication recommendations with documented efficacy for children under 5 years old of age with FXS. We examined data through a chart review for 45 children with FXS, 12–50 months old, using the Mullen Scales of Early Learning (MSEL) for baseline and longitudinal assessments. All children had clinical level of anxiety, language delays based on MSEL scores, and similar early learning composite (ELC) scores at their first visit to our clinic. Incidence of autism spectrum disorder (ASD) was similar in both groups. There were 11 children who were treated with sertraline, and these patients were retrospectively compared to 34 children who were not treated with sertraline by chart review. The baseline assessments were done at ages ranging from 18 to 44 months (mean 26.9, SD 7.99) and from 12 to 50 months (mean 29.94, SD 8.64) for treated and not treated groups, respectively. Mean rate of improvement in both expressive and receptive language development was significantly higher in the group who was treated with sertraline ( < 0 . 0 0 0 1 and = 0 . 0 0 7 1 , resp.). This data supports the need for a controlled trial of sertraline treatment in young children with FXS. En ligne : http://dx.doi.org/10.1155/2012/104317 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=178
in Autism Research and Treatment > (March 2012) . - 8 p.[article] Sertraline May Improve Language Developmental Trajectory in Young Children with Fragile X Syndrome: A Retrospective Chart Review [Texte imprimé et/ou numérique] / Tri Indah WINARNI, Auteur ; Weerasak CHONCHAIYA, Auteur ; Evan ADAMS, Auteur ; Jacky W. AU, Auteur ; Yi MU, Auteur ; Susan M. RIVERA, Auteur ; Danh V. NGUYEN, Auteur ; Randi J. HAGERMAN, Auteur . - 2012 . - 8 p.
Langues : Anglais (eng)
in Autism Research and Treatment > (March 2012) . - 8 p.
Index. décimale : PER Périodiques Résumé : Young children with fragile X syndrome (FXS) often experience anxiety, irritability, and hyperactivity related to sensory hyperarousal. However, there are no medication recommendations with documented efficacy for children under 5 years old of age with FXS. We examined data through a chart review for 45 children with FXS, 12–50 months old, using the Mullen Scales of Early Learning (MSEL) for baseline and longitudinal assessments. All children had clinical level of anxiety, language delays based on MSEL scores, and similar early learning composite (ELC) scores at their first visit to our clinic. Incidence of autism spectrum disorder (ASD) was similar in both groups. There were 11 children who were treated with sertraline, and these patients were retrospectively compared to 34 children who were not treated with sertraline by chart review. The baseline assessments were done at ages ranging from 18 to 44 months (mean 26.9, SD 7.99) and from 12 to 50 months (mean 29.94, SD 8.64) for treated and not treated groups, respectively. Mean rate of improvement in both expressive and receptive language development was significantly higher in the group who was treated with sertraline ( < 0 . 0 0 0 1 and = 0 . 0 0 7 1 , resp.). This data supports the need for a controlled trial of sertraline treatment in young children with FXS. En ligne : http://dx.doi.org/10.1155/2012/104317 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=178 A solution to limitations of cognitive testing in children with intellectual disabilities: the case of fragile X syndrome / D. HESSL in Journal of Neurodevelopmental Disorders, 1-1 (March 2009)
[article]
Titre : A solution to limitations of cognitive testing in children with intellectual disabilities: the case of fragile X syndrome Type de document : Texte imprimé et/ou numérique Auteurs : D. HESSL, Auteur ; D. V. NGUYEN, Auteur ; C. GREEN, Auteur ; Alyssa D. CHAVEZ, Auteur ; F. TASSONE, Auteur ; Randi J. HAGERMAN, Auteur ; D. SENTURK, Auteur ; A. SCHNEIDER, Auteur ; A. LIGHTBODY, Auteur ; A. L. REISS, Auteur ; S. HALL, Auteur Article en page(s) : p.33-45 Langues : Anglais (eng) Mots-clés : Assessment FMR1 gene Fmrp Iq Mental retardation Index. décimale : PER Périodiques Résumé : Intelligence testing in children with intellectual disabilities (ID) has significant limitations. The normative samples of widely used intelligence tests, such as the Wechsler Intelligence Scales, rarely include an adequate number of subjects with ID needed to provide sensitive measurement in the very low ability range, and they are highly subject to floor effects. The IQ measurement problems in these children prevent characterization of strengths and weaknesses, poorer estimates of cognitive abilities in research applications, and in clinical settings, limited utility for assessment, prognosis estimation, and planning intervention. Here, we examined the sensitivity of the Wechsler Intelligence Scale for Children (WISC-III) in a large sample of children with fragile X syndrome (FXS), the most common cause of inherited ID. The WISC-III was administered to 217 children with FXS (age 6-17 years, 83 girls and 134 boys). Using raw norms data obtained with permission from the Psychological Corporation, we calculated normalized scores representing each participant's actual deviation from the standardization sample using a z-score transformation. To validate this approach, we compared correlations between the new normalized scores versus the usual standard scores with a measure of adaptive behavior (Vineland Adaptive Behavior Scales) and with a genetic measure specific to FXS (FMR1 protein or FMRP). The distribution of WISC-III standard scores showed significant skewing with floor effects in a high proportion of participants, especially males (64.9%-94.0% across subtests). With the z-score normalization, the flooring problems were eliminated and scores were normally distributed. Furthermore, we found correlations between cognitive performance and adaptive behavior, and between cognition and FMRP that were very much improved when using these normalized scores in contrast to the usual standardized scores. The results of this study show that meaningful variation in intellectual ability in children with FXS, and probably other populations of children with neurodevelopmental disorders, is obscured by the usual translation of raw scores into standardized scores. A method of raw score transformation may improve the characterization of cognitive functioning in ID populations, especially for research applications. En ligne : http://dx.doi.org/10.1007/s11689-008-9001-8 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=341
in Journal of Neurodevelopmental Disorders > 1-1 (March 2009) . - p.33-45[article] A solution to limitations of cognitive testing in children with intellectual disabilities: the case of fragile X syndrome [Texte imprimé et/ou numérique] / D. HESSL, Auteur ; D. V. NGUYEN, Auteur ; C. GREEN, Auteur ; Alyssa D. CHAVEZ, Auteur ; F. TASSONE, Auteur ; Randi J. HAGERMAN, Auteur ; D. SENTURK, Auteur ; A. SCHNEIDER, Auteur ; A. LIGHTBODY, Auteur ; A. L. REISS, Auteur ; S. HALL, Auteur . - p.33-45.
Langues : Anglais (eng)
in Journal of Neurodevelopmental Disorders > 1-1 (March 2009) . - p.33-45
Mots-clés : Assessment FMR1 gene Fmrp Iq Mental retardation Index. décimale : PER Périodiques Résumé : Intelligence testing in children with intellectual disabilities (ID) has significant limitations. The normative samples of widely used intelligence tests, such as the Wechsler Intelligence Scales, rarely include an adequate number of subjects with ID needed to provide sensitive measurement in the very low ability range, and they are highly subject to floor effects. The IQ measurement problems in these children prevent characterization of strengths and weaknesses, poorer estimates of cognitive abilities in research applications, and in clinical settings, limited utility for assessment, prognosis estimation, and planning intervention. Here, we examined the sensitivity of the Wechsler Intelligence Scale for Children (WISC-III) in a large sample of children with fragile X syndrome (FXS), the most common cause of inherited ID. The WISC-III was administered to 217 children with FXS (age 6-17 years, 83 girls and 134 boys). Using raw norms data obtained with permission from the Psychological Corporation, we calculated normalized scores representing each participant's actual deviation from the standardization sample using a z-score transformation. To validate this approach, we compared correlations between the new normalized scores versus the usual standard scores with a measure of adaptive behavior (Vineland Adaptive Behavior Scales) and with a genetic measure specific to FXS (FMR1 protein or FMRP). The distribution of WISC-III standard scores showed significant skewing with floor effects in a high proportion of participants, especially males (64.9%-94.0% across subtests). With the z-score normalization, the flooring problems were eliminated and scores were normally distributed. Furthermore, we found correlations between cognitive performance and adaptive behavior, and between cognition and FMRP that were very much improved when using these normalized scores in contrast to the usual standardized scores. The results of this study show that meaningful variation in intellectual ability in children with FXS, and probably other populations of children with neurodevelopmental disorders, is obscured by the usual translation of raw scores into standardized scores. A method of raw score transformation may improve the characterization of cognitive functioning in ID populations, especially for research applications. En ligne : http://dx.doi.org/10.1007/s11689-008-9001-8 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=341 Symptoms of Autism in Males with Fragile X Syndrome: A Comparison to Nonsyndromic ASD Using Current ADI-R Scores / Andrea MCDUFFIE in Journal of Autism and Developmental Disorders, 45-7 (July 2015)
[article]
Titre : Symptoms of Autism in Males with Fragile X Syndrome: A Comparison to Nonsyndromic ASD Using Current ADI-R Scores Type de document : Texte imprimé et/ou numérique Auteurs : Andrea MCDUFFIE, Auteur ; Angela John THURMAN, Auteur ; Randi J. HAGERMAN, Auteur ; Leonard ABBEDUTO, Auteur Année de publication : 2015 Article en page(s) : p.1925-1937 Langues : Anglais (eng) Mots-clés : Autism spectrum disorder Fragile X syndrome ADI-R Index. décimale : PER Périodiques Résumé : Symptoms of autism are frequent in males with fragile X syndrome (FXS), but it is not clear whether symptom profiles differ from those of nonsyndromic ASD. Using individual item scores from the Autism Diagnostic Inventory-Revised, we examined which current symptoms of autism differed in boys with FXS relative to same-aged boys diagnosed with nonsyndromic ASD. In addition, different subsamples of participants were matched on autism diagnostic status and severity of autism symptoms. Between-group comparisons revealed that boys with FXS showed significantly less impairment in Social Smiling than did age-, diagnostic-, and severity-matched boys with nonsyndromic ASD. Severity-matched boys with FXS showed more impairment in complex mannerisms than did boys with nonsyndromic ASD. Behavioral differences between FXS and nonsyndromic ASD may be of theoretical importance in understanding the causes and correlates of ASD in FXS and in developing and implementing appropriate treatments. En ligne : http://dx.doi.org/10.1007/s10803-013-2013-6 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=261
in Journal of Autism and Developmental Disorders > 45-7 (July 2015) . - p.1925-1937[article] Symptoms of Autism in Males with Fragile X Syndrome: A Comparison to Nonsyndromic ASD Using Current ADI-R Scores [Texte imprimé et/ou numérique] / Andrea MCDUFFIE, Auteur ; Angela John THURMAN, Auteur ; Randi J. HAGERMAN, Auteur ; Leonard ABBEDUTO, Auteur . - 2015 . - p.1925-1937.
Langues : Anglais (eng)
in Journal of Autism and Developmental Disorders > 45-7 (July 2015) . - p.1925-1937
Mots-clés : Autism spectrum disorder Fragile X syndrome ADI-R Index. décimale : PER Périodiques Résumé : Symptoms of autism are frequent in males with fragile X syndrome (FXS), but it is not clear whether symptom profiles differ from those of nonsyndromic ASD. Using individual item scores from the Autism Diagnostic Inventory-Revised, we examined which current symptoms of autism differed in boys with FXS relative to same-aged boys diagnosed with nonsyndromic ASD. In addition, different subsamples of participants were matched on autism diagnostic status and severity of autism symptoms. Between-group comparisons revealed that boys with FXS showed significantly less impairment in Social Smiling than did age-, diagnostic-, and severity-matched boys with nonsyndromic ASD. Severity-matched boys with FXS showed more impairment in complex mannerisms than did boys with nonsyndromic ASD. Behavioral differences between FXS and nonsyndromic ASD may be of theoretical importance in understanding the causes and correlates of ASD in FXS and in developing and implementing appropriate treatments. En ligne : http://dx.doi.org/10.1007/s10803-013-2013-6 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=261 Targeted treatments in autism and fragile X syndrome / Kağan GURKAN C. in Research in Autism Spectrum Disorders, 6-4 (October-December 2012)
[article]
Titre : Targeted treatments in autism and fragile X syndrome Type de document : Texte imprimé et/ou numérique Auteurs : Kağan GURKAN C., Auteur ; Randi J. HAGERMAN, Auteur Année de publication : 2012 Article en page(s) : p.1311-1320 Langues : Anglais (eng) Mots-clés : Fragile X syndrome Autism mGluR GABA Treatment Index. décimale : PER Périodiques Résumé : Autism is a neurodevelopmental disorder consisting of a constellation of symptoms that sometimes occur as part of a complex disorder characterized by impairments in social interaction, communication and behavioral domains. It is a highly disabling disorder and there is a need for treatment targeting the core symptoms. Although autism is accepted as highly heritable, there is no genetic cure at this time. Autism is shown to be linked to several genes and is a feature of some complex genetic disorders, including fragile X syndrome (FXS), fragile X premutation involvement, tuberous sclerosis and Rett syndrome. The term autism spectrum disorders (ASDs) covers autism, Asperger syndrome and pervasive developmental disorders (PDD-NOS) and the etiologies are heterogeneous. In recent years, targeted treatments have been developed for several disorders that have a known specific genetic cause leading to autism. Since there are significant molecular and neurobiological overlaps among disorders, targeted treatments developed for a specific disorder may be helpful in ASD of unknown etiology. Examples of this are two drug classes developed to treat FXS, Arbaclofen, a GABAB agonist, and mGluR5 antagonists, and both may be helpful in autism without FXS. The mGluR5 antagonists are also likely to have a benefit in the aging problems of fragile X premutation carriers, the fragile X-associated tremor ataxia syndrome (FXTAS) and the Parkinsonism that can occur in aging patients with fragile X syndrome. Targeted treatments in FXS which has a well known genetic etiology may lead to new targeted treatments in autism. En ligne : http://dx.doi.org/10.1016/j.rasd.2012.05.007 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=165
in Research in Autism Spectrum Disorders > 6-4 (October-December 2012) . - p.1311-1320[article] Targeted treatments in autism and fragile X syndrome [Texte imprimé et/ou numérique] / Kağan GURKAN C., Auteur ; Randi J. HAGERMAN, Auteur . - 2012 . - p.1311-1320.
Langues : Anglais (eng)
in Research in Autism Spectrum Disorders > 6-4 (October-December 2012) . - p.1311-1320
Mots-clés : Fragile X syndrome Autism mGluR GABA Treatment Index. décimale : PER Périodiques Résumé : Autism is a neurodevelopmental disorder consisting of a constellation of symptoms that sometimes occur as part of a complex disorder characterized by impairments in social interaction, communication and behavioral domains. It is a highly disabling disorder and there is a need for treatment targeting the core symptoms. Although autism is accepted as highly heritable, there is no genetic cure at this time. Autism is shown to be linked to several genes and is a feature of some complex genetic disorders, including fragile X syndrome (FXS), fragile X premutation involvement, tuberous sclerosis and Rett syndrome. The term autism spectrum disorders (ASDs) covers autism, Asperger syndrome and pervasive developmental disorders (PDD-NOS) and the etiologies are heterogeneous. In recent years, targeted treatments have been developed for several disorders that have a known specific genetic cause leading to autism. Since there are significant molecular and neurobiological overlaps among disorders, targeted treatments developed for a specific disorder may be helpful in ASD of unknown etiology. Examples of this are two drug classes developed to treat FXS, Arbaclofen, a GABAB agonist, and mGluR5 antagonists, and both may be helpful in autism without FXS. The mGluR5 antagonists are also likely to have a benefit in the aging problems of fragile X premutation carriers, the fragile X-associated tremor ataxia syndrome (FXTAS) and the Parkinsonism that can occur in aging patients with fragile X syndrome. Targeted treatments in FXS which has a well known genetic etiology may lead to new targeted treatments in autism. En ligne : http://dx.doi.org/10.1016/j.rasd.2012.05.007 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=165 The Fragile X Syndrome / Edward GOLDSON in Developmental Medicine & Child Neurology, 34-9 (September 1992)
[article]
Titre : The Fragile X Syndrome Type de document : Texte imprimé et/ou numérique Auteurs : Edward GOLDSON, Auteur ; Randi J. HAGERMAN, Auteur Année de publication : 1992 Article en page(s) : p.826-832 Langues : Anglais (eng) Index. décimale : PER Périodiques Résumé : We have begun to appreciate that the extent of this disorder is much wider than merely mental retardation. It is also a common cause of learning and emotional problems in mildly affected female carriers with normal IQS. These children present an enormous challenge to all child-care providers, be they in medicine, education, or in various therapy disciplines. Early identification is essential, and the key to effective management is an innovative and multidisciplinary approach. As disciplines become more familiar with and knowledgeable about this frequently occurring form of mental retardation or cause of learning disabilities, they should become more adept at early identification and early referral for support and intervention. Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=138
in Developmental Medicine & Child Neurology > 34-9 (September 1992) . - p.826-832[article] The Fragile X Syndrome [Texte imprimé et/ou numérique] / Edward GOLDSON, Auteur ; Randi J. HAGERMAN, Auteur . - 1992 . - p.826-832.
Langues : Anglais (eng)
in Developmental Medicine & Child Neurology > 34-9 (September 1992) . - p.826-832
Index. décimale : PER Périodiques Résumé : We have begun to appreciate that the extent of this disorder is much wider than merely mental retardation. It is also a common cause of learning and emotional problems in mildly affected female carriers with normal IQS. These children present an enormous challenge to all child-care providers, be they in medicine, education, or in various therapy disciplines. Early identification is essential, and the key to effective management is an innovative and multidisciplinary approach. As disciplines become more familiar with and knowledgeable about this frequently occurring form of mental retardation or cause of learning disabilities, they should become more adept at early identification and early referral for support and intervention. Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=138 Use of Emotional Cues for Lexical Learning: A Comparison of Autism Spectrum Disorder and Fragile X Syndrome / Angela John THURMAN in Journal of Autism and Developmental Disorders, 45-4 (April 2015)
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