A Comparison of Behavioral and Emotional Characteristics in Children with Autism, Prader-Willi Syndrome, and Williams Syndrome

ERIC Educational Resources Information Center

Dimitropoulos, Anastasia; Ho, Alan Y.; Klaiman, Cheryl; Koenig, Kathy; Schultz, Robert T.

2009-01-01

In order to investigate unique and shared characteristics and to determine factors predictive of group classification, quantitative comparisons of behavioral and emotional problems were assessed using the Developmental Behavior Checklist (DBC-P) and the Vineland Adaptive Behavior Scales in autistic disorder, Williams syndrome (WS), and…

Evaluation of a Behavioral Treatment Package to Reduce Sleep Problems in Children with Angelman Syndrome

ERIC Educational Resources Information Center

Allen, Keith D.; Kuhn, Brett R.; DeHaai, Kristi A.; Wallace, Dustin P.

2013-01-01

The purpose of this investigation was to evaluate the effectiveness of a behavioral treatment package to reduce chronic sleep problems in children with Angelman Syndrome. Participants were five children, 2-11 years-of-age. Parents maintained sleep diaries to record sleep and disruptive nighttime behaviors. Actigraphy was added to provide…

Asperger Syndrome and Difficult Moments: Practical Solutions for Tantrums, Rage, and Meltdowns.

ERIC Educational Resources Information Center

Myles, Brenda Smith; Southwick, Jack

This book discusses characteristics of individuals with Asperger syndrome that affect behavior, and presents strategies for preventing behavioral problems. Chapter 1, "An Overview of the Characteristics of Asperger Syndrome that May Impact Behavior," discusses specific characteristics that affect cognition, language, socialization, sensory…

Feeding, eating and behavioral disturbances in Prader-Willi syndrome and non-syndromal obesity.

PubMed

Sonnengrün, Lilli; Schober, Celestina; Vogel, Mandy; Hiemisch, Andreas; Döhnert, Mirko; Hilbert, Anja; Kiess, Wieland

2016-08-01

Although most individuals with Prader-Willi syndrome (PWS) are obese, little is known about the impact of obesity-related psychosocial factors in PWS. In the present study we compared feeding, eating, and behavioral disturbances in children and adolescents with PWS, peers with non-syndromal obesity, and normal weight controls. Twelve persons with PWS, aged 7-22 years, age- and gender-matched obese and normal weight individuals were analyzed regarding parental feeding practices, eating disturbances, and behavioral problems via standardized questionnaires. Parents of individuals with PWS reported significantly more restrictive feeding and monitoring than did parents of obese or normal weight children without PWS (p<0.05). Social problems were more common in the obese and the PWS group than in the normal-weight group (p<0.05). Behavioral problems were significantly correlated with parental restrictive feeding practices. Our data show that children and adolescents with PWS are affected by psychosocial problems, and that restrictive feeding practices might be associated with more severe behavioral problems. Further studies in larger samples will be necessary to replicate these results and possibly provide new therapeutic approaches for the management of PWS.

Maladaptive and Compulsive Behavior in Prader-Willi Syndrome: New Insights from Older Adults

ERIC Educational Resources Information Center

Dykens, Elisabeth M.

2004-01-01

Although maladaptive and compulsive behaviors are increasingly well-described in young persons with Prader-Willi syndrome, it is unclear how these problems manifest in older adults with this syndrome. In Part I, I compared maladaptive and compulsive behaviors in 45 older adults with Prader-Willi syndrome (ages 30 to 50 years) to 195 children,…

Emotional and Behavioral Problems among Impoverished Kenyan Youth: Factor Structure and Sex-Differences

PubMed Central

Harder, Valerie S.; Mutiso, Victoria N.; Khasakhala, Lincoln I.; Burke, Heather M.; Rettew, David C.; Ivanova, Masha Y.; Ndetei, David M.

2014-01-01

Data on youth emotional and behavioral problems from societies in Sub-Saharan Africa are lacking. This may be due to the fact that few youth mental health assessments have been tested for construct validity of syndrome structure across multicultural societies that include developing countries, and almost none have been tested in Sub-Saharan Africa. The Youth Self-Report (YSR), for example, has shown great consistency of its syndrome structure across many cultures, yet data from only one developing country in Sub-Saharan Africa have been included. In this study, we test the factor structure of YSR syndromes among Kenyan youth ages 11–18 years from an informal settlement in Nairobi, Kenya and examine sex-differences in levels of emotional and behavioral problems. We find the eight syndrome structure of the YSR to fit these data well (Root Mean Square Error of Approximation=.049). While Kenyan girls have significantly higher internalizing (Anxious/Depressed, Withdrawn/Depressed, Somatic) problem scores than boys, these differences are of similar magnitude to published multicultural findings. The results support the generalizability of the YSR syndrome structure to Kenyan youth and are in line with multicultural findings supporting the YSR as an assessment of emotional and behavioral problems in diverse societies. PMID:25419046

Patterns of Depressive Symptoms and Social Relating Behaviors Differ Over Time From Other Behavioral Domains for Young People With Down Syndrome

PubMed Central

Foley, Kitty-Rose; Bourke, Jenny; Einfeld, Stewart L.; Tonge, Bruce J.; Jacoby, Peter; Leonard, Helen

2015-01-01

Abstract People with intellectual disabilities are at a higher risk for experiencing behavioral, emotional, and psychiatric problems in comparison with the general population. People with Down syndrome have been reported as experiencing fewer behavioral problems than others with intellectual disability, although still at a greater level than the non-intellectually disabled population, except for depression and Alzheimer disease. The aim of this study was to describe the trajectories of subscales of behavior, including depressive symptoms, communication disturbance, anxiety, disruptiveness, and social relating abilities, for young adults with Down syndrome. Families of young adults with Down syndrome living in Perth, Western Australia, participated in a questionnaire study over 8 years, 2004 (n = 255), 2009 (n = 191), and 2011 (n = 188). Questionnaires collected information about young person characteristics and family functioning. The parent-completed Developmental Behavior Checklist-Adult (DBC-A) and Developmental Behavior Checklist-Primary Carer Version (DBC-P) were used to measure emotional and behavioral problems. These measures include the following subscales: disruptive, communication and anxiety disturbances, self-absorbed, antisocial, depressive, and social relating. DBC score declined from 2004 to 2011 reflecting an improvement in behavior in the self-absorbed (coeff −0.011, 95% confidence interval (CI) −0.031, −0.008), anxiety (coef −0.009 95%CI −0.129, −0.006), communication disturbances (coeff −0.008, 95% CI −0.012, −0.005) and disruptive/antisocial behavior (coeff −0.013, 95% CI −0.016, −0.009) subscales. Subscales for depressive symptoms and social relating problems decreased less (coeff −0.003, 95% CI −0.007, −0.0001) (coeff −0.003 95% CI −0.007, 0.001). Young people who were lower functioning were reported as exhibiting significantly more behavioral problems across every subscale when compared with those who were higher functioning. Behavior of young adults with Down syndrome improves over time but depressive symptoms and social relating behavior problems persist into adulthood. It is possible that those with persistent depressive symptoms are at a high risk for developing depressive illness in adulthood. Identifying young people with Down syndrome who are at risk for developing depression in adult life has implications for prevention and early treatment. PMID:25984682

A Comparison of Emotional-Behavioral Problems of Siblings at the Age Range of 3-9 Year Old Children with Autism and Down Syndrome.

PubMed

Pourbagheri, Nahid; Mirzakhani, Navid; Akbarzadehbaghban, Alireza

2018-01-01

Children's emotional-behavioral problems will have a huge impact on their future. Such problems are more seen in the siblings of children with special needs. The present study aimed to compare emotional-behavioral problems in the healthy siblings of autistic children with the healthy siblings of children with Down syndrome in order to identify such children in Iran. This descriptive study was carried out in Tehran, Iran in 2016 on 174 healthy children aged between 3 and 9 yr old among whom 58 cases had autistic siblings, 58 cases had siblings with Down syndrome, and 58 cases had typically development siblings. The participants were selected using convenience sampling technique. All volunteer parents filled in SDQ Questionnaire . The study results were calculated using independent sample t -test, two-way ANOVA, and Tukey post hoc test by SPSS. The mean overall score of Strengths and Difficulties Questionnaire was reported as 17.98±6.19 in the autism group, 11.01±6.56 in the Down syndrome group and 4.43±4.34 in the healthy group. There was a significant difference among autism, Down syndrome, and healthy groups. In the group of siblings with Down syndrome, the problems were significantly more in the age range of 3 to 7 yr old ( P <0.05). However, no significant difference was observed in the scores of males and females ( P >0.05). Siblings with autism or Down syndrome can have especial psychological effects on healthy children in families in the way that such effect will be more with autistic children. Therefore, formulating beneficial strategies for their parents is used to prevent emotional behavioral problems.

Adaptive Skills, Behavior Problems, and Parenting Stress in Mothers of Boys with Fragile X Syndrome

ERIC Educational Resources Information Center

Sarimski, Klaus

2010-01-01

The relationship of temperament, atypical behaviors, and adaptive behavior of young boys with Fragile X syndrome on mothers' parenting stress was analyzed. Twenty-six boys with Fragile X syndrome (30-88 months of age) participated. The overall development of the participants was significantly delayed with a specific profile of adaptive behaviors…

Preschool Psychopathology Reported by Parents in 23 Societies: Testing the Seven-Syndrome Model of the Child Behavior Checklist for Ages 1.5–5

PubMed Central

Ivanova, Masha Y.; Achenbach, Thomas M.; Rescorla, Leslie A.; Harder, Valerie S.; Ang, Rebecca P.; Bilenberg, Niels; Bjarnadottir, Gudrun; Capron, Christiane; De Pauw, Sarah S.W.; Dias, Pedro; Dobrean, Anca; Doepfner, Manfred; Duyme, Michele; Eapen, Valsamma; Erol, Nese; Esmaeili, Elaheh Mohammad; Ezpeleta, Lourdes; Frigerio, Alessandra; Gonçalves, Miguel M.; Gudmundsson, Halldor S.; Jeng, Suh-Fang; Jetishi, Pranvera; Jusiene, Roma; Kim, Young-Ah; Kristensen, Solvejg; Lecannelier, Felipe; Leung, Patrick W.L.; Liu, Jianghong; Montirosso, Rosario; Oh, Kyung Ja; Plueck, Julia; Pomalima, Rolando; Shahini, Mimoza; Silva, Jaime R.; Simsek, Zynep; Sourander, Andre; Valverde, Jose; Van Leeuwen, Karla G.; Woo, Bernardine S.C.; Wu, Yen-Tzu; Zubrick, Stephen R.; Verhulst, Frank C.

2014-01-01

Objective To test the fit of a seven-syndrome model to ratings of preschoolers' problems by parents in very diverse societies. Method Parents of 19,106 children 18 to 71 months of age from 23 societies in Asia, Australasia, Europe, the Middle East, and South America completed the Child Behavior Checklist for Ages 1.5–5 (CBCL/1.5–5). Confirmatory factor analyses were used to test the seven-syndrome model separately for each society. Results The primary model fit index, the root mean square error of approximation (RMSEA), indicated acceptable to good fit for each society. Although a six-syndrome model combining the Emotionally Reactive and Anxious/Depressed syndromes also fit the data for nine societies, it fit less well than the seven-syndrome model for seven of the nine societies. Other fit indices yielded less consistent results than the RMSEA. Conclusions The seven-syndrome model provides one way to capture patterns of children's problems that are manifested in ratings by parents from many societies. Clinicians working with preschoolers from these societies can thus assess and describe parents' ratings of behavioral, emotional, and social problems in terms of the seven syndromes. The results illustrate possibilities for culture–general taxonomic constructs of preschool psychopathology. Problems not captured by the CBCL/1.5–5 may form additional syndromes, and other syndrome models may also fit the data. PMID:21093771

Preschool psychopathology reported by parents in 23 societies: testing the seven-syndrome model of the child behavior checklist for ages 1.5-5.

PubMed

Ivanova, Masha Y; Achenbach, Thomas M; Rescorla, Leslie A; Harder, Valerie S; Ang, Rebecca P; Bilenberg, Niels; Bjarnadottir, Gudrun; Capron, Christiane; De Pauw, Sarah S W; Dias, Pedro; Dobrean, Anca; Doepfner, Manfred; Duyme, Michele; Eapen, Valsamma; Erol, Nese; Esmaeili, Elaheh Mohammad; Ezpeleta, Lourdes; Frigerio, Alessandra; Gonçalves, Miguel M; Gudmundsson, Halldor S; Jeng, Suh-Fang; Jetishi, Pranvera; Jusiene, Roma; Kim, Young-Ah; Kristensen, Solvejg; Lecannelier, Felipe; Leung, Patrick W L; Liu, Jianghong; Montirosso, Rosario; Oh, Kyung Ja; Plueck, Julia; Pomalima, Rolando; Shahini, Mimoza; Silva, Jaime R; Simsek, Zynep; Sourander, Andre; Valverde, Jose; Van Leeuwen, Karla G; Woo, Bernardine S C; Wu, Yen-Tzu; Zubrick, Stephen R; Verhulst, Frank C

2010-12-01

To test the fit of a seven-syndrome model to ratings of preschoolers' problems by parents in very diverse societies. Parents of 19,106 children 18 to 71 months of age from 23 societies in Asia, Australasia, Europe, the Middle East, and South America completed the Child Behavior Checklist for Ages 1.5-5 (CBCL/1.5-5). Confirmatory factor analyses were used to test the seven-syndrome model separately for each society. The primary model fit index, the root mean square error of approximation (RMSEA), indicated acceptable to good fit for each society. Although a six-syndrome model combining the Emotionally Reactive and Anxious/Depressed syndromes also fit the data for nine societies, it fit less well than the seven-syndrome model for seven of the nine societies. Other fit indices yielded less consistent results than the RMSEA. The seven-syndrome model provides one way to capture patterns of children's problems that are manifested in ratings by parents from many societies. Clinicians working with preschoolers from these societies can thus assess and describe parents' ratings of behavioral, emotional, and social problems in terms of the seven syndromes. The results illustrate possibilities for culture-general taxonomic constructs of preschool psychopathology. Problems not captured by the CBCL/1.5-5 may form additional syndromes, and other syndrome models may also fit the data. Copyright © 2010 American Academy of Child and Adolescent Psychiatry. Published by Elsevier Inc. All rights reserved.

Structural Equivalence of Involvement in Problem Behavior by Adolescents across Racial Groups Using Multiple Group Confirmatory Factor Analysis.

ERIC Educational Resources Information Center

Williams, James H.; And Others

1996-01-01

Problem behavior theory predicts that adolescent problem behaviors are manifestations of a single behavioral syndrome. This study tested the validity of the theory across racial groups. Results indicate that multiple pathways are necessary to account for the problem behaviors and they support previous research indicating system response bias in…

Psychopathology from adolescence into young adulthood: an 8-year follow-up study.

PubMed

Ferdinand, R F; Verhulst, F C

1995-11-01

This study investigated the stability of behavioral and emotional problems from adolescence into young adulthood. Subjects from the general population (N = 459), aged 13-16 years, were evaluated initially with the Child Behavior Checklist (completed by parents) and 8 years later with the Young Adult Self-Report. The scoring format and factor structure of the two assessment instruments are similar; syndromes constructed from the two instruments are based on parents', teachers', and self-report information derived from large clinical samples. Signs of maladjustment also were assessed at follow-up through interviews. Of the individuals with total problem scores in the deviant range on the Child Behavior Checklist, 27.3% had total problem scores in the deviant range on the Young Adult Self-Report at follow-up. The probability of having a total problem score in the deviant range at follow-up was raised 7.4-fold by having deviant-range scores on the Child Behavior Checklist somatic complaints and anxious/depressed syndromes (simultaneously) at the initial assessment. Referral to mental health services was predicted by deviant-range scores on the anxious/depressed syndrome, while suicide attempts were predicted by deviance on the withdrawn syndrome. Adolescent problems tended to persist into young adulthood to a moderate degree. High rates of withdrawal from social contacts, anxiety or depression, somatic complaints without known medical origin, social problems, attention problems, delinquent behavior, and aggressive behavior during adolescence were risk factors for specific types of psychopathology and maladjustment at 8-year follow-up. The presence of psychopathology in adolescence should not be regarded as normative.

Mental Health Problems in Adults with Williams Syndrome

ERIC Educational Resources Information Center

Stinton, Chris; Elison, Sarah; Howlin, Patricia

2010-01-01

Although many researchers have investigated emotional and behavioral difficulties in individuals with Williams syndrome, few have used standardized diagnostic assessments. We examined mental health problems in 92 adults with Williams syndrome using the Psychiatric Assessment Schedule for Adults with Developmental Disabilities--PAS-ADD (Moss,…

Behavior and adaptive functioning in adolescents with Down syndrome: specifying targets for intervention.

PubMed

Jacola, Lisa M; Hickey, Francis; Howe, Steven R; Esbensen, Anna; Shear, Paula K

2014-01-01

Research suggests that adolescents with Down syndrome experience increased behavior problems as compared to age matched peers; however, few studies have examined how these problems relate to adaptive functioning. The primary aim of this study was to characterize behavior in a sample of adolescents with Down syndrome using two widely-used caregiver reports: the Behavioral Assessment System for Children, 2 nd Edition (BASC-2) and Child Behavioral Checklist (CBCL). The clinical utility of the BASC-2 as a measure of behavior and adaptive functioning in adolescents with Down syndrome was also examined. Fifty-two adolescents with Down syndrome between the ages of 12 and 18 (24 males) completed the Peabody Picture Vocabulary Test, 4 th Edition (PPVT-IV) as an estimate of cognitive ability. Caregivers completed the BASC-2 and the CBCL for each participant. A significant proportion of the sample was reported to demonstrate behavior problems, particularly related to attention and social participation. The profile of adaptive function was variable, with caregivers most frequently rating impairment in skills related to activities of daily living and functional communication. Caregiver ratings did not differ by gender and were not related to age or estimated cognitive ability. Caregiver ratings of attention problems on the BASC-2 accounted for a significant proportion of variance in Activities of Daily Living ( Adj R 2 = 0.30) , Leadership ( Adj R 2 = 0.30) Functional Communication ( Adj R 2 = 0.28, Adaptability ( Adj R 2 = 0.29), and Social Skills ( Adj R 2 = 0.17). Higher frequencies of symptoms related to social withdrawal added incremental predictive validity for Functional Communication, Leadership, and Social Skills. Convergent validity between the CBCL and BASC-2 was poor when compared with expectations based on the normative sample. Our results confirm and extend previous findings by describing relationships between specific behavior problems and targeted areas of adaptive function. Findings are novel in that they provide information about the clinical utility of the BASC-2 as a measure of behavior and adaptive skills in adolescents with Down syndrome. The improved specification of behavior and adaptive functioning will facilitate the design of targeted intervention, thus improving functional outcomes and overall quality of life for individuals with Down syndrome and their families.

Behavior and adaptive functioning in adolescents with Down syndrome: specifying targets for intervention

PubMed Central

Jacola, Lisa M.; Hickey, Francis; Howe, Steven R.; Esbensen, Anna; Shear, Paula K.

2016-01-01

Background Research suggests that adolescents with Down syndrome experience increased behavior problems as compared to age matched peers; however, few studies have examined how these problems relate to adaptive functioning. The primary aim of this study was to characterize behavior in a sample of adolescents with Down syndrome using two widely-used caregiver reports: the Behavioral Assessment System for Children, 2nd Edition (BASC-2) and Child Behavioral Checklist (CBCL). The clinical utility of the BASC-2 as a measure of behavior and adaptive functioning in adolescents with Down syndrome was also examined. Methods Fifty-two adolescents with Down syndrome between the ages of 12 and 18 (24 males) completed the Peabody Picture Vocabulary Test, 4th Edition (PPVT-IV) as an estimate of cognitive ability. Caregivers completed the BASC-2 and the CBCL for each participant. Results A significant proportion of the sample was reported to demonstrate behavior problems, particularly related to attention and social participation. The profile of adaptive function was variable, with caregivers most frequently rating impairment in skills related to activities of daily living and functional communication. Caregiver ratings did not differ by gender and were not related to age or estimated cognitive ability. Caregiver ratings of attention problems on the BASC-2 accounted for a significant proportion of variance in Activities of Daily Living (Adj R2 = 0.30), Leadership (Adj R2 = 0.30) Functional Communication (Adj R2 = 0.28, Adaptability (Adj R2 = 0.29), and Social Skills (Adj R2 = 0.17). Higher frequencies of symptoms related to social withdrawal added incremental predictive validity for Functional Communication, Leadership, and Social Skills. Convergent validity between the CBCL and BASC-2 was poor when compared with expectations based on the normative sample. Conclusion Our results confirm and extend previous findings by describing relationships between specific behavior problems and targeted areas of adaptive function. Findings are novel in that they provide information about the clinical utility of the BASC-2 as a measure of behavior and adaptive skills in adolescents with Down syndrome. The improved specification of behavior and adaptive functioning will facilitate the design of targeted intervention, thus improving functional outcomes and overall quality of life for individuals with Down syndrome and their families. PMID:28539987

Behavior and Adaptive Functioning in Adolescents with Down Syndrome: Specifying Targets for Intervention

ERIC Educational Resources Information Center

Jacola, Lisa M.; Hickey, Francis; Howe, Steven R.; Esbensen, Anna; Shear, Paula K.

2014-01-01

Adolescents with Down syndrome can demonstrate increased behavior problems as compared with typical peers. Few studies have explored whether behavior impacts adaptive functioning. Caregiver report from the Behavioral Assessment System for Children, 2nd Edition (BASC-2; Reynolds & Kamphaus, 2004) and the Child Behavioral Checklist (CBCL;…

Long-Term Impact of Parental Well-Being on Adult Outcomes and Dementia Status in Individuals with Down Syndrome

ERIC Educational Resources Information Center

Esbensen, Anna J.; Mailick, Marsha R.; Silverman, Wayne

2013-01-01

Parental characteristics were significant predictors of health, functional abilities, and behavior problems in adults with Down syndrome ("n" ?=? 75) over a 22-year time span, controlling for initial levels and earlier changes in these outcomes. Lower levels of behavior problems were predicted by improvements in maternal depressive…

The Fears, Phobias and Anxieties of Children with Autism Spectrum Disorders and Down Syndrome: Comparisons with Developmentally and Chronologically Age Matched Children

ERIC Educational Resources Information Center

Evans, David W.; Canavera, Kristin; Kleinpeter, F. Lee; Maccubbin, Elise; Taga, Ken

2005-01-01

This study compared the fears and behavior problems of 25 children with an autism spectrum disorder (ASD), 43 children with Down syndrome (DS), 45 mental age (MA) matched children, and 37 chronologically age (CA) matched children. Children's fears, phobias, anxieties and behavioral problems were assessed using parent reports. Significant…

Behavioral Phenotype of Fragile X Syndrome in Adolescence and Adulthood

PubMed Central

Smith, Leann E.; Barker, Erin T.; Seltzer, Marsha Mailick; Abbeduto, Leonard; Greenberg, Jan S.

2012-01-01

The present study explored the behavioral profile of individuals with fragile X syndrome during adolescence and adulthood. Individuals with both fragile X syndrome and autism (n = 30) were compared with (a) individuals diagnosed with fragile X syndrome (but not autism; n = 106) and (b) individuals diagnosed with autism (but not fragile X syndrome; n = 135) on measures of autism symptoms, adaptive functioning, behavior problems, and psychological symptoms. Results indicated that individuals dually diagnosed with fragile X syndrome and autism displayed greater communication and social reciprocity impairments than individuals with fragile X syndrome only. Individuals in the dually diagnosed group also exhibited higher levels of repetitive and challenging behaviors than either comparison group, suggesting a unique profile of vulnerability for those diagnosed with both fragile X syndrome and autism. PMID:22264109

Psychiatric and Behavioral Disorders in Persons with Down Syndrome

ERIC Educational Resources Information Center

Dykens, Elisabeth M.

2007-01-01

Similar to the state of the broader intellectual disabilities field, many gaps exist in the research and treatment of mental health concerns in people with Down syndrome. This review summarizes key findings on the type and prevalence of behavior and emotional problems in children, adolescents, and adults with Down syndrome. Such findings include…

Behavioral Profiles in Phelan-McDermid Syndrome: Focus on Mental Health

ERIC Educational Resources Information Center

Shaw, Steven R.; Rahman, Amira; Sharma, Akanksha

2011-01-01

Phelan-McDermid syndrome (PMS) is a multiple congenital anomalies and intellectual disabilities syndrome associated with a deletion of chromosome 22 terminal band 13.3. The deletion is associated with severe intellectual disabilities, absent or delayed speech, behavior problems, and autism. The objective of this study was to provide a detailed…

Full syndrome and subthreshold attention-deficit/hyperactivity disorder in a Korean community sample: comorbidity and temperament findings.

PubMed

Cho, Soo-Churl; Kim, Boong-Nyun; Kim, Jae-Won; Rohde, Luis Augusto; Hwang, Jun-Won; Chungh, Dong-Seon; Shin, Min-Sup; Lyoo, In Kyoon; Go, Bock-Ja; Lee, Sang-Eun; Kim, Hyo-Won

2009-07-01

The main objective of this study was to investigate the comorbid disorders and temperamental profiles of full syndrome and subthreshold attention-deficit/hyperactivity disorder (ADHD). A sample of 2,493 students was randomly selected from six representative elementary schools in Seoul, Korea. Among 245 children with full syndrome and subthreshold ADHD diagnosed by the diagnostic interview schedule for children-4th version, parents of 185 subjects (mean age 9.0 +/- 1.7 years) and of a random sample of 185 age- and gender-matched non-ADHD children have completed the parent's version of the children behavior checklist (CBCL) and the juvenile temperament and character inventory (JTCI). The prevalence rates of full syndrome and subthreshold ADHD were, respectively, 5.90% (95% confidence interval = 4.74-7.06) and 9.00% (95% confidence interval = 7.58-10.41). Subthreshold ADHD cases did not differ from full syndrome ADHD in any JTCI profile, showing high novelty seeking/low persistence/low self-directedness than controls. Subthreshold ADHD also showed increased risk for externalizing disorders and higher scores in eight CBCL scales (somatic complaints, anxious/depressed, social problems, attention problems, delinquent behaviors, aggressive behaviors, externalizing problems and total behavioral problems) compared to the controls. These results support the clinical relevance of subthreshold ADHD in Asian culture. Increased clinical awareness for children with subthreshold ADHD is needed.

Anxiety, attention problems, hyperactivity, and the Aberrant Behavior Checklist in fragile X syndrome.

PubMed

Wheeler, Anne; Raspa, Melissa; Bann, Carla; Bishop, Ellen; Hessl, David; Sacco, Pat; Bailey, Donald B

2014-01-01

Behavior problems are a common challenge for individuals with fragile X syndrome (FXS) and constitute the primary clinical outcome domain in trials testing new FXS medications. However, little is known about the relationship between caregiver-reported behavior problems and co-occurring conditions such as anxiety and attention problems. In this study, 350 caregivers, each with at least one son or daughter with full-mutation FXS, rated one of their children with FXS using the Aberrant Behavior Checklist-Community Version (ABC-C); the Anxiety subscale of the Anxiety, Depression, and Mood Scale; and the Attention/Hyperactivity Items from the Symptom Inventories. In addition to examining family consequences of these behaviors, this study also sought to replicate psychometric findings for the ABC-C in FXS, to provide greater confidence for its use in clinical trials with this population. Psychometric properties and baseline ratings of problem behavior were consistent with other recent studies, further establishing the profile of problem behavior in FXS. Cross-sectional analyses suggest that selected dimensions of problem behavior, anxiety, and hyperactivity are age related; thus, age should serve as an important control in any studies of problem behavior in FXS. Measures of anxiety, attention, and hyperactivity were highly associated with behavior problems, suggesting that these factors at least coincide with problem behavior. However, these problems generally did not add substantially to variance in caregiver burden predicted by elevated behavior problems. The results provide further evidence of the incidence of problem behaviors and co-occurring conditions in FXS and the impact of these behaviors on the family. © 2013 Wiley Periodicals, Inc.

Mental Illness, Behavior Problems, and Social Behavior in Adults with Down Syndrome

ERIC Educational Resources Information Center

Straccia, Claudio; Baggio, Stéphanie; Barisnikov, Koviljka

2014-01-01

Little is known about the behavioral characteristics of adults with Down syndrome (DS) without dementia. The main purpose of this study was to investigate the psychopathology and social behavior among adults with DS compared to adults with nonspecific intellectual disability (NSID). Thirty-four adults with DS were individually matched with 34…

Psychopathology and behavior problems in children and adolescents with Williams syndrome: Distinctive relationships with cognition.

PubMed

Osório, Ana A C; Rossi, Natália F; Gonçalves, Óscar F; Sampaio, Adriana; Giacheti, Célia M

2017-08-01

Several studies have documented the high prevalence of psychopathology and behavior problems in Williams syndrome (WS). However, the links between cognitive development and such symptoms need further clarification. Our study aims to expand current knowledge on levels of behavior problems and its links to cognition in a sample of Brazilian individuals with WS. A total of 25 children and adolescents with WS and their parents participated in this study. The participants' IQs were assessed with the Wechsler Scales of Intelligence (for children or adults) and parental reports of psychopathology/behavior problems were collected using the Child Behavior Checklist (CBCL). The presence of clinically significant attention problems was a main feature in our sample of children and adolescents with WS. In the children, higher IQ scores were found to be significantly associated with less externalizing problems, while in the adolescents cognitive abilities were found to be associated with less internalizing symptoms. These results provide further insight into the links between psychopathology and behavior problems and cognitive abilities in WS, and suggest the need to take age into consideration when analyzing such relationships.

Integrating Behavioral Health and Primary Care for Comorbid Behavioral and Medical Problems

ClinicalTrials.gov

2018-01-10

Arthritis; Asthma; Chronic Obstructive Lung Disease; Diabetes; Heart Failure; Hypertension; Anxiety; Chronic Pain; Depression; Fibromyalgia; Insomnia; Irritable Bowel Syndrome; Problem Drinking; Substance Use Disorder

Coping with the Usual Family Diet: Eating Behaviour and Food Choices of Children with Down's Syndrome, Autistic Spectrum Disorders or Cri du Chat Syndrome and Comparison Groups of Siblings.

ERIC Educational Resources Information Center

Collins, Margaret S. R.; Kyle, Rosalind; Smith, Suzanne; Laverty, Anne; Roberts, Sylvia; Eaton-Evans, Jill

2003-01-01

A study of 405 Irish children (ages 2-18) with Down syndrome, autism, and cri du chat syndrome, and 280 siblings found most had some level of self-feeding skills. Chewing problems were reported for some children, including siblings of children with Down syndrome and autism. Behavioral problems at meals were common. (Contains references.)…

The Turner Syndrome: Cognitive Deficits, Affective Discrimination, and Behavior Problems.

ERIC Educational Resources Information Center

McCauley, Elizabeth; And Others

1987-01-01

The study attemped to link cognitive and social problems seen in girls with Turner syndrome by assessing the girls' ability to process affective cues. Seventeen 9- to 17-year-old girls diagnosed with Turner syndrome were compared to a matched control group on a task which required interpretation of affective intention from facial expression.…

Autism, ADHD, Mental Retardation and Behavior Problems in 100 Individuals with 22q11 Deletion Syndrome

ERIC Educational Resources Information Center

Niklasson, Lena; Rasmussen, Peder; Oskarsdottir, Solveig; Gillberg, Christopher

2009-01-01

This study assessed the prevalence and type of associated neuropsychiatric problems in children and adults with 22q11 deletion syndrome. One-hundred consecutively referred individuals with 22q11 deletion syndrome were given in-depth neuropsychiatric assessments and questionnaires screens. Autism spectrum disorders (ASDs) and/or attention…

Frontal behavioral syndromes in Prader-Willi syndrome.

PubMed

Ogura, Kaeko; Shinohara, Mayumi; Ohno, Kousaku; Mori, Etsuro

2008-08-01

Prader-Willi syndrome (PWS) is a genetically determined neurodevelopmental disorder presenting with behavioral problems including hyperphagia, emotional aberration, and compulsion-like behaviors. This combination of behavioral problems is likely to be caused by damage to the orbitofrontal cortices and anterior temporal lobes or to circuits involving them. To investigate the prevalence of eating and non-eating behavioral disturbances in PWS by using assessment tools developed originally for patients with frontotemporal dementia and with frontal lobe injury. The questionnaire consisted of 35 questions related to three categories of behavior: eating behaviors (including four domains: appetite, food preference, eating habits, and other oral behaviors), stereotypy (including four domains: roaming, speaking, movements, and daily rhythm), and collecting behaviors. It was administered in Japan to the parents of 250 individuals aged 1-42 years with a clinical diagnosis of PWS. The prevalence rates of symptoms in all categories were high. Each domain involved in eating behaviors was significantly correlated with stereotypy and collecting behaviors. The prevalence rates and severity scores of some eating and non-eating behaviors were higher in the older groups. Abnormal eating behaviors, stereotyped behaviors, and collecting behaviors were common in the PWS subjects. There was also a potential link between abnormal eating and non-eating behaviors related to frontal behavioral syndromes. It is likely that these behavioral abnormalities reflect dysfunction of the orbitofrontal cortices and anterior temporal lobes.

Parenting Stress in CHARGE Syndrome and the Relationship with Child Characteristics.

PubMed

Wulffaert, Josette; Scholte, Evert M; Dijkxhoorn, Yvette M; Bergman, Jorieke E H; van Ravenswaaij-Arts, Conny M A; van Berckelaer-Onnes, Ina A

2009-08-01

This study investigates the parental perception of stress related to the upbringing of children with CHARGE syndrome and its association with behavioral and physical child characteristics. Parents of 22 children completed the Nijmegen Parenting Stress Index-Short, Developmental Behavior Checklist, and Dutch Vineland Screener 0-12 and reported their child's problems with hearing, vision and ability to speak. Parenting stress was high in 59% of the subjects. Behavioral problems on the depression, autism, self-absorbed and disruptive behavior scales correlated positively with parenting stress. A non-significant trend was found, namely higher stress among the parents of non-speaking children. No associations were found with other child characteristics, i.e. level of adaptive functioning and intellectual disability, auditory and visual problems, deafblindness, gender, and age. Raising a child with CHARGE syndrome is stressful; professional support is therefore essential for this population. More research into other possible influencing characteristics is needed to improve family-oriented interventions. Since CHARGE is a rare syndrome, closer international collaboration is needed, not only to expand the group of study subjects to increase statistical power, but also to harmonize research designs and measurement methods to improve the validity, the reliability, and the generalization of the findings.

Do Peer Relations in Adolescence Influence Health in Adulthood? Peer Problems in the School Setting and the Metabolic Syndrome in Middle-Age

PubMed Central

Gustafsson, Per E.; Janlert, Urban; Theorell, Töres; Westerlund, Hugo; Hammarström, Anne

2012-01-01

While the importance of social relations for health has been demonstrated in childhood, adolescence and adulthood, few studies have examined the prospective importance of peer relations for adult health. The aim of this study was to examine whether peer problems in the school setting in adolescence relates to the metabolic syndrome in middle-age. Participants came from the Northern Swedish Cohort, a 27-year cohort study of school leavers (effective n = 881, 82% of the original cohort). A score of peer problems was operationalized through form teachers’ assessment of each student’s isolation and popularity among school peers at age 16 years, and the metabolic syndrome was measured by clinical measures at age 43 according to established criteria. Additional information on health, health behaviors, achievement and social circumstances were collected from teacher interviews, school records, clinical measurements and self-administered questionnaires. Logistic regression was used as the main statistical method. Results showed a dose-response relationship between peer problems in adolescence and metabolic syndrome in middle-age, corresponding to 36% higher odds for the metabolic syndrome at age 43 for each SD higher peer problems score at age 16. The association remained significant after adjustment for health, health behaviors, school adjustment or family circumstances in adolescence, and for psychological distress, health behaviors or social circumstances in adulthood. In analyses stratified by sex, the results were significant only in women after adjustment for covariates. Peer problems were significantly related to all individual components of the metabolic syndrome. These results suggest that unsuccessful adaption to the school peer group can have enduring consequences for metabolic health. PMID:22761778

Change in the Behavioral Phenotype of Adolescents and Adults with FXS: Role of the Family Environment

ERIC Educational Resources Information Center

Smith, Leann E.; Hong, Jinkuk; Greenberg, Jan S.; Mailick, Marsha R.

2016-01-01

The present study examined trajectories of adaptive behavior, behavior problems, psychological symptoms, and autism symptoms in adolescents and adults with fragile X syndrome (n = 147) over a three-year period. Adaptive behavior significantly increased over time, particularly for adolescents, and the severity of behavior problems decreased over…

Viewing Social Scenes: A Visual Scan-Path Study Comparing Fragile X Syndrome and Williams Syndrome

ERIC Educational Resources Information Center

Williams, Tracey A.; Porter, Melanie A.; Langdon, Robyn

2013-01-01

Fragile X syndrome (FXS) and Williams syndrome (WS) are both genetic disorders which present with similar cognitive-behavioral problems, but distinct social phenotypes. Despite these social differences both syndromes display poor social relations which may result from abnormal social processing. This study aimed to manipulate the location of…

Feeding and Swallowing Dysfunction in Genetic Syndromes

ERIC Educational Resources Information Center

Cooper-Brown, Linda; Copeland, Sara; Dailey, Scott; Downey, Debora; Petersen, Mario Cesar; Stimson, Cheryl; Van Dyke, Don C.

2008-01-01

Children with genetic syndromes frequently have feeding problems and swallowing dysfunction as a result of the complex interactions between anatomical, medical, physiological, and behavioral factors. Feeding problems associated with genetic disorders may also cause feeding to be unpleasant, negative, or even painful because of choking, coughing,…

Behavior in children with Prader-Willi syndrome before and during growth hormone treatment: a randomized controlled trial and 8-year longitudinal study.

PubMed

Lo, Sin T; Siemensma, Elbrich P C; Festen, Dederieke A M; Collin, Philippe J L; Hokken-Koelega, Anita C S

2015-09-01

Information on behavior of children with Prader-Willi syndrome (PWS) and the effect of growth hormone (GH) treatment is scarce. Parents report less problem behavior during GH treatment. Forty-two pre-pubertal children, aged 3.5-14 years were studied in a randomized controlled GH trial (RCT) during 2 years, followed by a longitudinal study during 8 years of GH treatment. Behavior was measured annually by the Developmental Behavior Checklist for children with intellectual disability (DBC) and a Dutch questionnaire to evaluate social behavioral problems in children, the Children's Social Behavior Questionnaire (CSBQ). Problem behavior measured by the DBC in children with PWS was similar compared to peers with comparable intellectual disability. Scores on 'Social disabilities' subscale were however significantly higher compared to the DBC total score (p < 0.01). A lower IQ was associated with more self-absorbed behavior, more communication problems and more problem behavior in general. Problem behavior measured by the CSBQ was similar compared to peers with a comparable intellectual disability, but children with PWS scored significantly higher on the 'Not tuned', 'Understanding', and 'Stereotyped' subscales than the CSBQ total score (p < 0.05 for all subscales and p = 0.001 for the 'Not tuned'-subscale). There were no significant effects of GH treatment during the RCT and 8 years of GH treatment. Children with PWS showed similar problem behavior as a reference population with a comparable intellectual disability. Social problems were the most pronounced within-problem behavior in PWS. In contrast to our expectations and parents reports, our study shows no improvement but also no deterioration of behavioral problems in children with PWS during long-term GH treatment.

Intellectual Profiles in KBG-Syndrome: A Wechsler Based Case-Control Study

PubMed Central

van Dongen, Linde C. M.; Wingbermühle, Ellen; Oomens, Wouter; Bos-Roubos, Anja G.; Ockeloen, Charlotte W.; Kleefstra, Tjitske; Egger, Jos I. M.

2017-01-01

KBG syndrome is a neurodevelopmental disorder (NDD) caused by loss-of-function of the ANKRD11 gene. The core phenotype comprises developmental delay (DD)/ intellectual disability (ID) and several specific facial dysmorphisms. In addition, both ADHD- and ASD-related symptoms have been mentioned. For the correct understanding of these developmental and behavioral characteristics however, it is of great importance to apply objective measures, which seldom has been done in patients with KBG syndrome. In this study, intelligence profiles of patients with KBG syndrome (n = 18) were compared with a control group comprising patients with NDD caused by various other genetic defects (n = 17), by means of the Wechsler scales. These scales were also used to measure speed of information processing, working memory, verbal comprehension and perceptual reasoning. No significant differences were found in the global level of intelligence of patients with KBG syndrome as compared to the patient genetic control group. The same was true for Wechsler subtest results. Hence, behavioral problems associated with KBG syndrome cannot directly be related to or explained by a specific intelligence profile. Instead, specific assessment of neurocognitive functions should be performed to clarify the putative behavioral problems as observed in this syndrome. PMID:29311865

Psychopharmacology in Fragile X Syndrome--Present and Future

ERIC Educational Resources Information Center

Berry-Kravis, Elizabeth; Potanos, Kristina

2004-01-01

In addition to cognitive disability, fragile X syndrome (FXS) is associated with behavioral problems that are often functionally limiting. There are few controlled trials to guide treatment; however, available information does suggest that medications can be quite helpful for a number of categories of behavioral disturbance in FXS. Specifically,…

Clobazam and Aggression-Related Adverse Events in Pediatric Patients With Lennox-Gastaut Syndrome.

PubMed

Paolicchi, Juliann M; Ross, Gail; Lee, Deborah; Drummond, Rebecca; Isojarvi, Jouko

2015-10-01

Lennox-Gastaut syndrome is an intractable epileptic encephalopathy marked by frequent drop seizures. Most patients develop moderate intellectual disability and behavioral problems, including hyperactivity, aggressiveness, insecurity, and autistic features. Treatment with benzodiazepines, including clobazam, may increase aggression/behavioral problems in patients with Lennox-Gastaut syndrome. Post hoc analyses of data from the OV-1012 trial assessed the potential for behavioral effects with clobazam treatment in pediatric (2 to 18 years) patients with Lennox-Gastaut syndrome. OV-1012 was a phase 3, randomized, double-blind, parallel-group trial comprising a 4-week baseline period, 3-week titration period, and a 12-week maintenance period. Data from 194 patients were analyzed for a history of aggression/behavioral problems, occurrence of aggression-related adverse events, and by assessment of potential drug-related effects on four behavior domains of the Child Behavior Checklist. Twenty-nine aggression-related adverse events were reported for 27 (13.9%) patients. Similar percentages of clobazam-treated patients with and without a history of aggressive behavior experienced an aggression-related adverse event (16.7% versus 15.5%, respectively). In the medium- and high-dosage clobazam groups, onset of aggression-related adverse effects occurred within the 3-week titration period with 63.2% resolving by the end of the study. Aggression-related adverse event onset and resolution were similar for the low-dosage clobazam and placebo groups. Analysis of baseline to postbaseline T scores for the behavior domains of the Child Behavior Checklist indicated no significant differences between clobazam and placebo. Post hoc analyses indicate that the overall rate of aggression with clobazam treatment was low and dosage dependent. Clobazam treatment was effective in reducing drop seizures regardless of aggression experience. Copyright © 2015 Elsevier Inc. All rights reserved.

Dental management of patient with Williams Syndrome - A case report.

PubMed

Wong, Daniel; Ramachandra, Srinivas Sulugodu; Singh, Ashish Kumar

2015-01-01

Williams syndrome is a multisystemic rare genetic disorder caused by deletion of 26-28 genes in the long arm of chromosome 7. It is characterized by developmental and physical abnormalities including congenital cardiovascular abnormalities, mental retardation, neurological features, growth deficiency, genitourinary manifestations, gastrointestinal problems, musculoskeletal problems, unique behavioral characteristics, and dental problems. Dental abnormalities include malocclusion, hypodontia, malformed teeth, taurodontism, pulp stones, increased space between teeth, enamel hypoplasia, and high prevalence of dental caries. Authors report a 17-year-old female patient with underlying Williams syndrome. Oral features and problems seen in the patient are listed. Malocclusion and screwdriver shaped teeth were noticed. Generalized widening of the periodontal ligament space with vital teeth was seen. This finding has not been reported in cases of Williams syndrome earlier. Precautions taken during dental treatment in patients with Williams syndrome are also discussed.

Discrepancies in Parent and Teacher Ratings of Social-Behavioral Functioning of Children with Chromosome 22q11.2 Deletion Syndrome: Implications for Assessment

ERIC Educational Resources Information Center

Shashi, Vandana; Wray, Emily; Schoch, Kelly; Curtiss, Kathleen; Hooper, Stephen R.

2013-01-01

Children with 22q11.2 deletion syndrome exhibit high rates of social-behavioral problems, particularly in the internalizing domain, indicating an area in need of intervention. The current investigation was designed to obtain information regarding parent and teacher ratings of the social-emotional behavior of children with 22q11DS. Using the Child…

Tourette Syndrome (TS): Other Concerns and Conditions

MedlinePlus

... with friends. Learn more about ADHD » Obsessive-Compulsive Behaviors People with obsessive-compulsive behaviors have unwanted thoughts ( ... compulsive behaviors. Diagnosing OCD » Learn more about OCD » Behavior or Conduct Problems About 1 in 4 children ...

Cross-syndrome comparison of real-world executive functioning and problem solving using a new problem-solving questionnaire.

PubMed

Camp, Joanne S; Karmiloff-Smith, Annette; Thomas, Michael S C; Farran, Emily K

2016-12-01

Individuals with neurodevelopmental disorders like Williams syndrome and Down syndrome exhibit executive function impairments on experimental tasks (Lanfranchi, Jerman, Dal Pont, Alberti, & Vianello, 2010; Menghini, Addona, Costanzo, & Vicari, 2010), but the way that they use executive functioning for problem solving in everyday life has not hitherto been explored. The study aim is to understand cross-syndrome characteristics of everyday executive functioning and problem solving. Parents/carers of individuals with Williams syndrome (n=47) or Down syndrome (n=31) of a similar chronological age (m=17 years 4 months and 18 years respectively) as well as those of a group of younger typically developing children (n=34; m=8years 3 months) completed two questionnaires: the Behavior Rating Inventory of Executive Function (BRIEF; Gioia, Isquith, Guy, & Kenworthy, 2000) and a novel Problem-Solving Questionnaire. The rated likelihood of reaching a solution in a problem solving situation was lower for both syndromic groups than the typical group, and lower still for the Williams syndrome group than the Down syndrome group. The proportion of group members meeting the criterion for clinical significance on the BRIEF was also highest for the Williams syndrome group. While changing response, avoiding losing focus and maintaining perseverance were important for problem-solving success in all groups, asking for help and avoiding becoming emotional were also important for the Down syndrome and Williams syndrome groups respectively. Keeping possessions in order was a relative strength amongst BRIEF scales for the Down syndrome group. Results suggest that individuals with Down syndrome tend to use compensatory strategies for problem solving (asking for help and potentially, keeping items well ordered), while for individuals with Williams syndrome, emotional reactions disrupt their problem-solving skills. This paper highlights the importance of identifying syndrome-specific problem-solving strengths and difficulties to improve effective functioning in everyday life. Copyright © 2016 Elsevier Ltd. All rights reserved.

Psychological Well-Being of Mothers and Siblings in Families of Girls and Women with Rett Syndrome

ERIC Educational Resources Information Center

Cianfaglione, Rina; Hastings, Richard P.; Felce, David; Clarke, Angus; Kerr, Michael P.

2015-01-01

Few published studies have reported on the psychological well-being of family members of individuals with Rett syndrome (RTT). Eighty-seven mothers of girls and women with RTT completed a questionnaire survey about their daughters' behavioral phenotype, current health, and behavior problems, and their own and a sibling's well-being. Mothers…

International Comparisons of Behavioral and Emotional Problems in Preschool Children: Parents' Reports from 24 Societies

ERIC Educational Resources Information Center

Rescorla, Leslie A.; Achenbach, Thomas M.; Ivanova, Masha Y.; Harder, Valerie S.; Otten, Laura; Bilenberg, Niels; Bjarnadottir, Gudrun; Capron, Christiane; De Pauw, Sarah S. W.; Dias, Pedro; Dobrean, Anca; Dopfner, Manfred; Duyme, Michel; Eapen, Valsamma; Erol, Nese; Esmaeili, Elaheh Mohammad; Ezpeleta, Lourdes; Frigerio, Alessandra; Fung, Daniel S. S.; Goncalves, Miguel; Gudmundsson, Halldor; Jeng, Suh-Fang; Jusiene, Roma; Kim, Young Ah; Kristensen, Solvejg; Liu, Jianghong; Lecannelier, Felipe; Leung, Patrick W. L.; Machado, Barbara Cesar; Montirosso, Rosario; Oh, Kyung Ja; Ooi, Yoon Phaik; Pluck, Julia; Pomalima, Rolando; Pranvera, Jetishi; Schmeck, Klaus; Shahini, Mimoza; Silva, Jaime R.; Simsek, Zeynep; Sourander, Andre; Valverde, Jose; van der Ende, Jan; Van Leeuwen, Karla G.; Wu, Yen-Tzu; Yurdusen, Sema; Zubrick, Stephen R.; Verhulst, Frank C.

2011-01-01

International comparisons were conducted of preschool children's behavioral and emotional problems as reported on the Child Behavior Checklist for Ages 1 1/2-5 by parents in 24 societies (N = 19,850). Item ratings were aggregated into scores on syndromes; "Diagnostic and Statistical Manual of Mental Disorders"-oriented scales; a Stress…

The Structure of Problem Behavior in a Sample of Maltreated Youths

ERIC Educational Resources Information Center

Culhane, Sara E.; Taussig, Heather N.

2009-01-01

Studies of adolescent community samples suggest that substance use, risky sexual behavior, delinquency, and other problem behaviors can be explained in part by a single, underlying factor or syndrome. Of current interest is the generalizability of these findings to subgroups or special populations of youths who may be at high risk for problem…

Families of Children with Prader-Willi Syndrome: Stress-Support and Relations to Child Characteristics.

ERIC Educational Resources Information Center

Hodapp, Robert M.; And Others

1997-01-01

This study examined stress-support in 42 families of 3- to 18-year-old children with Prader-Willi Syndrome. While children's age, intelligence quotient, and degree of obesity were not related to familial stress, families experienced greater stress when children showed more behavior problems overall, more externalizing and internalizing problems,…

The fears, phobias and anxieties of children with autism spectrum disorders and Down syndrome: comparisons with developmentally and chronologically age matched children.

PubMed

Evans, David W; Canavera, Kristin; Kleinpeter, F Lee; Maccubbin, Elise; Taga, Ken

2005-01-01

This study compared the fears and behavior problems of 25 children with an autism spectrum disorder (ASD), 43 children with Down syndrome (DS), 45 mental age (MA) matched children, and 37 chronologically age (CA) matched children. Children's fears, phobias, anxieties and behavioral problems were assessed using parent reports. Significant differences emerged across the diagnostic groups on a variety of fears. Children with ASD were reported to have more situation phobias and medical fears, but fewer fears of harm/injury compared to all other groups. The groups also differed in terms of the pattern of correlations between fears, phobias, anxieties and behavior problems. For children with ASD, fears, phobias and anxieties were closely related to problem behaviors, whereas fears, phobias, and anxieties were less related to behavioral symptoms for the other groups of subjects. Such findings suggest that children with ASD exhibit a distinct profile of fear and anxiety compared to other mental age and chronologically age-matched children, and these fears are related to the symptoms associated with ASD.

The Efficacy of Social Skills Treatment for Children with Asperger Syndrome

ERIC Educational Resources Information Center

Elder, Lisa M.; Caterino, Linda C.; Chao, Janet; Shaknai, Dina; De Simone, Gina

2006-01-01

Children with Asperger Syndrome present with significant social skills deficits, which may contribute to clinical problems such as anxiety, depression, and/or other behavioral disorders. This article provides a description of the nature of Asperger Syndrome and provides possible treatment interventions, specifically focusing on the efficacy of…

Family Environment and Behavior Problems in Children, Adolescents, and Adults with Fragile X Syndrome

ERIC Educational Resources Information Center

Greenberg, Jan S.; Seltzer, Marsha Mailick; Baker, Jason K.; Smith, Leann E.; Warren, Steven F.; Brady, Nancy; Hong, Jinkuk

2012-01-01

We examine how the family environment is associated with aspects of the Fragile X syndrome phenotype during childhood, adolescence, and adulthood. Mothers of children (n = 48), adolescents (n = 85), and adults (n = 34) with Fragile X syndrome participated in a multisite study. For children and adults with Fragile X syndrome, the presence of warmth…

Longitudinal Course of Behavioural and Emotional Problems of Young Persons with Prader-Willi, Fragile X, Williams and Down Syndromes.

ERIC Educational Resources Information Center

Einfeld, Stewart; Tonge, Bruce; Turner, Gillian; Parmenter, Trevor; Smith, Arabella

1999-01-01

A comparison of levels of emotional and behavioral disturbance in 599 children and adolescents with four genetically determined causes of intellectual disability and an epidemiologically derived control group found in a 4-year follow-up study that persons with Down syndrome had significantly less behavior disturbance than controls and those with…

Ten-Year Time Trends in Emotional and Behavioral Problems of Dutch Children Referred for Youth Care

ERIC Educational Resources Information Center

Veerman, Jan Willem; De Meyer, Ronald

2012-01-01

Emotional and behavioral problems assessed with the "Child Behavior Checklist" (CBCL) were analyzed from 2,739 Dutch children referred to Families First (FF) or Intensive Family Treatment (IFT) from 1999 to 2008, to examine time trends. From the year 2004 onward, six of the eight CBCL-syndrome scales yielded significant decreases from the…

The Co-occurrence of Gambling with Substance Use and Conduct Disorder among Youth in the U.S

PubMed Central

Barnes, Grace M.; Welte, John W.; Hoffman, Joseph H.; Tidwell, Marie-Cecile O.

2013-01-01

The co-occurrence of gambling with substance use and conduct disorder was examined in a representative U.S. household sample of 2,274 youth 14 to 21 years old. The findings show that problem gambling occurs within a problem behavior syndrome with other substance use behaviors and conduct disorder. Male gender, being black, and being Hispanic were found to be significant in predicting problem gambling over and above the effects of all four substance use and conduct disorder variables. Clinical interventions for one specific problem behavior in youth should consider assessing the other problem behaviors as well. PMID:21314760

Maternal Sensitivity and Overt Aggression in Young Children with Down Syndrome

ERIC Educational Resources Information Center

Niccols, Alison; Milligan, Karen; Chisholm, Vivienne; Atkinson, Leslie

2011-01-01

Children with genetic syndromes offer a unique opportunity to combine genetic and environmental approaches to the study of aggression. Children with genetic syndromes associated with developmental delay are at increased risk for behavior problems, but little is known about risk and resilience factors. In this study, we examined maternal…

Prader–Willi Syndrome: Genetics and Behavior

PubMed Central

Thompson, Travis; Butler, Merlin G.; MacLean, William E.; Joseph, Beth

2016-01-01

Since its inception, the John F. Kennedy Center has attempted to overcome developmental problems, which create restrictive barriers to the participation of individuals with specific disabilities in our broader society. Some of Nicholas Hobbs’s earliest efforts involved developing strategies for preventing children’s emotional and behavior problems, which interfered with their later full participation in society. Other investigators in the Kennedy Center explored ways of reducing dysfunctional repetitive movement problems and self-injury commonly associated with autism and severe mental retardation. We have become concerned about a group of people who have the potential to live largely independently (or semi-independently), to work at meaningful jobs in the community, and to make full use of the same recreational and leisure opportunities as other members of society but who are prevented from doing so because of a life-threatening behavior problem. Prader–Willi syndrome (PWS) is a genetic developmental disability characterized by a group of specific behavioral features of which an insatiable appetite is the most striking. PWS is the most commonly known genetic cause of obesity. The eating disorder associated with PWS can be so severe as to be life threatening, including eating to the point of stomach rupture and death. Though a cluster of commonly covarying clinical features are exhibited by people with this syndrome, only the eating disorder is common to all affected individuals. PWS shares behavioral features with other disorders and disabilities, such as obsessive compulsive disorder and autism, but only PWS includes the unique combination of characteristics that distinguish this syndrome. Because eating disorders such as bulimia and anorexia nervosa also share features with PWS, any light that could be shed on the causes and treatment of the eating disorder in PWS could potentially have far-reaching implications for other eating disorders as well. In this article, we review the behavioral, cognitive, and other psychological features of PWS and explore their relationships to known genetic mechanisms. PMID:27594721

Tourette Syndrome: Overview and Classroom Interventions. A Complex Neurobehavioral Disorder Which May Involve Learning Problems, Attention Deficit Hyperactivity Disorder, Obsessive Compulsive Symptoms, and Stereotypical Behaviors.

ERIC Educational Resources Information Center

Fisher, Ramona A.; Collins, Edward C.

Tourette Syndrome is conceptualized as a neurobehavioral disorder, with behavioral aspects that are sometimes difficult for teachers to understand and deal with. The disorder has five layers of complexity: (1) observable multiple motor, vocal, and cognitive tics and sensory involvement; (2) Attention Deficit Hyperactivity Disorder; (3)…

Culture, parenting, and child behavioral problems: a comparative study of cross-cultural immigrant families and native-born families in Taiwan.

PubMed

Yang, Hao-Jan; Kuo, Yi-Jin; Wang, Lee; Yang, Chien-Ying

2014-08-01

Little is known about the interplay of cultural, parenting, and sociodemographic/socioeconomic factors on children's behavioral problems, especially within culturally mixed families in Chinese society. This study compares the presence of behavioral problems between children from families with an immigrant mother and those from native-born families in a randomly selected sample of 957 children aged 6 to 12 years from three counties in central Taiwan. Behavioral problems were assessed using the Child Behavior Checklist completed by parents and the Teacher's Report Form. Parenting styles were assessed using the Parental Bonding Instrument completed by children. Children of immigrant mothers had higher scores for all behavioral syndromes based on the parent's report. However, in the teacher's report a difference was only observed for withdrawn/depressed syndrome. Children of immigrant mothers were more likely, and children with high paternal care were less likely, to have internalizing and total problems in the parent's report. For the teacher's report, only high education in fathers was associated with decreased internalizing and total problems in children. These findings suggest that children growing up in a cross-cultural environment with an immigrant mother, as opposed to a native-born Taiwanese family environment, are more likely to have higher internalizing problems and total behavioral problem scores, due to a number of cultural, parenting, and sociodemographic factors. Children's behaviors appear to be more influenced by fathers' than mothers' parenting styles, regardless of family type. The study findings imply that unequal health and social conditions exist between cross-cultural and native-born families. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

Sex differences and gender-invariance of mother-reported childhood problem behavior.

PubMed

van der Sluis, Sophie; Polderman, Tinca J C; Neale, Michael C; Verhulst, Frank C; Posthuma, Danielle; Dieleman, Gwen C

2017-09-01

Prevalence and severity of childhood behavioral problems differ between boys and girls, and in psychiatry, testing for gender differences is common practice. Population-based studies show that many psychopathology scales are (partially) Measurement Invariance (MI) with respect to gender, i.e. are unbiased. It is, however, unclear whether these studies generalize towards clinical samples. In a psychiatric outpatient sample, we tested whether the Child Behavior Checklist 6-18 (CBCL) is unbiased with respect to gender. We compared mean scores across gender of all syndrome scales of the CBCL in 3271 patients (63.3% boys) aged 6-18. Second, we tested for MI on both the syndrome scale and the item-level using a stepwise modeling procedure. Six of the eight CBCL syndrome scales included one or more gender-biased items (12.6% of all items), resulting in slight over- or under-estimation of the absolute gender difference in mean scores. Two scales, Somatic Complaints and Rule-breaking Behavior, contained no biased items. The CBCL is a valid instrument to measure gender differences in problem behavior in children and adolescents from a clinical sample; while various gender-biased items were identified, the resulting bias was generally clinically irrelevant, and sufficient items per subscale remained after exclusion of biased items. Copyright © 2016 John Wiley & Sons, Ltd. Copyright © 2016 John Wiley & Sons, Ltd.

Incontinence in persons with Down Syndrome.

PubMed

Niemczyk, Justine; von Gontard, Alexander; Equit, Monika; Medoff, David; Wagner, Catharina; Curfs, Leopold

2017-08-01

To assess the rates of incontinence and associated psychological problems in children, adolescents and adults with Down Syndrome, a genetic syndrome caused by partial or complete triplication (trisomy) of chromosome 21 and characterized by typical facial features, a physical growth delay and mild or moderate intellectual disability. Three hundred and seventeen persons with Down Syndrome (4-51 years) were recruited through a German parent support group (59.6% male, mean age 19.2 years). The Parental Questionnaire: Enuresis/Urinary Incontinence, the Incontinence Questionnaire-Pediatric Lower Urinary Tract Symptoms, as well as the Developmental Behavior Checklist (DBC) for parents or for adults were filled out by parents or care-givers. 17.2% of the sample had nocturnal enuresis, 15.9% had daytime urinary incontinence, and 14.2% had fecal incontinence. Incontinence was present in 64.0% of young children (4-12 years), 10.3% of teens (13-17 years), 12.8% of young adults (18-30 years) and in 22.4% of older adults (>30 years). 13.6% of children and 8.4% of adults had a DBC score in the clinical range. 19.5% of children and 27.8% of adults with incontinence had behavioral problems. There was a significant association between nocturnal enuresis, daytime urinary incontinence and clinical DBC scores in adults. Incontinence in Down Syndrome is mainly present in young children and increases in older adults. Behavioral comorbidity is associated with incontinence only in adults with Down Syndrome. Screening and treatment of incontinence in individuals with Down Syndrome is recommended. © 2016 Wiley Periodicals, Inc.

PARENTAL REPORTS OF EARLY SOCIOEMOTIONAL AND BEHAVIORAL PROBLEMS: DOES THE FATHER'S VIEW MAKE A DIFFERENCE?

PubMed

Alakortes, Jaana; Fyrstén, Jenni; Bloigu, Risto; Carter, Alice S; Moilanen, Irma K; Ebeling, Hanna E

2017-05-01

Although both mothers and fathers are essential sources of information to address early socioemotional/behavioral (SEB) problems, there continues to be a dearth of studies considering both parental views. A sample of 208 toddlers (M age = 19.3 months) was recruited through public child health centers. Both parents of 172 toddlers (76 boys, 96 girls) completed the Child Behavior Checklist (CBCL) 1-5 (T.M. Achenbach & L.A. Rescorla, 2000; Finnish translation by F. Almqvist, ). Correspondence (intraclass correlation coefficients; ICCs) between the maternal and paternal CBCL ratings was good (.64) for the Internalizing and excellent (.76) for the Externalizing and Total Problems scores whereas ICCs varied from .45 for the Withdrawn to .76 for the Sleep Problems and Aggressive Behavior syndrome scores. Regarding discrepancies, mothers consistently reported higher CBCL scale scores than did fathers. Most significant differences between the parental ratings were found on the Aggressive Behavior syndrome, Externalizing, and Total Problems scales. Interparental rating discrepancies increased with elevations in the corresponding CBCL scale scores. Positive correlations were found between maternal, but not paternal, parenting stress and interparental rating discrepancies on the CBCL. The observed differences between maternal and paternal ratings highlight the importance of gathering reports from both parents when assessing early SEB problems. The findings are more profoundly discussed in the article. © 2017 Michigan Association for Infant Mental Health.

Attentional Disengagement in Adults with Williams Syndrome

ERIC Educational Resources Information Center

Lense, Miriam D.; Key, Alexandra P.; Dykens, Elisabeth M.

2011-01-01

Williams syndrome (WS) is a neurodevelopmental disorder characterized by a distinctive behavioral and cognitive profile, including widespread problems with attention. However, the specific nature of their attentional difficulties, such as inappropriate attentional allocation and/or poor attentional disengagement abilities, has yet to be…

Advances in the Treatment of Fragile X Syndrome

PubMed Central

Hagerman, Randi J.; Berry-Kravis, Elizabeth; Kaufmann, Walter E.; Ono, Michele Y.; Tartaglia, Nicole; Lachiewicz, Ave; Kronk, Rebecca; Delahunty, Carol; Hessl, David; Visootsak, Jeannie; Picker, Jonathan; Gane, Louise; Tranfaglia, Michael

2010-01-01

The FMR1 mutations can cause a variety of disabilities, including cognitive deficits, attention-deficit/hyperactivity disorder, autism, and other socioemotional problems, in individuals with the full mutation form (fragile X syndrome) and distinct difficulties, including primary ovarian insufficiency, neuropathy and the fragile X-associated tremor/ataxia syndrome, in some older premutation carriers. Therefore, multigenerational family involvement is commonly encountered when a proband is identified with a FMR1 mutation. Studies of metabotropic glutamate receptor 5 pathway antagonists in animal models of fragile X syndrome have demonstrated benefits in reducing seizures, improving behavior, and enhancing cognition. Trials of metabotropic glutamate receptor 5 antagonists are beginning with individuals with fragile X syndrome. Targeted treatments, medical and behavioral interventions, genetic counseling, and family supports are reviewed here. PMID:19117905

[Cerebellar cognitive affective syndrome secondary to a cerebellar tumour].

PubMed

Domínguez-Carral, J; Carreras-Sáez, I; García-Peñas, J J; Fournier-Del Castillo, C; Villalobos-Reales, J

2015-01-01

Cerebellar cognitive affective syndrome is characterized by disturbances of executive function, impaired spatial cognition, linguistic difficulties, and personality change. The case of an 11 year old boy is presented, with behavior problems, learning difficulties and social interaction problems. In the physical examination he had poor visual contact, immature behavior, reduced expressive language and global motor disability with gait dyspraxia, with no defined cerebellar motor signs. In the neuropsychological evaluation he has a full scale overall intellectual quotient of 84, with signs of cerebellar cognitive affective syndrome. A tumour affecting inferior cerebellar vermis was observed in the magnetic resonance imaging, which had not significantly grown during 5 years of follow up. The cerebellum participates in controlling cognitive and affective functions. Cerebellar pathology must be considered in the differential diagnosis of children with cognitive or learning disorder with associated behavioral and emotional components. Copyright © 2013 Asociación Española de Pediatría. Published by Elsevier Espana. All rights reserved.

Change in the Behavioral Phenotype of Adolescents and Adults with FXS: Role of the Family Environment.

PubMed

Smith, Leann E; Hong, Jinkuk; Greenberg, Jan S; Mailick, Marsha R

2016-05-01

The present study examined trajectories of adaptive behavior, behavior problems, psychological symptoms, and autism symptoms in adolescents and adults with fragile X syndrome (n = 147) over a three-year period. Adaptive behavior significantly increased over time, particularly for adolescents, and the severity of behavior problems decreased over time. Family environmental factors predicted phenotypic variables net of gender, intellectual disability status, and medication use. Maternal warmth was associated with higher levels of adaptive behavior, lower levels of autism symptoms, and decreases in behavior problems over time. Maternal depressive symptoms and criticism were associated with higher levels of psychological symptoms. Implications for interventions are discussed.

Quality of life in adolescents and adults with CHARGE syndrome.

PubMed

Hartshorne, Nancy; Hudson, Alexandra; MacCuspie, Jillian; Kennert, Benjamin; Nacarato, Tasha; Hartshorne, Timothy; Blake, Kim

2016-08-01

Health-related Quality of Life and the Impact of Childhood Neurologic Disability Scale were collected for 53 patients with CHARGE syndrome aged 13-39 years with a mean academic level of 4th grade. The most prevalent new and ongoing issues included bone health issues, sleep apnea, retinal detachment, anxiety, and aggression. Sleep issues were significantly correlated with anxiety, self-abuse, conduct problems, and autistic-like behaviors. Problems with overall health, behavior, and balance most affected the number of social activities in the individual's life. Sensory impairment most affected relationships with friends. Two contrasting case studies are presented and demonstrate that the quality of life exists on a broad spectrum in CHARGE syndrome, just as its physical features range from mild to very severe. A multitude of factors, including those beyond the physical manifestations, such as anxiety and sleep problems, influence quality of life and are important areas for intervention. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

Behavioral problems and parenting style among Taiwanese children with autism and their siblings.

PubMed

Gau, Susan Shur-Fen; Chou, Miao-Churn; Lee, Ju-Chin; Wong, Ching-Ching; Chou, Wen-Jiun; Chen, Ming-Fang; Soong, Wei-Tsuen; Wu, Yu-Yu

2010-02-01

The purpose of the present study was to investigate the behavioral problems and parenting style among children with autism and their siblings in an ethnic Chinese population. A total of 151 children with DSM-IV autistic disorder, aged 3-12, 134 siblings without autism, and 113 normally developing controls were recruited. Both parents reported their parenting styles and psychological status and mothers also reported children's behavioral problems. Children with autism had significantly more severe behavioral problems and obtained less affection and more overprotection and authoritarian controlling from their parents than the other two groups. Compared to the controls, unaffected siblings showed some behavioral problems, and obtained less maternal care. Withdrawal and attention, social, and thought problems were the most associated behavioral syndromes to distinguish children with autism from those without. In addition to children with autism, who have a wide range of behavioral problems and impaired parent-child interactions, their siblings may be at risk for such problems.

The Psychoeducational Characteristics of Children with Turner Syndrome.

ERIC Educational Resources Information Center

Rovet, Joanne F.

1993-01-01

This study compared psychoeducational characteristics of 67 children (ages 6-16) with Turner syndrome and 27 nonaffected controls. Subjects exhibited selective impairments in visuospatial and memory areas; significant underachievement in arithmetic; poor social competence; and increased behavior problems, particularly in the area of hyperactivity.…

Sexual initiation and emotional/behavioral problems in Taiwanese adolescents: a multivariate response profile analysis.

PubMed

Chan, Chia-Hua; Ting, Te-Tien; Chen, Yen-Tyng; Chen, Chuan-Yu; Chen, Wei J

2015-04-01

This study aimed to investigate the relations of adolescent sexual experiences (particularly early initiation) to a spectrum of emotional/behavioral problems and to probe possible gender difference in such relationships. The 10th (N = 8,842) and 12th (N = 10,083) grade students, aged 16-19 years, participating in national surveys in 2005 and 2006 in Taiwan were included for this study. A self-administered web-based questionnaire was designed to collect information on sociodemographic characteristics, sexual experience, substance use, and the Youth Self-Report Form. For the sexually experienced adolescents, their sexual initiation was classified as early initiation (<16 years) or non-early initiation (16-19 years). Gender-specific multivariate response profile regression was used to examine the relationship between sexual experience and the behavioral syndromes. Externalizing problems, including Rule-breaking Behavior and Aggressive Behavior, were strongly associated with sexual initiation in adolescence; the magnitude of the association increased for earlier sexual initiation, especially for females. As to internalizing problems, the connection was rather heterogeneous. The scores on some syndromes, such as Somatic Complaints and Anxious/Depressed, were higher only for females with early or non-early sexual initiation whereas the score on Withdrawn, along with Social Problems that is neither internalizing nor externalizing, was lower for the sexually experienced adolescents than for the sexually inexperienced ones. We concluded that earlier sexual initiation was associated with a wider range of behavioral problems in adolescents for both genders, yet the increased risk with emotional problems was predominately found in females.

Sleep in children with autism spectrum disorder.

PubMed

Kotagal, Suresh; Broomall, Eileen

2012-10-01

Children with autism spectrum disorder demonstrate an increased prevalence of difficulties with sleep initiation and maintenance. The consequences may include alterations in daytime behavior, memory, and learning in patients, and significant stress in caretakers. The dysregulation of melatonin synthesis, sensitization to environmental stimuli, behavioral insomnia syndromes, delayed sleep phase syndrome, rapid eye movement sleep behavior disorder, and comorbid anxiety, depression, and epilepsy comprise common etiologic factors. The clinical assessment of sleep problems in this population and a management algorithm are presented. Copyright © 2012 Elsevier Inc. All rights reserved.

Child Behavior Checklist Juvenile Bipolar Disorder (CBCL-JBD) and CBCL Posttraumatic Stress Problems (CBCL-PTSP) Scales Are Measures of a Single Dysregulatory Syndrome

ERIC Educational Resources Information Center

Ayer, Lynsay; Althoff, Robert; Ivanova, Masha; Rettew, David; Waxler, Ellen; Sulman, Julie; Hudziak, James

2009-01-01

Background: The Child Behavior Checklist Juvenile Bipolar Disorder (CBCL-JBD) profile and Posttraumatic Stress Problems (CBCL-PTSP) scale have been used to assess juvenile bipolar disorder (JBD) and posttraumatic stress disorder (PTSD), respectively. However, their validity is questionable according to previous research. Both measures are…

Behavior of 10 patients with FG Syndrome (Opitz-Kaveggia Syndrome) and the p.R961W Mutation in the MED12 Gene

PubMed Central

Graham, John M; Visootsak, Jeannie; Dykens, Elisabeth; Huddleston, Lillie; Clark, Robin D; Jones, Kenneth L; Moeschler, John B; Opitz, John M; Morford, Jackie; Simensen, Richard; Rogers, R. Curtis; Schwartz, Charles E; Friez, Michael J; Stevenson, Roger E

2011-01-01

Opitz and Kaveggia [1974] reported on a family of five affected males with distinctive facial appearance, mental retardation, macrocephaly, imperforate anus and hypotonia. Risheg et al. [2007] identified an identical mutation (p.R961W) in MED12 in six families with Opitz-Kaveggia syndrome, including a surviving affected man from the family reported in 1974. The previously defined behavior phenotype of hyperactivity, affability, and excessive talkativeness is very frequent in young boys with this mutation, along with socially oriented, attention-seeking behaviors. We present case studies of two older males with FG syndrome and the p.R961W mutation to illustrate how their behavior changes with age. We also characterize the behavior of eight additional individuals with FG syndrome and this recurrent mutation in MED12 using the Vineland Adaptive Behavior Scales 2nd ed., the Reiss Profile of Fundamental Goals and Motivation Sensitivities, and the Achenbach Child Behavior Checklist. Males with this MED12 mutation had deficits in communication skills compared to their socialization and daily living skills. In addition, they were at increased risk for maladaptive behavior, with a propensity towards aggression, anxiety, and inattention. Based on the behavior phenotype in 10 males with this recurrent MED12 mutation, we offer specific recommendations and interventional strategies. Our findings reinforce the importance of testing for the p.R961W MED12 mutation in males who are suspected of having developmental and behavioral problems with a clinical phenotype that is consistent with FG syndrome. PMID:18973276

Parental characteristics, parenting style, and behavioral problems among chinese children with Down syndrome, their siblings and controls in Taiwan.

PubMed

Gau, Susan Shur-Fen; Chiu, Yen-Nan; Soong, Wei-Tsuen; Lee, Ming-Been

2008-09-01

The literature has documented maternal distress and behavioral problems among children with Down syndrome (DS), however, little is known about paternal adjustment and behavioural problems among the siblings of children with DS. Here, we examined parental psychopathology, parenting style and emotional/behavioral problems among children with DS, their siblings, and controls in Taiwan. We recruited 45 families of children with DS (age, 2-4 years) and 50 families of normally developing children (age, 3-5 years). If there were more than two children in the case family, the sibling whose age was closest to the child with DS was recruited (age, 3-8 years). Both parents completed self-administered measures of their personality characteristics, psychopathology, family functioning, parenting styles, and child behavioral problems, using the Chinese versions of the Maudsley Personality Inventory, Brief Symptom Rating Scale, Family Adaptability and Cohesion Evaluation Scale, Parental Bonding Instrument, and Child Behavioral Checklist, respectively. Children with DS demonstrated significantly more severe symptoms than normal children of a wide range of behavioral problems such as attention problems, delinquency, social problems, somatic complaints, thought problems, and withdrawal compared with the other two groups, and obtained similar parental treatment, except for paternal overprotection. Their parents suffered from more psychopathology and their mothers were less often employed than their counterparts. The siblings of children with DS obtained less overprotection from their mothers than children with DS and less maternal care and control than normal children. There was no difference in emotional/behavioral problems between the siblings and normal controls. Our findings suggest that in addition to the physical, educational and psychological needs of children with DS, the psychological care of their mothers, fathers and siblings also needs to be evaluated. Moreover, parenting counseling should focus not only on children with DS, but their siblings as well.

Behavioral Profiles of Children With Williams Syndrome From Spain and the United States: Cross-Cultural Similarities and Differences.

PubMed

Pérez-García, Débora; Brun-Gasca, Carme; Pérez-Jurado, Luis A; Mervis, Carolyn B

2017-03-01

To identify similarities and differences in the behavioral profile of children with Williams syndrome from Spain (n = 53) and the United States (n = 145), we asked parents of 6- to 14-year-olds with Williams syndrome to complete the Child Behavior Checklist 6-18. The distribution of raw scores was significantly higher for the Spanish sample than the American sample for all of the higher-order factors and half of both the empirically based and Diagnostic and Statistical Manual of Mental Disorders (DSM)-oriented scales. In contrast, analyses based on country-specific T-scores indicated that the distribution for the Spanish sample was significantly higher than for the American sample only on the Social Problems scale. No gender differences were found. Genetic and cultural influences on children's behavior and cultural influences on parental ratings of behavior are discussed.

Weinberg's syndrome: a disorder of attention and behavior problems needing further research.

PubMed

Brumback, R A

2000-07-01

A subset of inattentive children have an underlying problem in sustaining wakefulness ("vigilance"). This disorder of vigilance, termed Weinberg's syndrome, is characterized by difficulty in maintaining wakefulness and alertness as evidenced by (among other symptoms) motor restlessness (fidgeting and moving about, yawning and stretching, talkativeness) and complaints of tiredness. During tasks requiring concentration (continuous mental activity) such as reading, children with Weinberg's syndrome will daydream, lose interest, complain of boredom, and become increasingly restless. Napping, while infrequent, usually is not refreshing. A distinct personality described by family members and friends as kind, affectionate, compassionate, or "angelic" also seems to characterize this condition. Weinberg's syndrome has a familial pattern suggesting autosomal-dominant inheritance. Additional neurophysiologic, pharmacotherapeutic, epidemiologic, and genetic studies will be necessary for a full understanding of Weinberg's syndrome.

Serving Clients with Tourette Syndrome: A Manual for Service Providers.

ERIC Educational Resources Information Center

Meyers, Abbey S.

The booklet is intended to provide an overview of Tourette Syndrome, (TS), a neurological movement disorder characterized by multiple involuntary tics, uncontrollable vocalizations, and a range of behavior problems including attention deficit and hyperactivity. Typical characteristics of TS are described along with information on etiology,…

Fetal Alcohol Syndrome and the Developing Socio-Emotional Brain

ERIC Educational Resources Information Center

Niccols, Alison

2007-01-01

Fetal alcohol syndrome (FAS) is currently recognized as the most common known cause of mental retardation, affecting from 1 to 7 per 1000 live-born infants. Individuals with FAS suffer from changes in brain structure, cognitive impairments, and behavior problems. Researchers investigating neuropsychological functioning have identified deficits in…

TPH2 G/T Polymorphism Is Associated with Hyperphagia, IQ, and Internalizing Problems in Prader-Willi Syndrome

ERIC Educational Resources Information Center

Dykens, Elisabeth M.; Roof, Elizabeth; Bittel, Douglas; Butler, Merlin G.

2011-01-01

Background: Prader-Willi syndrome (PWS) is a genetic, neurodevelopmental disorder characterized by intellectual disabilities, growth hormone dysregulation, hyperphagia, increased risks of morbid obesity, compulsive behaviors, and irritability. As aberrant serotonergic functioning is strongly implicated in PWS, we examined associations between the…

Prader-Willi Syndrome. ARC Q&A #101-52.

ERIC Educational Resources Information Center

Arc, Arlington, TX.

This fact sheet uses a question-and-answer format to summarize what is known about Prader-Willi Syndrome (PWS), a complex genetic disorder resulting in short stature, mental retardation or learning disabilities, incomplete sexual development, characteristic behavior problems, low muscle tone, and an involuntary urge to eat constantly, which…

Preschool Psychopathology Reported by Parents in 23 Societies: Testing the Seven-Syndrome Model of the Child Behavior Checklist for Ages 1.5-5

ERIC Educational Resources Information Center

Ivanova, Masha Y.; Achenbach, Thomas M.; Rescorla, Leslie A.; Harder, Valerie S.; Ang, Rebecca P.; Bilenberg, Niels; Bjarnadottir, Gudrun; Capron, Christiane; De Pauw, Sarah S. W.; Dias, Pedro; Dobrean, Anca; Doepfner, Manfred; Duyme, Michele; Eapen, Valsamma; Erol, Nese; Esmaeili, Elaheh Mohammad; Ezpeleta, Lourdes; Frigerio, Alessandra; Goncalves, Miguel M.; Gudmundsson, Halldor S.; Jeng, Suh-Fang; Jetishi, Pranvera; Jusiene, Roma; Kim, Young-Ah; Kristensen, Solvejg; Lecannelier, Felipe; Leung, Patrick W. L.; Liu, Jianghong; Montirosso, Rosario; Oh, Kyung Ja; Plueck, Julia; Pomalima, Rolando; Shahini, Mimoza; Silva, Jaime R.; Simsek, Zynep; Sourander, Andre; Valverde, Jose; Van Leeuwen, Karla G.; Woo, Bernardine S. C.; Wu, Yen-Tzu; Zubrick, Stephen R.; Verhulst, Frank C.

2010-01-01

Objective: To test the fit of a seven-syndrome model to ratings of preschoolers' problems by parents in very diverse societies. Method: Parents of 19,106 children 18 to 71 months of age from 23 societies in Asia, Australasia, Europe, the Middle East, and South America completed the Child Behavior Checklist for Ages 1.5-5 (CBCL/1.5-5). Confirmatory…

Behavior Modification of Retarded Preschool Children.

ERIC Educational Resources Information Center

Yamaguchi, Kaoru

1977-01-01

In a study of behavior modification two Down's syndrome preschool children, the first a 5-year-old boy with autistic behavior patterns and the second a 4-year-old girl whose behavior problem was to reject other children, were examined. The first S was engaged in ball catching activities with a teacher with positive reinforcement (playing the S's…

Gilles de la Tourette syndrome as a paradigmatic neuropsychiatric disorder.

PubMed

Cavanna, Andrea E

2018-05-21

Gilles de la Tourette syndrome is a chronic and complex tic disorder accompanied by specific behavioral problems in the majority of patients. With its multifaceted interplay between motion and emotion, this condition is a paradigmatic example of the science and art of clinical neuropsychiatry. This review article encompasses the clinical phenomenology of motor and vocal tics and associated sensory experiences (premonitory urges), as well as the behavioral spectrum of the most common comorbidities, including obsessive-compulsive disorder, attention-deficit and hyperactivity disorder, affective symptoms, and impulsivity. Knowledge of the contributions of both tics and behavioral problems to patients' health-related quality of life across the lifespan should assist treating clinicians in formulating a targeted management plan. Although the exact pathophysiology of Gilles de la Tourette syndrome remains elusive, research into therapeutic interventions has expanded the range of available interventions across multiple domains. A thorough understanding of the neurology and psychiatry of this condition is of key importance to meet the needs of this patient population, from the formulation of an accurate diagnosis to the implementation of effective treatment strategies.

A Follow-Up Study of Adults Who Were Clinically Diagnosed as Hyperkinetic in Childhood.

ERIC Educational Resources Information Center

Beck, Mitchell A.

A followup study involving 24 Ss was conducted to investigate the extent to which hyperkinesis, and its associate behavioral problems, persist into adulthood in children who have been clinically diagnosed as having the hyperkinetic behavioral syndrome. Five areas were considered: behavioral change, educational attainment, treatment, occupational…

Severe Affective and Behavioural Dysregulation Is Associated with Significant Psychosocial Adversity and Impairment

ERIC Educational Resources Information Center

Jucksch, Viola; Salbach-Andrae, Harriet; Lenz, Klaus; Goth, Kirstin; Dopfner, Manfred; Poustka, Fritz; Freitag, Christine M.; Lehmkuhl, Gerd; Lehmkuhl, Ulrike; Holtmann, Martin

2011-01-01

Background: Recently, a highly heritable behavioral phenotype of simultaneous deviance on the Anxious/Depressed, Attention Problems, and Aggressive Behavior syndrome scales has been identified on the Child Behavior Checklist (CBCL-Dysregulation Profile, CBCL-DP). This study aims to investigate psychosocial adversity and impairment of the CBCL-DP.…

Gender Differences in the Behavioral Symptom Severity of Prader-Willi Syndrome.

PubMed

Gito, Masao; Ihara, Hiroshi; Ogata, Hiroyuki; Sayama, Masayuki; Murakami, Nobuyuki; Nagai, Toshiro; Ayabe, Tadayuki; Oto, Yuji; Shimoda, Kazutaka

2015-01-01

This study measured gender differences in Prader-Willi syndrome (PWS) in regard to the severity of behavioral symptoms. The Food Related Problem Questionnaire (FRPQ), the Aberrant Behavior Checklist Japanese Version, the Childhood Routines Inventory, the Pervasive Developmental Disorders Autism Society Japan Rating Scale, and Japanese ADHD-RS were administered to PWS patients (45 males aged 6 to 58 and 37 females aged 6 to 45). To examine the effects that gender and genotype have on the severity of each symptom, two-way ANOVAs were conducted. Significant interactions were found only in regard to FRPQ scores, such as FRPQ total score (F(1, 78) = 8.43, p < 0.01). The FRPQ of male deletion (DEL) individuals was higher than that of female DEL and male mUPD. The FRPQ of male maternal uniparental disomy (mUPD) was lower than that of female mUPD. In terms of problem behaviors, routines, autistic behaviors, and hyperactivity, no significant differences were found. Food-related behaviors in DEL were more severe in males, although those in mUPD were less severe in males.

The Role of Government and NGO in Promoting Wellness of People with Down Syndrome

ERIC Educational Resources Information Center

Jiar, Yeo Kee; Handayani, Lina; Xi, Lu

2014-01-01

People with Down Syndrome (PWDS) experience cognitive delays indicated by difficulties with cognition, long-term memory and non-verbal problem solving skills. PWDS have specific speech and language impairments which affect all aspects of development. Some children develop difficult behaviors which cause family stress and affect social and…

Pragmatic Inferences in High-Functioning Adults with Autism and Asperger Syndrome

ERIC Educational Resources Information Center

Pijnacker, Judith; Hagoort, Peter; Buitelaar, Jan; Teunisse, Jan-Pieter; Geurts, Bart

2009-01-01

Although people with autism spectrum disorders (ASD) often have severe problems with pragmatic aspects of language, little is known about their pragmatic reasoning. We carried out a behavioral study on high-functioning adults with autistic disorder (n = 11) and Asperger syndrome (n = 17) and matched controls (n = 28) to investigate whether they…

Physiological Correlates of Maternal Responsivity in Mothers of Preschoolers with Fragile X Syndrome

ERIC Educational Resources Information Center

Robinson, Ashley N.; Roberts, Jane E.; Brady, Nancy C.; McQuillin, Samuel D.; Warren, Steven F.

2016-01-01

The present study examined the relationship between salivary cortisol and maternal responsiveness in mothers of boys with fragile X syndrome (FXS). Maternal responsivity is strongly associated with child outcomes, and children with FXS are at risk for compromised development due to intellectual disability and problem behavior. Increased…

Social Functioning among Girls with Fragile X or Turner Syndrome and Their Sisters.

ERIC Educational Resources Information Center

Mazzocco, Michele M. M.; Baumgardner, Thomas; Freund, Lisa S.; Reiss, Allan L.

1998-01-01

Social behaviors among girls (ages 6-16) with fragile X (n=8) or Turner syndrome (n=9) were examined to address the role of family environment versus biological determinants of social dysfunction. Compared to their sisters, subjects had lower IQS and higher rating of social and attention problems. (Author/CR)

Modeling Family Dynamics in Children with Fragile X Syndrome

ERIC Educational Resources Information Center

Hall, Scott S.; Burns, David D.; Reiss, Allan L.

2007-01-01

Few studies have examined the impact of children with genetic disorders and their unaffected siblings on family functioning. In this study, the reciprocal causal links between problem behaviors and maternal distress were investigated in 150 families containing a child with fragile X syndrome (FXS) and an unaffected sibling. Both children's…

Leisure Activities in Prader-Wlli Syndrome: Implications for Health, Cognition and Adaptive Functioning

ERIC Educational Resources Information Center

Dykens, Elisabeth M.

2014-01-01

Although hyperphagia and compulsivity in Prader-Willi syndrome (PWS) are well described, recreation and adaptive skills are relatively unexplored. Parents of 123 participants with PWS (4--48 years) completed measures of their child's adaptive, recreation, and problem behaviors. Offspring received cognitive testing. Watching TV was the most…

Deficits in Social Attribution Ability in Prader-Willi Syndrome

ERIC Educational Resources Information Center

Koenig, Kathleen; Klin, Ami; Schultz, Robert

2004-01-01

Prader-Willi syndrome (PWS), a genetic form of mental retardation, involves a myriad of physical and behavioral problems. Poor social adjustment has been reported, but the origin of this difficulty is unknown. The Social Attribution Task, a measure of one's ability to make appropriate social attributions from an ambiguous visual display [Klin…

Sexual Abuse: Somatic and Emotional Reactions.

ERIC Educational Resources Information Center

Rimza, Mary Ellen; And Others

1988-01-01

Chart reviews and telephone interviews with 72 sexual abuse victims found that 48 of the children had symptoms similar to the "rape trauma" syndrome. Two-thirds of victims commonly had somatic complaints (such as abdominal pain) and emotional/behavioral problems (runaway behavior, suicide attempts). (DB)

Maternal support for autonomy: relationships with persistence for children with Down syndrome and typically developing children.

PubMed

Gilmore, Linda; Cuskelly, Monica; Jobling, Anne; Hayes, Alan

2009-01-01

Maternal behaviors and child mastery behaviors were examined in 25 children with Down syndrome and 43 typically developing children matched for mental age (24-36 months). During a shared problem-solving task, there were no group differences in maternal directiveness or support for autonomy, and mothers in the two groups used similar verbal strategies when helping their child. There were also no group differences in child mastery behaviors, measured as persistence with two optimally challenging tasks. However, the two groups differed in the relationships of maternal style with child persistence. Children with Down syndrome whose mothers were more supportive of their autonomy in the shared task displayed greater persistence when working independently on a challenging puzzle, while children of highly directive mothers displayed lower levels of persistence. For typically developing children, persistence was unrelated to maternal style, suggesting that mother behaviors may have different causes or consequences in the two groups.

Assessing local instrument reliability and validity: a field-based example from northern Uganda.

PubMed

Betancourt, Theresa S; Bass, Judith; Borisova, Ivelina; Neugebauer, Richard; Speelman, Liesbeth; Onyango, Grace; Bolton, Paul

2009-08-01

This paper presents an approach for evaluating the reliability and validity of mental health measures in non-Western field settings. We describe this approach using the example of our development of the Acholi psychosocial assessment instrument (APAI), which is designed to assess depression-like (two tam, par and kumu), anxiety-like (ma lwor) and conduct problems (kwo maraco) among war-affected adolescents in northern Uganda. To examine the criterion validity of this measure in the absence of a traditional gold standard, we derived local syndrome terms from qualitative data and used self reports of these syndromes by indigenous people as a reference point for determining caseness. Reliability was examined using standard test-retest and inter-rater methods. Each of the subscale scores for the depression-like syndromes exhibited strong internal reliability ranging from alpha = 0.84-0.87. Internal reliability was good for anxiety (0.70), conduct problems (0.83), and the pro-social attitudes and behaviors (0.70) subscales. Combined inter-rater reliability and test-retest reliability were good for most subscales except for the conduct problem scale and prosocial scales. The pattern of significant mean differences in the corresponding APAI problem scale score between self-reported cases vs. noncases on local syndrome terms was confirmed in the data for all of the three depression-like syndromes, but not for the anxiety-like syndrome ma lwor or the conduct problem kwo maraco.

Behavioral phenotype in adults with Prader-Willi syndrome.

PubMed

Sinnema, Margje; Einfeld, Stewart L; Schrander-Stumpel, Constance T R M; Maaskant, Marian A; Boer, Harm; Curfs, Leopold M G

2011-01-01

Prader-Willi syndrome (PWS) is characterized by temper tantrums, impulsivity, mood fluctuations, difficulty with change in routine, skinpicking, stubbornness and aggression. Many studies on behavior in PWS are limited by sample size, age range, a lack of genetically confirmed diagnosis of PWS and inconsistent assessment of behavior. The aim of this study was to explore systematically the relation between behavioral problems and age groups, genetic subtypes and BMI categories in an adult PWS population. Participants were contacted via the Dutch Prader-Willi Parent Association and through physicians specialized in persons with ID. Behaviors were studied using the Developmental Behavior Checklist for Adults (DBC-A). The forms were completed by the main caregivers of 98 adults with a genetically confirmed diagnosis of PWS. Differences between age groups were statistically significant (ANOVA, p=0.03). DBC-A total scores were higher in the consecutive age groups, with the most behavioral problems in the oldest age groups. Differences between genetic subtypes were also statistically significant (ANOVA, p<0.01). Persons with mUPD had higher total scores on the DBC-A than persons with a deletion. Those with a Type I deletion showed higher total DBC-A scores than persons with a Type II deletion. There were no statistically significant differences in DBC-A total scores between the different BMI categories. Individuals with a BMI<25 had higher scores on the self-absorbed subscale compared to persons with a BMI between 25 and 30. Unlike previous descriptions of the behavioral phenotype in adults with PWS, we did not find a reduction in behavioral problems in older adults. Therefore, special attention should be paid to behavioral problems as part of general management of adults with PWS. Longitudinal studies are warranted to gain more insight into the natural history and course of behavioral problems in adults and older people with PWS over the long term and possible risk and preventive factors. Copyright © 2010 Elsevier Ltd. All rights reserved.

Difficulty eating and significant weight loss in joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type.

PubMed

Baeza-Velasco, Carolina; Van den Bossche, Thomas; Grossin, Daniel; Hamonet, Claude

2016-06-01

Joint Hypermobility Syndrome, also known as Ehlers-Danlos Syndrome Hypermobility Type (JHS/EDS-HT), is a heritable disorder of connective tissue, common but poorly known by the medical community. Although generalized joint hypermobility and fragility of tissues have been described as core features, recent research highlights the multisystemic nature of JHS/EDS-HT, which presents with a wide range of articular and extra-articular symptoms. Among these, gastrointestinal problems, temporomandibular disorders, and smell and taste abnormalities are common among those affected, having significant implications for eating. The present work reviews the literature linking JHS/EDS-HT and eating problems. Two illustrative case reports, in which JHS/EDS-HT manifestations contribute to developing and maintaining disturbed eating behaviors and significant weight loss, are presented.

Spoiled child syndrome.

PubMed

McIntosh, B J

1989-01-01

People often speak of children as being "spoiled" and many parents worry about the possibility of spoiling their infants and children. Many pediatricians, however, are uncomfortable with this term because it is a poorly defined and derogatory expression. Some would even deny that infants and children can be spoiled. Avoiding the use of the expression spoiled can create difficulties in communicating with parents concerned about their children's behavior. In this article, the spoiled child syndrome will be defined and those patterns of behavior that characterize it will be distinguished from other patterns of difficult behavior which may be confused with it. The spoiled child syndrome is characterized by excessive self-centered and immature behavior, resulting from the failure of parents to enforce consistent, age-appropriate limits. Many of the problem behaviors that cause parental concern are unrelated to spoiling as properly understood. Such behaviors are often age-related normal behaviors, reactions to family stresses, or patterns of behavior determined by factors inherent in the child. Pediatricians can provide counseling and reassurance for such behaviors and, by helping parents understand the etiology of true spoiling, can encourage the use of behavior modification techniques for its prevention and treatment.

Reduced gamma-aminobutyric acid is associated with emotional and behavioral problems in Prader-Willi syndrome.

PubMed

Rice, Lauren J; Lagopoulos, Jim; Brammer, Michael; Einfeld, Stewart L

2016-12-01

Prader-Willi syndrome (PWS) is characterized by infantile hypotonia, hypogonadism, small hands and feet, distinct facial features and usually intellectual impairment. The disorder is associated with severe behavioral disturbances which include hyperphagia leading to morbid obesity, temper outbursts, skin-picking, and compulsive behaviors. While the brain mechanisms that underpin these disturbances are unknown these behaviors suggest a lack of inhibition and thus gamma-aminobutyric acid (GABA), the main inhibitory neurotransmitter may be implicated. In the present study, we investigated in vivo brain GABA and its relationship with emotion and behavior in individuals with PWS. Single voxel proton magnetic resonance spectroscopy (1H-MRS) was performed on 15 individuals with PWS and 15 age- and gender-matched typically developing controls. GABA levels were measured in the parieto-occipital lobe. All other metabolite levels (N-acetyl aspartate, myo-Inositol, glutathione, glutamate, and glutamine + glutamate) were measured in the anterior cingulate cortex (ACC). GABA levels were significantly lower in the participants with PWS who had clinically significant emotional and behavioral problems relative to typically developing control participants and participants with PWS who did not have emotional and behavioral problems within the clinically significant range. GABA levels were negatively correlated with total behavioral problem scores as well as temper outbursts, skin-picking, depression, social relating difficulties, and a tendency to be self-absorbed. Our data suggests that alterations of the GABAergic system may play an important role in aspects of the pathophysiology of PWS. Pathological mechanism found in PWS may be relevant to understanding the control of similar behaviors in the general population. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

A Comparative Study of Behavior Problems among Left-Behind Children, Migrant Children and Local Children.

PubMed

Hu, Hongwei; Gao, Jiamin; Jiang, Haochen; Jiang, Haixia; Guo, Shaoyun; Chen, Kun; Jin, Kaili; Qi, Yingying

2018-04-01

This study aims to estimate the prevalence of behavioral problems among left-behind children, migrant children and local children in China, and to compare the risks of behavioral problems among the three types of children. Data on 4479 children aged 6-16 used in this study were from a survey conducted in China in 2017. The school-age version of the Children Behavior Checklist was used to measure children's behavioral problems. Descriptive analysis, correlation analysis, and logistic regressions were conducted. The prevalence of behavioral problems was 18.80% and 13.59% for left-behind children and migrant children, respectively, both of which were higher than that of local children. Logistic regression analysis showed that after adjustments for individual and environmental variables, the likelihood of total, internalizing and externalizing behavior problems for left-behind children and migrant children were higher than those for local children; left-behind children had a higher likelihood of internalizing problems than externalizing problems, while migrant children had a higher prevalence of externalizing problems. Left-behind children had a higher prevalence of each specific syndrome than migrant and local children. Both individual and environmental factors were associated with child behavioral problems, and family migration may contribute to the increased risks. Left-behind and migrant children were more vulnerable than local children to behavioral problems.

A Comparative Study of Behavior Problems among Left-Behind Children, Migrant Children and Local Children

PubMed Central

Hu, Hongwei; Gao, Jiamin; Jiang, Haochen; Jiang, Haixia; Guo, Shaoyun; Chen, Kun; Jin, Kaili; Qi, Yingying

2018-01-01

This study aims to estimate the prevalence of behavioral problems among left-behind children, migrant children and local children in China, and to compare the risks of behavioral problems among the three types of children. Data on 4479 children aged 6–16 used in this study were from a survey conducted in China in 2017. The school-age version of the Children Behavior Checklist was used to measure children’s behavioral problems. Descriptive analysis, correlation analysis, and logistic regressions were conducted. The prevalence of behavioral problems was 18.80% and 13.59% for left-behind children and migrant children, respectively, both of which were higher than that of local children. Logistic regression analysis showed that after adjustments for individual and environmental variables, the likelihood of total, internalizing and externalizing behavior problems for left-behind children and migrant children were higher than those for local children; left-behind children had a higher likelihood of internalizing problems than externalizing problems, while migrant children had a higher prevalence of externalizing problems. Left-behind children had a higher prevalence of each specific syndrome than migrant and local children. Both individual and environmental factors were associated with child behavioral problems, and family migration may contribute to the increased risks. Left-behind and migrant children were more vulnerable than local children to behavioral problems. PMID:29614783

Behavioral and emotional problems in Chinese children of divorced parents.

PubMed

Liu, X; Guo, C; Okawa, M; Zhai, J; Li, Y; Uchiyama, M; Neiderhiser, J M; Kurita, H

2000-07-01

This study examined the behavioral problems in Chinese children of divorced parents. A total of 58 children of divorce and 116 gender-, age-, and school class-matched controls were ascertained from a general population sample of children aged 6 through 15 years. Parents completed the Child Behavior Checklist (CBCL), and teachers completed the Teacher's Report Form (TRF) and Conners Hyperkinesis Index. Parent-reported problem scores on the CBCL total scale and each subscale, and prevalence of all CBCL syndromes except for Somatic Complaints, were significantly higher in children of divorce than in controls. Teacher-reported problem scores on the TRF total scale and Social and Attention Problems and prevalence of Attention Problems were significantly different for the 2 groups of children. Social competence was rated significantly lower in children of divorce than in controls. Discriminant function analysis showed that behavioral problems in children of divorce were characterized by aggressive behavior, withdrawal, and social problems. The findings emanating from China provide the first evidence of the link between parental divorce and children's psychopathology and clarify the psychopathological dimensions in Chinese children of divorced parents.

Sexual Preoccupation Behavior in Parkinson's Disease.

PubMed

Bronner, Gila; Hassin-Baer, Sharon; Gurevich, Tanya

2017-01-01

People with Parkinson's disease (PD) present with problematic sexual behaviors that are often misunderstood or ignored. Sexual problems in PD are part of a non-motor syndrome, and they play a  prominent role in the life of affected individuals and their partners. Based on our considerable clinical experience, we describe four common types of sexual preoccupation behaviors in people with PD: (1) sexual behavior with underlying sexual dysfunction, (2) sexual desire discrepancy with partner after restored desire, (3) hypersexuality and compulsive sexual behavior, and (4) sexual behavior with underlying restless genital syndrome. We also suggest methods of assessing and diagnosing these sexual behaviors, and propose alternative possible treatments for people with PD and their partners/caregivers. Understanding these four behavioral types will assist healthcare professionals in explaining and educating people with PD and their partners, contribute to decreased stress and tension between them, and help them manage these sexual issues.

Examining the Relationship between Heart Rate and Problem Behavior: A Case Study of Severe Skin Picking in Prader-Willi Syndrome

ERIC Educational Resources Information Center

Hall, Scott S.; Hammond, Jennifer L.; Hustyi, Kristin M.

2013-01-01

Few studies have examined the relationship between heart rate and self-injurious behavior (SIB) shown by individuals with IDD (intellectual and developmental disabilities). In this single-case study, we simultaneously monitored heart rate and activity levels during a functional analysis of severe skin picking behavior exhibited by a young man with…

Using Video Modeling, Prompting, and Behavior-Specific Praise to Increase Moderate-to-Vigorous Physical Activity for Young Children with down Syndrome

ERIC Educational Resources Information Center

Adamo, Elyse K.; Wu, Jenny; Wolery, Mark; Hemmeter, Mary Louise; Ledford, Jennifer R.; Barton, Erin E.

2015-01-01

Children with Down syndrome may be at increased risk of problems associated with inactivity. Early intervention to increase physical activity may lead to increased participation in typical activities and long-term increases in quality of life (e.g., decreased likelihood of obesity-related illness). A multi-component intervention, including video…

Neuropsychiatry of 18q{sup {minus}} syndrome

DOE Office of Scientific and Technical Information (OSTI.GOV)

Mahr, R.N.; Moberg, P.J.; Campbell, H.

Our understanding of neuropsychiatric abnormalities in patients with deletions of the long arm of chromosome 18 (18q{sup {minus}} syndrome) is based mainly on sporadic case reports. We characterized the neuropsychiatric phenotype in 27 patients across a wide age range (2-47 years) with breakpoints ranging from 18q22.3-18q21.2. Adaptive behavior scores (Vineland Composite) were significantly higher in females than in males (62 {+-} 5 vs. 43 {+-} 3). Intelligence ranged from borderline to severely deficient (IQ, 73-<40), with academic achievement similarly impaired. Performance in specific neuropsychological functions, including attention, novel problem solving, memory, language, visuomotor integration, and fine motor dexterity, was consistentlymore » in the moderately-to-severely impaired range. Behavioral problems were common in both sexes, including aggressivity, hyperactivity, and temper tantrums. Contrary to the few previous reports, we found no evidence of psychosis in any patient. In a subset of patients selected on the basis of no prior knowledge of behavioral problems, 1 of 16 patients (61%) had autism, as defined by the Autistic Diagnostic Interview-Revised (ADI-R). Thus, the prevalence of autism in 18q{sup {minus}} syndrome is probably no greater than that in other developmental disabilities with a similar level of cognitive impairment. In contrast to what has been believed since 18q{sup {minus}} was first described 30 years ago, we found no relationship between chromosome deletion size and any measure of cognition or behavior; nor were there any correlations between any of these measures with the presence or absence of abnormalities on MRI or somatosensory-evoked potentials. 38 refs., 3 figs., 2 tabs.« less

Is there a conflicted surrogate syndrome affecting quality of care in nursing homes?

PubMed

Kidder, Samuel W; Smith, David A

2006-03-01

Is there a point at which family complaints about care cease to be constructive and become excessive and counterproductive? Do excessive complaint behaviors represent a "conflicted surrogate syndrome" that is indicative of psychopathology in the family member or family system? Can this psychopathology result in avoidance behavior by the nursing staff sufficient to result in poor care? While many family/resident complaints are valid and should be viewed as constructive there are occasions when excessive complaints by the family of a nursing facility resident are a result of psychiatric illness or psychological problems in the family member(s) or are evidence of an abnormality in the family system. This series of brief case reports is offered to create discussion of what might be termed a "conflicted surrogate syndrome" that may result in avoidance behavior by staff and consequent poor care.

Neurobehavioral phenotype in Prader-Willi syndrome.

PubMed

Whittington, Joyce; Holland, Anthony

2010-11-15

The focus of this article is on the lifetime development of people with Prader-Willi syndrome (PWS) and specifically on the neurobehavioral phenotype. We consider studies of this aspect of the phenotype (the "behavioral phenotype" of the syndrome) that have confirmed that there are specific behaviors and psychiatric disorders, the propensities to which are increased in those with PWS, and cannot be accounted for by other variables such as IQ or adaptive behavior. Beginning with a description of what is observed in people with PWS, we review the evolving PWS phenotype and consider how some aspects of the phenotype might be best explained, and how this complex phenotype may relate to the equally complex genotype. We then consider in more detail some of the neurobehavioral aspects of the phenotype listed above that raise the greatest management problems for parents and carers. © 2010 Wiley-Liss, Inc.

Testosterone replacement therapy to improve secondary sexual characteristics and body composition without adverse behavioral problems in adult male patients with Prader-Willi syndrome: an observational study.

PubMed

Kido, Yasuhiro; Sakazume, Satoru; Abe, Yoshiko; Oto, Yuji; Itabashi, Hisashi; Shiraishi, Masahisa; Yoshino, Atsunori; Tanaka, Yuriko; Obata, Kazuo; Murakami, Nobuyuki; Nagai, Toshiro

2013-09-01

Prader-Willi syndrome (PWS), a complex genetic disorder, arises from suppressed expression of paternally inherited imprinted genes on chromosome 15q11-q13. Characteristics include short stature, intellectual disability, behavioral problems, hypogonadism, obesity, and reduced bone and muscle mass. Testosterone replacement (TR) remains controversial due to concerns regarding behavioral problems. To evaluate the effects of TR on secondary sexual characteristics, body composition, and behavior in adult males with PWS, 22 male PWS patients over the age of 16 with behavioral scores of less than grade 4 on the Modified Overt Aggression Scale (MOAS) underwent monthly intramuscular TR (125 mg). Pubertal change, body composition and behavior were evaluated before and after 24 months of therapy. Serum testosterone, LH, and FSH did not change. Increased pubic hair was observed in 16 of 22 patients (72.7%). Percent body fat decreased from 47.55 ± 2.06% to 39.75 ± 1.60% (n = 18) (P = 0.018). Bone mineral density increased from 0.8505 ± 0.0426 g/cm(2) to 0.9035 ± 0.0465 g/cm(2) (n = 18) (P = 0.036), and lean body mass increased from 18093.4 ± 863.0 g to 20312.1 ± 1027.2 g (n = 18) (P = 0.009). The MOAS was unchanged, from 4.5 ± 2.0 at the beginning of the study to 3.0 ± 1.7 at the end of study indicating no increase in aggression. No behavioral problems were observed. Based on this pilot study, TR with 125 mg monthly is a potentially safe and useful intervention for adult males with PWS. Copyright © 2013 Wiley Periodicals, Inc.

[The relationship between the abnormal behavior and serum C-reactive protein in children with obstructive sleep apnea-hypopnea syndrome].

PubMed

Wang, Yan; Li, Yanzhong; Wang, Xin

2009-12-01

To explore the pathogenesis of abnormal behavior in children with obstructive sleep apnea-hypopnea syndrome (OSAHS). The behavioral problems and C-reactive protein were measured in 40 children with OSAHS and 30 children with habitual snoring who underwent overnight Polysomnography, 40 cases of healthy children for the control group. The ratio of abnormal behavior in OSAHS and habitual snoring children was significantly higher than that of the healthy control group, while no significant difference between the two groups. The content of C-reactive protein in OSAHS children (4.24 mg/L) was significantly higher than habitual snoring (2.76 mg/L) and healthy control group (1.27 mg/L); in habitual snoring children C-reactive protein was higher than in healthy control group. The content of serum C-reactive protein in OSAHS children accompanied by abnormal behavior (4.63 mg/L) was significantly higher than that without abnormal behavior (3.23 mg/L). The content of serum C-reactive protein content in habitual snoring children accompanied by abnormal behavior (3.63 mg/L) was significantly higher than that without abnormal behavior (1.76 mg/L). OSAHS and habitual snoring children have more behavior problems. C-reactive protein levels are higher in children with OSAHS and habitual snoring, and the levels of C-reactive protein are related to the abnormal behavior in these children.

Errorless Compliance Training: Success-Focused Behavioral Treatment of Children with Asperger Syndrome

ERIC Educational Resources Information Center

Ducharme, Joseph M.; Sanjuan, Elena; Drain, Tammy

2007-01-01

Errorless compliance training is a noncoercive, success-focused approach to treatment of problem behavior in children. The intervention involves graduated exposure of a child to increasingly more challenging requests at a slow enough rate to ensure that noncompliance rarely occurs, providing parents with many opportunities to reinforce cooperative…

Coping with Dravet syndrome: parental experiences with a catastrophic epilepsy.

PubMed

Nolan, Kathleen J; Camfield, Carol S; Camfield, Peter R

2006-09-01

The aim of this study was to investigate how parents cope with and care for a child with Dravet syndrome, a severe myoclonic epilepsy with three distinct stages. Twenty-four parents of children with Dravet syndrome participated in a semi-structured interview and 17 completed the Impact of Childhood Neurologic Disability Scale (ICND) questionnaire. Children included 11 males and 13 females aged 2 to 24 years (mean age 10y 2mo [SD 5y 8mo]). Stage 1 of the syndrome was generally very difficult. Uncertainty about the diagnosis was the primary cause of stress. Seizure control was worst during this time. The primary concern in Stage 2 remained seizure control but developmental, behavioral, and sleep issues also emerged. Negative effects were noted in parents' relationships with others. Stage 3 brought better seizure control but decreased cognitive level, increased behavioral problems, and increasing social isolation for parents. Respite and relief care were hard to obtain at each stage. ICND scores mirrored the findings of the interviews. The three stages of Dravet syndrome present serious challenges for parents. Seizures are persistent and severe but developmental, behavioral, and sleep issues add to the stress. In the absence of successful medical treatment for Dravet syndrome, further attention needs to be paid to helping families cope with the disorder.

Using Problem-Solving Frameworks to Address Challenging Behavior of Students with High-Functioning Autism and/or Asperger Syndrome

ERIC Educational Resources Information Center

O'Connor, Karen V.; Stichter, Janine P.

2011-01-01

Students with high-functioning autism and/or Asperger Syndrome (HFA/AS) are characterized by difficulties with communication as well as impairments in social interaction skills. Students with HFA/AS have been shown to generate solutions that are lower quality (e.g., less socially appropriate) than those of their typical peers and also to have…

Prenatal Exposure to Phthalate Esters and Behavioral Syndromes in Children at 8 Years of Age: Taiwan Maternal and Infant Cohort Study

PubMed Central

Lien, Yin-Ju; Ku, Hsiu-Ying; Su, Pen-Hua; Chen, Suh-Jen; Chen, Hsiao-Yen; Liao, Pao-Chi; Chen, Wei-J.

2014-01-01

Background: Few studies have shown an association between prenatal phthalate exposure and adverse effects on neurodevelopment and behavior in young children. Objectives: We aimed to assess the relationship between prenatal exposure to phthalate esters and behavior syndromes in children at 8 years of age. Methods: A total of 122 mother–child pairs from the general population in central Taiwan were studied from 2000 to 2009. Mono-methyl phthalate (MMP), mono-ethyl phthalate (MEP), mono-butyl phthalate (MBP), mono-benzyl phthalate (MBzP), and three di-(2-ethylhexyl) phthalate (DEHP) metabolites—mono-2-ethylhexyl, mono-2-ethyl-5-hydroxyhexyl, and mono-2-ethyl-5-oxohexyl phthalates (MEHP, MEHHP, and MEOHP)—were measured in maternal urine collected during the third trimester of pregnancy using liquid chromatography–electrospray ionization–tandem mass spectrometry. Behavioral syndromes of children at 8 years of age were evaluated using the Child Behavior Checklist (CBCL). Associations between log10-transformed creatinine-corrected phthalate concentrations and standardized scores of the CBCL were estimated using linear regression models or multinomial logistic regressions with adjustments for potential confounders. Results: Externalizing problem scores were significantly higher in association with a 1-unit increase in log10-transformed creatinine-corrected concentrations of maternal MBP (β = 4.29; 95% CI: 0.59, 7.99), MEOHP (β = 3.74; 95% CI: 1.33, 6.15), and MEHP (β = 4.28 ; 95% CI: 0.03, 8.26) after adjusting for the child’s sex, intelligence, and family income. Meanwhile, MBP and MEOHP were significantly associated with Delinquent Behavior and Aggressive Behavior scores. The same pattern was found for borderline and/or clinical ranges. Conclusions: Our findings suggest positive associations between maternal DEHP and dibutyl phthalate (DBP) exposure and externalizing domain behavior problems in 8-year-old children. Citation: Lien YJ, Ku HY, Su PH, Chen SJ, Chen HY, Liao PC, Chen WJ, Wang SL. 2015. Prenatal exposure to phthalate esters and behavioral syndromes in children at 8 years of age: Taiwan Maternal and Infant Cohort Study. Environ Health Perspect 123:95–100; http://dx.doi.org/10.1289/ehp.1307154 PMID:25280125

Indian story on semen loss and related Dhat syndrome.

PubMed

Prakash, Om; Kar, Sujit Kumar; Sathyanarayana Rao, T S

2014-10-01

India is a country of many religions and ancient cultures. Indian culture is largely directed by the Vedic culture since time immemorial. Later Indian culture is influenced by Buddhism, Islam, and Christianity. Indian belief system carries the footprints of these cultures. Every culture describes human behaviors and an interpretation of each human behavior is largely influenced by the core cultural belief system. Sexuality is an important domain which is colored by different cultural colors. Like other cultures, Indian culture believes "semen" as the precious body fluid which needs to be preserved. Most Indian beliefs consider loss of semen as a threat to the individual. Ancient Indian literature present semen loss as a negative health related event. Dhat syndrome (related to semen loss) is a culture-bound syndrome seen in the natives of Indian subcontinent. This article gathers the Indian concepts related to semen loss. It also outlines belief systems behind problems of Dhat syndrome.

Indian story on semen loss and related Dhat syndrome

PubMed Central

Prakash, Om; Kar, Sujit Kumar; Sathyanarayana Rao, T. S.

2014-01-01

India is a country of many religions and ancient cultures. Indian culture is largely directed by the Vedic culture since time immemorial. Later Indian culture is influenced by Buddhism, Islam, and Christianity. Indian belief system carries the footprints of these cultures. Every culture describes human behaviors and an interpretation of each human behavior is largely influenced by the core cultural belief system. Sexuality is an important domain which is colored by different cultural colors. Like other cultures, Indian culture believes “semen” as the precious body fluid which needs to be preserved. Most Indian beliefs consider loss of semen as a threat to the individual. Ancient Indian literature present semen loss as a negative health related event. Dhat syndrome (related to semen loss) is a culture-bound syndrome seen in the natives of Indian subcontinent. This article gathers the Indian concepts related to semen loss. It also outlines belief systems behind problems of Dhat syndrome. PMID:25568479

Noonan syndrome.

PubMed

Bhambhani, Vikas; Muenke, Maximilian

2014-01-01

Noonan syndrome is a common genetic disorder that causes multiple congenital abnormalities and a large number of potential health conditions. Most affected individuals have characteristic facial features that evolve with age; a broad, webbed neck; increased bleeding tendency; and a high incidence of congenital heart disease, failure to thrive, short stature, feeding difficulties, sternal deformity, renal malformation, pubertal delay, cryptorchidism, developmental or behavioral problems, vision problems, hearing loss, and lymphedema. Familial recurrence is consistent with an autosomal dominant mode of inheritance, but most cases are due to de novo mutations. Diagnosis can be made on the basis of clinical features, but may be missed in mildly affected patients. Molecular genetic testing can confirm diagnosis in 70% of cases and has important implications for genetic counseling and management. Most patients with Noonan syndrome are intellectually normal as adults, but some may require multidisciplinary evaluation and regular follow-up care. Age-based Noonan syndrome-specific growth charts and treatment guidelines are available.

A partial loss of function allele of Methyl-CpG-binding protein 2 predicts a human neurodevelopmental syndrome

PubMed Central

Samaco, Rodney C.; Fryer, John D.; Ren, Jun; Fyffe, Sharyl; Chao, Hsiao-Tuan; Sun, Yaling; Greer, John J.; Zoghbi, Huda Y.; Neul, Jeffrey L.

2008-01-01

Rett Syndrome, an X-linked dominant neurodevelopmental disorder characterized by regression of language and hand use, is primarily caused by mutations in methyl-CpG-binding protein 2 (MECP2). Loss of function mutations in MECP2 are also found in other neurodevelopmental disorders such as autism, Angelman-like syndrome and non-specific mental retardation. Furthermore, duplication of the MECP2 genomic region results in mental retardation with speech and social problems. The common features of human neurodevelopmental disorders caused by the loss or increase of MeCP2 function suggest that even modest alterations of MeCP2 protein levels result in neurodevelopmental problems. To determine whether a small reduction in MeCP2 level has phenotypic consequences, we characterized a conditional mouse allele of Mecp2 that expresses 50% of the wild-type level of MeCP2. Upon careful behavioral analysis, mice that harbor this allele display a spectrum of abnormalities such as learning and motor deficits, decreased anxiety, altered social behavior and nest building, decreased pain recognition and disrupted breathing patterns. These results indicate that precise control of MeCP2 is critical for normal behavior and predict that human neurodevelopmental disorders will result from a subtle reduction in MeCP2 expression. PMID:18321864

Advances in research on the neurological and neuropsychiatric phenotype of Klinefelter syndrome.

PubMed

Savic, Ivanka

2012-04-01

Klinefelter syndrome, 47,XXY is the most common chromosomal aberration among men. It represents a naturally occurring human model for studies of both X-chromosome gene expression and potential androgen effects on brain development and function. The aim of this review is to combine available brain imaging and behavioral data to provide an overview of what we have learned about the neural underpinnings of cognitive, emotional and behavioral dysunctions in Klinefelter syndrome. The behavioral phenotype of 47,XXY is characterized by language, executive and psychomotor dysfunction, as well as socioemotional impairment. The prevalence of schizophrenia, attention deficit hyperactivity disorder, autism spectrum disorders and affective regulation problems is increased. Neuroimaging studies of children and adults with Klinefelter syndrome syndrome show characteristic structural changes from typical individuals. There are increases in the grey matter volume of the sensorimotor and parietooccipital regions, as well as significant reductions in amygdala, hippocampal, insular, temporal and inferior-frontal grey matter volumes. Widespread white matter abnormalities have been revealed, with reductions in some areas (including anterior cingulate, bilaterally) but increases in others (such as left parietal lobe). Mechanisms underlying these developmental anomalies could include imbalance in gene dosage relative to typical men or women, as well as the potential consequence of endocrinological deficits. Studies of Klinefelter syndrome could generate important information about the impact of anomalies in sex chromosome gene regulation on the development of cerebral grey and white matter and, ultimately, on human behavior.

[Mania associated with Usher syndrome type II].

PubMed

Praharaj, Samir Kumar; Acharya, Mahima; Sarvanan, Arul; Kongasseri, Sreejayan; Behere, Rishikesh V; Sharma, P S V N

2012-01-01

Usher syndrome (or Hallgren syndrome) is an autosomal recessive genetic disorder characterized by sensorineural deafness, retinitis pigmentosa, and variable vestibular deficit; Usher syndrome type II is the most common form. Various neuropsychiatric disorders have been reported to occur in those with Usher syndrome, including schizophrenia-like disorder, atypical psychosis, recurrent depressive illness, neurotic disorder, and mental retardation; however, bipolar disorder is not common in those with Usher syndrome. Herein we describe a 30-year-old male with Usher syndrome type II that developed features indicative of a probable manic episode. The patient had complete remission of symptoms in response to treatment with olanzapine 20 mg d-1. In persons with dual sensory impairment there are inherent problems with assessment and diagnosis is difficult due to their limited communication abilities. The diagnosis of Usher syndrome depends heavily on behavioral observation and disturbances in vegetative functions.

Genetics Home Reference: 7q11.23 duplication syndrome

MedlinePlus

... with this condition have low-average to average intelligence. Intellectual disability or borderline intellectual ability occur in ... Rarely, people with this disorder have above-average intelligence. Behavioral problems associated with this condition include anxiety ...

Progression of Challenging Behaviors in Children and Adolescents with Autism Spectrum Disorders as Measured by the Autism Spectrum Disorders-Problem Behaviors for Children (ASD-PBC)

ERIC Educational Resources Information Center

Matson, Johnny L.; Mahan, Sara; Hess, Julie A.; Fodstad, Jill C.; Neal, Daniene

2010-01-01

This study examined the effect of age on challenging behaviors among 167 children, ages 3-14 years, with Autistic Disorder, Pervasive Developmental Disorder Not Otherwise Specified, or Asperger's syndrome. Results of a MANOVA indicated that there were no significant differences between young children, children, and young adolescents on any of the…

Parenting and children's externalizing problems in substance-abusing families.

PubMed

Stanger, Catherine; Dumenci, Levent; Kamon, Jody; Burstein, Marcy

2004-09-01

This study tested associations in path models among positive and negative parenting and children's rule-breaking behavior, aggressive and oppositional behavior, and attention problems for families with a drug-dependent parent. A structural model tested relations between parenting and children's externalizing problems for 251 families with 399 children between the ages of 6 and 18, controlling for nonindependence of ratings at the family level. The model also tested potential moderators, including child age, gender, and ethnicity (White vs. other), and caregiver gender (families with a female substance-abusing caregiver vs. families with a male substance-abusing caregiver). Results indicated that caregiver ratings of monitoring predicted rule-breaking behavior and use of inconsistent discipline predicted ratings of all 3 externalizing syndromes, after controlling parenting and externalizing problems for the effects of the moderators and after controlling significant relations among types of parenting and types of externalizing problems.

Behavioral, Cognitive and Neural Markers of Asperger Syndrome

PubMed Central

Faridi, Farnaz; Khosrowabadi, Reza

2017-01-01

Asperger syndrome (AS) is a subtype of Autism Spectrum Disorder (ASD) characterized by major problems in social and nonverbal communication, together with limited and repetitive forms of behavior and interests. The linguistic and cognitive development in AS is preserved which help us to differentiate it from other subtypes of ASD. However, significant effects of AS on cognitive abilities and brain functions still need to be researched. Although a clear cut pathology for Asperger has not been identified yet, recent studies have largely focused on brain imaging techniques to investigate AS. In this regard, we carried out a systematic review on behavioral, cognitive, and neural markers (specifically using MRI and fMRI) studies on AS. In this paper, behavior, motor skills and language capabilities of individuals with Asperger are compared to those in healthy controls. In addition, common findings across MRI and fMRI based studies associated with behavior and cognitive disabilities are highlighted. PMID:29167722

Behavioral, Cognitive and Neural Markers of Asperger Syndrome.

PubMed

Faridi, Farnaz; Khosrowabadi, Reza

2017-01-01

Asperger syndrome (AS) is a subtype of Autism Spectrum Disorder (ASD) characterized by major problems in social and nonverbal communication, together with limited and repetitive forms of behavior and interests. The linguistic and cognitive development in AS is preserved which help us to differentiate it from other subtypes of ASD. However, significant effects of AS on cognitive abilities and brain functions still need to be researched. Although a clear cut pathology for Asperger has not been identified yet, recent studies have largely focused on brain imaging techniques to investigate AS. In this regard, we carried out a systematic review on behavioral, cognitive, and neural markers (specifically using MRI and fMRI) studies on AS. In this paper, behavior, motor skills and language capabilities of individuals with Asperger are compared to those in healthy controls. In addition, common findings across MRI and fMRI based studies associated with behavior and cognitive disabilities are highlighted.

Fragile X syndrome: A review of clinical management

PubMed Central

Lozano, Reymundo; Azarang, Atoosa; Wilaisakditipakorn, Tanaporn; Hagerman, Randi J

2016-01-01

Summary The fragile X mental retardation 1 gene, which codes for the fragile X mental retardation 1 protein, usually has 5 to 40 CGG repeats in the 5′ untranslated promoter. The full mutation is the almost always the cause of fragile X syndrome (FXS). The prevalence of FXS is about 1 in 4,000 to 1 in 7,000 in the general population although the prevalence varies in different regions of the world. FXS is the most common inherited cause of intellectual disability and autism. The understanding of the neurobiology of FXS has led to many targeted treatments, but none have cured this disorder. The treatment of the medical problems and associated behaviors remain the most useful intervention for children with FXS. In this review, we focus on the non-pharmacological and pharmacological management of medical and behavioral problems associated with FXS as well as current recommendations for follow-up and surveillance. PMID:27672537

Externalizing and internalizing syndromes in relation to school truancy among adolescents in high-risk urban schools.

PubMed

Nik Jaafar, Nik Ruzyanei; Tuti Iryani, Mohammad Daud; Wan Salwina, Wan Ismail; Fairuz Nazri, Abdul Rahman; Kamal, Nor Azlin; Prakash, Reddy Jaya; Shah, Shamsul Azhar

2013-04-01

To examine the relationship between externalizing/internalizing syndromes and school truancy among Form Four (10th grade) students attending "high-risk" schools in Kuala Lumpur. This is a cross-sectional study conducted upon 16-year-old adolescents attending three high-risk schools in Kuala Lumpur. A total of 373 students completed self-administered questionnaires on the sociodemographic variables and externalizing/internalizing syndromes. The number of truant-days per student during the study period was obtained from the school records. The mean internalizing score, externalizing score and total problem score of those who were truant were found to be significantly higher (P < 0.05) than those who were non-truant. Multiple logistic regressions showed externalizing syndrome (odds ratio [OR] = 1.044; confidence interval [CI] = 1.012-1.078, P = 0.018) significantly predicts truancy but not the internalizing syndrome. Two other psychosocial factors, namely, having divorced parents (OR = 2.495, CI = 1.058-5.886, P = 0.037) and did not understand or were uncertain of the purpose of schooling (OR = 2.621, CI = 1.265-5.433, P = 0.010) were also showed to be stronger predictors of truancy compared to externalizing/internalizing syndromes. Truancy has significant association to externalizing syndrome, in addition to other significant psychosocial variables like parental and schooling factors. This finding indicated the need for comprehensive assessment of students presenting with truant behavior, including the identification of associated psychological and behavioral problems which highlights the roles of mental health professionals in tackling truancy. Copyright © 2013 Wiley Publishing Asia Pty Ltd.

Behavioral correlation with television watching and videogame playing among children in the United Arab Emirates.

PubMed

Yousef, Said; Eapen, Valsamma; Zoubeidi, Taoufik; Mabrouk, Abdelazim

2014-08-01

Television viewing and videogame use (TV/VG) appear to be associated with some childhood behavioral problems. There are no studies addressing this problem in the United Arab Emirates. One hundred ninety-seven school children (mean age, 8.7 ± 2.1 years) were assessed. Child Behavior Checklist (CBCL) subscale scores and socio-demographic characteristics were compared between children who were involved with TV/VG more than 2 hours/day and those involved less than 2 hours/day (the recommended upper limit by The American Academy of Pediatrics). Thirty-seven percent of children who were involved with TV/VG time of more than 2 hours/day scored significantly higher on CBCL syndrome scales of withdrawn, social problems, attention problems, delinquent behavior, aggressive behavior, internalizing problems, externalizing problems and the CBCL total scores compared with their counterparts. Moreover, these children were younger in birth order and had fewer siblings. After controlling for these confounders using logistic regression, we found that TV/VG time more than 2 hours/day was positively associated with withdrawn (p = 0.008), attention problem (p = 0.037), externalizing problems (p = 0.007), and CBCL total (p = 0.014). Involvement with TV/VG for more than 2 hours/day is associated with more childhood behavioral problems. Counteracting negative effects of the over-involvement with TV/VG in children requires increased parental awareness.

Obesity and its relationship to addictions: is overeating a form of addictive behavior?

PubMed

Barry, Danielle; Clarke, Megan; Petry, Nancy M

2009-01-01

Obesity is a major public health problem and notoriously difficult to treat. There are many parallels between obesity/overeating and addictions to alcohol and drugs. This paper discusses similarities between obesity and addictive disorders, including common personality characteristics, disruptive behavior syndromes, and brain mechanisms. Although there are important differences between overeating and other addictive behaviors, an addiction model of overeating may effectively inform prevention and treatment of obesity.

Obesity and Its Relationship to Addictions: Is Overeating a Form of Addictive Behavior?

PubMed Central

Barry, Danielle; Clarke, Megan; Petry, Nancy M.

2010-01-01

Obesity is a major public health problem and notoriously difficult to treat. There are many parallels between obesity/overeating and addictions to alcohol and drugs. This paper discusses similarities between obesity and addictive disorders, including common personality characteristics, disruptive behavior syndromes, and brain mechanisms. Although there are important differences between overeating and other addictive behaviors, an addiction model of overeating may effectively inform prevention and treatment of obesity. PMID:19874165

Brief Review of Current Research in FXS: Implications for Treatment with Psychotropic Medication

ERIC Educational Resources Information Center

Valdovinos, Maria G.

2007-01-01

The purpose of this paper is to provide a brief review of current research in fragile X syndrome (FXS) with regards to the morphology and behavioral phenotype associated with FXS and the use of psychotropic medication for the treatment of behavior problems (e.g., aggression) often seen in FXS (full mutation). The lack of production of the fragile…

Executive Functioning Mediates the Effect of Behavioral Problems on Depression in Mothers of Children with Developmental Disabilities

ERIC Educational Resources Information Center

Chan, Wai; Smith, Leann E.; Greenberg, Jan S.; Hong, Jinkuk; Mailick, Marsha R.

2017-01-01

The present investigation explored long-term relationships of behavioral symptoms of adolescents and adults with developmental disabilities with the mental health of their mothers. Fragile X premutation carrier mothers of an adolescent or adult child with fragile X syndrome (n = 95), and mothers of a grown child with autism (n = 213) were…

Post-Institutional Autistic Syndrome in Romanian adoptees.

PubMed

Hoksbergen, René; ter Laak, Jan; Rijk, Kathinka; van Dijkum, Cor; Stoutjesdijk, Felicia

2005-10-01

Romanian adoptees have a background of severe neglect. International research has shown that this can give rise to symptoms of autistic behavior. Rutter et al. (1999, Journal of Child Psychology Psychiatry, 40(4), 537-549.) refers to "quasi-autistic patterns", and Federici (1998, Help for the hopelss child: A guide for families. Alexandria: Federici & Assocoates.) to Post-Institutional Autistic Syndrome (PIAS). Eighty Romanian adoptees, averaging 8 years of age, who had resided in the Netherlands for 5 years were studied. Parent interviews and the Auti-R scale showed the extent to which the children exhibited PIAS. In one third of these children we observed (in addition to other behavioral problems) stereotypic behaviors and communication and language disorders. Our findings resembled the Rutter et al. (1999, Journal of Child Psychology Psychiatry, 40(4), 537-549). data. Six of the children were classified within the autistic spectrum pursuant to the Auti-R, and seven within the so-called intermediate group. No difference was found between the girls and the boys. Children who had been in their adoptive families for 5 years or more showed fewer behavior problems than children who had been in their adoptive families for four or less years.

Obsessive-compulsive symptoms in Prader-Willi and "Prader-Willi-Like" patients.

PubMed

State, M W; Dykens, E M; Rosner, B; Martin, A; King, B H

1999-03-01

To compare obsessive-compulsive (OC) symptoms in patients with Prader-Willi syndrome (PWS) and symptoms in a group of patients presenting with "Prader-Willi-like" features but without the genetic abnormalities associated with PWS. 16 patients aged 4 through 20 years were evaluated in a clinic specializing in the assessment and management of behavioral and food-related problems in PWS. Eight patients were found to have key features of the syndrome but did not have a PWS genotype. These PWS-like subjects were matched to 8 clinic patients with a confirmed deletion of the PWS critical region of the paternally derived chromosome 15. All subjects were evaluated for obesity, IQ, food-related problems, maladaptive behaviors, and non-food-related OC symptoms. There were no differences between the 2 groups with respect to measures of obesity, IQ, food-related difficulties, or overall maladaptive behaviors. The PWS group showed significantly greater numbers of OC symptoms and greater symptom severity. Patients with PWS have elevated numbers of OC symptoms and significant symptom-related impairment which are not explained by developmental delay, food-related difficulties, or obesity. OC symptoms are part of a behavioral phenotype that accompanies deletions on the proximal long arm of chromosome 15 in PWS.

Taste perception and sensory sensitivity: Relationship to feeding problems in boys with Barth Syndrome.

PubMed

Reynolds, Stacey; Kreider, Consuelo M; Meeley, Lauren E; Bendixen, Roxanna M

2015-03-01

Feeding problems are common in boys with Barth syndrome and may contribute to the population's propensity for growth delay and muscle weakness. The purpose of this study was to quantify and describe these feeding issues and examine altered taste perception and sensory sensitivity as contributing factors. A cross-sectional, two-group comparison design was used to examine feeding preferences and behaviors, chemical taste perception, and sensory sensitivities in fifty boys with (n=24) and without (n=26) Barth ages 4-17 years. Taste perception was measured using chemical test strips saturated with phenylthiocarbamide (PTC) and sodium benzoate (NaB). Feeding problems were documented by parents using a Food Inventory, while sensory sensitivities were recorded using a Short Sensory Profile. Boys with Barth differed significantly from typical peers with regards to problem feeding behaviors. For boys with Barth, food refusal and food selectivity were identified as being present in 50% the sample, while 70% of had identified problems related to gagging or swallowing foods. About half of all Barth families noted that their child's eating habits did not match the family's and that separate meals were often prepared. As demonstrated in previous research, about 50% of boys with Barth demonstrated probable or definite differences in taste/smell sensitivity, which was significantly higher than controls. On tests of chemical taste perception, boys with Barth were significantly more likely to be supertasters to PTC and non-tasters to NaB. Taster-status did not directly relate to the presence of feeding problems, however, taste/smell sensitivity did significantly relate to food selectivity by type and texture. Results indicate feeding problems in at least 50-70% of boys with Barth syndrome, and suggest that behaviors are often present before 6 months of age. Differences in taste perception may influence dietary choices in boys with Barth, particularly their craving of salty foods. Taste/smell sensitivity also appears to influence food selectivity, and therefore may be important to consider in this population, particularly in light of dietary influences on cardiac function, energy consumption, and overall growth.

[Neuropsychiatric aspects of Prader-Willi syndrome – a review].

PubMed

Briegel, Wolfgang

2018-05-01

Prader-Willi Syndrome (PWS) is caused by the absence of paternal expression of imprinted genes in the region at 15q11–q13. With an estimated birth incidence of 1/15 000 – 1/30 000, PWS is one of the more frequent genetic syndromes among humans. Typical physical features include neonatal hypotonia and feeding problems, hypogonadism, hyperphagia in later childhood with consecutive obesity, and short stature. Most people with PWS show a mild to moderate intellectual disability. Furthermore, lability of mood, temper tantrums, skin-picking, and compulsive behaviors are quite typical for subjects with PWS. Psychotic disorders have also been found to be quite common in adulthood. This manuscript reviews current knowledge about the etiology, physical features, developmental aspects, behavioral phenotype, and psychiatric disorders that occur as well as existing psychopharmacological and psychotherapeutic interventions.

Neurocognitive findings in Prader-Willi syndrome and early-onset morbid obesity.

PubMed

Miller, Jennifer; Kranzler, John; Liu, Yijun; Schmalfuss, Ilona; Theriaque, Douglas W; Shuster, Jonathan J; Hatfield, Ann; Mueller, O Thomas; Goldstone, Anthony P; Sahoo, Trilochan; Beaudet, Arthur L; Driscoll, Daniel J

2006-08-01

To examine whether early-onset morbid obesity is associated with cognitive impairment, neuropathologic changes, and behavioral problems. This case-control study compared head MRI scans and cognitive, achievement, and behavioral evaluations of subjects with Prader-Willi syndrome (PWS), early-onset morbid obesity (EMO), and normal-weight sibling control subjects from both groups. Head MRI was done on 17 PWS, 18 EMO, and 21 siblings, and cognitive, achievement, and behavioral evaluations were done on 19 PWS, 17 EMO, and 24 siblings. The mean General Intellectual Ability score of the EMO group was 77.4 +/- 17.8; PWS, 63.3 +/- 14.2; and control subjects, 106.4 +/- 13.0. Achievement scores for the three groups were EMO, 78.7 +/- 18.8; PWS, 71.2 +/- 17.0; and control subjects, 104.8 +/- 17.0. Significant negative behaviors and poor adaptive skills were found in the EMO group. White matter lesions were noted on brain MRI in 6 subjects with PWS and 5 with EMO. None of the normal-weight control subjects had these findings. Individuals with EMO have significantly lower cognitive function and more behavioral problems than control subjects with no history of childhood obesity. Both EMO and PWS subjects have white matter lesions on brain MRI that have not previously been described.

Bowel obsession syndrome in a patient with ulcerative colitis.

PubMed

Porcelli, Piero; Leandro, Gioacchino

2007-01-01

Gastroenterologists are often faced with the diagnostic problem of differentiating acute symptoms of ulcerative colitis from functional intestinal disorders. Bowel obsession syndrome (BOS) is an OCD-like, functional syndrome characterized by fear of fecal incontinence and compulsive behaviors of evacuation-checking. Only sparse case studies on treatment of BOS with antidepressants have been published. This is the first study on successful psychotherapy of a male patient with ulcerative colitis overlapping functional bowel symptoms and marked symptoms of BOS. Clinical recognition of BOS may help clinicians in differential diagnosis, prevent unnecessary investigations, and give patients the most appropriate treatment.

[Fournier syndrome: the perception of the patient].

PubMed

Cavalini, Fernanda; Moriya, Tokico Murakawa; Pelá, Nilza Teresa Rotter

2002-06-01

The purpose of the present investigation was to identify the perception of the Fournier syndrome's patients about their disease and caregivers. Data was collected by means of interviews with a structured routine and the analysis was based on the André's analysis of prose. According to the patients, the syndrome causes intense pain, edema, fever and wounds, demanding surgical treatment, dressings and bringing physical, economical and familiar problems. The caregivers' attitudes and behaviors were described as having more negative aspects than good ones. The necessity of a better training of the caregivers in the cognitive, psychomotor and affective sense is evident.

The link between drinking and gambling among undergraduate university students.

PubMed

Hodgins, David C; Racicot, Stephanie

2013-09-01

The purpose of this research was to explore different aspects of the link between alcohol use and gambling among undergraduate university students (N = 121). Potential aspects of the link examined included level of involvement in each behavior, consequences, motives for involvement, and impaired control over involvement. Results confirmed that drinking and gambling among university students are associated, consistent with the expectations of a problem syndrome model. The strongest link was between general dimensions of problematic involvement for both behaviors. Students who drink to cope and have other indicators of alcohol problems are more likely to gamble to cope, gamble to win money, and have higher gambling involvement and gambling-related problems. However, the salience of drinking and gambling to cope in this relationship is an interesting finding that needs further exploration and extension to other problem behaviors. PsycINFO Database Record (c) 2013 APA, all rights reserved.

Behavioral problems among patients in skilled nursing facilities.

PubMed Central

Zimmer, J G; Watson, N; Treat, A

1984-01-01

This survey of a 33 per cent random sample (1,139) of 3,456 patients in 42 skilled nursing facilities (SNFs) in upstate New York yielded 64.2 per cent with significant behavioral problems. Of these, 257 (22.6 per cent) of the total sample had what were defined as "serious" problems (i.e., excluding those with only impaired judgment and/or physical restraint orders). Details of the problem behaviors of this group, their previous history, current management, frequency of psychiatric consultation, and adequacy of documentation were analyzed. Median age was the same as the general SNF population, a slightly lower proportion was female, and, while 66.5 per cent had diagnoses indicating organic brain syndrome, very few had specific psychiatric diagnoses, and only 4.7 per cent had been admitted from a psychiatric facility. The attending physician had noted the behavioral problem in the record in only 9.7 per cent and had requested psychiatric consultation in 14.8 per cent of these "serious" cases. The need for more staff training in mental health care, and more physician and psychiatric consultative assistance are discussed. PMID:6476166

Viewing social scenes: a visual scan-path study comparing fragile X syndrome and Williams syndrome.

PubMed

Williams, Tracey A; Porter, Melanie A; Langdon, Robyn

2013-08-01

Fragile X syndrome (FXS) and Williams syndrome (WS) are both genetic disorders which present with similar cognitive-behavioral problems, but distinct social phenotypes. Despite these social differences both syndromes display poor social relations which may result from abnormal social processing. This study aimed to manipulate the location of socially salient information within scenes to investigate the visual attentional mechanisms of: capture, disengagement, and/or general engagement. Findings revealed that individuals with FXS avoid social information presented centrally, at least initially. The WS findings, on the other hand, provided some evidence that difficulties with attentional disengagement, rather than attentional capture, may play a role in the WS social phenotype. These findings are discussed in relation to the distinct social phenotypes of these two disorders.

Contributors to Adult Sibling Relationships and Intention to Care of Siblings of Individuals With Down Syndrome.

PubMed

Cuskelly, Monica

2016-05-01

The contribution of childhood sibling relationships to adult sibling relationships and intention to provide care was investigated in a sample in which one member of each dyad had Down syndrome. Thirty-nine adult siblings of an adult with Down syndrome who had participated in a study of sibling relationships in childhood/adolescence provided data about the quality of current relationships and of their intention to provide care for their brother/sister with Down syndrome in the future. Only behavior problems in the child with Down syndrome predicted warmth of the current adult relationship. Although adult sibling relationships were reported to be warm, the quality of neither the current nor the past relationship was associated with the reported intention to provide care.

Associations between maternal and paternal depressive symptoms and early child behavior problems: Testing a mutually adjusted prospective longitudinal model.

PubMed

Narayanan, Martina K; Nærde, Ane

2016-05-15

While there is substantial empirical work on maternal depression, less is known about how mothers' and fathers' depressive symptoms compare in their association with child behavior problems in early childhood. In particular, few studies have examined unique relationships in the postpartum period by controlling for the other parent, or looked at longitudinal change in either parent's depressive symptoms across the first living years as a predictor of child problems. We examined depressive symptoms in parents at 6, 12, 24, 36 and 48 months following childbirth, and child behavior problems at 48 months. Linear growth curve analysis was used to model parents' initial levels and changes in symptoms across time and their associations with child outcomes. Mothers' depressive symptoms at 6 months predicted behavior problems at 48 months for all syndrome scales, while fathers' did not. Estimates for mothers' symptoms were significantly stronger on all subscales. Change in fathers' depressive symptoms over time was a significantly larger predictor of child aggressive behavior than corresponding change in mothers'. No interaction effects between parents' symptoms on behavior problems appeared, and few child gender differences. Child behavior was assessed once precluding tests for bidirectional effects. We only looked at linear change in parental symptoms. Mothers' postpartum depressive symptoms are a stronger predictor for early child behavior problems than fathers'. Change in fathers' depressive symptoms across this developmental period was uniquely and strongly associated with child aggressive problems, and should therefore be addressed in future research and clinical practice. Copyright © 2016 Elsevier B.V. All rights reserved.

Women and AIDS-Related Concerns

PubMed Central

Cochran, Susan D.; Mays, Vickie M.

2014-01-01

Acquired Immunodeficiency Syndrome (AIDS) has important implications for the practice of psychology. As the epidemic continues, the role of behavior change and psychosocial factors in the spread and transmission of Human Immunodeficiency Virus (HIV) infections assumes increasing significance. Psychologists, as behavior change experts, have a special and challenging role to play in educating the public, particularly women, about AIDS. This article examines AIDS- and HIV-related concerns in women with a focus on the personal dilemmas for the practicing psychologist, problems in health behavior advocacy, and methods and pitfalls in modifying sexual behaviors. PMID:2930055

Behavior in Prader-Willi syndrome: relationship to genetic subtypes and age.

PubMed

Dykens, Elisabeth M; Roof, Elizabeth

2008-09-01

Some behavioral features of Prader-Willi syndrome (PWS) are associated with the major genetic subtypes of this disorder. While most agree that those with maternal uniparental disomy (UPD) have a distinctive cognitive and psychiatric profile, findings are more controversial regarding possible differences among persons who vary in paternal deletion size. Caregivers of 88 persons with PWS aged 5 to 51 years (M = 22 years) were administered measures of problem behavior, compulsivity, hyperphagia, and adaptive skills. The sample was well characterized as having relatively large, Type I (n = 26) or smaller, Type II (n = 29) deletions, or UPD (n = 33). No significant behavioral differences were found between the Type I versus Type II deletion groups. Within each genetic subtype, however, differences emerged in how advancing age related to behavior. Although age did not emerge as a significant correlate of behavior in the Type II or UPD groups, in the Type I group age was consistently associated with lower problem behaviors, adaptive skills, and externalizing symptoms. Although differences between deletion subtypes were not found, significant within-subtype differences emerged in relationships between age and behavior. Negative associations between age and behavior in the Type I group only may relate to non-imprinted genes that are deleted in Type I but not Type II cases, including CYFIP1. Altered expression of CYFIP1 is seen in other developmental disabilities, including 15q disorders, and haploinsufficiency of CYFIP1 in Type I PWS cases may be associated with age-related phenotypic effects. Findings underscore the importance of a life-span perspective in phenotypic research.

Childhood internalizing and externalizing problems predict the onset of clinical panic attacks over adolescence: the TRAILS study.

PubMed

Mathyssek, Christina M; Olino, Thomas M; Verhulst, Frank C; van Oort, Floor V A

2012-01-01

Panic attacks are a source of individual suffering and are an independent risk factor for later psychopathology. However, much less is known about risk factors for the development of panic attacks, particularly during adolescence when the incidence of panic attacks increases dramatically. We examined whether internalizing and externalizing problems in childhood predict the onset of panic attacks in adolescence. This study is part of the TRacking Adolescents' Individual Lives Survey (TRAILS), a Dutch longitudinal population cohort study (N = 1,584). Internalizing and Externalizing Problems were collected using the Youth Self-Report (YSR) and the parent-report Child Behavior Checklist (CBCL) at baseline (age 10-12). At age 18-20, DSM-IV defined panic attacks since baseline were assessed with the Composite International Diagnostic Interview (CIDI). We investigated whether early adolescent Internalizing and Externalizing Problems predicted panic attacks between ages 10-20 years, using survival analysis in univariate and multivariate models. There were N = 314 (19.8%) cases who experienced at least one DSM-IV defined panic attack during adolescence and N = 18 (1.2%) who developed panic disorder during adolescence. In univariate analyses, CBCL Total Problems, Internalizing Problems and three of the eight syndrome scales predicted panic attack onset, while on the YSR all broad-band problem scales and each narrow-band syndrome scale predicted panic attack onset. In multivariate analyses, CBCL Social Problems (HR 1.19, p<.05), and YSR Thought Problems (HR 1.15, p<.05) and Social Problems (HR 1.26, p<.01) predicted panic attack onset. Risk indicators of panic attack include the wide range of internalizing and externalizing problems. Yet, when adjusted for co-occurring problem behaviors, Social Problems were the most consistent risk factor for panic attack onsets in adolescence.

Aging in Prader-Willi syndrome: twelve persons over the age of 50 years.

PubMed

Sinnema, Margje; Schrander-Stumpel, Constance T R M; Maaskant, Marian A; Boer, Harm; Curfs, Leopold M G

2012-06-01

The life expectancy of persons with Prader-Willi syndrome (PWS) has increased in recent years. Because of the paucity of reports on older persons with PWS, the natural history, the onset, and type of age-related problems are poorly understood. Twelve persons with a genetically confirmed diagnosis of PWS aged over 50 years are described (4 deletion; 8 mUPD). Data on physical, behavioral, psychiatric, and aging characteristics were collected through semi-structured interviews with the individuals with PWS and their main carers. Cardiovascular diseases, diabetes, dermatological, and orthopedic problems were common physical complaints in older people with PWS. Functioning in activities of daily living, psychological functioning, physical functions, and care dependence were substantially worse in the older age group (50+) compared to the control group (18-49 years). Seven out of eight persons with mUPD had a history of psychiatric illness. Behavioral problems were observed in the older age group. Given the combination of age-related physical morbidity, physical appearance, behavioral and psychiatric problems, and functional decline in our cohort, we hypothesize that premature aging occurs in PWS. The care for older people with PWS requires a lifespan approach that recognizes the presence, progression, and consequences of specific morbidity. Special medical surveillance of people with PWS from 40 years onwards would ensure that intervention and support is offered with respect to specific areas of decline at the earliest possible time. Copyright © 2012 Wiley Periodicals, Inc.

Syndromes of Self-Reported Psychopathology for Ages 18-59 in 29 Societies.

PubMed

Ivanova, Masha Y; Achenbach, Thomas M; Rescorla, Leslie A; Tumer, Lori V; Ahmeti-Pronaj, Adelina; Au, Alma; Maese, Carmen Avila; Bellina, Monica; Caldas, J Carlos; Chen, Yi-Chuen; Csemy, Ladislav; da Rocha, Marina M; Decoster, Jeroen; Dobrean, Anca; Ezpeleta, Lourdes; Fontaine, Johnny R J; Funabiki, Yasuko; Guðmundsson, Halldór S; Harder, Valerie S; de la Cabada, Marie Leiner; Leung, Patrick; Liu, Jianghong; Mahr, Safia; Malykh, Sergey; Maras, Jelena Srdanovic; Markovic, Jasminka; Ndetei, David M; Oh, Kyung Ja; Petot, Jean-Michel; Riad, Geylan; Sakarya, Direnc; Samaniego, Virginia C; Sebre, Sandra; Shahini, Mimoza; Silvares, Edwiges; Simulioniene, Roma; Sokoli, Elvisa; Talcott, Joel B; Vazquez, Natalia; Zasepa, Ewa

2015-06-01

This study tested the multi-society generalizability of an eight-syndrome assessment model derived from factor analyses of American adults' self-ratings of 120 behavioral, emotional, and social problems. The Adult Self-Report (ASR; Achenbach and Rescorla 2003) was completed by 17,152 18-59-year-olds in 29 societies. Confirmatory factor analyses tested the fit of self-ratings in each sample to the eight-syndrome model. The primary model fit index (Root Mean Square Error of Approximation) showed good model fit for all samples, while secondary indices showed acceptable to good fit. Only 5 (0.06%) of the 8,598 estimated parameters were outside the admissible parameter space. Confidence intervals indicated that sampling fluctuations could account for the deviant parameters. Results thus supported the tested model in societies differing widely in social, political, and economic systems, languages, ethnicities, religions, and geographical regions. Although other items, societies, and analytic methods might yield different results, the findings indicate that adults in very diverse societies were willing and able to rate themselves on the same standardized set of 120 problem items. Moreover, their self-ratings fit an eight-syndrome model previously derived from self-ratings by American adults. The support for the statistically derived syndrome model is consistent with previous findings for parent, teacher, and self-ratings of 1½-18-year-olds in many societies. The ASR and its parallel collateral-report instrument, the Adult Behavior Checklist (ABCL), may offer mental health professionals practical tools for the multi-informant assessment of clinical constructs of adult psychopathology that appear to be meaningful across diverse societies.

5p13 microduplication syndrome: a new case and better clinical definition of the syndrome.

PubMed

Novara, Francesca; Alfei, Enrico; D'Arrigo, Stefano; Pantaleoni, Chiara; Beri, Silvana; Achille, Valentina; Sciacca, Francesca L; Giorda, Roberto; Zuffardi, Orsetta; Ciccone, Roberto

2013-01-01

Chromosome 5p13 duplication syndrome (OMIM #613174), a contiguous gene syndrome involving duplication of several genes on chromosome 5p13 including NIPBL (OMIM 608667), has been described in rare patients with developmental delay and learning disability, behavioral problems and peculiar facial dysmorphisms. 5p13 duplications described so far present with variable sizes, from 0.25 to 13.6 Mb, and contain a variable number of genes. Here we report another patient with 5p13 duplication syndrome including NIPBL gene only. Proband's phenotype overlapped that reported in patients with 5p13 microduplication syndrome and especially that of subjects with smaller duplications. Moreover, we better define genotype-phenotype relationship associated with this duplication and confirmed that NIPBL was likely the major dosage sensitive gene for the 5p13 microduplication phenotype. Copyright © 2012 Elsevier Masson SAS. All rights reserved.

Prader-Willi syndrome as a model of human hyperphagia.

PubMed

Tauber, Maithe; Diene, Gwenaelle; Mimoun, Emmanuelle; Çabal-Berthoumieu, Sophie; Mantoulan, Carine; Molinas, Catherine; Muscatelli, F; Salles, Jean Pierre

2014-01-01

Prader-Willi syndrome (PWS), first described in 1956, is considered as a paradigm of a neurodevelopmental disorder with severe and early obesity with hyperphagia and impaired satiety. The improved knowledge in the natural history and recent data on genetics offer new perspectives for understanding the metabolic and endocrine dysfunctions and possibly for treatment. Natural history of the disease has been described due to the early diagnosis performed in the first months of life and various nutritional phases have been described. In addition, there is clear evidence that the abnormal feeding behavior is included in the behavioral problems. Brain imaging studies have shown that some brain regions may be important in PWS. The role of SNORD116 gene cluster is detailed and its links with circadian rhythm and brain and hypothalamus development. Pathophysiology of the abnormal ghrelin levels and of OT dysfunction is documented. While no effect on appetite and weight regulation has been reported with ghrelin antagonists, OT has been shown to improve some of the behavioral problems in adults. We discuss our hypothesis of an abnormal ghrelin/OT/dopamine pathway which may explain the switch of nutritional phases and behavior. These new aspects offer an opportunity for therapeutic use and possible early intervention. © 2014 S. Karger AG, Basel.

Syndromes of collateral-reported psychopathology for ages 18-59 in 18 Societies

PubMed Central

Ivanova, Masha Y.; Achenbach, Thomas M.; Rescorla, Leslie A.; Turner, Lori V.; Árnadóttir, Hervör Alma; Au, Alma; Caldas, J. Carlos; Chaalal, Nebia; Chen, Yi Chuen; da Rocha, Marina M.; Decoster, Jeroen; Fontaine, Johnny R.J.; Funabiki, Yasuko; Guðmundsson, Halldór S.; Kim, Young Ah; Leung, Patrick; Liu, Jianghong; Malykh, Sergey; Marković, Jasminka; Oh, Kyung Ja; Petot, Jean-Michel; Samaniego, Virginia C.; Silvares, Edwiges Ferreira de Mattos; Šimulionienė, Roma; Šobot, Valentina; Sokoli, Elvisa; Sun, Guiju; Talcott, Joel B.; Vázquez, Natalia; Zasępa, Ewa

2017-01-01

The purpose was to advance research and clinical methodology for assessing psychopathology by testing the international generalizability of an 8-syndrome model derived from collateral ratings of adult behavioral, emotional, social, and thought problems. Collateral informants rated 8,582 18–59-year-old residents of 18 societies on the Adult Behavior Checklist (ABCL). Confirmatory factor analyses tested the fit of the 8-syndrome model to ratings from each society. The primary model fit index (Root Mean Square Error of Approximation) showed good model fit for all societies, while secondary indices (Tucker Lewis Index, Comparative Fit Index) showed acceptable to good fit for 17 societies. Factor loadings were robust across societies and items. Of the 5,007 estimated parameters, 4 (0.08%) were outside the admissible parameter space, but 95% confidence intervals included the admissible space, indicating that the 4 deviant parameters could be due to sampling fluctuations. The findings are consistent with previous evidence for the generalizability of the 8-syndrome model in self-ratings from 29 societies, and support the 8-syndrome model for operationalizing phenotypes of adult psychopathology from multi-informant ratings in diverse societies. PMID:29399019

Behavior problems in children at time of first recognized seizure and changes over the following 3 years.

PubMed

Austin, J K; Perkins, S M; Johnson, C S; Fastenau, P S; Byars, A W; deGrauw, T J; Dunn, D W

2011-08-01

The purposes of this 36-month study of children with first recognized seizures were: (1) to describe baseline differences in behavior problems between children with and without prior unrecognized seizures; (2) to identify differences over time in behavior problems between children with seizures and their healthy siblings; (3) to identify the proportions of children with seizures and healthy siblings who were consistently at risk for behavior problems for 36 months; and (4) to identify risk factors for behavior problems 36 months following the first recognized seizure. Risk factors explored included demographic (child age and gender, caregiver education), neuropsychological (IQ, processing speed), seizure (epileptic syndrome, use of antiepileptic drug, seizure recurrence), and family (family mastery, satisfaction with family relationships, parent response) variables. Participants were 300 children aged 6 through 14 years with a first recognized seizure and 196 healthy siblings. Data were collected from medical records, structured interviews, self-report questionnaires, and neuropsychological testing. Behavior problems were measured using the Child Behavior Checklist and the Teacher's Report Form. Data analyses included descriptive statistics and linear mixed models. Children with prior unrecognized seizures were at higher risk for behavior problems at baseline. As a group, children with seizures showed a steady reduction in behavior problems over time. Children with seizures were found to have significantly more behavior problems than their siblings over time, and significantly more children with seizures (11.3%) than siblings (4.6%) had consistent behavior problems over time. Key risk factors for child behavior problems based on both caregivers and teachers were: less caregiver education, slower initial processing speed, slowing of processing speed over the first 36 months, and a number of family variables including lower levels of family mastery or child satisfaction with family relationships, lower parent support of the child's autonomy, and lower parent confidence in their ability to discipline their child. Children with new-onset seizures who are otherwise developing normally have higher rates of behavior problems than their healthy siblings; however, behavior problems are not consistently in the at-risk range in most children during the first 3 years after seizure onset. When children show behavior problems, family variables that might be targeted include family mastery, parent support of child autonomy, and parents' confidence in their ability to handle their children's behavior.

Reliability and Validity of an Interviewer-Administered Adaptation of the Youth Self-Report for Mental Health Screening of Vulnerable Young People in Ethiopia.

PubMed

Geibel, Scott; Habtamu, Kassahun; Mekonnen, Gebeyehu; Jani, Nrupa; Kay, Lynnette; Shibru, Julyata; Bedilu, Lake; Kalibala, Samuel

2016-01-01

Evaluate the reliability and validity of the Youth Self-Report (YSR) as a screening tool for mental health problems among young people vulnerable to HIV in Ethiopia. A cross-sectional assessment of young people currently receiving social services. Young people age 15-18 participated in a study where a translated and adapted version of the YSR was administered by trained nurses, followed by an assessment by Ethiopian psychiatrists. Internal reliability of YSR syndrome scales were assessed using Chronbach's alpha. Test-retest reliability was assessed through repeating the YSR one month later. To assess validity, analysis of the sensitivity and specificity of the YSR compared to the psychiatrist assessment was conducted. Across the eight syndrome scales, the YSR best measured the diagnosis of anxiety/depression and social problems among young women, and attention problems among young men. Among individual YSR syndrome scales, internal reliability ranged from unacceptable (Chronback's alpha = 0.11, rule-breaking behavior among young women) to good (α≥0.71, anxiety/depression among young women). Anxiety/depression scores of ≥8.5 among young women also had good sensitivity (0.833) and specificity (0.754) to predict a true diagnosis. The YSR syndrome scales for social problems among young women and attention problems among young men also had fair consistency and validity measurements. Most YSR scores had significant positive correlations between baseline and post-one month administration. Measures of reliability and validity for most other YSR syndrome scales were fair to poor. The adapted, personally administered, Amharic version of the YSR has sufficient reliability and validity in identifying young vulnerable women with anxiety/depression and/or social problems, and young men with attention problems; which were the most common mental health disorders observed by psychiatrists among the migrant populations in this study. Further assessment of the applicability of the YSR among vulnerable young people for less common disorders in Ethiopia is needed.

Velo-Cardio-Facial Syndrome: 30 Years of Study

PubMed Central

Shprintzen, Robert J.

2009-01-01

Velo-cardio-facial syndrome is one of the names that has been attached to one of the most common multiple anomaly syndromes in humans. The labels DiGeorge sequence, 22q11 deletion syndrome, conotruncal anomalies face syndrome, CATCH 22, and Sedlačková syndrome have all been attached to the same disorder. Velo-cardio-facial syndrome has an expansive phenotype with more than 180 clinical features described that involve essentially every organ and system. The syndrome has drawn considerable attention because a number of common psychiatric illnesses are phenotypic features including attention deficit disorder, schizophrenia, and bipolar disorder. The expression is highly variable with some individuals being essentially normal at the mildest end of the spectrum, and the most severe cases having life-threatening and life-impairing problems. The syndrome is caused by a microdeletion from chromosome 22 at the q11.2 band. Although the large majority of affected individuals have identical 3 megabase deletions, less than 10% of cases have smaller deletions of 1.5 or 2.0 megabases. The 3 megabase deletion encompasses a region containing 40 genes. The syndrome has a population prevalence of approximately 1:2,000 in the U.S., although incidence is higher. Although initially a clinical diagnosis, today velo-cardio-facial syndrome can be diagnosed with extremely high accuracy by fluorescence in situ hybridization (FISH) and several other laboratory techniques. Clinical management is age dependent with acute medical problems such as congenital heart disease, immune disorders, feeding problems, cleft palate, and developmental disorders occupying management in infancy and preschool years. Management shifts to cognitive, behavioral, and learning disorders during school years, and then to the potential for psychiatric disorders including psychosis in late adolescence and adult years. Although the majority of people with velo-cardio-facial syndrome do not develop psychosis, the risk for severe psychiatric illness is 25 times higher for people affected with velo-cardio-facial syndrome than the general population. Therefore, interest in understanding the nature of psychiatric illness in the syndrome remains strong. PMID:18636631

SATB2-associated syndrome: Mechanisms, phenotype, and practical recommendations.

PubMed

Zarate, Yuri A; Fish, Jennifer L

2017-02-01

The SATB2-associated syndrome is a recently described syndrome characterized by developmental delay/intellectual disability with absent or limited speech development, craniofacial abnormalities, behavioral problems, dysmorphic features, and palatal and dental abnormalities. Alterations of the SATB2 gene can result from a variety of different mechanisms that include contiguous deletions, intragenic deletions and duplications, translocations with secondary gene disruption, and point mutations. The multisystemic nature of this syndrome demands a multisystemic approach and we propose evaluation and management guidelines. The SATB2-associated syndrome registry has now been started and that will allow gathering further clinical information and refining the provided surveillance recommendations. © 2016 The Authors. American Journal of Medical Genetics Part A Published by Wiley Periodicals, Inc. © 2016 The Authors. American Journal of Medical Genetics Part A Published by Wiley Periodicals, Inc.

Effects of Adenotonsillectomy on Parent-Reported Behavior in Children With Obstructive Sleep Apnea

PubMed Central

Thomas, Nina Hattiangadi; Xanthopoulos, Melissa S.; Kim, Ji Young; Shults, Justine; Escobar, Emma; Giordani, Bruno; Hodges, Elise; Chervin, Ronald D.; Paruthi, Shalini; Rosen, Carol L.; Taylor, Gerry H.; Arens, Raanan; Katz, Eliot S.; Beebe, Dean W.; Redline, Susan; Radcliffe, Jerilynn

2017-01-01

Abstract Objectives: The childhood obstructive sleep apnea syndrome (OSAS) is associated with behavioral abnormalities. Studies on the effects of OSAS treatment on behavior are conflicting, with few studies using a randomized design. Further, studies may be confounded by the inclusion of behavioral outcome measures directly related to sleep. The objective of this study was to determine the effect of adenotonsillectomy on behavior in children with OSAS. We hypothesized that surgery would improve behavioral ratings, even when sleep symptom items were excluded from the analysis. Methods: This was a secondary analysis of Child Behavior Checklist (CBCL) data, with and without exclusion of sleep-specific items, from the Childhood Adenotonsillectomy Trial (CHAT). CBCL was completed by caregivers of 380 children (7.0+1.4 [range 5–9] years) with OSAS randomized to early adenotonsillectomy (eAT) versus 7 months of watchful waiting with supportive care (WWSC). Results: There was a high prevalence of behavioral problems at baseline; 16.6% of children had a Total Problems score in the clinically abnormal range. At follow-up, there were significant improvements in Total Problems (p < .001), Internalizing Behaviors (p = .04), Somatic Complaints (p = .01), and Thought Problems (p = .01) in eAT vs. WWSC participants. When specific sleep-related question items were removed from the analysis, eAT showed an overall improvement in Total (p = .02) and Other (p = .01) problems. Black children had less improvement in behavior following eAT than white children, but this difference attenuated when sleep-related items were excluded. Conclusions: This large, randomized trial showed that adenotonsillectomy for OSAS improved parent-rated behavioral problems, even when sleep-specific behavioral issues were excluded from the analysis. PMID:28199697

Effects of Adenotonsillectomy on Neurocognitive Function in Pediatric Obstructive Sleep Apnea Syndrome

PubMed Central

Horiuchi, Fumie; Oka, Yasunori; Komori, Kenjiro; Tokui, Yasumasa; Matsumoto, Teruhisa; Ueno, Shu-ichi

2014-01-01

Obstructive sleep apnea syndrome (OSAS) in children does not only present with symptoms of sleep disturbances but also with associated symptoms such as growth failure, enuresis, academic learning difficulties, and behavioral problems, including attention deficit/hyperactivity disorder- (ADHD-) like symptoms. We evaluated neurocognitive functions before and after adenotonsillectomy in a patient with OSAS. An 11-year-old boy suspected of having ADHD with nocturnal enuresis was referred for evaluation. He was found to have adenotonsillar hypertrophy. Presence of snoring was evident only after detailed medical interview. Polysomnography confirmed the diagnosis of OSAS, which was subsequently treated by adenotonsillectomy. The apnea/hypopnea index decreased from 21.9 at baseline to 1.8 after surgery, and the frequency of enuresis fell from almost nightly to 2-3 times per month. Neurocognitive and behavioral assessment after the treatment of OSAS showed significant improvement in cognitive functions, especially attention capacity and considerable amelioration of behavioral problems including ADHD-like symptoms. As the most common cause of pediatric OSAS is adenotonsillar hypertrophy, medical interview and oropharyngeal examination should always be performed in children suspected of having ADHD. The necessity of sleep evaluation for children with ADHD-like symptoms was also emphasized. PMID:24971187

Mental Illness in Persons with Mental Retardation: ARC Facts.

ERIC Educational Resources Information Center

Weber, Linda R.; Wimmer, Sharon

This brief factsheet presents information on mental illness in mentally retarded persons. The most prevalent disorders found in this population are schizophrenia, organic brain syndrome, adjustment disorders, personality disorders, depression, and behavioral problems. Few standardized methods of assessment exist for the diagnosis of mental illness…

Clinical management of poor adherence to CPAP: motivational enhancement.

PubMed

Aloia, Mark S; Arnedt, J Todd; Riggs, Raine L; Hecht, Jacki; Borrelli, Belinda

2004-01-01

Adherence to continuous positive airway pressure (CPAP) in patients with sleep apnea hypopnea syndrome (SAHS) is poor. Previous studies have attempted to identify specific barriers to treatment, but none has identified the sole cause for the problem. We outline a behavioral approach to the problem of CPAP adherence that is based on the theories of the transtheoretical model and social cognitive theory. We used these theories to guide the development of an intervention based on the methods of motivational interviewing. We present our motivational enhancement therapy for CPAP (ME-CPAP) here, with some brief pilot data to show its efficacy. Finally, we outline some strengths and weaknesses of taking a behavior change approach to the problem of poor CPAP adherence.

Phenotypic and Molecular Convergence of 2q23.1 Deletion Syndrome with Other Neurodevelopmental Syndromes Associated with Autism Spectrum Disorder

PubMed Central

Mullegama, Sureni V.; Alaimo, Joseph T.; Chen, Li; Elsea, Sarah H.

2015-01-01

Roughly 20% of autism spectrum disorders (ASD) are syndromic with a well-established genetic cause. Studying the genes involved can provide insight into the molecular and cellular mechanisms of ASD. 2q23.1 deletion syndrome (causative gene, MBD5) is a recently identified genetic neurodevelopmental disorder associated with ASD. Mutations in MBD5 have been found in ASD cohorts. In this study, we provide a phenotypic update on the prevalent features of 2q23.1 deletion syndrome, which include severe intellectual disability, seizures, significant speech impairment, sleep disturbance, and autistic-like behavioral problems. Next, we examined the phenotypic, molecular, and network/pathway relationships between nine neurodevelopmental disorders associated with ASD: 2q23.1 deletion Rett, Angelman, Pitt-Hopkins, 2q23.1 duplication, 5q14.3 deletion, Kleefstra, Kabuki make-up, and Smith-Magenis syndromes. We show phenotypic overlaps consisting of intellectual disability, speech delay, seizures, sleep disturbance, hypotonia, and autistic-like behaviors. Molecularly, MBD5 possibly regulates the expression of UBE3A, TCF4, MEF2C, EHMT1 and RAI1. Network analysis reveals that there could be indirect protein interactions, further implicating function for these genes in common pathways. Further, we show that when MBD5 and RAI1 are haploinsufficient, they perturb several common pathways that are linked to neuronal and behavioral development. These findings support further investigations into the molecular and pathway relationships among genes linked to neurodevelopmental disorders and ASD, which will hopefully lead to common points of regulation that may be targeted toward therapeutic intervention. PMID:25853262

Parenting Stress and Emotional/Behavioral Problems in Adolescents with Primary Headache.

PubMed

Operto, Francesca Felicia; Craig, Francesco; Peschechera, Antonia; Mazza, Roberta; Lecce, Paola Alessandra; Margari, Lucia

2017-01-01

Primary headache is a frequent and disabling disorder, common among children and adolescents, and it is a painful syndrome often accompanied by functional impairment and associated with emotional and behavior problems. The aim of this study was to investigate parenting stress and emotional/behavioral problems in adolescents affected by primary headache compared with healthy adolescents. The study population consisted of 35 adolescents and a control group of 23 healthy subjects. The assessment included the administration of clinical standardized scales such as Parent Stress Index-Short Form, Pediatric Migraine Disability Assessment Score Questionnaire, and Child Behavior Checklist (CBCL). Headache group and control group did not differ in terms of parenting stress ( p  = 0.29). On the contrary, headache group showed more internalizing problems ( p  = 0.023), affective problems ( p  = 0.01), anxious ( p  = 0.001), and somatic complaints ( p  < 0.001) compared with control group. In addition, we found a significant correlation between PSI domains and specific CBCL subscales in the headache group. The findings emphasize the need for expanded intervention in the clinical treatment of pediatric headache, a treatment that may also include the family members. Further research is needed.

Treatments for fragile X syndrome: a closer look at the data.

PubMed

Hall, Scott S

2009-01-01

Research into the determinants and developmental course of fragile X syndrome (FXS) has made remarkable progress over the last 25 years. However, treatments to ameliorate the symptoms of FXS have been less forthcoming. While there is optimism in the field that the pace of intervention research is quickening, there has been a bias toward psychopharmacological approaches to treatment. A closer look at the data from those investigations reveals a paucity of evidence that medications can improve intellectual and adaptive functioning in FXS, or decrease associated behavioral and/or emotional issues. Work in other related disorders (e.g., autism) has shown that dramatic improvements in intellectual and adaptive functioning, as well as behavioral and emotional problems, can occur if intensive behavioral treatment is begun early in the child's life. It is hoped that future research efforts will evaluate these intensive early intervention strategies in children with FXS, perhaps in combination with pharmacological approaches.

Williams Syndrome and 15q Duplication: Coincidence versus Association.

PubMed

Khokhar, Aditi; Agarwal, Swashti; Perez-Colon, Sheila

2017-01-01

Williams syndrome is a multisystem disorder caused by contiguous gene deletion in 7q11.23, commonly associated with distinctive facial features, supravalvular aortic stenosis, short stature, idiopathic hypercalcemia, developmental delay, joint laxity, and a friendly personality. The clinical features of 15q11q13 duplication syndrome include autism, mental retardation, ataxia, seizures, developmental delay, and behavioral problems. We report a rare case of a girl with genetically confirmed Williams syndrome and coexisting 15q duplication syndrome. The patient underwent treatment for central precocious puberty and later presented with primary amenorrhea. The karyotype revealed 47,XX,+mar. FISH analysis for the marker chromosome showed partial trisomy/tetrasomy for proximal chromosome 15q (15p13q13). FISH using an ELN -specific probe demonstrated a deletion in the Williams syndrome critical region in 7q11.23. To our knowledge, a coexistence of Williams syndrome and 15q duplication syndrome has not been reported in the literature. Our patient had early pubertal development, which has been described in some patients with Williams syndrome. However, years later after discontinuing gonadotropin-releasing hormone analogue treatment, she developed primary amenorrhea.

Comparison of ADHD Symptom Subtypes as Source-Specific Syndromes

ERIC Educational Resources Information Center

Gadow, Kenneth D.; Drabick, Deborah A. G.; Loney, Jan; Sprafkin, Joyce; Salisbury, Helen; Azizian, Allen; Schwartz, Joseph

2004-01-01

Background: This study examines differences between the three subtypes of attention-deficit/hyperactivity disorder (ADHD), inattentive (I), hyperactive-impulsive (H), and combined (C), in a heterogeneous sample of 248 boys (ages 6 to 10 years) with emotional and behavioral problems who were recruited for participation in a diagnostic study.…

From aggression to autism: new perspectives on the behavioral sequelae of monoamine oxidase deficiency.

PubMed

Bortolato, Marco; Floris, Gabriele; Shih, Jean C

2018-05-10

The two monoamine oxidase (MAO) enzymes, A and B, catalyze the metabolism of monoamine neurotransmitters, such as serotonin, norepinephrine, and dopamine. The phenotypic outcomes of MAO congenital deficiency have been studied in humans and animal models, to explore the role of these enzymes in behavioral regulation. The clinical condition caused by MAOA deficiency, Brunner syndrome, was first described as a disorder characterized by overt antisocial and aggressive conduct. Building on this discovery, subsequent studies were focused on the characterization of the role of MAOA in the neurobiology of antisocial conduct. MAO A knockout mice were found to display high levels of intermale aggression; however, further analyses of these mutants unveiled additional behavioral abnormalities mimicking the core symptoms of autism-spectrum disorder. These findings were strikingly confirmed in newly reported cases of Brunner syndrome. The role of MAOB in behavioral regulation remains less well-understood, even though Maob-deficient mice have been found to exhibit greater behavioral disinhibition and risk-taking responses, supporting previous clinical studies showing associations between low MAO B activity and impulsivity. Furthermore, lack of MAOB was found to exacerbate the severity of psychopathological deficits induced by concurrent MAOA deficiency. Here, we summarize how the convergence of clinical reports and behavioral phenotyping in mutant mice has helped frame a complex picture of psychopathological features in MAO-deficient individuals, which encompass a broad spectrum of neurodevelopmental problems. This emerging knowledge poses novel conceptual challenges towards the identification of the endophenotypes shared by autism-spectrum disorder, antisocial behavior and impulse-control problems, as well as their monoaminergic underpinnings.

Syndromes of Self-Reported Psychopathology for Ages 18–59 in 29 Societies

PubMed Central

Achenbach, Thomas M.; Rescorla, Leslie A.; Tumer, Lori V.; Ahmeti-Pronaj, Adelina; Au, Alma; Maese, Carmen Avila; Bellina, Monica; Caldas, J. Carlos; Chen, Yi-Chuen; Csemy, Ladislav; da Rocha, Marina M.; Decoster, Jeroen; Dobrean, Anca; Ezpeleta, Lourdes; Fontaine, Johnny R. J.; Funabiki, Yasuko; Guðmundsson, Halldór S.; Harder, Valerie s; de la Cabada, Marie Leiner; Leung, Patrick; Liu, Jianghong; Mahr, Safia; Malykh, Sergey; Maras, Jelena Srdanovic; Markovic, Jasminka; Ndetei, David M.; Oh, Kyung Ja; Petot, Jean-Michel; Riad, Geylan; Sakarya, Direnc; Samaniego, Virginia C.; Sebre, Sandra; Shahini, Mimoza; Silvares, Edwiges; Simulioniene, Roma; Sokoli, Elvisa; Talcott, Joel B.; Vazquez, Natalia; Zasepa, Ewa

2017-01-01

This study tested the multi-society generalizability of an eight-syndrome assessment model derived from factor analyses of American adults’ self-ratings of 120 behavioral, emotional, and social problems. The Adult Self-Report (ASR; Achenbach and Rescorla 2003) was completed by 17,152 18–59-year-olds in 29 societies. Confirmatory factor analyses tested the fit of self-ratings in each sample to the eight-syndrome model. The primary model fit index (Root Mean Square Error of Approximation) showed good model fit for all samples, while secondary indices showed acceptable to good fit. Only 5 (0.06%) of the 8,598 estimated parameters were outside the admissible parameter space. Confidence intervals indicated that sampling fluctuations could account for the deviant parameters. Results thus supported the tested model in societies differing widely in social, political, and economic systems, languages, ethnicities, religions, and geographical regions. Although other items, societies, and analytic methods might yield different results, the findings indicate that adults in very diverse societies were willing and able to rate themselves on the same standardized set of 120 problem items. Moreover, their self-ratings fit an eight-syndrome model previously derived from self-ratings by American adults. The support for the statistically derived syndrome model is consistent with previous findings for parent, teacher, and self-ratings of 1½–18-year-olds in many societies. The ASR and its parallel collateral-report instrument, the Adult Behavior Checklist (ABCL), may offer mental health professionals practical tools for the multi-informant assessment of clinical constructs of adult psychopathology that appear to be meaningful across diverse societies. PMID:29805197

Prader-Willi-like phenotype: investigation of 1p36 deletion in 41 patients with delayed psychomotor development, hypotonia, obesity and/or hyperphagia, learning disabilities and behavioral problems.

PubMed

D'Angelo, Carla S; Da Paz, José A; Kim, Chong A; Bertola, Débora R; Castro, Claudia I E; Varela, Monica C; Koiffmann, Célia P

2006-01-01

Monosomy 1p36 is one of the most commonly observed mental retardation (MR) syndromes that results in a clinically recognizable phenotype including delayed psychomotor development and/or MR, hypotonia, epilepsy, hearing loss, growth delay, microcephaly, deep-set eyes, flat nasal bridge and pointed chin. Besides, a Prader-Willi syndrome (PWS)-like phenotype has been described in patients with 1p36 monosomy. Forty-one patients presenting hypotonia, developmental delay, obesity and/or hyperphagia and behavioral problems who tested negative for PWS were investigated by FISH and/or microsatellite markers. Twenty-six were analyzed with a 1p-specific subtelomeric probe, and one terminal deletion was identified. Thirty patients (15 of which also studied by FISH) were investigated by microsatellite markers, and no interstitial 1p36 deletion was found. Our patient presenting the 1p36 deletion did not have the striking features of this monosomy, but her clinical and behavioral features were quite similar to those observed in patients with PWS, except for the presence of normal sucking at birth. The extent of the deletion could be limited to the most terminal 2.5 Mb of 1p36, within the chromosomal region 1p36.33-1p36.32, that is smaller than usually seen in monosomy 1p36 patients. Therefore, chromosome 1p36.33 deletion should be investigated in patients with hypotonia, developmental delay, obesity and/or hyperphagia and behavioral problems who test negative for PWS.

Increase in child behavior problems among urban Brazilian 4-year olds: 1993 and 2004 Pelotas birth cohorts

PubMed Central

Matijasevich, Alicia; Murray, Elizabeth; Stein, Alan; Anselmi, Luciana; Menezes, Ana M; Santos, Iná S; Barros, Aluísio JD; Gigante, Denise P; Barros, Fernando C; Victora, Cesar G

2014-01-01

Background There are an increasing number of reports on time trends in child and adolescent psychological problems but none from low- and middle-income countries, and very few covering the preschool period. The aim was to investigate changes in preschool behavioral/emotional problems in two birth cohorts from a middle-income country born 11 years apart. Methods We analyzed data from the 1993 and 2004 Pelotas birth cohort studies from Brazil. A subsample of 4-year olds from the 1993 cohort (634) and all 4-year olds from the 2004 cohort (3750) were assessed for behavioral/emotional problems through maternal report using the Child Behavior Checklist (CBCL). Response rates in these two population-based cohorts were above 90%. Results We found a significant increase in CBCL total problems, internalizing and externalizing mean scores over the 11-year period. For 1993 and 2004 Pelotas cohorts, respectively, CBCL mean values (SE) total problems scores were 27.9 (0.8) and 34.7 (0.3); for internalizing scores, 5.7 (0.2) and 6.3 (0.1) and for externalizing scores, 12.4 (0.4) and 15.5 (0.1). After adjusting for confounding variables, the largest increase from 1993 to 2004 was identified in the aggressive behavior syndrome score (Cohen's d = .50), followed by the externalizing problem score (Cohen's d = .40) and CBCL total problem score (Cohen's d = .36), respectively. The rise in child psychological problems was more marked in children from families with fewer assets and with less educated mothers. Conclusions Our findings provide evidence for a substantial increase in preschool behavioral problems among children in Brazil over an 11-year period. PMID:24735354

International comparisons of behavioral and emotional problems in preschool children: parents' reports from 24 societies.

PubMed

Rescorla, Leslie A; Achenbach, Thomas M; Ivanova, Masha Y; Harder, Valerie S; Otten, Laura; Bilenberg, Niels; Bjarnadottir, Gudrun; Capron, Christiane; De Pauw, Sarah S W; Dias, Pedro; Dobrean, Anca; Döpfner, Manfred; Duyme, Michel; Eapen, Valsamma; Erol, Nese; Esmaeili, Elaheh Mohammad; Ezpeleta, Lourdes; Frigerio, Alessandra; Fung, Daniel S S; Gonçalves, Miguel; Guðmundsson, Halldór; Jeng, Suh-Fang; Jusiené, Roma; Ah Kim, Young; Kristensen, Solvejg; Liu, Jianghong; Lecannelier, Felipe; Leung, Patrick W L; Machado, Bárbara César; Montirosso, Rosario; Ja Oh, Kyung; Ooi, Yoon Phaik; Plück, Julia; Pomalima, Rolando; Pranvera, Jetishi; Schmeck, Klaus; Shahini, Mimoza; Silva, Jaime R; Simsek, Zeynep; Sourander, Andre; Valverde, José; van der Ende, Jan; Van Leeuwen, Karla G; Wu, Yen-Tzu; Yurdusen, Sema; Zubrick, Stephen R; Verhulst, Frank C

2011-01-01

International comparisons were conducted of preschool children's behavioral and emotional problems as reported on the Child Behavior Checklist for Ages 1½-5 by parents in 24 societies (N = 19,850). Item ratings were aggregated into scores on syndromes; Diagnostic and Statistical Manual of Mental Disorders-oriented scales; a Stress Problems scale; and Internalizing, Externalizing, and Total Problems scales. Effect sizes for scale score differences among the 24 societies ranged from small to medium (3-12%). Although societies differed greatly in language, culture, and other characteristics, Total Problems scores for 18 of the 24 societies were within 7.1 points of the omnicultural mean of 33.3 (on a scale of 0-198). Gender and age differences, as well as gender and age interactions with society, were all very small (effect sizes < 1%). Across all pairs of societies, correlations between mean item ratings averaged .78, and correlations between internal consistency alphas for the scales averaged .92, indicating that the rank orders of mean item ratings and internal consistencies of scales were very similar across diverse societies.

International Comparisons of Behavioral and Emotional Problems in Preschool Children: Parents’ Reports From 24 Societies

PubMed Central

Rescorla, Leslie A.; Achenbach, Thomas M.; Ivanova, Masha Y.; Harder, Valerie S.; Otten, Laura; Bilenberg, Niels; Bjarnadottir, Gudrun; Capron, Christiane; De Pauw, Sarah S. W.; Dias, Pedro; Dobrean, Anca; Döpfner, Manfred; Duyme, Michel; Eapen, Valsamma; Erol, Nese; Esmaeili, Elaheh Mohammad; Ezpeleta, Lourdes; Frigerio, Alessandra; Fung, Daniel S. S.; Gonçalves, Miguel; Guđmundsson, Halldór; Jeng, Suh-Fang; Jusiené, Roma; Kim, Young Ah; Kristensen, Solvejg; Liu, Jianghong; Lecannelier, Felipe; Leung, Patrick W. L.; Machado, Bárbara César; Montirosso, Rosario; Oh, Kyung Ja; Ooi, Yoon Phaik; Plück, Julia; Pomalima, Rolando; Pranvera, Jetishi; Schmeck, Klaus; Shahini, Mimoza; Silva, Jaime R.; Simsek, Zeynep; Sourander, Andre; Valverde, José; van der Ende, Jan; Van Leeuwen, Karla G.; Wu, Yen-Tzu; Yurdusen, Sema; Zubrick, Stephen R.; Verhulst, Frank C.

2014-01-01

International comparisons were conducted of preschool children’s behavioral and emotional problems as reported on the Child Behavior Checklist for Ages 1½–5 by parents in 24 societies (N =19,850). Item ratings were aggregated into scores on syndromes; Diagnostic and Statistical Manual of Mental Disorders–oriented scales; a Stress Problems scale; and Internalizing, Externalizing, and Total Problems scales. Effect sizes for scale score differences among the 24 societies ranged from small to medium (3–12%). Although societies differed greatly in language, culture, and other characteristics, Total Problems scores for 18 of the 24 societies were within 7.1 points of the omnicultural mean of 33.3 (on a scale of 0–198). Gender and age differences, as well as gender and age interactions with society, were all very small (effect sizes <1%). Across all pairs of societies, correlations between mean item ratings averaged .78, and correlations between internal consistency alphas for the scales averaged .92, indicating that the rank orders of mean item ratings and internal consistencies of scales were very similar across diverse societies. PMID:21534056

Prader-Willi syndrome: a case report with atypical developmental features.

PubMed

Sewaybricker, Letícia E; Guaragna-Filho, Guilherme; Paula, Georgette B; Andrade, Juliana G R; Tincani, Bruna J; D'Souza-Li, Lília; Lemos-Marini, Sofia H V; Maciel-Guerra, Andréa T; Guerra-Júnior, Gil

2014-09-01

To describe the case of a male Prader-Willi syndrome (PWS) patient with atypical development features. We report the case of a male adolescent with confirmed diagnosis of PWS which presents atypical phenotype. The patient progressed with spontaneous and complete pubertal development, stature in the normal range, and weight control without any pharmacological treatment, except metformin. PWS is an imprinting paternally inherited disorder of 15q11-13 characterized by hypotonia in infant age, hyperphagia, varied degrees of mental retardation, behavior problems, hypogonadism, short stature, and other less common findings.

Reintegrating Troops with Mild Traumatic Brain Injury (mTBI) into their Communities: Understanding the Scope and Timeline of Post-Deployment Driving Problems

DTIC Science & Technology

2015-10-01

behaviors and anxieties among post- deployed SMs with and without traumatic brain injury (TBI), post-traumatic stress syndrome (PTSD) or TBI with...post- traumatic stress syndrome (TBI/PTSD). The goal was to compare SMs who were post-deployment to SMs who had not served in OEF/OIF/OND, however all...in situations when SM would typically drive (p=.02) with TBI/PTSD reporting this more common than TBI and 0Dx. • Move to middle of road or onto

Treatment of Tourette syndrome.

PubMed

Kurlan, Roger M

2014-01-01

Tourette's syndrome (TS) consists of chronic motor and phonic tics and characteristically begins in childhood. The tics can be disabling and commonly associated behavioral comorbities such as attention deficit hyperactivity disorder (ADHD) and obsessive-compulsive disorder (OCD), can also cause problems in daily functioning. The underlying etiology and neurobiology of TS remain unknown although genetic factors appear to be important, cortical control of basal ganglia motor function appears to be disturbed and neurochemical abnormalities, particularly involving dopamine neurotransmission, are likely present. The treatment of TS involves appropriate education and support. Tics can be treated with habit reversal cognitive behavioral therapy, medications (most commonly alpha agonists and antipsychotics), local intramuscular injections of botulinum toxin and some severe, refractory cases have responded to deep brain stimulation surgery (DBS). It is important to appropriately diagnose and treat comorbid behavioral disorders that are disrupting function. OCD can be treated with cognitive behavioral therapy, selective serotonin reuptake inhibitors, and atypical antipsychotics. DBS has become a treatment option for patients with disabling OCD despite other therapies. ADHD is treated with appropriate classroom accommodations, behavioral therapy, alpha agonists, atomoxetine or methylphenidate-containing stimulant drugs.

[Schizophrenia--a life shortening disease].

PubMed

Munitz, Hanan

2010-08-01

The life expectancy of schizophrenic patients is shortened by about 20%. This alarming finding is mainly caused by an increased rate of the metabolic syndrome which is inherent in schizophrenic patients and is increased by the use of antipsychotic drugs, mainly second generation drugs. Another reason is a high rate of suicide and violent death. There is a controversy about the relationship of malignant disease and schizophrenia. Problems in the health behavior of patients should also be considered, mainly lack of exercise, high smoking rate, inappropriate diet and overweight. Psychiatrists and family physicians tend to be passive in dealing with this problem. This article proposes activism of the medical profession aimed at improving health behavior, early detection of physical problems and increased awareness of the medical profession, patients and their families.

A Behavior Analytic Approach to Exploratory Motor Behavior: How Can Caregivers Teach EM Behavior to Infants with Down Syndrome?

ERIC Educational Resources Information Center

Bauer, Sara M.; Jones, Emily A.

2014-01-01

Impairment in exploratory motor (EM) behavior is part of the Down syndrome behavioral phenotype. Exploratory motor behavior may be a pivotal skill for early intervention with infants with Down syndrome. Exploratory motor impairments are often attributed to general delays in motor development in infants with Down syndrome. A behavior analytic…

Neurocognitive and neurobehavioral disabilities in Epilepsy with Electrical Status Epilepticus in slow sleep (ESES) and related syndromes.

PubMed

Raha, Sarbani; Shah, Urvashi; Udani, Vrajesh

2012-11-01

The aims of this study were to assess the cognitive and behavioral problems of patients with Epilepsy with Electrical Status Epilepticus in slow sleep (ESES) and related syndromes and to review their EEG (electroencephalography) findings and treatment options. Fourteen patients with ESES were evaluated and treated in 2010. Nine children had continuous spike and wave during slow-wave sleep (CSWS)/ESES syndrome, 3 had Atypical BECTS (benign epilepsy with centrotemporal spikes), 1 had Opercular syndrome, and 1 had Landau-Kleffner syndrome. The duration of ESES ranged from 6 to 52 months. Eleven (91%) children had behavioral issues, most prominent being hyperactivity. Seven of the 13 children (53%) showed evidence of borderline to moderate cognitive impairment. A total of 28 EEG findings of ESES were analyzed for SWI (spike-wave index). Antiepileptic drugs received by the patients included valproate, clobazam, levetiracetam, and others. Eleven patients had been treated with oral steroids and it was found to be efficacious in seven (63%). Disabilities caused by ESES affect multiple domains. Patients with an SWI>50% should be followed up frequently with neuropsychological assessments. Steroids appear to be effective, although there is a need to standardize the dose and duration of treatment. Copyright © 2012 Elsevier Inc. All rights reserved.

The 7q11.23 Microduplication Syndrome: A Clinical Report with Review of Literature

PubMed Central

Abbas, Elham; Cox, Devin M.; Smith, Teri; Butler, Merlin G.

2016-01-01

We report a 14-year-old adolescent girl with selective mutism (SM) and a 7q11.23 microduplication detected by chromosomal microarray (CMA) analysis and reviewed the literature from 18 published clinical reports. Our patient had specific phobias, SM, extreme anxiety, obesity, cutis marmorata, and a round appearing face with a short neck and over folded ears. We reviewed the published clinical, cognitive, behavioral, and cytogenetic findings grouped by speech and language delay, growth and development, craniofacial, clinical, and behavior and cognitive features due to the 7q11.23 microduplication. This microduplication syndrome is characterized by speech delay (91%), social anxiety (42%), attention deficit hyperactivity disorder (ADHD, 37%), autism spectrum disorder (29%), and separation anxiety (13%). Other findings include abnormal brain imaging (80%), congenital heart and vascular defects (54%), and mild intellectual disability (38%). We then compared the phenotype with Williams–Beuren syndrome (WBS) which is due to a deletion of the same chromosome region. Both syndromes have abnormal brain imaging, hypotonia, delayed motor development, joint laxity, mild intellectual disability, ADHD, autism, and poor visuospatial skills but opposite or dissimilar findings regarding speech and behavioral patterns, cardiovascular problems, and social interaction. Those with WBS are prone to have hyperverbal speech, lack of stranger anxiety, and supravalvular aortic stenosis while those with the 7q11.23 microduplication have speech delay, SM, social anxiety, and are prone to aortic dilatation. PMID:27617154

The 7q11.23 Microduplication Syndrome: A Clinical Report with Review of Literature.

PubMed

Abbas, Elham; Cox, Devin M; Smith, Teri; Butler, Merlin G

2016-09-01

We report a 14-year-old adolescent girl with selective mutism (SM) and a 7q11.23 microduplication detected by chromosomal microarray (CMA) analysis and reviewed the literature from 18 published clinical reports. Our patient had specific phobias, SM, extreme anxiety, obesity, cutis marmorata, and a round appearing face with a short neck and over folded ears. We reviewed the published clinical, cognitive, behavioral, and cytogenetic findings grouped by speech and language delay, growth and development, craniofacial, clinical, and behavior and cognitive features due to the 7q11.23 microduplication. This microduplication syndrome is characterized by speech delay (91%), social anxiety (42%), attention deficit hyperactivity disorder (ADHD, 37%), autism spectrum disorder (29%), and separation anxiety (13%). Other findings include abnormal brain imaging (80%), congenital heart and vascular defects (54%), and mild intellectual disability (38%). We then compared the phenotype with Williams-Beuren syndrome (WBS) which is due to a deletion of the same chromosome region. Both syndromes have abnormal brain imaging, hypotonia, delayed motor development, joint laxity, mild intellectual disability, ADHD, autism, and poor visuospatial skills but opposite or dissimilar findings regarding speech and behavioral patterns, cardiovascular problems, and social interaction. Those with WBS are prone to have hyperverbal speech, lack of stranger anxiety, and supravalvular aortic stenosis while those with the 7q11.23 microduplication have speech delay, SM, social anxiety, and are prone to aortic dilatation.

Endocrine manifestations and management of Prader-Willi syndrome.

PubMed

Emerick, Jill E; Vogt, Karen S

2013-08-21

Prader-Willi syndrome (PWS) is a complex genetic disorder, caused by lack of expression of genes on the paternally inherited chromosome 15q11.2-q13. In infancy it is characterized by hypotonia with poor suck resulting in failure to thrive. As the child ages, other manifestations such as developmental delay, cognitive disability, and behavior problems become evident. Hypothalamic dysfunction has been implicated in many manifestations of this syndrome including hyperphagia, temperature instability, high pain threshold, sleep disordered breathing, and multiple endocrine abnormalities. These include growth hormone deficiency, central adrenal insufficiency, hypogonadism, hypothyroidism, and complications of obesity such as type 2 diabetes mellitus. This review summarizes the recent literature investigating optimal screening and treatment of endocrine abnormalities associated with PWS, and provides an update on nutrition and food-related behavioral intervention. The standard of care regarding growth hormone therapy and surveillance for potential side effects, the potential for central adrenal insufficiency, evaluation for and treatment of hypogonadism in males and females, and the prevalence and screening recommendations for hypothyroidism and diabetes are covered in detail. PWS is a genetic syndrome in which early diagnosis and careful attention to detail regarding all the potential endocrine and behavioral manifestations can lead to a significant improvement in health and developmental outcomes. Thus, the important role of the provider caring for the child with PWS cannot be overstated.

Munchausen by Proxy (MBP) Maltreatment: An International Educational Challenge.

ERIC Educational Resources Information Center

Lasher, Louisa J.

2003-01-01

This article is an introduction to a special section on Munchausen Syndrome by Proxy (MSBP) as a form of child maltreatment. In MSBP the perpetrator has deliberately induced, fabricated, or exaggerated a physical and/or psychological-behavioral-mental health problem in another. The article stresses the importance of obtaining an MSBP finding of…

Validity of "DSM-IV" Syndromes in Preschoolers with Autism Spectrum Disorders

ERIC Educational Resources Information Center

Lecavalier, Luc; Gadow, Kenneth D.; Devincent, Carla J.; Houts, Carrie R.; Edwards, Michael C.

2011-01-01

Behavior and emotional problems are often present in very young children with autism spectrum disorders (ASDs) but their nosology has been the object of scant empirical attention. The objective of this study was to assess the construct validity of select "Diagnostic and Statistical Manual of Mental Disorders" ("DSM)"--defined…

Maladaptive behaviour in Prader-Willi syndrome in adult life.

PubMed

Clarke, D J; Boer, H; Chung, M C; Sturmey, P; Webb, T

1996-04-01

Thirty adults with Prader-Willi syndrome (PWS) were compared with 30 adults with non-specific learning disability matched for age, sex and severity of mental retardation. Maladaptive behaviour was assessed with the Aberrant Behavior Checklist (ABC), a 58-item structured interview which rates behaviours from 0 (not a problem) to 3 (severe problem) and which yields five factors (I) irritability, agitation; (II) lethargy, withdrawal; (III) stereotypic behavior; (IV) hyperactivity, non-compliance; and (V) inappropriate speech). The PWS sample had significantly higher factor I (P < 0.001) and factor V (P < 0.05) scores. The PWS sample had mean scores above 1 for 17 ABC items; the contrast subjects had no mean scores above 1. The factor I scores for the PWS sample were similar to those of inpatients in hospital facilities for adults with mental retardation and mental illness or severely challenging behaviour. The results support previous work, and extend it by suggesting that temper tantrums, self-injury, impulsiveness, lability of mood, inactivity and repetitive speech are characteristic behaviours in PWS in adult life. Studies of the reasons for heterogeneity in behaviour are now needed.

22q11.2 deletion syndrome: behaviour problems of children and adolescents and parental stress.

PubMed

Briegel, W; Schneider, M; Schwab, K Otfried

2008-11-01

22q11.2 deletion syndrome can be associated with a variety of somatic symptoms, developmental delays and psychiatric disorders. At present, there is little information on behaviour problems, parental stress and possible relations between these factors. Therefore, this study investigates behaviour problems of children and adolescents with 22q11.2DS, and their primary caregivers' stress. Parents of 4-17 year old subjects known to the German 22q11.2 deletion syndrome foundation were anonymously asked to fill out several questionnaires, e.g. the Child Behavior Checklist 4-18 (CBCL/4-18). The primary caregivers of 77/126 children [43 males, 34 females, mean age: 8;0 (4;0-16;11) years] sent back filled-out questionnaires. Forty-six of 76 subjects were rated as clinical on at least one of the CBCL-scales. Males had significantly higher scores on the total problems scale and the internalizing problems scale than females. The patients' age correlated with several CBCL-scales. Eleven of 49 subjects were suspicious of an autism spectrum disorder. Compared with the general population, but not with other parents of mentally and/or physically handicapped children, the primary caregivers experienced higher levels of stress, but showed normal life satisfaction. In spite of high rates of clinical behaviour problems among children and adolescents with 22q11.2DS and despite increased parental stress, most primary caregivers seem to have effective coping strategies, e.g. partnership support, to sustain normal levels of life satisfaction.

Childhood Internalizing and Externalizing Problems Predict the Onset of Clinical Panic Attacks over Adolescence: The TRAILS Study

PubMed Central

Mathyssek, Christina M.; Olino, Thomas M.; Verhulst, Frank C.; van Oort, Floor V. A.

2012-01-01

Background Panic attacks are a source of individual suffering and are an independent risk factor for later psychopathology. However, much less is known about risk factors for the development of panic attacks, particularly during adolescence when the incidence of panic attacks increases dramatically. We examined whether internalizing and externalizing problems in childhood predict the onset of panic attacks in adolescence. Method This study is part of the TRacking Adolescents’ Individual Lives Survey (TRAILS), a Dutch longitudinal population cohort study (N = 1,584). Internalizing and Externalizing Problems were collected using the Youth Self-Report (YSR) and the parent-report Child Behavior Checklist (CBCL) at baseline (age 10–12). At age 18–20, DSM-IV defined panic attacks since baseline were assessed with the Composite International Diagnostic Interview (CIDI). We investigated whether early adolescent Internalizing and Externalizing Problems predicted panic attacks between ages 10–20 years, using survival analysis in univariate and multivariate models. Results There were N = 314 (19.8%) cases who experienced at least one DSM-IV defined panic attack during adolescence and N = 18 (1.2%) who developed panic disorder during adolescence. In univariate analyses, CBCL Total Problems, Internalizing Problems and three of the eight syndrome scales predicted panic attack onset, while on the YSR all broad-band problem scales and each narrow-band syndrome scale predicted panic attack onset. In multivariate analyses, CBCL Social Problems (HR 1.19, p<.05), and YSR Thought Problems (HR 1.15, p<.05) and Social Problems (HR 1.26, p<.01) predicted panic attack onset. Conclusion Risk indicators of panic attack include the wide range of internalizing and externalizing problems. Yet, when adjusted for co-occurring problem behaviors, Social Problems were the most consistent risk factor for panic attack onsets in adolescence. PMID:23251576

Relationships of Social Context and Identity to Problem Behavior among High-Risk Hispanic Adolescents

PubMed Central

Schwartz, Seth J.; Mason, Craig A.; Pantin, Hilda; Wang, Wei; Brown, C. Hendricks; Campo, Ana; Szapocznik, José

2008-01-01

The present study was designed to examine the extent to which (a) family and school functioning and (b) personal and ethnic identity is associated with conduct problems, drug use, and sexual risk taking in a sample of 227 high-risk Hispanic adolescents. Adolescents participated in the study with their primary parents, who were mostly mothers. Adolescents completed measures of family and school functioning, personal and ethnic identity, conduct problems, and drug use. Parents completed measures of family functioning and adolescent conduct problems. Results indicated that school functioning and personal identity confusion were related to alcohol use, illicit drug use, and sexual risk taking indirectly through adolescent reports of conduct problems. Adolescent reports of family functioning were related to alcohol use, illicit drug use, and sexual risk taking through school functioning and conduct problems. Results are discussed in terms of the problem behavior syndrome and in terms of the finding of relative independence of contextual and identity variables vis-à-vis conduct problems, substance use, and sexual risk taking. PMID:19412356

Effects of Adenotonsillectomy on Parent-Reported Behavior in Children With Obstructive Sleep Apnea.

PubMed

Thomas, Nina Hattiangadi; Xanthopoulos, Melissa S; Kim, Ji Young; Shults, Justine; Escobar, Emma; Giordani, Bruno; Hodges, Elise; Chervin, Ronald D; Paruthi, Shalini; Rosen, Carol L; Taylor, Gerry H; Arens, Raanan; Katz, Eliot S; Beebe, Dean W; Redline, Susan; Radcliffe, Jerilynn; Marcus, Carole L

2017-04-01

The childhood obstructive sleep apnea syndrome (OSAS) is associated with behavioral abnormalities. Studies on the effects of OSAS treatment on behavior are conflicting, with few studies using a randomized design. Further, studies may be confounded by the inclusion of behavioral outcome measures directly related to sleep. The objective of this study was to determine the effect of adenotonsillectomy on behavior in children with OSAS. We hypothesized that surgery would improve behavioral ratings, even when sleep symptom items were excluded from the analysis. This was a secondary analysis of Child Behavior Checklist (CBCL) data, with and without exclusion of sleep-specific items, from the Childhood Adenotonsillectomy Trial (CHAT). CBCL was completed by caregivers of 380 children (7.0+1.4 [range 5-9] years) with OSAS randomized to early adenotonsillectomy (eAT) versus 7 months of watchful waiting with supportive care (WWSC). There was a high prevalence of behavioral problems at baseline; 16.6% of children had a Total Problems score in the clinically abnormal range. At follow-up, there were significant improvements in Total Problems (p < .001), Internalizing Behaviors (p = .04), Somatic Complaints (p = .01), and Thought Problems (p = .01) in eAT vs. WWSC participants. When specific sleep-related question items were removed from the analysis, eAT showed an overall improvement in Total (p = .02) and Other (p = .01) problems. Black children had less improvement in behavior following eAT than white children, but this difference attenuated when sleep-related items were excluded. This large, randomized trial showed that adenotonsillectomy for OSAS improved parent-rated behavioral problems, even when sleep-specific behavioral issues were excluded from the analysis. © Sleep Research Society 2017. Published by Oxford University Press on behalf of the Sleep Research Society. All rights reserved. For permissions, please e-mail journals.permissions@oup.com.

Validating office-based screening for psychosocial strengths and difficulties among youths in foster care.

PubMed

Jee, Sandra H; Szilagyi, Moira; Conn, Anne-Marie; Nilsen, Wendy; Toth, Sheree; Baldwin, Constance D; Szilagyi, Peter G

2011-05-01

To assess the effectiveness of social-emotional screening in the primary care setting for youths in foster care. The setting was a primary care practice for all youth in home-based foster care in 1 county. Subjects were youths, aged 11 to 17 years, and their foster parents; both completed a Strengths and Difficulties Questionnaire at well-child visits. The Strengths and Difficulties Questionnaire is a previously validated 25-item tool that has 5 domains: emotional symptoms; conduct problems; hyperactivity/inattention; peer problems; and prosocial behaviors and an overall total difficulties score. We first compared youth versus parent Strengths and Difficulties Questionnaire scores and then assessed the accuracy of these Strengths and Difficulties Questionnaire scores by comparing them in a subsample of youths (n = 50) with results of home-based structured clinical interviews using the Children's Interview for Psychiatric Syndromes. Of 138 subjects with both youth and parent reports, 78% had prosocial behaviors (strengths), and 70% had 1 or more social-emotional problems. Parents reported significantly more conduct problems (38% vs 16%; P < .0001) and total difficulties (30% vs 16%; P = .002) than did youth. The Strengths and Difficulties Questionnaire had better agreement with the Children's Interview for Psychiatric Syndromes (n = 50) for any Strengths and Difficulties Questionnaire-identified problem for combined youth and foster-parent reports (93%), compared with youth report alone (54%) or parent report alone (71%). Although most youths in foster care have social-emotional problems, most have strengths as well. Youth and foster-parent perspectives on these problems differ. Systematic social-emotional screening in primary care that includes both youth and parent reports can identify youths who may benefit from services.

Gender differences in suicidal expressions and their determinants among young people in Cambodia, a post-conflict country*

PubMed Central

2011-01-01

Background Suicide among young people is a global public health problem, but adequate information on determinants of suicidal expression is lacking in middle and low income countries. Young people in transitional economies are vulnerable to psychosocial stressors and suicidal expressions. This study explores the suicidal expressions and their determinants among high school students in Cambodia, with specific focus on gender differences. Methods A sample of 320 young people, consisting of 153 boys and 167 girls between 15-18 years of age, was randomly selected from two high schools in Cambodia. Their self-reported suicidal expressions, mental health problems, life-skills dimensions, and exposure to suicidal behavior in others were measured using the Youth Self-Report (YSR), Life-Skills Development Scale (LSDS)-Adolescent Form, and Attitude Towards Suicide (ATTS) questionnaires. Results Suicidal plans were reported more often by teenage boys than teenage girls (M = 17.3%, F = 5.6%, p = 0.001), whereas girls reported more attempts (M = 0.6%, F = 7.8%, p = 0.012). Young men scored significantly higher on rule-breaking behavior than young women (p = 0.001), whereas young women scored higher on anxious/depression (p = 0.000), withdrawn/depression (p = 0.002), somatic complaints (p = 0.034), social problems (p = 0.006), and internalizing syndrome (p = 0.000). Young men exposed to suicide had significantly higher scores for internalizing syndrome compared to those unexposed (p = 0.001), while young women exposed to suicide scored significantly higher on both internalizing (p = 0.001) and externalizing syndromes (p = 0.012). Any type of exposure to suicidal expressions increased the risk for own suicidal expressions in both genders (OR = 2.04, 95% CI = 1.06-3.91); among young women, however, those exposed to suicide among friends and partners were at greater risk for the serious suicidal expressions (OR = 2.79, 95% CI = 1.00-7.74). Life skills dimension scores inversely correlated with externalizing syndrome in young men (p = 0.026) and internalizing syndrome in young women (p = 0.001). Conclusions The significant gender differences in suicidal expressions and their determinants in Cambodian teenagers highlight the importance of culturally appropriate and gender-specific suicide prevention programs. School-based life skills promotion may indirectly influence the determinants for suicidal expressions, particularly among young women with internalizing syndrome in Cambodia. PMID:21418649

Sex differences in the behavior of children with the 22q11 deletion syndrome

PubMed Central

Sobin, Christina; Kiley-Brabeck, Karen; Monk, Samantha Hadley; Khuri, Jananne; Karayiorgou, Maria

2009-01-01

High rates of psychiatric impairment in adults with 22q11DS suggest that behavioral trajectories of children with 22q11DS may provide critical etiologic insights. Past findings that report DSM diagnoses are extremely variable; moreover sex differences in behavior have not yet been examined. Dimensional CBCL ratings from 82 children, including 51 with the 22q11DS and 31 control siblings were analyzed. Strikingly consistent with rates of psychiatric impairment among affected adults, 25% percent of children with 22q11DS had high CBCL scores for Total Impairment, and 20% had high CBCL Internalizing Scale scores. Males accounted for 90% of high Internalizing scores and 67% of high Total Impairment scores. Attention and Social Problems were ubiquitous; more affected males than females (23% vs. 4%) scored high on Thought Problems. With regard to CBCL/DSM overlap, 20% of affected males as compared with 0 affected females had one or more high CBCL ratings in the absence of a DSM diagnosis. Behaviors of children with 22q11DS are characterized by marked sex differences when rated dimensionally, with significantly more males experiencing Internalizing and Thought Problems. Categorical diagnoses do not reflect behavioral differences between male and female children with 22q11DS, and may miss significant behavior problems in 20% of affected males. PMID:19217670

Autistic-spectrum disorders in Down syndrome: further delineation and distinction from other behavioral abnormalities.

PubMed

Carter, John C; Capone, George T; Gray, Robert M; Cox, Christiane S; Kaufmann, Walter E

2007-01-05

The present study extends our previous work characterizing the behavioral features of autistic-spectrum disorder (ASD) in Down syndrome (DS) using the Aberrant Behavior Checklist (ABC) and Autism Behavior Checklist (AutBehav). We examined which specific behaviors distinguished the behavioral phenotype of DS + ASD from other aberrant behavior disorders in DS, by determining the relative contribution of ABC and AutBehav subscales and items to the diagnosis of ASD. A total of 127 subjects (aged 2-24 years; mean age: 8.4 years; approximately 70% male), comprising: a cohort of 64 children and adolescents with DS and co-morbid ASD (DS + ASD), 19 with DS and stereotypic movement disorder (DS + SMD), 18 with DS and disruptive behaviors (DS + DB), and 26 with DS and no co-morbid behavior disorders (DS + none) were examined using the aforementioned measures of aberrant behavior. We found that subjects with DS + ASD showed the most severe aberrant behavior, especially stereotypy compared to DS + none and lethargy/social withdrawal and relating problems compared to DS + SMD. Specifically, relatively simple stereotypic behavior differentiated DS + ASD from DS + DB, whereas odd/bizarre stereotypic and anxious behavior characterized DS + ASD relative to DS + SMD and DS + none. Additionally, in a subset of subjects with DS + ASD and anxiety, social withdrawal was particularly pronounced. Overall, our findings indicate that a diagnosis of DS + ASD represents a distinctive set of aberrant behaviors marked by characteristic odd/bizarre stereotypic behavior, anxiety, and social withdrawal.

Gilles de la Tourette's syndrome in a patient with 47(XXX) syndrome: a case report.

PubMed

Chiappedi, Matteo; de Vincenzi, Silvia; Dolci, Roberta; De Luca, Sara; Bejor, Maurizio

2011-11-05

To the best of our knowledge, this is the first report of a comorbidity between Gilles de la Tourette's syndrome and 47 (XXX) syndrome. The clinical picture of Gilles de la Tourette's Syndrome is well described, while 47 (XXX) syndrome is much more rare and has a broader spectrum of possible phenotypic presentations. An Italian Caucasian girl was referred at the age of 11 to our Rehabilitation Center for anxiety and learning difficulties. The girl had already been diagnosed as having 47(XXX) syndrome; she had some rather typical features of the chromosomal abnormality, but she also showed a high level of anxiety and the presence of motor and vocal tics. When an accurate history was taken, a diagnosis of Gilles de la Tourette's Syndrome emerged. The possible interaction between peculiar features of these two syndromes in terms of neuropsychological and affective functioning is both interesting for the specific case and to hypothesize models of rehabilitation for patients with one or both syndromes. Executive functions are specifically reduced in both syndromes, therefore it might be hard to discriminate the contribution of each one to the general impairment; the same applies to anxiety. Moreover, mental retardation (with a significantly lower verbal cognitive functioning) poses relevant problems when suggesting cognitive behavioral or psychoeducational rehabilitative approaches.

PTSD, alcohol dependence, and conduct problems: Distinct pathways via lability and disinhibition.

PubMed

Simons, Jeffrey S; Simons, Raluca M; O'Brien, Carol; Stoltenberg, Scott F; Keith, Jessica A; Hudson, Jaime A

2017-01-01

This study tested the role of affect lability and disinhibition in mediating associations between PTSD symptoms and two forms of alcohol-related problems, dependence syndrome symptoms (e.g., impaired control over consumption) and conduct problems (e.g., assault, risk behaviors). Genotype at the serotonin transporter linked polymorphic region (5-HTTLPR) was hypothesized to moderate associations between traumatic stress and PTSD symptoms. In addition, the study tested whether childhood traumatic stress moderated associations between combat trauma and PTSD symptoms. Participants were 270 OIF/OEF/OND veterans. The hypothesized model was largely supported. Participants with the low expression alleles of 5-HTTLPR (S or L G ) exhibited stronger associations between childhood (but not combat) traumatic stress and PTSD symptoms. Affect lability mediated the associations between PTSD symptoms and alcohol dependence symptoms. Behavioral disinhibition mediated associations between PTSD symptoms and conduct related problems. Conditional indirect effects indicated stronger associations between childhood traumatic stress and lability, behavioral disinhibition, alcohol consumption, AUD symptoms, and associated conduct problems via PTSD symptoms among those with the low expression 5-HTTLPR alleles. However, interactions between combat trauma and either childhood trauma or genotype were not significant. The results support the hypothesis that affect lability and behavioral disinhibition are potential intermediate traits with distinct associations with AUD and associated externalizing problems. Copyright © 2016 Elsevier Ltd. All rights reserved.

Teachers' Assumptions Regarding the Severity, Causes, and Outcomes of Behavioral Problems in Preschoolers: Implications for Referral.

ERIC Educational Resources Information Center

Walker, Elaine; And Others

1984-01-01

Asked preschool teachers (N=100) to rate severity, long-term outcome, stability, and importance of constitutional and environmental determinants for case vignettes describing three syndromes: aggression, hyperactivity, and withdrawal, and to judge the need for referral. Results indicated little evidence of sex bias in teachers' evaluations of the…

Chronic Snoring and Sleep in Children: A Demonstration of Sleep Disruption

ERIC Educational Resources Information Center

Lopes, M. Cecilia; Guilleminault, Christian

2007-01-01

Objective: Chronic snoring that does not adhere to the criteria for a diagnosis of obstructive sleep apnea syndrome may be associated with learning and behavioral problems. We investigated the sleep structure of chronic snorers who had an apnea-hypopnea index of less than 1 event per hour and analyzed the cyclic alternating pattern. Methods:…

Sleep in Neurodevelopmental Disorders

PubMed Central

Esbensen, Anna J; Schwichtenberg, Amy J

2017-01-01

Individuals with intellectual and developmental disabilities (IDD) experience sleep problems at higher rates than the general population. Although individuals with IDD are a heterogeneous group, several sleep problems cluster within genetic syndromes or disorders. This review summarizes the prevalence of sleep problems experienced by individuals with Angelman syndrome, Cornelia de Lange syndrome, Cri du Chat syndrome, Down syndrome, fragile X syndrome, Prader-Willi syndrome, Smith-Magenis syndrome, Williams syndrome, autism spectrum disorder, and idiopathic IDD. Factors associated with sleep problems and the evidence for sleep treatments are reviewed for each neurodevelopmental disorder. Sleep research advancements in neurodevelopmental disorders are reviewed, including the need for consistency in defining and measuring sleep problems, considerations for research design and reporting of results, and considerations when evaluating sleep treatments. PMID:28503406

Behavioral profile of adults with Prader-Willi syndrome: correlations with individual and environmental variables.

PubMed

Jauregi, Joseba; Laurier, Virginie; Copet, Pierre; Tauber, Maithé; Thuilleaux, Denise

2013-08-06

Maladaptive behavior has been reported as a phenotypical feature in Prader-Willi syndrome (PWS). It severely limits social adaptation and the quality of life of children and adults with the syndrome. Different factors have been linked with the intensity and form of these behavioral disturbances but there is no consensus about the cause. Consequently, there is still controversy regarding management strategies and there is a need for new data. The behavior of 100 adults with PWS attending a dedicated center was assessed using the Developmental Behavior Checklist for Adults (DBC-A) and the PWS-specific Hyperphagia Questionnaire. The DBC-A was completed separately by trained caregivers at the center and relatives or caregivers in a natural setting. Genotype, gender, age, degree of obesity and cognitive impairment were analyzed as variables with a hypothetical influence on behavioral features. Patients showed a relatively high rate of behavioral disturbances other than hyperphagia. Disruptive and social relating were the highest scoring DBC-A subscales whereas anxiety/antisocial and self-absorbed were the lowest. When hospital caregiver and natural caregiver scores were compared, scores for the latter were higher for all subscales except for disruptive and anxiety/antisocial. These effects of institutional management were underlined. In the DBC-A, 22 items have descriptive indications of PWS behavior and were used for further comparisons and correlation analysis. In contrast to previous reports, rates of disturbed behavior were lower in patients with a deletion genotype. However, the behavioral profile was similar for both genotypes. No differences were found in any measurement when comparing type I and type II deletions. The other analyzed variables showed little relevance. Significant rates of behavioral disorders were highlighted and their typology described in a large cohort of adults with PWS. The deletion genotype was related to a lower severity of symptoms. Some major behavioral problems, such as hyperphagia, may be well controlled if living circumstances are adapted to the specific requirements of individuals with PWS.

Behavioral profile of adults with Prader-Willi syndrome: correlations with individual and environmental variables

PubMed Central

2013-01-01

Background Maladaptive behavior has been reported as a phenotypical feature in Prader–Willi syndrome (PWS). It severely limits social adaptation and the quality of life of children and adults with the syndrome. Different factors have been linked with the intensity and form of these behavioral disturbances but there is no consensus about the cause. Consequently, there is still controversy regarding management strategies and there is a need for new data. Methods The behavior of 100 adults with PWS attending a dedicated center was assessed using the Developmental Behavior Checklist for Adults (DBC-A) and the PWS-specific Hyperphagia Questionnaire. The DBC-A was completed separately by trained caregivers at the center and relatives or caregivers in a natural setting. Genotype, gender, age, degree of obesity and cognitive impairment were analyzed as variables with a hypothetical influence on behavioral features. Results Patients showed a relatively high rate of behavioral disturbances other than hyperphagia. Disruptive and social relating were the highest scoring DBC-A subscales whereas anxiety/antisocial and self-absorbed were the lowest. When hospital caregiver and natural caregiver scores were compared, scores for the latter were higher for all subscales except for disruptive and anxiety/antisocial. These effects of institutional management were underlined. In the DBC-A, 22 items have descriptive indications of PWS behavior and were used for further comparisons and correlation analysis. In contrast to previous reports, rates of disturbed behavior were lower in patients with a deletion genotype. However, the behavioral profile was similar for both genotypes. No differences were found in any measurement when comparing type I and type II deletions. The other analyzed variables showed little relevance. Conclusions Significant rates of behavioral disorders were highlighted and their typology described in a large cohort of adults with PWS. The deletion genotype was related to a lower severity of symptoms. Some major behavioral problems, such as hyperphagia, may be well controlled if living circumstances are adapted to the specific requirements of individuals with PWS. PMID:23919902

Higher plasma orexin a levels in children with Prader-Willi syndrome compared with healthy unrelated sibling controls.

PubMed

Manzardo, Ann M; Johnson, Lisa; Miller, Jennifer L; Driscoll, Daniel J; Butler, Merlin G

2016-09-01

Prader-Willi syndrome (PWS) is a rare genetic neurodevelopmental disorder associated with maladaptive social behavior, hyperphagia and morbid obesity. Orexin A is a hypothalamic neuropeptide important as a homeostatic regulator of feeding behavior and in energy metabolism through actions in the lateral hypothalamus. Dysregulation of orexin signaling may contribute to behavioral problems and hyperphagia seen in PWS and we sought to assess orexin A levels in PWS relative to controls children. Morning fasting plasma orexin A levels were analyzed in 23 children (aged 5-11 years) with genetically confirmed PWS and 18 age and gender matched healthy unrelated siblings without PWS. Multiplex immune assays utilized the Milliplex Human Neuropeptide Magnetic panel and the Luminex platform. Natural log-transformed orexin A data were analyzed using general linear model adjusting for diagnosis, gender, age, total body fat, and body mass index (BMI). Plasma orexin A levels were significantly higher (P < 0.006) in children with PWS (average ±SD = 1,028 pg/ml ± 358) compared with unrelated siblings (average ±SD = 609 pg/ml ± 351; P < 0.001). Orexin A levels correlated with age in females and were significantly elevated in PWS even after these effects were controlled. These findings support the hypothesis that dysregulation of orexin signaling may contribute to behavioral problems and hyperphagia in PWS. Further studies are warranted to better understand the complex relationship between orexin A levels and the problematic behaviors consistently found in individuals with PWS. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

Higher plasma orexin A levels in children with Prader-Willi syndrome compared with healthy unrelated sibling controls.

PubMed

Manzardo, Ann M; Johnson, Lisa; Miller, Jennifer L; Driscoll, Daniel J; Butler, Merlin G

2016-08-01

Prader-Willi syndrome (PWS) is a rare genetic neurodevelopmental disorder associated with maladaptive social behavior, hyperphagia, and morbid obesity. Orexin A is a hypothalamic neuropeptide important as a homeostatic regulator of feeding behavior and in energy metabolism through actions in the lateral hypothalamus. Dysregulation of orexin signaling may contribute to behavioral problems and hyperphagia seen in PWS and we sought to assess orexin A levels in PWS relative to controls children. Morning fasting plasma orexin A levels were analyzed in 23 children (aged 5-11 years) with genetically confirmed PWS and 18 age and gender matched healthy unrelated siblings without PWS. Multiplex immune assays utilized the Milliplex Human Neuropeptide Magnetic panel and the Luminex platform. Natural log-transformed orexin A data were analyzed using general linear model adjusting for diagnosis, gender, age, total body fat and body mass index (BMI). Plasma orexin A levels were significantly higher (P < 0.006) in children with PWS (average ±SD = 1028 pg/ml ± 358) compared with unrelated siblings (average ±SD = 609 pg/ml ± 351; P < 0.001). Orexin A levels correlated with age in females and were significantly elevated in PWS even after these effects were controlled. These findings support the hypothesis that dysregulation of orexin signaling may contribute to behavioral problems and hyperphagia in PWS. Further studies are warranted to better understand the complex relationship between orexin A levels and the problematic behaviors consistently found in individuals with PWS. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

Psychological Well-being in Fathers of Adolescents and Young Adults with Down Syndrome, Fragile X Syndrome, and Autism.

PubMed

Hartley, Sigan L; Seltzer, Marsha Mailick; Head, Lara; Abbeduto, Leonard

2012-04-01

The psychological well-being of fathers of children with developmental disabilities remains poorly understood. The present study examined depressive symptoms, pessimism, and coping in fathers of adolescents and young adults with Down syndrome (DS; n = 59), autism spectrum disorders (ASDs; n = 135), and fragile X syndrome (FXS; n = 46) Fathers of sons/daughters with ASDs reported a higher level of depressive symptoms than the other groups of fathers. Fathers of sons/daughters with DS reported a lower level of pessimism than the other groups of fathers. There were no group differences in paternal coping style. Group differences in paternal depressive symptoms and pessimism were, in part, related to differences in paternal age, the child's behavior problems, risk of having additional children with a disability, and maternal depressive symptoms. Findings from this study can be used to educate providers and design services for fathers during the later parenting years.

Children with Usher syndrome: mental and behavioral disorders.

PubMed

Dammeyer, Jesper

2012-03-27

Mental and behavioral disorders among adults with Usher syndrome have been discussed and reported in some case studies but no research has been reported on children with Usher syndrome. This article investigates the prevalence and characteristics of mental and behavioral disorders among 26 children, 3-17 years of age, with Usher syndrome. Six of the 26 children were diagnosed with a mental or behavioral disorder (1 with schizophrenia and mild mental retardation, 1 with atypical autism and severe mental retardation, 1 with atypical autism and mild mental retardation, 1 with mild mental retardation, and 2 with conduct disorder). Another 3 children had had a mental or behavioral disorder previously in their childhood. Even though vision impairment first manifests in late childhood, some children with Usher syndrome seem to develop mental and behavioral disorders during childhood. The aetiology and treatment of mental and behavioral disorders among children with Usher syndrome are discussed. Children with Usher syndrome and their parents may need clinical support during early childhood to prevent development of mental and behavioral disorders.

Violence and Abuse Among HIV-Infected Women and Their Children in Zambia

PubMed Central

Murray, Laura K.; Haworth, Alan; Semrau, Katherine; Singh, Mini; Aldrovandi, Grace M.; Sinkala, Moses; Thea, Donald M.; Bolton, Paul A.

2009-01-01

HIV and violence are two major public health problems increasingly shown to be connected and relevant to international mental health issues and HIV-related services. Qualitative research is important due to the dearth of literature on this association in developing countries, cultural influences on mental health syndromes and presentations, and the sensitive nature of the topic. The study presented in this paper sought to investigate the mental health issues of an HIV-affected population of women and children in Lusaka, Zambia, through a systematic qualitative study. Two qualitative methods resulted in the identification of three major problems for women: domestic violence (DV), depression-like syndrome, and alcohol abuse; and children: defilement, DV, and behavior problems. DV and sexual abuse were found to be closely linked to HIV and alcohol abuse. This study shows the local perspective of the overlap between violence and HIV. Results are discussed in relation to the need for violence and abuse to be addressed as HIV services are implemented in sub-Saharan Africa. PMID:16909070

Clinically significant psychiatric symptoms among male carriers of the fragile X premutation, with and without FXTAS, and the mediating influence of executive functioning.

PubMed

Grigsby, Jim; Brega, Angela G; Bennett, Rachael E; Bourgeois, James A; Seritan, Andreea L; Goodrich, Glenn K; Hagerman, Randi J

2016-08-01

To clarify the neuropsychiatric phenotype of fragile X-associated tremor/ataxia syndrome (FXTAS), and assess the extent to which it is mediated by the dysexecutive syndrome that is a major feature of the disorder. We examined the prevalence of clinically meaningful psychiatric symptoms among male carriers of the fragile X premutation, with and without FXTAS, in comparison with men with a normal allele. Measures included the Neuropsychiatric Inventory (NPI), Symptom Checklist-90-R (SCL-90-R), and the Behavioral Dyscontrol Scale, a measure of executive functioning. Between-group differences were evaluated using logistic regression, followed by a mediation analysis with ordinary least squares regression to assess the contribution of dysexecutive syndrome to the observed psychiatric domains. Men with FXTAS showed higher rates of clinically significant symptoms overall and in specific domains: somatization, obsessive compulsive, depression, anxiety, psychoticism, agitation/aggression, apathy/indifference, irritability, and nighttime behavior problems. Post hoc analyses suggested that findings of psychoticism among men with FXTAS may be associated with participants' accurate acknowledgment of cognitive and physical dysfunction, rather than reflecting psychosis. Asymptomatic carriers showed no evidence of clinically significant psychiatric symptoms, but when all carriers were compared with men having a normal FMR1 allele, executive function deficits were found to mediate scores in several domains on both NPI and SCL-90-R. Building on prior research, the results provide evidence that the psychiatric phenotype for men includes clinically meaningful depression, hostility, and irritability, in association with behavioral and attentional disinhibition. It is likely that these problems reflect the effects of impaired executive functioning.

Tourette Syndrome and Chronic Tic Disorders: The Clinical Spectrum Beyond Tics.

PubMed

Martino, Davide; Ganos, Christos; Pringsheim, Tamara M

2017-01-01

The clinical surveillance and active management of Tourette syndrome (TS) and other primary chronic tic disorders cannot be limited to tics, as these patients manifest a spectrum of sensory-, behavioral-, cognitive-, and sleep-related problems that have a major impact on their functioning and quality of life, influencing enormously clinical decision making on a routine basis. The sensory phenomena of primary tic disorders consist of premonitory urges and heightened sensitivity to external somatosensory and interoceptive stimuli. Recent evidence suggests that raised interoceptive awareness may be related to the classical premonitory urges associated with tics. The burden of behavioral comorbidities is very important in determining the degree of disability of patients with primary tic disorders. Only 10%-15% of these patients presents exclusively with a tic disorder. Obsessive-compulsive disorder (OCD) are common in TS, and the clinical distinction between compulsions and complex tics may be difficult in some cases. "Tic-related OCD" represents a phenomenologically characteristic subtype of OCD, also associated with "just right" phenomena. Probably the presence of comorbid attention deficit/hyperactivity disorder is the main determinant of cognitive dysfunction in TS patients and influences heavily also the risk of developing disruptive behaviors. Mood and anxiety disorders, impulse control disorders, rage attacks, "impulsive" tic-like behaviors (e.g., nonobscene socially inappropriate behaviors, and self-injurious behaviors), and autism spectrum disorders complete the wide psychopathological spectrum of primary chronic tic disorders. Moreover, specific sleep abnormalities have been reported in TS patients, although more research is needed on this specific clinical problem. As in other areas of clinical neuroscience, a comprehensive approach to both motor and nonmotor aspects of this group of disorders will help personalizing treatment interventions and, ultimately, improve quality of care. © 2017 Elsevier Inc. All rights reserved.

Deletion and duplication within the p11.2 region of chromosome 17

DOE Office of Scientific and Technical Information (OSTI.GOV)

McCorquodale, D.J.; McCorquodale, M.; Bereziouk, O.

1994-09-01

A 7 1/2-year-old male patient presented with mild mental retardation, speech delay, hyperactivity, behavioral problems, mild facial hypoplasia, short broad hands, digital anomalies, and self-injurious behavior. Chromosomes obtained from peripheral blood cells revealed a deletion of 17p11.2 in about 40% of the metaphases examined, suggesting that the patient had Smith-Magenis Syndrome. A similar pattern of mosaicism in peripheral blood cells, but not in fibroblasts in which all cells displayed the deletion, has been previously reported. Since some cases of Smith-Magenis Syndrome have a deletion that extends into the region associated with Charcot-Marie-Tooth (CMT) Syndrome, we examined interphase cells with amore » CMT1A-specific probe by the method of fluorescence in situ hybridization. The CMT1A region was not deleted, but about 40% of the cells gave signals indicating a duplication of the CMT1A region. The patient has not presented neuropathies associated with CMT at this time. Future tracking of the patient should be informative.« less

[Caregiver Stress in Foster and Adoptive Parents of Children with Fetal Alcohol Spectrum Disorders].

PubMed

Sarimski, Klaus

2014-01-01

Caregiver Stress in Foster and Adoptive Parents of Children with Fetal Alcohol Spectrum Disorders. Foster and adoptive parents of 71 children with fetal alcohol syndrome (FAS) report on developmental and behavioral characteristics, family stress, coping resources and their satisfaction with support. The data reveal an elevated rate of social and emotional problems in the children. In spite positive individual and social resources, the foster and adoptive parents feel a high level of caregiver stress. 30 % of them rate the support they receive from pediatric, therapeutic or educational services as lower than expected. Specifically, they miss early information on the diagnosis, professional knowledge and support for the special challenges of education and managing behavioral problems in their collaboration with social support agencies.

A disease specific questionnaire for assessing behavior in individuals with Prader-Willi syndrome.

PubMed

Avrahamy, Hamutal; Pollak, Yehuda; Shriki-Tal, Liron; Genstil, Larry; Hirsch, Harry J; Gross-Tsur, Varda; Benarroch, Fortu

2015-04-01

Prader-Willi syndrome (PWS) is a genetic multisystem disorder with various medical, cognitive, behavioral and psychiatric problems. PWS is caused by the lack of expression of paternal genes on chromosome 15q2-q13 due to a deletion (70-75%), uniparental disomy (25-30%) or imprinting center defect (<5%). The common PWS behavioral and psychiatric characteristics are very typical in all ethnicities and were reported worldwide. Still, each individual has a specific profile of these common traits and the severity of his or her symptoms varies over time. Behavioral problems are the most important factor affecting the quality of life of both the individuals and their families. There is a need for a standardized tool to assess the specific behavioral profile of each individual and its present severity, in order to enable physicians to tailor the specific treatment needed and assist in a more accurate clinical follow up. To the best of our knowledge no such a tool has been standardized and published. We developed, based on the literature (mainly Forster and Gourash's paradigm) and our clinical experience, a 37 item disease specific questionnaire, the "PWS Behavioral Questionnaire" (PWSBQ) for assessing behavior in PWS patients. The purpose of the present study was to validate this tool in the entire adolescent and adult PWS population in Israel. The PWSBQ focuses on five major domains-abnormal emotional regulation, food-seeking related behavior, lack of flexibility, oppositional behavior and interpersonal problems and lastly body related behaviors. Caregivers of all Hebrew speaking individuals with PWS over the age of 12 years attending the Israeli national multidisciplinary PWS clinic were recruited. Of the 54 eligible individuals, 53 participated. They were interviewed with the PWSBQ and in addition filled the "Hyperphagia Questionnaire" and the "Child Behavioral Checklist" (CBCL). After verifying the questionnaire's content validity, all items on the PWSBQ were analyzed for internal reliability by calculating Cronbach's α. Criterion validity was evaluated by correlation testing with regard to the Hyperphagia Questionnaire and CBCL. In order to assess the questionnaire's interpretability, the correlation between the PWSBQ and the "Clinical Global Impression" (CGI) scores was evaluated. The PWSBQ total score was positively correlated with both the CBCL total score and the CGI score (0.662 and 0.549, p<0.001 respectively). Of the five domains, four had acceptable internal reliability (excluding the body related behaviors domain, which was thus removed from the total score). Criterion validity was established for the four domains remaining in the statistical analysis (abnormal emotional regulation, food seeking related behavior, lack of flexibility and oppositional behavior and interpersonal problems). Our findings suggest that the PWSBQ is a valid and reliable tool for the assessment of current behavioral problems among individuals with PWS. Although further research is needed in order to verify PWSBQ's ability to identify changes in the behavioral status of a given individual, it can now be used both in research and in a clinical setting, enabling the physician to plan the most suitable treatment based on the current behavioral status. Copyright © 2014 Elsevier Inc. All rights reserved.

Parent training education program: a pilot study, involving families of children with Prader-Willi syndrome.

PubMed

Kodra, Yllka; Kondili, Loreta A; Ferraroni, Alessia; Serra, Maria Antonietta; Caretto, Flavia; Ricci, Maria Antonietta; Taruscio, Domenica

2016-01-01

Prader-Willi syndrome (PWS) is a rare genetic disorder characterized by severe hypotonia during the neonatal period and the first two years of life, the onset of hyperphagia with a risk of obesity during infancy and adulthood, learning difficulties and behavioral or severe psychiatric problems. This complex disease has severe consequences and difficult management issues also for patients' families. Parents of children with PWS need appropriate psychoeducational intervention in order to better manage their children with PWS. The purpose of this study was the implementation and evaluation of a PWS psychoeducational parent training program. The Italian National Center for Rare Diseases implemented a pilot parent training program offered to parents of children with PWS. The intervention's effects was evaluated using questionnaires comprised of 11 items rated on a 7 point Likert scale. The intervention was offered to 43 parents. The behavior problems management, dietary restrictions, autonomy and relationships were indicated by parents as the priority topics which needed to be addressed. Evaluations, immediately post-intervention and after 6 months, were reported by parents, fulfilling specific questionnaires. 90% of parents involved in the study, appreciated the methodology, 86% felt more informed about PWS, 47-62% felt more capable to better approach behaviour's problems, 20-25% felt better about the child's health situation and future expectations. Feeling more capable to help the child autonomy and relationships were reported in 62% and 63% of parents respectively, which decreased significantly (p < 0.05) according to the evaluation 6 months after the intervention. Younger age of parents (< 44 years of age) was significantly correlated with better understanding on how to help the child's autonomy (OR: 0.05; CI: 0.04-0.8) and to better collaborate with the child's teachers (OR: 0.02; CI: 0.001-0.9). Parent training is a promising intervention for parents of children with behavior's problems. Interventions with a behaviorally oriented program, addressed to parents of PWS affected children, is a useful tool in increasing their ability to manage the problems related to the disease.

Antisocial Behavioral Syndromes and Past-Year Physical Health Among Adults in the United States: Results from the National Epidemiologic Survey on Alcohol and Related Conditions

PubMed Central

Goldstein, Risë B.; Dawson, Deborah A.; Chou, S. Patricia; Ruan, W. June; Saha, Tulshi D.; Pickering, Roger P.; Stinson, Frederick S.; Grant, Bridget F.

2010-01-01

Objective To describe associations of DSM-IV antisocial personality disorder (ASPD), conduct disorder without progression to ASPD (“CD only”), and syndromal antisocial behavior in adulthood without CD before age 15 (AABS, not a DSM-IV diagnosis) with past-year physical health status and hospital care utilization in the general U.S. adult population. Methods This report is based on the 2001-2002 National Epidemiologic Survey on Alcohol and Related Conditions (n=43,093, response rate=81%). Respondents were classified according to whether they met criteria for ASPD, AABS, “CD only,” or no antisocial syndrome. Associations of antisocial syndromes with physical health status and care utilization were examined using normal-theory and logistic regression. Results ASPD and AABS were significantly but modestly associated with total past-year medical conditions, coronary heart and gastrointestinal diseases, and numbers of inpatient hospitalizations, inpatient days, emergency department visits, and clinically significant injuries. ASPD was also associated with liver disease, arthritis, and lower scores on the Short Form-12 version 2 (SF-12v2) Physical Component, Role Physical, and Bodily Pain Scales. AABS was associated with noncoronary heart disease, lower scores on the SF-12v2 General Health and Vitality Scales, and, among men, arthritis. “CD only” was associated with single but not multiple inpatient hospitalizations, emergency department visits, and clinically significant injuries. Conclusions Estimates of burden related to antisocial behavioral syndromes need to consider associated physical health problems. Prevention and treatment guidelines for injuries and common chronic diseases may need to address comorbid antisociality, and interventions targeting antisociality may need to consider general health status, including prevention and management of injuries and chronic diseases. PMID:18348594

Behavioral and psychiatric disorders in Prader-Willi syndrome: a population study in Japan.

PubMed

Hiraiwa, Rika; Maegaki, Yoshihiro; Oka, Akira; Ohno, Kousaku

2007-10-01

Prader-Willi syndrome (PWS) is a genetically determined neurodevelopmental disorder characterized by mental retardation and distinct physical, behavioral, and psychiatric features. Based on parents' questionnaires, we examined the prevalence of behavioral and psychiatric disorders of 165 persons with PWS aged 2-31 years in Japan. The data were analyzed comparing four different age groups with PWS: group 1, 2-5 years (n=34); group 2, 6-11 years (n=57); group 3, 12-17 years (n=45); and group 4, 18-31 years (n=29). Further, we compared the results of our PWS group 4 with those of 42 age-, gender-, and intelligence level-matched intellectual disability (ID) individuals without PWS. Our results showed that repetitive speech and stubbornness were prominent from early childhood and other behavioral problems such as hyperphagia, stealing food, temper tantrums, lying, and emotional lability tended to be more frequent with age among persons with PWS. Moreover, young adults with PWS have significantly higher rates of behavioral and psychiatric disorders than IDs without PWS, such as stubbornness, hyperphagia, temper tantrums, self-injurious behavior (skin picking), hypersomnia, inactivity, and delusion. Degree of obesity was not necessarily related to behavioral and psychiatric features associated with PWS. Our findings revealed that persons with PWS are more vulnerable to behavioral and psychiatric disorders particularly in young adulthood compared to those with ID from other etiologies in Japan.

Bibliography on the Hyperkinetic Behavior Syndrome.

ERIC Educational Resources Information Center

Kirson, Tamara; And Others

The bibliography on the hyperkinetic behavior syndrome focuses on the behavior characteristics of and treatment for hyperactivity. Entries are divided into the following sections (sample subsections are in parentheses): general review of pediatric psychopharmacology; the hyperkinetic behavior syndrome (overview, diagnosis and evaluation,…

The Mental and Emotional Well-Being of Children: A Portrait of States and the Nation 2007. The National Survey of Children's Health

ERIC Educational Resources Information Center

US Department of Health and Human Services, 2010

2010-01-01

Children, like adults, may have mental health problems, including depression and anxiety. They may also have behavioral conditions, such as conduct disorders; cognitive disorders, such as autism spectrum disorder; or neurological conditions, such as Tourette Syndrome. Children may also be affected by delays in their physical, cognitive, or…

Children with Usher syndrome: mental and behavioral disorders

PubMed Central

2012-01-01

Background Mental and behavioral disorders among adults with Usher syndrome have been discussed and reported in some case studies but no research has been reported on children with Usher syndrome. Methods This article investigates the prevalence and characteristics of mental and behavioral disorders among 26 children, 3-17 years of age, with Usher syndrome. Results Six of the 26 children were diagnosed with a mental or behavioral disorder (1 with schizophrenia and mild mental retardation, 1 with atypical autism and severe mental retardation, 1 with atypical autism and mild mental retardation, 1 with mild mental retardation, and 2 with conduct disorder). Another 3 children had had a mental or behavioral disorder previously in their childhood. Conclusion Even though vision impairment first manifests in late childhood, some children with Usher syndrome seem to develop mental and behavioral disorders during childhood. The aetiology and treatment of mental and behavioral disorders among children with Usher syndrome are discussed. Children with Usher syndrome and their parents may need clinical support during early childhood to prevent development of mental and behavioral disorders. PMID:22449032

[Formula: see text]Executive functioning and its relation to ASD and ADHD symptomatology in 22q11.2 deletion syndrome.

PubMed

de Sonneville, Leo M J; Hidding, Elske; van Engeland, Herman; Vorstman, Jacob A S; Sijmens-Morcus, Monique E J; Swaab, Hanna

2018-01-01

Children with 22q11.2 deletion syndrome (22q11DS; velo-cardio-facial-syndrome) are at risk for the developmental disorders, attention deficit hyperactivity disorder (ADHD) and autism spectrum disorder (ASD). In this study, the relation between executive functioning (EF) and the severity of ADHD and ASD symptoms is examined, since EF is known to be important in relation to emotional and behavioral problems. The participants consist of 58 children (38 females) with a mean age of 13.5 years (SD 2.6). Standardized assessment was used to evaluate the severity of ASD and ADHD symptomatology. The major aspects of EF, i.e., cognitive flexibility, inhibition, sustained attention, distractibility, working memory and reaction speed, were evaluated. The profile of EF in 22q11DS was found to be characterized by weaker performance compared to the norms on all subdomains of EF. Poor cognitive flexibility and inhibition, as well as high distractibility, were found to be related to more severe ASD symptoms, while poor quality of sustained attention and high distractibility were found to be related to more severe ADHD symptoms. It is concluded that children with 22q11DS experience impairments in EF, and that the degree of impairment on specific EF subdomains is related to the severity of ASD and/or ADHD symptomatology. These results may help in defining the mediating role of neurocognitive dysfunctions in the development of social and behavioral problems in 22q11DS.

Gilles de la Tourette's syndrome in a patient with 47(XXX) syndrome: a case report

PubMed Central

2011-01-01

Introduction To the best of our knowledge, this is the first report of a comorbidity between Gilles de la Tourette's syndrome and 47 (XXX) syndrome. The clinical picture of Gilles de la Tourette's Syndrome is well described, while 47 (XXX) syndrome is much more rare and has a broader spectrum of possible phenotypic presentations. Case presentation An Italian Caucasian girl was referred at the age of 11 to our Rehabilitation Center for anxiety and learning difficulties. The girl had already been diagnosed as having 47(XXX) syndrome; she had some rather typical features of the chromosomal abnormality, but she also showed a high level of anxiety and the presence of motor and vocal tics. When an accurate history was taken, a diagnosis of Gilles de la Tourette's Syndrome emerged. Conclusions The possible interaction between peculiar features of these two syndromes in terms of neuropsychological and affective functioning is both interesting for the specific case and to hypothesize models of rehabilitation for patients with one or both syndromes. Executive functions are specifically reduced in both syndromes, therefore it might be hard to discriminate the contribution of each one to the general impairment; the same applies to anxiety. Moreover, mental retardation (with a significantly lower verbal cognitive functioning) poses relevant problems when suggesting cognitive behavioral or psychoeducational rehabilitative approaches. PMID:22054059

Sleep Disturbances Associated with Parkinson's Disease

PubMed Central

Suzuki, Keisuke; Miyamoto, Masayuki; Miyamoto, Tomoyuki; Iwanami, Masaoki; Hirata, Koichi

2011-01-01

Sleep disturbances are common problems affecting the quality life of Parkinson's disease (PD) patients and are often underestimated. The causes of sleep disturbances are multifactorial and include nocturnal motor disturbances, nocturia, depressive symptoms, and medication use. Comorbidity of PD with sleep apnea syndrome, restless legs syndrome, rapid eye movement sleep behavior disorder, or circadian cycle disruption also results in impaired sleep. In addition, the involvement of serotoninergic, noradrenergic, and cholinergic neurons in the brainstem as a disease-related change contributes to impaired sleep structures. Excessive daytime sleepiness is not only secondary to nocturnal disturbances or dopaminergic medication but may also be due to independent mechanisms related to impairments in ascending arousal system and the orexin system. Notably, several recent lines of evidence suggest a strong link between rapid eye movement sleep behavior disorder and the risk of neurodegenerative diseases such as PD. In the present paper, we review the current literature concerning sleep disorders in PD. PMID:21876839

A social competence intervention for young children with high functioning autism and Asperger syndrome: a pilot study.

PubMed

Minne, Elizabeth Portman; Semrud-Clikeman, Margaret

2012-11-01

The key features of Asperger Syndrome (AS) and high functioning autism (HFA) include marked and sustained impairment in social interactions. A multi-session, small group program was developed to increase social perception based on the assumption perceptual or interpretive problems underlying these social difficulties. Additionally, the group format espoused a play therapy orientation and the use of sociodramatic play was the primary therapeutic modality used. Qualitative analyses of the data resulted in an explanation of the key changes in social interactions that took place through the course of the intervention. Although each participant's experience in this group was unique, all children in this program demonstrated improvements in their social interactions, as they experienced development both emotionally and behaviorally. Findings suggest that, despite their rigid interests and behavior patterns, the social limitations of these children improved when provided with the necessary environmental resources.

A comprehensive team approach to the management of patients with Prader-Willi syndrome.

PubMed

Eiholzer, Urs; Whitman, Barbara Y

2004-09-01

Prader-Willi syndrome (PWS) is a genetic disorder characterized by extreme obesity accompanied by other, multisystem clinical manifestations encompassing both physical and behavioral/cognitive abnormalities. The multi-dimensional problems of patients with PWS cannot be treated with a single intervention and benefit from a team approach to management to optimize outcomes. Childhood stature below target height and reduced final height are some defining characteristics of PWS, and compelling evidence from growth hormone (GH) treatment trials suggests that hypothalamic GH deficiency exists. Treatment with GH has been shown to increase height velocity in children with PWS, decrease weight-for-height index values and body fat mass, and have a positive effect on lean body mass during at least the first year of therapy. In addition to medical concerns, the behavioral manifestations, including an uncorrectable deficit in appetite control, and cognitive limitations associated with PWS, require long-term multidisciplinary management.

Asperger syndrome related suicidal behavior: two case studies.

PubMed

Kocourkova, Jana; Dudova, Iva; Koutek, Jiri

2013-01-01

Asperger syndrome hinders adaptation to developmental challenges during childhood and adolescence, particularly with regard to interpersonal relationships. Individuals with Asperger syndrome display lack of empathy and limited ability to understand social and emotional exchanges with other people. Individuals with Asperger syndrome are significantly exposed to the risk of suicidal behavior, especially during adolescence. The authors describe cases of suicidal behavior in two adolescent boys with Asperger syndrome.

Natural history of Sanfilippo syndrome type A.

PubMed

Buhrman, Dakota; Thakkar, Kavita; Poe, Michele; Escolar, Maria L

2014-05-01

To describe the natural history of Sanfilippo syndrome type A. We performed a retrospective review of 46 children (21 boys, 25 girls) with Sanfilippo syndrome type A evaluated between January 2000 and April 2013. Assessments included neurodevelopmental evaluations, audiologic testing, and assessment of growth, adaptive behavior, cognitive behavior, motor function, and speech/language skills. Only the baseline evaluation was included for patients who received hematopoietic stem cell transplantation. Median age at diagnosis was 35 months, with a median delay between initial symptoms to diagnosis of 24 months. The most common initial symptoms were speech/language delay (48%), dysmorphology (22%), and hearing loss (20%). Early behavioral problems included perseverative chewing and difficulty with toilet training. All children developed sleep difficulties and behavioral changes (e.g., hyperactivity, aggression). More than 93% of the children experienced somatic symptoms such as hepatomegaly (67%), abnormal dentition (39%), enlarged tongue (37%), coarse facial features (76%), and protuberant abdomen (43%). Kaplan-Meier analysis showed a 60% probability of surviving past 17 years of age. Sanfilippo type A is characterized by severe hearing loss and speech delay, followed by a rapid decline in cognitive skills by 3 years of age. Significant somatic disease occurs in more than half of patients. Behavioral difficulties presented between 2 and 4 years of age during a rapid period of cognitive decline. Gross motor abilities are maintained during this period, which results in an active child with impaired cognition. Sleep difficulties are concurrent with the period of cognitive degeneration. There is currently an unacceptable delay in diagnosis, highlighting the need to increase awareness of this disease among clinicians.

Multi-informant reports of psychiatric symptoms among high-functioning adolescents with Asperger syndrome or autism.

PubMed

Hurtig, Tuula; Kuusikko, Sanna; Mattila, Marja-Leena; Haapsamo, Helena; Ebeling, Hanna; Jussila, Katja; Joskitt, Leena; Pauls, David; Moilanen, Irma

2009-11-01

The aim of the study was to examine psychiatric symptoms in high-functioning adolescents with autism spectrum disorders reported by multiple informants. Forty-three 11- to 17-year-old adolescents with Asperger syndrome (AS) or high-functioning autism (HFA) and 217 typically developed adolescents completed the Youth Self-Report (YSR), while their parents completed the Child Behavior Checklist (CBCL). Teachers of adolescents with AS/HFA completed the Teacher Report Form (TRF). The informants reported significantly more psychiatric symptoms, especially withdrawn, anxious/depressed, social and attention problems, in adolescents with AS/HFA than in controls. In contrast to findings in the general population, the psychiatric problems of adolescents with AS/HFA are well acknowledged by multiple informants, including self-reports. However, anxiety and depressive symptoms were more commonly reported by adolescents with AS/HFA and their teachers than their parents, indicating that some emotional distress may be hidden from their parents.

Sleep-like behavior and 24-h rhythm disruption in the Tc1 mouse model of Down syndrome.

PubMed

Heise, I; Fisher, S P; Banks, G T; Wells, S; Peirson, S N; Foster, R G; Nolan, P M

2015-02-01

Down syndrome is a common disorder associated with intellectual disability in humans. Among a variety of severe health problems, patients with Down syndrome exhibit disrupted sleep and abnormal 24-h rest/activity patterns. The transchromosomic mouse model of Down syndrome, Tc1, is a trans-species mouse model for Down syndrome, carrying most of human chromosome 21 in addition to the normal complement of mouse chromosomes and expresses many of the phenotypes characteristic of Down syndrome. To date, however, sleep and circadian rhythms have not been characterized in Tc1 mice. Using both circadian wheel-running analysis and video-based sleep scoring, we showed that these mice exhibited fragmented patterns of sleep-like behaviour during the light phase of a 12:12-h light/dark (LD) cycle with an extended period of continuous wakefulness at the beginning of the dark phase. Moreover, an acute light pulse during night-time was less effective in inducing sleep-like behaviour in Tc1 animals than in wild-type controls. In wheel-running analysis, free running in constant light (LL) or constant darkness (DD) showed no changes in the circadian period of Tc1 animals although they did express subtle behavioural differences including a reduction in total distance travelled on the wheel and differences in the acrophase of activity in LD and in DD. Our data confirm that Tc1 mice express sleep-related phenotypes that are comparable with those seen in Down syndrome patients with moderate disruptions in rest/activity patterns and hyperactive episodes, while circadian period under constant lighting conditions is essentially unaffected. © 2015 Medical Research Council. Genes, Brain and Behavior published by International Behavioural and Neural Genetics Society and John Wiley & Sons Ltd.

Structured hypocaloric diet is more effective than behavioral therapy in reducing metabolic syndrome in Mexican postmenopausal women: a randomized controlled trial.

PubMed

Perichart-Perera, Otilia; Balas-Nakash, Margie; Muñoz-Manrique, Cinthya; Legorreta-Legorreta, Jennifer; Rodríguez-Cano, Ameyalli; Mier-Cabrera, Jennifer; Aguilera-Pérez, Jesús Rafael

2014-07-01

This study aims to compare the effects of a lifestyle intervention using a behavioral therapy (BT) approach with the effects of a cardioprotective structured hypocaloric diet on metabolic syndrome in Mexican postmenopausal women. This study is a randomized clinical trial (2006-2009) of Mexican postmenopausal women with metabolic syndrome (Adult Treatment Panel III criteria) who were recruited from the Postmenopause Clinic of the National Institute of Perinatology in Mexico City. Women were assigned to one of two groups--group 1 (structured hypocaloric diet; n = 63): energy restriction (-300 to -500 kcal/d) emphasizing cardioprotective dietary changes; and group 2 (BT; n = 55): goal setting, problem-solving, and stimulus control to achieve cardioprotective dietary and lifestyle recommendations. Metabolic syndrome prevalence, as well as weight, waist circumference, fat mass, and fasting biochemical markers (glucose and lipid profile), were measured at baseline and at 2, 4, and 6 months after the intervention. Metabolic syndrome risk (relative risk and absolute risk reduction), mean differences between groups, and logistic regression were evaluated using Statistical Package for the Social Sciences software, version 17.0. A total of 118 women were studied (mean [SD] age, 53.81 [6.43] y). No baseline differences were observed between groups. At the end of the study, a higher reduction in metabolic syndrome prevalence was observed in group 1 (-38.1%) compared with group 2 (-12.7%; relative risk, 0.237; 95% CI, 0.092-0.608; P = 0.003). The effect was maintained even when adjusted by age, hormone therapy and antihypertensive drug use. A cardioprotective structured hypocaloric diet is more effective than the BT approach in reducing metabolic syndrome after 6 months of intervention. Both strategies have positive effects on different individual cardiovascular risk factors.

Behavioral phenotypes of genetic syndromes with intellectual disability: comparison of adaptive profiles.

PubMed

Di Nuovo, Santo; Buono, Serafino

2011-10-30

The study of distinctive and consistent behaviors in the most common genetic syndromes with intellectual disability is useful to explain abnormalities or associated psychiatric disorders. The behavioral phenotypes revealed outcomes totally or partially specific for each syndrome. The aim of our study was to compare similarities and differences in the adaptive profiles of the five most frequent genetic syndromes, i.e. Down syndrome, Williams syndrome, Angelman syndrome, Prader-Willi syndrome, and Fragile-X syndrome (fully mutated), taking into account the relation with chronological age and the overall IQ level. The research was carried out using the Vineland Adaptive Behavior Scale (beside the Wechsler Intelligence scales to obtain IQ) with a sample of 181 persons (107 males and 74 females) showing genetic syndromes and mental retardation. Syndrome-based groups were matched for chronological age and mental age (excluding the Angelman group, presenting with severe mental retardation). Similarities and differences in the adaptive profiles are described, relating them to IQs and maladaptive behaviors. The results might be useful in obtaining a global index of adjustment for the assessment of intellectual disability level as well as for educational guidance and rehabilitative plans. Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.

Socio-cognitive habilitation using the math interactive learning experience program for alcohol-affected children.

PubMed

Kable, Julie A; Coles, Claire D; Taddeo, Elles

2007-08-01

Fetal alcohol syndrome (FAS) has been recognized as a disabling condition with a significant impact on the neurobehavioral functioning of affected individuals, including cognition, behavior, and academic functioning, but little research has been performed on targeted interventions for these children. A socio-cognitive habilitative program focused on improving behavior and math functioning in children 3 to 10 years of age (n=61) was developed and evaluated. The intervention provided parental instruction on FAS, advocacy, and behavioral regulation via workshops and interactive math tutoring with children. All families received parental instruction and were then randomly assigned to either the math instruction or standard psychoeducational care groups. Satisfaction with workshops was very high, with over 90% agreeing that trainers were knowledgeable and materials easy to understand and helpful. Significant gains in knowledge were found for information provided in the instructional groups. At posttesting, caregivers reported fewer problem behaviors on the Achenbach Child Behavior Checklist, Internalizing Problem Behavior, Externalizing Problem Behavior, and Total Problem Behavior summary scales. After 5 months, both groups of children demonstrated gains in math knowledge but significantly higher gains were found in the group receiving direct math instruction. The math treatment group was also more likely to demonstrate a gain of over 1 standard deviation on any of the 4 math outcome measures used. These findings suggest that parents of children with fetal alcohol spectrum disorders (FAS(D)) benefit from instruction in understanding their child's alcohol-related neurological damage and strategies to provide positive behavioral supports and that targeted psychoeducational programs may be able to remediate some of the math deficits associated with prenatal alcohol exposure.

Preschool children with intellectual disability: syndrome specificity, behaviour problems, and maternal well-being.

PubMed

Eisenhower, A S; Baker, B L; Blacher, J

2005-09-01

Children with intellectual disability (ID) are at heightened risk for behaviour problems and diagnosed mental disorder. Likewise, mothers of children with ID are more stressed than mothers of typically developing children. Research on behavioural phenotypes suggests that different syndromes of ID may be associated with distinct child behavioural risks and maternal well-being risks. In the present study, maternal reports of child behaviour problems and maternal well-being were examined for syndrome-specific differences. The present authors studied the early manifestation and continuity of syndrome-specific behaviour problems in 215 preschool children belonging to 5 groups (typically developing, undifferentiated developmental delays, Down syndrome, autism, cerebral palsy) as well as the relation of syndrome group to maternal well-being. At age 3, children with autism and cerebral palsy showed the highest levels of behaviour problems, and children with Down syndrome and typically developing children showed the lowest levels. Mothers of children with autism reported more parenting stress than all other groups. These syndrome-specific patterns of behaviour and maternal stress were stable across ages 3, 4 and 5 years, except for relative increases in behaviour problems and maternal stress in the Down syndrome and cerebral palsy groups. Child syndrome contributed to maternal stress even after accounting for differences in behaviour problems and cognitive level. These results, although based on small syndrome groups, suggest that phenotypic expressions of behaviour problems are manifested as early as age 3. These behavioural differences were paralleled by differences in maternal stress, such that mothers of children with autism are at elevated risk for high stress. In addition, there appear to be other unexamined characteristics of these syndromes, beyond behaviour problems, which also contribute to maternal stress.

Longitudinal Profiles of Adaptive Behavior in Fragile X Syndrome

PubMed Central

Quintin, Eve-Marie; Jo, Booil; Lightbody, Amy A.; Hazlett, Heather Cody; Piven, Joseph; Hall, Scott S.; Reiss, Allan L.

2014-01-01

OBJECTIVE: To examine longitudinally the adaptive behavior patterns in fragile X syndrome. METHOD: Caregivers of 275 children and adolescents with fragile X syndrome and 225 typically developing children and adolescents (2–18 years) were interviewed with the Vineland Adaptive Behavior Scales every 2 to 4 years as part of a prospective longitudinal study. RESULTS: Standard scores of adaptive behavior in people with fragile X syndrome are marked by a significant decline over time in all domains for males and in communication for females. Socialization skills are a relative strength as compared with the other domains for males with fragile X syndrome. Females with fragile X syndrome did not show a discernible pattern of developmental strengths and weaknesses. CONCLUSIONS: This is the first large-scale longitudinal study to show that the acquisition of adaptive behavior slows as individuals with fragile X syndrome age. It is imperative to ensure that assessments of adaptive behavior skills are part of intervention programs focusing on childhood and adolescence in this condition. PMID:25070318

Perceived motor problems in daily life: Focus group interviews with people with Noonan syndrome and their relatives.

PubMed

Croonen, Ellen A; Harmsen, Mirjam; Van der Burgt, Ineke; Draaisma, Jos M; Noordam, Kees; Essink, Marlou; Nijhuis-van der Sanden, Maria W G

2016-09-01

Studies from a patient perspective on motor performance problems in Noonan syndrome in daily life are lacking. The aims of this study were to provide insight into the motor performance problems that people with Noonan syndrome and/or their relatives experienced, the major consequences they suffered, the benefits of interventions they experienced, and the experiences with healthcare professionals they mentioned. We interviewed 10 adults with Noonan syndrome (two were joined by their parent), and 23 mothers (five of whom had Noonan syndrome), nine fathers (one of whom had Noonan syndrome) and one cousin who reported on 28 children with Noonan syndrome. People with Noonan syndrome reported particular problems related to pain, decreased muscle strength, fatigue, and clumsiness, which had an evident impact on functioning in daily life. Most participants believed that problems with motor performance improved with exercise, appropriate physiotherapy guidance, and other supportive interventions. Nevertheless, people with Noonan syndrome and/or their relatives did not feel heard and supported and experienced no understanding of their problems by healthcare professionals. This was the first study from a patient perspective that described the motor performance problems in people with Noonan syndrome, the major consequences in daily life, the positive experiences of interventions and the miscommunication with healthcare professionals. To achieve optimal support, healthcare professionals, as well as people with Noonan syndrome and/or their relatives themselves, should be aware of these frequently presented problems with motor performance. Research on these different aspects is needed to better understand and support people with Noonan syndrome.© 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

Within-individual correlations reveal link between a behavioral syndrome, condition and cortisol in free-ranging Belding's ground squirrels

PubMed Central

Brooks, Katherine C.; Mateo, Jill. M.

2014-01-01

Animals often exhibit consistent individual differences in behavior (i.e. animal personality) and correlations between behaviors (i.e. behavioral syndromes), yet the causes of those patterns of behavioral variation remain insufficiently understood. Many authors hypothesize that state-dependent behavior produces animal personality and behavioral syndromes. However, empirical studies assessing patterns of covariation among behavioral traits and state variables have produced mixed results. New statistical methods that partition correlations into between-individual and residual within-individual correlations offer an opportunity to more sufficiently quantify relationships among behaviors and state variables to assess hypotheses of animal personality and behavioral syndromes. In a population of wild Belding's ground squirrels (Urocitellus beldingi) we repeatedly measured activity, exploration, and response to restraint behaviors alongside glucocorticoids and nutritional condition. We used multivariate mixed models to determine whether between-individual or within-individual correlations drive phenotypic relationships among traits. Squirrels had consistent individual differences for all five traits. At the between-individual level, activity and exploration were positively correlated whereas both traits negatively correlated with response to restraint, demonstrating a behavioral syndrome. At the within-individual level, condition negatively correlated with cortisol, activity and exploration. Importantly, this indicates that although behavior is state-dependent, which may play a role in animal personality and behavioral syndromes, feedback mechanisms between condition and behavior appear not to produce consistent individual differences in behavior and correlations between them. PMID:25598565

[Behavioral and cognitive profile of corpus callosum agenesia - Review].

PubMed

Lábadi, Beatrix; Beke, Anna Maria

2016-11-30

Agenesis of corpus callosum is a relatively frequent congenital cerebral malformation including dysplasia, total or partial absence of corpus callosum. The agenesis of corpus callosum can be occured in isolated form without accompanying somatic or central nervous system abnormalities and it can be associated with other central nervus system malformations. The behavioral and cognitive outcome is more favorable for patients with isolated agenesis of corpus callous than syndromic form of corpus callosum. The aim of this study is to review recent research on behavioral and social-cognitive functions in individuals with agenesis of corpus callosum. Developmental delay is common especially in higher-order cognitive and social functions. An internet database search was performed to identify publications on the subject. Fifty-five publications in English corresponded to the criteria. These studies reported deficits in language, social cognition and emotions in individuals with agenesis of corpus callosum which is known as primary corpus callous syndrome. The results indicate that individuals with agenesis of corpus callosum have deficiency in social-cognitive domain (recognition of emotions, weakness in paralinguistic aspects of language and mentalizing abilities). The impaired social cognition can be manifested in behavioral problems like autism and attention deficit hyperactivity disorder.

Cross Syndrome Comparison of Sleep Problems in Children with Down Syndrome and Williams Syndrome

ERIC Educational Resources Information Center

Ashworth, Anna; Hill, Catherine M.; Karmiloff-Smith, Annette; Dimitriou, Dagmara

2013-01-01

Based on previous findings of frequent sleep problems in children with Down syndrome (DS) and Williams syndrome (WS), the present study aimed to expand our knowledge by using parent report and actigraphy to define sleep problems more precisely in these groups. Twenty-two school-aged children with DS, 24 with WS and 52 typically developing (TD)…

Executive Functioning Mediates the Effect of Behavioral Problems on Depression in Mothers of Children With Developmental Disabilities

PubMed Central

Chan, Wai; Smith, Leann E.; Greenberg, Jan S.; Hong, Jinkuk; Mailick, Marsha R.

2017-01-01

The present investigation explored long-term relationships of behavioral symptoms of adolescents and adults with developmental disabilities with the mental health of their mothers. Fragile X premutation carrier mothers of an adolescent or adult child with fragile X syndrome (n = 95), and mothers of a grown child with autism (n = 213) were included. Behavioral symptoms at Time 1 were hypothesized to predict maternal depressive symptoms at Time 3 via maternal executive dysfunction at Time 2. Results provided support for the mediating pathway of executive dysfunction. Additionally, the association of behavioral symptoms with executive dysfunction differed across the two groups, suggesting that premutation carriers may be more susceptible to caregiving stress due to their genotype. PMID:28095060

Cognitive and behavioral heterogeneity in genetic syndromes.

PubMed

Pegoraro, Luiz F L; Steiner, Carlos E; Celeri, Eloisa H R V; Banzato, Claudio E M; Dalgalarrondo, Paulo

2014-01-01

this study aimed to investigate the cognitive and behavioral profiles, as well as the psychiatric symptoms and disorders in children with three different genetic syndromes with similar sociocultural and socioeconomic backgrounds. thirty-four children aged 6 to 16 years, with Williams-Beuren syndrome (n=10), Prader-Willi syndrome (n=11), and Fragile X syndrome (n=13) from the outpatient clinics of Child Psychiatry and Medical Genetics Department were cognitively assessed through the Wechsler Intelligence Scale for Children (WISC-III). Afterwards, a full-scale intelligence quotient (IQ), verbal IQ, performance IQ, standard subtest scores, as well as frequency of psychiatric symptoms and disorders were compared among the three syndromes. significant differences were found among the syndromes concerning verbal IQ and verbal and performance subtests. Post-hoc analysis demonstrated that vocabulary and comprehension subtest scores were significantly higher in Williams-Beuren syndrome in comparison with Prader-Willi and Fragile X syndromes, and block design and object assembly scores were significantly higher in Prader-Willi syndrome compared with Williams-Beuren and Fragile X syndromes. Additionally, there were significant differences between the syndromes concerning behavioral features and psychiatric symptoms. The Prader-Willi syndrome group presented a higher frequency of hyperphagia and self-injurious behaviors. The Fragile X syndrome group showed a higher frequency of social interaction deficits; such difference nearly reached statistical significance. the three genetic syndromes exhibited distinctive cognitive, behavioral, and psychiatric patterns. Copyright © 2013 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.

Advances in understanding behavioral phenotypes in neurogenetic syndromes.

PubMed

Harris, James C

2010-11-15

Syndrome-specific behavior was proposed by Langdon Down in his first clinical descriptions. Research interest followed but waned during the eugenics era when antisocial behavior was attributed to people with intellectual disability (ID) and the US Supreme Court legalized involuntary sterilization. When these claims were refuted and behavioral treatments introduced, their focus on environmental determination minimized the importance of biological research. The modern era began with the recognition that patterned behavior, for example, self-injury in Lesch-Nyhan syndrome and hyperphagia in PWS, was syndrome-specific, and when parent support groups pointed out syndrome-specific behavioral similarities in their children. Syndrome-specific rating scales and methodologies followed to allow behavioral comparisons between syndromes. The focus initially was on specific behaviors but with refinements in neuropsychological tests has expanded to include neurocognitive profiles. Greater clarification in genetic diagnoses has led to mutant mouse behavioral models and neurophysiologic and neuroimaging strategies have made possible the study of brain circuits. There is growing interest in investigating the developmental trajectory of behaviors from infancy to adulthood and old age. Because anxiety, mood disturbance, repetitive behaviors, and social deficits commonly occur in people with severe ID, those affected are often given multiple psychiatric diagnoses. This has led to considerable confusion in the literature. It is critical to focus on specific behaviors and cognitive patterns in research and not confuse psychiatric symptoms that lack precise definitions and involve multiple genes, the so-called psychiatric phenotype, with the more specific behavioral phenotype. New treatments based on knowledge of underlying neurobiology call for more fine-grained definition of behavior. © 2010 Wiley-Liss, Inc.

Tanning as an addictive behavior: a literature review.

PubMed

Nolan, Bridgit V; Taylor, Sarah L; Liguori, Anthony; Feldman, Steven R

2009-02-01

Recent studies have identified reinforcing properties associated with tanning and suggest a possible physiologic mechanism and addiction driving tanning behavior. This article attempts to synthesize the existing literature on tanning and addiction to investigate possible associations. We investigated a variety of substance dependence models to define what constitutes dependence/addiction and to determine how current studies on tanning meet these criteria. In some individuals, tanning has met Diagnostic and Statistical Manual criteria for a substance-related disorder or tanning-modified Cut Down, Annoyed, Guilt, Eye-opener criteria. Trial studies have demonstrated the induction of withdrawal symptoms in frequent tanners. Additional studies are needed to investigate the associated dependency and addiction more fully and to elucidate its similarities to other better-known addictive syndromes. Tanning is a problem behavior, both as a health risk and as a possible dependency. Future studies, especially in the area of cognitive mapping and cue-related stimuli are needed. Imaging studies may be important in elucidating whether the same areas of the brain are involved in tanning addiction as in other addictive syndromes.

Delayed puberty, eroticism, and sense of smell: A psychological study of hypogonadotropinism, osmatic and anosmatic (Kallmann's syndrome).

PubMed

Bobrow, N A; Money, J; Lewis, V G

1971-12-01

In 13 male patients with a diagnosis of hypogonadotropic hypogonadism, associated in five cases with verified hyposmia (Kallmann's syndrome), social development and participation were delayed. The patients associated the lack of visible signs of male puberty with their delayed sociosexual maturation. However, the evidence of physical maturation after treatment did not have the ameliorative effect on their social behavior one would have liked to predict. Dating behavior was limited before and after treatment for all the patients, and sexual interest was low even for the three married ones. The experience of falling in love was notably absent in the relationships described. Two characteristic social reaction patterns were social introversion and hostile rejection of agemates. Intellectual functioning and the incidence of personality pathology were not diagnostically noteworthy. None of the patients had any problems with gender identity except for erotic apathy which extended to masturbation. There was an absence of homosexuality. Possibly hypogonadotropic patients have a primary defect of the hypothalamus, manifesting itself as a dysfunction of the behavioral concomitants of puberty as well as a failure of hormonal puberty.

Gilles de la Tourette's syndrome in special education schools: a United Kingdom study.

PubMed

Eapen, V; Robertson, M M; Zeitlin, H; Kurlan, R

1997-06-01

In order to determine the prevalence of tic disorders in children with severe school problems requiring a residential facility and comparison groups of children in regular day schools, we performed direct clinical examinations for the presence of tics and Gilles de la Tourette's syndrome (GTS) in 20 children from a residential school for emotional and behavioral difficulties (EBD); 25 children from a residential school for learning disabilities; 17 "problem" children (PC) (identified by teachers as having academic or behaviour problems) and 19 normal children (NC) selected at random (using random numbers) from a regular school. Of the EBD students, 65% were judged to have definite tics as compared with 24% of students with learning difficulties (P < 0.05), 6% of PC (P < 0.003) and none of the NC (P < 0.0006) group. Most of the affected students met diagnostic criteria for GTS. Our findings suggest that GTS is commonly associated with the need for special education and that this association is particularly robust for children with severe school problems. In these children, the presence of tics may be an indicator of an underlying dysfunction of neurological development.

Tourette Syndrome and Tic Disorders

PubMed Central

Leckman, James F.

2005-01-01

Objective: This is a practical review of Tourette syndrome, including phenomenology, natural history, and state-of-the-art assessment and treatment. Method: Computerized literature searches were conducted under the keywords Tourette syndrome,tics, and children-adolescents. Results: Studies have documented the natural history of Tourette syndrome and its frequent co-occurrence with attention problems, obsessive-compulsive disorder (OCD), and a range of other mood and anxiety disorders, which are often of primary concern to patients and their families. Proper diagnosis and education are often very helpful for patients, parents, siblings, teachers, and peers. When necessary, available anti-tic treatments have proven efficacious. First-line options include the alpha adrenergic agents and the atypical neuroleptics, as well as behavioral interventions such as habit reversal. Conclusions: The study of tics and Tourette symdrome has led to the development of several pathophysiological models and helped in the development of management options. However, fully explanatory models are still needed that would allow for accurate prognostication in the course of illness and the development of improved treatments. PMID:21152158

Self-reported stress among adolescent siblings of individuals with autism spectrum disorder and Down syndrome.

PubMed

Shivers, Carolyn M; McGregor, Casey; Hough, Ashlea

2017-11-01

Despite the prevalence of studies showing increased stress among mothers of individuals with autism spectrum disorders, few studies have examined general stress among typically developing siblings. This study used an online survey to compare the levels of self-reported stress between adolescent siblings of individuals with autism spectrum disorder and Down syndrome. Sibling of individuals with autism reported significantly more overall stress than did siblings of individuals with Down syndrome, as well as more stress specifically attributed to the brother/sister with autism. The two groups did not differ on perceived social support from family and friends. In linear regression models, the disability group (autism vs Down syndrome) was significantly related to sibling stress above and beyond target child behavior problems, perceived social support, and demographic factors. These results help shed light on the daily experiences of adolescent siblings of individuals with autism and call for more research into potential interventions to address increased stress levels.

Pragmatic inferences in high-functioning adults with autism and Asperger syndrome.

PubMed

Pijnacker, Judith; Hagoort, Peter; Buitelaar, Jan; Teunisse, Jan-Pieter; Geurts, Bart

2009-04-01

Although people with autism spectrum disorders (ASD) often have severe problems with pragmatic aspects of language, little is known about their pragmatic reasoning. We carried out a behavioral study on high-functioning adults with autistic disorder (n = 11) and Asperger syndrome (n = 17) and matched controls (n = 28) to investigate whether they are capable of deriving scalar implicatures, which are generally considered to be pragmatic inferences. Participants were presented with underinformative sentences like "Some sparrows are birds". This sentence is logically true, but pragmatically inappropriate if the scalar implicature "Not all sparrows are birds" is derived. The present findings indicate that the combined ASD group was just as likely as controls to derive scalar implicatures, yet there was a difference between participants with autistic disorder and Asperger syndrome, suggesting a potential differentiation between these disorders in pragmatic reasoning. Moreover, our results suggest that verbal intelligence is a constraint for task performance in autistic disorder but not in Asperger syndrome.

Psychological Well-being in Fathers of Adolescents and Young Adults with Down Syndrome, Fragile X Syndrome, and Autism

PubMed Central

Hartley, Sigan L.; Seltzer, Marsha Mailick; Head, Lara; Abbeduto, Leonard

2011-01-01

The psychological well-being of fathers of children with developmental disabilities remains poorly understood. The present study examined depressive symptoms, pessimism, and coping in fathers of adolescents and young adults with Down syndrome (DS; n = 59), autism spectrum disorders (ASDs; n = 135), and fragile X syndrome (FXS; n = 46) Fathers of sons/daughters with ASDs reported a higher level of depressive symptoms than the other groups of fathers. Fathers of sons/daughters with DS reported a lower level of pessimism than the other groups of fathers. There were no group differences in paternal coping style. Group differences in paternal depressive symptoms and pessimism were, in part, related to differences in paternal age, the child’s behavior problems, risk of having additional children with a disability, and maternal depressive symptoms. Findings from this study can be used to educate providers and design services for fathers during the later parenting years. PMID:22611299

Modifiable Lifestyle Behaviors Are Associated With Metabolic Syndrome in a Taiwanese Population.

PubMed

Lin, Kuei-Man; Chiou, Jeng-Yuan; Ko, Shu-Hua; Tan, Jung-Ying; Huang, Chien-Ning; Liao, Wen-Chun

2015-11-01

To explore associations between metabolic syndrome and modifiable lifestyle behaviors among the adult population in Taiwan. This cross-sectional study analyzed data from a nationally representative sample that participated in the 2005-2008 Nutrition and Health Survey in Taiwan. The sample (2,337 participants older than 19 years) provided data on demographic characteristics, modifiable lifestyle behaviors, anthropometric measurements, and blood chemistry panel. These data were analyzed by descriptive statistics, univariate logistic regression, and multivariate logistic regression to determine factors associated with metabolic syndrome. Metabolic syndrome had a prevalence of 25.2%, and this prevalence increased with age. In univariate regression analysis, metabolic syndrome was associated with age, living with family members, educational level, and modifiable lifestyle behaviors (smoking, drinking, betel quid chewing, and physical activity). Individuals with a smoking history and currently chewing betel quid had the highest risk for metabolic syndrome. The risk for metabolic syndrome might be reduced by public health campaigns to encourage people to quit smoking cigarettes and chewing betel quid. Implementing more modifiable lifestyle behaviors in daily life will decrease metabolic syndrome in Taiwan. Considering that betel quid chewing and tobacco smoking interact to adversely affect metabolic syndrome risk, public health campaigns against both behaviors seem to be a cost-effective and efficient health promotion strategy to reduce the prevalence rate of metabolic syndrome. © 2015 Sigma Theta Tau International.

When somatization is not the only thing you suffer from: Examining comorbid syndromes using latent profile analysis, parenting practices and adolescent functioning.

PubMed

Scharf, Miri; Mayseless, Ofra; Rousseau, Sofie

2016-10-30

Understanding somatization presents a challenge to clinicians because it is often associated with other syndromes. We addressed somatization's comorbidity with other internalizing syndromes (anxiety, depression, withdrawal) using latent profile analysis. A representative sample of 3496 Israeli middle and high-school youths reported their internalizing symptoms, perceived parenting practices, psychosocial functioning, and health behaviors. Four profiles, similar across age and gender, were identified: overall-low (65.4%), moderately-high anxiety/depression/withdrawal (24.4%), high somatization (4.8%), and overall-high (5.4%). MANOVAs and follow-up ANOVAs revealed that for the most part the overall-high profile evinced the worst parenting, psychosocial functioning, and health behaviors (smoking and drinking), while the overall-low group evinced the best. For most variables the high somatization and moderately high profiles displayed midway results. However, the moderately-high profile reported higher levels of harsh parenting than the high somatization profile. The high somatization profile reported similar or higher levels of smoking, risk taking, vandalism, and rule violation than the overall-high group. High somatization, either alone or alongside anxiety, depression, and withdrawal, was associated with disruptive and risk-taking behaviors. This link might reflect problems in emotion and anger regulation and become stronger in adolescence because of dysregulation processes characterizing this period. Implications for practice are discussed. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

Trajectory and outcomes of speech language therapy in the Prader-Willi syndrome (PWS): case report.

PubMed

Misquiatti, Andréa Regina Nunes; Cristovão, Melina Pavini; Brito, Maria Claudia

2011-03-01

The aim of this study was to describe the trajectory and the outcomes of speech-language therapy in Prader-Willi syndrome through a longitudinal study of the case of an 8 year-old boy, along four years of speech-language therapy follow-up. The therapy sessions were filmed and documental analysis of information from the child's records regarding anamnesis, evaluation and speech-language therapy reports and multidisciplinary evaluations were carried out. The child presented typical characteristics of Prader-Willi syndrome, such as obesity, hyperfagia, anxiety, behavioral problems and self aggression episodes. Speech-language pathology evaluation showed orofacial hypotony, sialorrhea, hypernasal voice, cognitive deficits, oral comprehension difficulties, communication using gestures and unintelligible isolated words. Initially, speech-language therapy had the aim to promote the language development emphasizing social interaction through recreational activities. With the evolution of the case, the main focus became the development of conversation and narrative abilities. It were observed improvements in attention, symbolic play, social contact and behavior. Moreover, there was an increase in vocabulary, and evolution in oral comprehension and the development of narrative abilities. Hence, speech-language pathology intervention in the case described was effective in different linguistic levels, regarding phonological, syntactic, lexical and pragmatic abilities.

Exploration of Differences in Types of Sleep Disturbance and Severity of Sleep Problems between Individuals with Cri du Chat Syndrome, Down's Syndrome, and Jacobsen Syndrome: A Case Control Study

ERIC Educational Resources Information Center

Maas, Anneke P. H. M.; Didden, Robert; Korzilius, Hubert; Curfs, Leopold M. G.

2012-01-01

The prevalence of sleep problems in individuals with intellectual disability (ID) seems to vary between genetic syndromes associated with ID. Different types of sleep disturbances may indicate underlying causes of sleep problems and these types of sleep disturbances may vary between different genetic syndromes. We examined and compared five types…

The impact of eating behavior on psychological symptoms typical of reactive hypoglycemia. A pilot study comparing women with polycystic ovary syndrome to controls.

PubMed

Barry, John A; Bouloux, Pierre; Hardiman, Paul J

2011-08-01

The idea that diet can affect mood and behavior in women with polycystic ovary syndrome (PCOS) by altering blood glucose levels has become popular in recent years. This paper describes an online survey (N=462) of 24 women with PCOS, 299 healthy control women, 47 women who possibly had undiagnosed PCOS, and 92 men. The groups were compared for symptoms of mood and behavioral symptoms typical of reactive (postprandial) hypoglycemia. The outcome measures were two questionnaires that measure states associated with hypoglycemia: the Hypoglycemia Symptom Checklist-7 (HSC-7), which measures behavioral symptoms and the Mood Adjective Checklist (MACL), which measures emotional states. Controlling for age and body mass index (BMI) using between-groups analysis of covariance (ANCOVA), the women with PCOS scored significantly higher than the other three groups (p<0.001) on the outcome measures. These differences remained statistically significant in a subset of twelve women with PCOS compared to twelve healthy control women closely matched for age, BMI, and eating behavior. The findings are suggestive of hypoglycemia-related mood and behavioral problems in PCOS. Future research should test whether blood glucose levels correlate with these symptoms in PCOS, and whether a low glycemic index ('low-GI') diet improves the symptoms. Copyright © 2011 Elsevier Ltd. All rights reserved.

Hypersomnia in children: interface with psychiatric disorders.

PubMed

Kotagal, Suresh

2009-10-01

Patients being evaluated in child psychiatry clinics for behavior and mood disturbances frequently exhibit daytime sleepiness. Conversely, patients being evaluated for hypersomnia by sleep specialists may have depressed mood or hyperactive and aggressive behavior. The etiology of daytime sleepiness in children and adolescents is diverse and includes inadequate sleep hygiene, obstructive sleep apnea, delayed sleep phase syndrome, idiopathic hypersomnia, periodic hypersomnia, narcolepsy, and mood disorders per se. Treatment of a sleep disorder can have a favorable impact on alertness and quality of life. A high index of suspicion for sleep problems should be maintained in children and adolescents with psychiatric disorders.

Heart Activity and Autistic Behavior in Infants and Toddlers with Fragile X Syndrome

PubMed Central

Roberts, Jane E.; Tonnsen, Bridgette; Robinson, Ashley; Shinkareva, Svetlana V.

2014-01-01

The present study contrasted physiological arousal in infants and toddlers with fragile X syndrome to typically developing control participants and examined physiological predictors early in development to autism severity later in development in fragile X syndrome. Thirty-one males with fragile X syndrome (ages 8–40 months) and 25 age-matched control participants were included. The group with fragile X syndrome showed shorter interbeat intervals (IBIs), lower vagal tone (VT), and less modulation of IBI. Data suggested a nonlinear effect with IBI and autistic behavior; however, a linear effect with VT and autistic behavior emerged. These findings suggest that atypical physiological arousal emerges within the first year and predicts severity of autistic behavior in fragile X syndrome. These relationships are complex and dynamic, likely reflecting endogenous factors assumed to reflect atypical brain function secondary to reduced fragile X mental retardation protein. This research has important implications for the early identification and treatment of autistic behaviors in young children with fragile X syndrome. PMID:22515825

An under-diagnosed geriatric syndrome: sleep disorders among older adults.

PubMed

Tufan, Asli; Ilhan, Birkan; Bahat, Gulistan; Karan, Mehmet Akif

2017-06-01

Sleep disorders are commonly under-diagnosed in the geriatric population. We aimed to determine the prevalence of sleep problems among older adults admitted to the geriatrics out-patient clinic. Two hundred and three patients (136 female) older than 75 years of age were included in the study. Patients underwent comprehensive geriatric assessment, including identification of sleep problems using the Sleep Disturbance Scale, Rapid eye movement (REM) sleep behavior disorder (RBD) Single-Question Screen questionnaire (RBD1Q) and The Johns Hopkins Restless Leg Syndrome Severity Scale. Demographic and clinical data including age, sex, medications, comorbid diseases, body mass index and functional scores was noted. The mean age of the patients was 80.92±4.3 years. 35.5% of the patients had findings of REM-SBD and 32.5% of the patients had restless legs syndrome. Ninety-seven percent of the patients answered 'yes' to at least one of the sleep disturbance scale questions. There was no significant difference between male and female groups. We observed that sleep disorders were common among older adults. For this reason, the course and quality of sleep should be examined in all patients as a routine part of comprehensive geriatric assessment.

Zebrafish Models of Prader-Willi Syndrome: Fast Track to Pharmacotherapeutics

PubMed Central

Spikol, Emma D.; Laverriere, Caroline E.; Robnett, Maya; Carter, Gabriela; Wolfe, Erin; Glasgow, Eric

2016-01-01

Prader-Willi syndrome (PWS) is a rare genetic neurodevelopmental disorder characterized by an insatiable appetite, leading to chronic overeating and obesity. Additional features include short stature, intellectual disability, behavioral problems and incomplete sexual development. Although significant progress has been made in understanding the genetic basis of PWS, the mechanisms underlying the pathogenesis of the disorder remain poorly understood. Treatment for PWS consists mainly of palliative therapies; curative therapies are sorely needed. Zebrafish, Danio rerio, represent a promising way forward for elucidating physiological problems such as obesity and identifying new pharmacotherapeutic options for PWS. Over the last decade, an increased appreciation for the highly conserved biology among vertebrates and the ability to perform high-throughput drug screening has seen an explosion in the use of zebrafish for disease modeling and drug discovery. Here, we review recent advances in developing zebrafish models of human disease. Aspects of zebrafish genetics and physiology that are relevant to PWS will be discussed, and the advantages and disadvantages of zebrafish models will be contrasted with current animal models for this syndrome. Finally, we will present a paradigm for drug screening in zebrafish that is potentially the fastest route for identifying and delivering curative pharmacotherapies to PWS patients. PMID:27857842

Systematic review of the clinical and genetic aspects of Prader-Willi syndrome

PubMed Central

2011-01-01

Prader-Willi syndrome (PWS) is a complex multisystem genetic disorder that is caused by the lack of expression of paternally inherited imprinted genes on chromosome 15q11-q13. This syndrome has a characteristic phenotype including severe neonatal hypotonia, early-onset hyperphagia, development of morbid obesity, short stature, hypogonadism, learning disabilities, behavioral problems, and psychiatric problems. PWS is an example of a genetic condition caused by genomic imprinting. It can occur via 3 main mechanisms that lead to the absence of expression of paternally inherited genes in the 15q11.2-q13 region: paternal microdeletion, maternal uniparental disomy, and an imprinting defect. Over 99% of PWS cases can be diagnosed using DNA methylation analysis. Early diagnosis of PWS is important for effective long-term management. Growth hormone (GH) treatment improves the growth, physical phenotype, and body composition of patients with PWS. In recent years, GH treatment in infants has been shown to have beneficial effects on the growth and neurological development of patients diagnosed during infancy. There is a clear need for an integrated multidisciplinary approach to facilitate early diagnosis and optimize management to improve quality of life, prevent complications, and prolong life expectancy in patients with PWS. PMID:21503198

Systematic review of the clinical and genetic aspects of Prader-Willi syndrome.

PubMed

Jin, Dong Kyu

2011-02-01

Prader-Willi syndrome (PWS) is a complex multisystem genetic disorder that is caused by the lack of expression of paternally inherited imprinted genes on chromosome 15q11-q13. This syndrome has a characteristic phenotype including severe neonatal hypotonia, early-onset hyperphagia, development of morbid obesity, short stature, hypogonadism, learning disabilities, behavioral problems, and psychiatric problems. PWS is an example of a genetic condition caused by genomic imprinting. It can occur via 3 main mechanisms that lead to the absence of expression of paternally inherited genes in the 15q11.2-q13 region: paternal microdeletion, maternal uniparental disomy, and an imprinting defect. Over 99% of PWS cases can be diagnosed using DNA methylation analysis. Early diagnosis of PWS is important for effective long-term management. Growth hormone (GH) treatment improves the growth, physical phenotype, and body composition of patients with PWS. In recent years, GH treatment in infants has been shown to have beneficial effects on the growth and neurological development of patients diagnosed during infancy. There is a clear need for an integrated multidisciplinary approach to facilitate early diagnosis and optimize management to improve quality of life, prevent complications, and prolong life expectancy in patients with PWS.

[Care continuity for patients with Prader-Willi syndrome during transition from childhood to adulthood].

PubMed

Saitoh, Shinji

2010-01-01

Prader-Willi syndrome(PWS) is a complex multisystem genetic disorder, of which characteristic phenotypes include neonatal hypotonia, hyperphagia resulting in obesity, mental retardation, hypogonadism, and behavioral and psychiatric problems. The diagnosis can be obtained as early as during neonatal period thanks to development of genetic testing. Clinical features of PWS will change depending on age, although core phenotypes of hyperphagia, obesity and psychiatric issues stay for lifetime. Therefore, integrated multidisciplinary approach starting from neonatal period is mandatory to ensure optimal management to improve lifelong quality of life. For successful transition from childhood to adulthood, multidisciplinary team need to share clinical information, and should keep the same policy about food, environment and psychiatric issues.

Life Satisfaction Among Mothers of Individuals with Prader-Willi Syndrome.

PubMed

Shivers, Carolyn M; Leonczyk, Caroline L; Dykens, Elisabeth M

2016-06-01

Mothers of individuals with Prader-Willi syndrome (PWS) often experience numerous stressors, even when compared to mothers of children with other intellectual and developmental disabilities. Despite this, these mothers show great variability in self-reported life satisfaction. Using data from a longitudinal study of individuals with PWS and their families, the present study analyzed factors related to maternal life satisfaction, both cross-sectionally and over time. Results show that both child factors (e.g., behavior problems, hyperphagia) and maternal factors (e.g., stress, coping style) were significantly related to maternal life satisfaction. However, none of the tested variables predicted change in life satisfaction over time. Research and practice implications are discussed.

Sleep phenotypes in infants and toddlers with neurogenetic syndromes.

PubMed

Abel, Emily A; Tonnsen, Bridgette L

2017-10-01

Although sleep problems are well characterized in preschool- and school-age children with neurogenetic syndromes, little is known regarding the early emergence of these problems in infancy and toddlerhood. To inform syndrome-specific profiles and targets for intervention, we compared parent-reported sleep problems in infants and toddlers with Angelman syndrome (AS), Williams syndrome (WS), and Prader-Willi syndrome (PWS) with patterns observed among same-aged typically developing (TD) controls. Mothers of 80 children (18 AS, 19 WS, 19 PWS, and 24 TD) completed the Brief Infant Sleep Questionnaire. Primary dependent variables included (1) sleep onset latency, (2) total sleep duration, (3) daytime and nighttime sleep duration, and (4) sleep problem severity, as measured by both maternal impression and National Sleep Foundation guidelines. Sleep problems are relatively common in children with neurogenetic syndromes, with 41% of mothers reporting problematic sleep and 29% of children exhibiting abnormal sleep durations as per national guidelines. Across genetic subgroups, problems are most severe in children with AS and WS, particularly in relation to nighttime sleep duration. Although atypical sleep is characteristically reported in each syndrome later in development, infants and toddlers with PWS exhibited largely typical patterns, potentially indicating delayed onset of sleep problems in concordance with other medical features of PWS. Our findings suggest that sleep problems in neurogenetic syndromes emerge as early as infancy and toddlerhood, with variable profiles across genetic subgroups. This work underscores the importance of early sleep screenings as part of routine medical care of neurosyndromic populations and the need for targeted, syndrome-sensitive treatment. Copyright © 2017 Elsevier B.V. All rights reserved.

Treatment of the psychiatric problems associated with fragile X syndrome.

PubMed

Hagerman, Randi J; Polussa, Jonathan

2015-03-01

This work reviews recent research regarding treatment of fragile X syndrome (FXS), the most common inherited cause of intellectual disability and autism spectrum disorder. The phenotype includes anxiety linked to sensory hyperarousal, hyperactivity, and attentional problems consistent with attention deficit hyperactivity disorder and social deficits leading to autism spectrum disorder in 60% of boys and 25% of girls with FXS. Multiple targeted treatments for FXS have rescued the phenotype of the fmr1 knockout mouse, but few have been beneficial to patients with FXS. The failure of the metabotropic glutamate receptor 5 antagonists falls on the heels of the failure of Arbaclofen's efficacy in children and adults with autism or FXS. In contrast, efficacy has been demonstrated in a controlled trial of minocycline in children with FXS. Minocycline lowers the abnormally elevated levels of matrix metalloproteinase 9 in FXS. Acamprosate and lovastatin have been beneficial in open-label trials in FXS. The first 5 years of life may be the most efficacious time for intervention when combined with behavioral and/or educational interventions. Minocycline, acamprosate, lovastatin, and sertraline are treatments that can be currently prescribed and have shown benefit in children with FXS. Use of combined medical and behavioral interventions will likely be most efficacious for the treatment of FXS.

A Mixed Presentation of Serotonin Syndrome vs Neuroleptic Malignant Syndrome in a 12-Year-Old Boy.

PubMed

Sun, Christie; Sweet, Hannah; Minns, Alicia B; Shapiro, Desiree; Jenkins, Willough

2018-04-24

Neuroleptic malignant syndrome (NMS) and serotonin syndrome (SS) are serious medical conditions associated with commonly prescribed psychiatric medications. Although the mechanisms differ, they can be clinically difficult to distinguish. We report a case of a pediatric patient with complicated psychiatric history that developed features of both syndromes in the setting of polypharmacy. A 12-year-old boy with a history of developmental delay, attention-deficit hyperactivity disorder, and posttraumatic stress disorder presented to the emergency department with behavior changes consisting of delayed reactions, gait instability, drooling, and slowed movements. Ten days before presentation, his outpatient psychiatrist had made multiple medication changes including discontinuation of cyproheptadine (an appetite stimulant) and initiation of aripiprazole. On arrival, the patient was noted to be tachycardia and hypertensive for age. He was disoriented, intermittently agitated, and tremulous with increased tonicity, clonus in the lower extremities, and mydriasis. He was supportively treated with lorazepam and intravenous fluids while discontinuing potential offending agents. His course was complicated by hypertension and agitation managed with dexmedetomidine infusion and benzodiazepines. His mental status, tremors, and laboratory values began to improve over the next 2 days, and eventually transitioned to the inpatient psychiatric unit on hospital day 7. Diagnosis of NMS or SS can be difficult when there is overlap between syndromes, particularly in the setting of multiple potential offending agents or underlying developmental delay. In addition, pediatric patients may present atypically as compared with adult patients with the same condition. The use of antipsychotic medications for young children with behavioral problems has risen dramatically in the last decade, increasing their risk for developing SS or NMS.

Effects of Social Context on Social Interaction and Self-Injurious Behavior in Cornelia de Lange Syndrome

ERIC Educational Resources Information Center

Arron, Kate; Oliver, Chris; Hall, Scott; Sloneem, Jenny; Forman, Debbie; McClintock, Karen

2006-01-01

Cornelia de Lange syndrome is reported to be associated with self-injurious behavior (SIB) and social avoidance. We used analog methodology to examine the effect of manipulating adult social contact on social communicative behaviors and SIB in 16 children with this syndrome. For 9 participants engagement behavior was related to levels of adult…

Cooccurrence of Post-Intensive Care Syndrome Problems Among 406 Survivors of Critical Illness.

PubMed

Marra, Annachiara; Pandharipande, Pratik P; Girard, Timothy D; Patel, Mayur B; Hughes, Christopher G; Jackson, James C; Thompson, Jennifer L; Chandrasekhar, Rameela; Ely, Eugene Wesley; Brummel, Nathan E

2018-05-21

To describe the frequency of cooccurring newly acquired cognitive impairment, disability in activities of daily livings, and depression among survivors of a critical illness and to evaluate predictors of being free of post-intensive care syndrome problems. Prospective cohort study. Medical and surgical ICUs from five U.S. centers. Patients with respiratory failure or shock, excluding those with preexisting cognitive impairment or disability in activities of daily livings. None. At 3 and 12 months after hospital discharge, we assessed patients for cognitive impairment, disability, and depression. We categorized patients into eight groups reflecting combinations of cognitive, disability, and mental health problems. Using multivariable logistic regression, we modeled the association between age, education, frailty, durations of mechanical ventilation, delirium, and severe sepsis with the odds of being post-intensive care syndrome free. We analyzed 406 patients with a median age of 61 years and an Acute Physiology and Chronic Health Evaluation II of 23. At 3 and 12 months, one or more post-intensive care syndrome problems were present in 64% and 56%, respectively. Nevertheless, cooccurring post-intensive care syndrome problems (i.e., in two or more domains) were present in 25% at 3 months and 21% at 12 months. Post-intensive care syndrome problems in all three domains were present in only 6% at 3 months and 4% at 12 months. More years of education was associated with greater odds of being post-intensive care syndrome free (p < 0.001 at 3 and 12 mo). More severe frailty was associated with lower odds of being post-intensive care syndrome free (p = 0.005 at 3 mo and p = 0.048 at 12 mo). In this multicenter cohort study, one or more post-intensive care syndrome problems were present in the majority of survivors, but cooccurring problems were present in only one out of four. Education was protective from post-intensive care syndrome problems and frailty predictive of the development of post-intensive care syndrome problems. Future studies are needed to understand better the heterogeneous subtypes of post-intensive care syndrome and to identify modifiable risk factors.

[Orbitofrontal cortex and morality].

PubMed

Funayama, Michitaka; Mimura, Masaru

2012-10-01

Research on the neural substrates of morality is a recently emerging field in neuroscience. The anatomical structures implicated to play a role in morality include the frontal lobe, temporal lobe, cingulate gyrus, amygdala, hippocampus, and basal ganglia. In particular, the orbitofrontal or ventromedial prefrontal areas are thought to be involved in decision-making, and damage to these areas is likely to cause decision-making deficits and/or problems in impulsive control, which may lead to antisocial and less moral behaviors. In this article, we focus on case presentation and theory development with regard to moral judgment. First, we discuss notable cases and syndromes developing after orbitofrontal/ventromedial prefrontal damage, such as the famous cases of Gage and EVR, cases of childhood orbitofrontal damage, forced collectionism, squalor syndrome, and hypermoral syndrome. We then review the proposed theories and neuropsychological mechanisms underlying decision-making deficits following orbitofrontal/ventromedial prefrontal damage, including the somatic-marker hypothesis, reversal learning, preference judgment, theory of mind, and moral dilemma.

Sensory motor and functional skills of dizygotic twins: one with Smith-Magenis syndrome and a twin control.

PubMed

Smith, Michaele R; Hildenbrand, Hanna; Smith, Ann C M

2009-01-01

Smith-Magenis syndrome (SMS), the result of an interstitial deletion within chromosome 17p11.2, is a disorder that may include minor dysmorphic features, brachydactyly, short stature, hypotonia, speech delays, cognitive deficits, signs of peripheral neuropathy, scoliosis, and neurobehavioral problems including sleep disturbances and maladaptive repetitive and self-injurious behaviors. Physical and occupational therapists provide services for children who have the syndrome, whose genetic disorder is frequently not identified or diagnosed before 1 year of age. A comprehensive physical and occupational therapy evaluation was completed in nonidentical twins with one having SMS, using the Sensory Profile; Brief Assessment of Motor Function (BAMF); Peabody Developmental Motor Scales, Second Edition (PDMS-2); and Pediatric Evaluation of Disability Inventory (PEDI). This provides a framework for conducting assessments to enhance early detection and interdisciplinary management with this specialized population.

Culturally Sensitive Education Can Decrease Hispanic Workers' Risk of Metabolic Syndrome.

PubMed

Marks, Steven

2016-04-08

Metabolic syndrome is a continuing problem in the United States, and the Hispanic population is at increased risk of developing complications from this disease process. Many of the workers at a South Jersey yacht-building company are Hispanic, and a culturally sensitive education program was implemented to improve their overall health. Program assessments included the Latino Dietary Behavior Questionnaire (LDBQ), anthropomorphic measurements, and laboratory studies both pre and post intervention. The intervention included a Spanish-language lecture on metabolic syndrome and healthy nutrition, followed by an interactive culturally appropriate cooking demonstration for the participants and their significant others. Program participants showed reductions in body mass index, blood pressure, lipid levels, and hemoglobin A1c, and more knowledge of healthy nutrition as measured by the LDBQ. This finding supports culturally sensitive education programs to improve the health of Hispanic populations. © 2016 The Author(s).

Copy number variations in patients with electrical status epilepticus in sleep.

PubMed

Kevelam, Sietske H G; Jansen, Floor E; Binsbergen, Ellen van; Braun, Kees P J; Verbeek, Nienke E; Lindhout, Dick; Poot, Martin; Brilstra, Eva H

2012-02-01

Electrical status epilepticus in sleep syndrome is the association of the electroencephalographic pattern and deficits in language or global cognitive function and behavioral problems. The etiology is often unknown, but genetic risk factors have been implicated. Array-based comparative genomic hybridization was used to identify copy number variations in 13 children with electrical status epilepticus in sleep syndrome to identify possible underlying risk factors. Seven copy number variations were detected in 4 of the 13 patients, which consisted of 6 novel gains and 1 loss, the recurrent 15q13.3 microdeletion. Two patients carried a probable pathogenic copy number variation containing a gene involved in the cholinergic pathway. Genetic aberrations in patients with electrical status epilepticus in sleep syndrome can provide an entry in the investigation of the etiology of electrical status epilepticus in sleep. However, further studies are needed to confirm our findings.

Links between Sleep and Daytime Behaviour Problems in Children with Down Syndrome

ERIC Educational Resources Information Center

Esbensen, A. J.; Hoffman, E. K.; Beebe, D. W.; Byars, K. C.; Epstein, J.

2018-01-01

Background: In the general population, sleep problems have an impact on daytime performance. Despite sleep problems being common among children with Down syndrome, the impact of sleep problems on daytime behaviours in school-age children with Down syndrome is an understudied topic. Our study examined the relationship between parent-reported and…

Behavioral Phenotype of Fragile X Syndrome in Adolescence and Adulthood

ERIC Educational Resources Information Center

Smith, Leann E.; Barker, Erin T.; Seltzer, Marsha Mailick; Abbeduto, Leonard; Greenberg, Jan S.

2012-01-01

The present study explored the behavioral profile of individuals with fragile X syndrome during adolescence and adulthood. Individuals with both fragile X syndrome and autism (n = 30) were compared with (a) individuals diagnosed with fragile X syndrome (but not autism; n = 106) and (b) individuals diagnosed with autism (but not fragile X syndrome;…

Behavioral and psychological signs and symptoms of dementia: a practicing psychiatrist's viewpoint

PubMed Central

E. Mintzer, Jacobo; F. Mirski, Dario; S. Hoernig, Kathleen

2000-01-01

Alzheimer's disease typically presents with two often overlapping syndromes, one cognitive, the other behavioral. The behavioral syndrome is characterized by psychosis, aggression, depression, anxiety, agitation, and other common if less well-defined symptoms subsumed under the umbrella entity “behavioral and psychological symptoms of dementia” (BPSD), itself divided into a number of subsyndromes: psychosis, circadian rhythm (sleepwake) disturbance, depression, anxiety, and agitation, it is BPSD with its impact on care providers that ultimately precipitates the chain of events resulting in long-term institutional care. The treatment challenge involves eliminating unmet medical needs (undiagnosed hip fracture and asymptomatic urinary tract infection or pneumonia). Pharmacologic intervention relies on risperidone and, increasingly cholinesterase inhibitors for the control of psychosis (but with response rates of only 65% at tolerable doses), olanzapine and risperidone for anxiety, and carbamazepine and valproic acid for agitation. However, evidence increasingly favors nonpharmacologic interventions, to the extent that these should now be considered as the foundation of BPSD treatment. Problem behaviors are viewed as meaningful responses to unmet needs in the therapeutic milieu. Because the progression and impact of BPSD varies between patients, interventions must be explored, designed, implemented, and assessed on an individual basis. They include: family support and education, psychotherapy reality orientation, validation therapy, reminiscence and life review, behavioral interventions, therapeutic activities and creative arts therapies, environmental considerations (including restraint-free facilities), behavioral intensive care units, and workplace design and practices that aid the ongoing management of professional caregiver stress. PMID:22034243

Neuropsychology of traumatic brain injury: An expert overview.

PubMed

Azouvi, P; Arnould, A; Dromer, E; Vallat-Azouvi, C

Traumatic brain injury (TBI) is a serious healthcare problem, and this report is a selective review of recent findings on the epidemiology, pathophysiology and neuropsychological impairments following TBI. Patients who survive moderate-to-severe TBI frequently suffer from a wide range of cognitive deficits and behavioral changes due to diffuse axonal injury. These deficits include slowed information-processing and impaired long-term memory, attention, working memory, executive function, social cognition and self-awareness. Mental fatigue is frequently also associated and can exacerbate the consequences of neuropsychological deficits. Personality and behavioral changes can include combinations of impulsivity and apathy. Even mild TBI raises specific problems: while most patients recover within a few weeks or months, a minority of patients may suffer from long-lasting symptoms (post-concussion syndrome). The pathophysiology of such persistent problems remains a subject of debate, but seems to be due to both injury-related and non-injury-related factors. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

Individualism-Collectivism, Social Self-Control and Adolescent Substance Use and Risky Sexual Behavior.

PubMed

Pokhrel, Pallav; Bennett, Brooke L; Regmi, Sakshi; Idrisov, Bulat; Galimov, Artur; Akhmadeeva, Leila; Sussman, Steve

2018-06-07

Individualism and collectivism are cultural syndromes that have been associated with adolescent problem behavior in studies conducted in the U.S. and Southeast Asia. However, research investigating the mechanisms of how cultural orientation impacts health risk behaviors has been limited. This study tested a new model explaining the relationship between cultural orientation (i.e., individualism, collectivism) and adolescent problem behavior (i.e., substance use and risky sex) in terms of interpersonal self-regulation (i.e., social self-control). As such, the study is rooted in theories of the role of culture in developing self-regulation. Participants were high school students (N = 716) from the Bashkirtostan Republic of the Russian Federation. Adolescents from the Russian Federation tend to show high prevalence of cigarette smoking and binge drinking. People of the Russian Federation in general are traditionally collectivist in orientation, although increased globalization and post-Soviet capitalism may indicate high individualist values in younger generation Russians. Using path analysis we found that in addition to having direct effects, higher individualism indirectly affected substance use and risky sexual behavior through social self-control and negative life events. Higher collectivism was found to have a direct protective effect on risky sexual behavior and a direct effect on social self-control. However, collectivism was not found to have indirect effects on substance use or risky sexual behavior. Higher individualism appears to function as a risk factor for adolescent problem behavior and this relationship may be mediated by lower social self-control. Culturally-tailored prevention programs utilizing the individualism-collectivism framework may benefit from addressing social self-control.

Microdeletion/microduplication of proximal 15q11.2 between BP1 and BP2: a susceptibility region for neurological dysfunction including developmental and language delay.

PubMed

Burnside, Rachel D; Pasion, Romela; Mikhail, Fady M; Carroll, Andrew J; Robin, Nathaniel H; Youngs, Erin L; Gadi, Inder K; Keitges, Elizabeth; Jaswaney, Vikram L; Papenhausen, Peter R; Potluri, Venkateswara R; Risheg, Hiba; Rush, Brooke; Smith, Janice L; Schwartz, Stuart; Tepperberg, James H; Butler, Merlin G

2011-10-01

The proximal long arm of chromosome 15 has segmental duplications located at breakpoints BP1-BP5 that mediate the generation of NAHR-related microdeletions and microduplications. The classical Prader-Willi/Angelman syndrome deletion is flanked by either of the proximal BP1 or BP2 breakpoints and the distal BP3 breakpoint. The larger Type I deletions are flanked by BP1 and BP3 in both Prader-Willi and Angelman syndrome subjects. Those with this deletion are reported to have a more severe phenotype than individuals with either Type II deletions (BP2-BP3) or uniparental disomy 15. The BP1-BP2 region spans approximately 500 kb and contains four evolutionarily conserved genes that are not imprinted. Reports of mutations or disturbed expression of these genes appear to impact behavioral and neurological function in affected individuals. Recently, reports of deletions and duplications flanked by BP1 and BP2 suggest an association with speech and motor delays, behavioral problems, seizures, and autism. We present a large cohort of subjects with copy number alteration of BP1 to BP2 with common phenotypic features. These include autism, developmental delay, motor and language delays, and behavioral problems, which were present in both cytogenetic groups. Parental studies demonstrated phenotypically normal carriers in several instances, and mildly affected carriers in others, complicating phenotypic association and/or causality. Possible explanations for these results include reduced penetrance, altered gene dosage on a particular genetic background, or a susceptibility region as reported for other areas of the genome implicated in autism and behavior disturbances.

Changing patterns of adolescent sexual behavior: consequences for health and development.

PubMed

Friedman, H L

1992-07-01

Sexuality is a fundamental quality of human life, important for health, happiness, individual development, and indeed for the preservation of the human race. During the dynamic period of adolescence in which the passage from childhood to maturity takes place, sexuality takes on new dimensions; feelings become more intense, relationships become more complex, and the consequences of sexual behavior are radically altered. This not only affects the behavior of young people but also of those who interact with them, their families and peers, and those who work in the health, education, youth, social welfare, and other sectors. In the contemporary world the conditions of life for many young people have also changed, and with it patterns of sexual behavior. In general, earlier puberty, later marriage, a decline in the family leading to less control and more autonomy, and intense exposure to sexual stimuli via the mass media and travel across cultural boundaries have made pre-marital adolescent sexual activity more common. This has added to traditional problems of early marriage, newer problems of early pregnancy, childbirth, and induced abortion outside of marriage, sexually transmitted diseases, and human immunodeficiency syndrome infection leading to acquired immunodeficiency syndrome. But the work of the World Health Organization (WHO), along with many others in the field, strongly suggests that given appropriate information and services, trust and equity between the sexes, young people will behave responsibly and well. In this paper some of the findings from methods developed by WHO for research, training, advocacy, and evaluation, and findings in relation to patterns and determinants of sexual and reproductive health and development will be described, and future directions suggested.

Age-related perception of stature, acceptance of therapy, and psychosocial functioning in human growth hormone-treated girls with Turner's syndrome.

PubMed

Lagrou, K; Xhrouet-Heinrichs, D; Heinrichs, C; Craen, M; Chanoine, J P; Malvaux, P; Bourguignon, J P

1998-05-01

This study evaluated the perception of stature, acceptance of therapy, and psychosocial functioning in relation to age at onset and time on treatment during 2 yr of GH therapy in 31 girls with Turner's syndrome grouped by age (group A: 3.7-5.8 yr, n = 9; group B: 7.2-11.8 yr, n = 13; group C: 12.5-16.4 yr, n = 9). The growth response after 2 yr was significant in the 3 groups when calculated in terms of growth norms for untreated Turner girls (mean increase in height SD score: +1.2, +1.5, and +1.1, respectively). The effect was less marked in terms of growth norms for normal girls, particularly in group B (+0.5 SD score). Height was perceived as a problem by most patients, except in the youngest girls at the start of treatment (group A) and in the majority of the adolescents after 2 yr of GH therapy (group C), without evidence of relation to growth response during therapy. The GH injections were fairly well accepted by all patients, except those younger than 6 yr. In all patients, expected adult height was unrealistic and became more realistic with age, whereas no consistent changes were observed in relation to growth response to GH therapy. The Child Behavior Checklist revealed elevated mean scores at the behavioral subscales of attention problems (group A and B), social problems, withdrawal, and anxiety-depression (most obviously in group B). No significant changes were seen during GH therapy. In group C, an elevated mean social problem score at the Youth Self Report and a low mean social self-esteem score at the Self-Esteem Inventory were observed before therapy and showed a significant improvement during 2 yr of GH treatment. These results, however, might be biased due to an increase in social desirability during therapy. We conclude that the perception of height, acceptance of GH therapy, and psychosocial functioning in girls with Turner's syndrome show important differences between age groups, with only slight changes observed during GH therapy.

Impact of Tourette Syndrome on School Measures in a Nationally Representative Sample.

PubMed

Claussen, Angelika H; Bitsko, Rebecca H; Holbrook, Joseph R; Bloomfield, Jeanette; Giordano, Kathy

2018-05-01

Children with Tourette syndrome (TS) are at risk for a variety of co-occurring conditions and learning and school problems. The purpose of this study was to determine the impact of TS and co-occurring conditions on school measures. Parent-reported data from the 2007-2008 and 2011-2012 National Survey of Children's Health were combined (n = 129,353 children aged 6-17 yrs). Parent report of health care provider diagnosis of TS; co-occurring mental, emotional, and behavioral conditions; learning and language conditions; and school measures were assessed. School measures included type of school, individual education plan (IEP), number of school days missed, school problems, doing well in school, doing homework, and repeating a grade. Children with TS were compared with those who never had TS on school measures accounting for co-occurring conditions. After adjusting for demographics, compared with children without TS, children currently with TS were more likely to have an IEP, have a parent contacted about school problems, and not complete homework. After further adjusting for co-occurring conditions, only IEP status remained statistically significant. Compared with children with mild TS, children with moderate or severe TS were more likely to have an IEP, repeat a grade, encounter school problems, and not care about doing well in school. Tourette syndrome severity and co-occurring conditions are associated with school challenges and educational service needs. Awareness among health care providers, teachers and parents of the potential challenges related to both TS and co-occurring conditions would help to best support the child's education.

Down Syndrome: A Current Perspective. Bulletin 1762.

ERIC Educational Resources Information Center

Louisiana State Dept. of Education, Baton Rouge.

This informational document is intended to help answer questions commonly asked by families, professionals, and the general public concerning Down Syndrome. Medical problems associated with Down Syndrome include heart conditions, upper respiratory disorders, ear infections and hearing loss, vision/eye problems, cervical problems, and…

The Dysexecutive Questionnaire advanced: item and test score characteristics, 4-factor solution, and severity classification.

PubMed

Bodenburg, Sebastian; Dopslaff, Nina

2008-01-01

The Dysexecutive Questionnaire (DEX, , Behavioral assessment of the dysexecutive syndrome, 1996) is a standardized instrument to measure possible behavioral changes as a result of the dysexecutive syndrome. Although initially intended only as a qualitative instrument, the DEX has also been used increasingly to address quantitative problems. Until now there have not been more fundamental statistical analyses of the questionnaire's testing quality. The present study is based on an unselected sample of 191 patients with acquired brain injury and reports on the data relating to the quality of the items, the reliability and the factorial structure of the DEX. Item 3 displayed too great an item difficulty, whereas item 11 was not sufficiently discriminating. The DEX's reliability in self-rating is r = 0.85. In addition to presenting the statistical values of the tests, a clinical severity classification of the overall scores of the 4 found factors and of the questionnaire as a whole is carried out on the basis of quartile standards.

The Impact of Group Drumming on Social-Emotional Behavior in Low-Income Children

PubMed Central

Ho, Ping; Tsao, Jennie C. I.; Bloch, Lian; Zeltzer, Lonnie K.

2011-01-01

Low-income youth experience social-emotional problems linked to chronic stress that are exacerbated by lack of access to care. Drumming is a non-verbal, universal activity that builds upon a collectivistic aspect of diverse cultures and does not bear the stigma of therapy. A pretest-post-test non-equivalent control group design was used to assess the effects of 12 weeks of school counselor-led drumming on social-emotional behavior in two fifth-grade intervention classrooms versus two standard education control classrooms. The weekly intervention integrated rhythmic and group counseling activities to build skills, such as emotion management, focus and listening. The Teacher's Report Form was used to assess each of 101 participants (n = 54 experimental, n = 47 control, 90% Latino, 53.5% female, mean age 10.5 years, range 10–12 years). There was 100% retention. ANOVA testing showed that intervention classrooms improved significantly compared to the control group in broad-band scales (total problems (P < .01), internalizing problems (P < .02)), narrow-band syndrome scales (withdrawn/depression (P < .02), attention problems (P < .01), inattention subscale (P < .001)), Diagnostic and Statistical Manual of Mental Disorders-oriented scales (anxiety problems (P < .01), attention deficit/hyperactivity problems (P < .01), inattention subscale (P < .001), oppositional defiant problems (P < .03)), and other scales (post-traumatic stress problems (P < .01), sluggish cognitive tempo (P < .001)). Participation in group drumming led to significant improvements in multiple domains of social-emotional behavior. This sustainable intervention can foster positive youth development and increase student-counselor interaction. These findings underscore the potential value of the arts as a therapeutic tool. PMID:21660091

Carer-Reported Contemporary Health Problems in People with Severe and Profound Intellectual Disability and Genetic Syndromes

ERIC Educational Resources Information Center

Berg, Katy; Arron, Kate; Burbidge, Cheryl; Moss, Joanna; Oliver, Chris

2007-01-01

Identifying health problems in people with severe and profound disabilities and genetic syndromes can be problematic, and unidentified or unmanaged health problems may adversely impact an individual's quality of life. The authors studied carer-reported contemporary health problems in three genetic syndromes (Angelman, Cornelia de Lange, and Cri du…

Prevalence and risk factors of depression in childhood and adolescence as seen in four districts of North-Eastern Uganda.

PubMed

Kinyanda, Eugene; Kizza, Ruth; Abbo, Catherine; Ndyanabangi, Sheila; Levin, Jonathan

2013-04-05

Millions of African children are having to grow up under harsh and adverse psychosocial conditions but it's not fully understood how this negative psychosocial environment is affecting their mental health. This paper examines the prevalence and risk factors of depression in childhood and adolescence as seen in a community sample derived from four disadvantaged districts in north-eastern Uganda. 1587 children were assessed using a structured instrument administered by trained psychiatric nurses to collect data on psychiatric disorders (DSM IV criteria), adverse psychosocial factors and socio-demographic factors. The point prevalence of depressive disorder syndromes (DDS) in this study was 8.6% (95% CI 7.2%-10.1%) with a point prevalence for major depressive episode of 7.6% (95% CI 6.3%-9.0%) and dysthymia of 2.1% (95% CI 1.5%-3.0%). At multiple logistic regression, the factors that were independently significantly associated with DDS were: district (representing ecological factors), nature of living arrangements, domestic violence and psychiatric co-morbidities/psychiatric problems of emotional distress (assessed by the SDQ), suicidality and marginally, anxiety disorder syndromes, eating disorder syndromes, motor disorder syndromes and behavioral and developmental disorder syndromes (the later being protective against depression). Disadvantaged north-eastern Uganda had a high prevalence of childhood depressive disorders. Ecological factors, markers of the quality of the child-principal caregiver relationship (nature of living arrangements and domestic violence) and the presence of psychiatric co-morbidities/psychiatric problems were the important independent determinants of childhood depression in this study.

PET scan perfusion imaging in the Prader–Willi syndrome: new insights into the psychiatric and social disturbances

PubMed Central

Mantoulan, Carine; Payoux, Pierre; Diene, Gwenaëlle; Glattard, Mélanie; Rogé, Bernadette; Molinas, Catherine; Sevely, Annick; Zilbovicius, Monica; Celsis, Pierre; Tauber, Maïthé

2011-01-01

The Prader–Willi syndrome (PWS), a rare multisystem genetic disease, leads to severe disabilities, such as morbid obesity, endocrine dysfunctions, psychiatric disorders, and social disturbances. We explored the whole brain of patients with PWS to detect abnormalities that might explain the behavioral and social disturbances, as well as the psychiatric disorders of these patients. Nine patients with PWS (six males, three females; mean age 16.4 years) underwent a positron emission tomography (PET) scan with H215O as a tracer to measure regional cerebral blood flow (rCBF). The images were compared with those acquired from nine controls (six males, three females; mean age 21.2 years). A morphologic magnetic resonance imaging (MRI) was also performed in PWS patients, and their cognitive and behavioral skills were assessed with Wechsler Intelligence Scale for Children III and the Child Behavior Check List (CBCL). The MRI images showed no evident anatomic abnormalities, whereas PET scans revealed hypoperfused brain regions in PWS patients compared with controls, particularly in the anterior cingulum and superior temporal regions. We observed a significant relationship (P<0.05) between rCBF in the hypoperfused regions and CBCL scores. The functional consequences of these perfusion abnormalities in specific brain regions might explain the behavioral and social problems observed in these individuals. PMID:20588317

Use of levetiracetam to treat tics in children and adolescents with Tourette syndrome.

PubMed

Awaad, Yasser; Michon, Anne Marie; Minarik, Sarah

2005-06-01

Some drugs currently used to treat tics in pediatric patients have drawbacks, including the risk of side effects. New therapeutic options with better safety profiles are needed. Levetiracetam is an antiepileptic drug with atypical mechanisms of action that might be beneficial for this indication. We evaluated the effects of levetiracetam on motor and vocal tics, behavior, and school performance in children and adolescents with tics and Tourette syndrome (TS). Sixty patients, < or =18 years of age, with tics and TS were enrolled in this prospective, open-label study. The initial starting dose of levetiracetam was 250 mg/day. The dosage was titrated over 3 weeks to 1,000 to 2,000 mg/day. Clinical outcomes were assessed with the Clinical Global Impression Scale, Yale Global Tic Severity Scale, and Revised Conners' Parent Rating Scale. Behavior and school performance were also recorded. All 60 patients showed improvements based on all of the scales used, and 43 patients improved with regard to behavior and school performance. Levetiracetam was generally well tolerated. Three patients discontinued treatment because of exaggeration of preexisting behavioral problems. Levetiracetam may be useful in treating tics in children and adolescents. Given its established safety profile, levetiracetam is a candidate for evaluation in a well-controlled trial. (c) 2005 Movement Disorder Society.

PET scan perfusion imaging in the Prader-Willi syndrome: new insights into the psychiatric and social disturbances.

PubMed

Mantoulan, Carine; Payoux, Pierre; Diene, Gwenaëlle; Glattard, Mélanie; Rogé, Bernadette; Molinas, Catherine; Sevely, Annick; Zilbovicius, Monica; Celsis, Pierre; Tauber, Maïthé

2011-01-01

The Prader-Willi syndrome (PWS), a rare multisystem genetic disease, leads to severe disabilities, such as morbid obesity, endocrine dysfunctions, psychiatric disorders, and social disturbances. We explored the whole brain of patients with PWS to detect abnormalities that might explain the behavioral and social disturbances, as well as the psychiatric disorders of these patients. Nine patients with PWS (six males, three females; mean age 16.4 years) underwent a positron emission tomography (PET) scan with H(2)(15)O as a tracer to measure regional cerebral blood flow (rCBF). The images were compared with those acquired from nine controls (six males, three females; mean age 21.2 years). A morphologic magnetic resonance imaging (MRI) was also performed in PWS patients, and their cognitive and behavioral skills were assessed with Wechsler Intelligence Scale for Children III and the Child Behavior Check List (CBCL). The MRI images showed no evident anatomic abnormalities, whereas PET scans revealed hypoperfused brain regions in PWS patients compared with controls, particularly in the anterior cingulum and superior temporal regions. We observed a significant relationship (P<0.05) between rCBF in the hypoperfused regions and CBCL scores. The functional consequences of these perfusion abnormalities in specific brain regions might explain the behavioral and social problems observed in these individuals.

International epidemiology of child and adolescent psychopathology ii: integration and applications of dimensional findings from 44 societies.

PubMed

Rescorla, Leslie; Ivanova, Masha Y; Achenbach, Thomas M; Begovac, Ivan; Chahed, Myriam; Drugli, May Britt; Emerich, Deisy Ribas; Fung, Daniel S S; Haider, Mariam; Hansson, Kjell; Hewitt, Nohelia; Jaimes, Stefanny; Larsson, Bo; Maggiolini, Alfio; Marković, Jasminka; Mitrović, Dragan; Moreira, Paulo; Oliveira, João Tiago; Olsson, Martin; Ooi, Yoon Phaik; Petot, Djaouida; Pisa, Cecilia; Pomalima, Rolando; da Rocha, Marina Monzani; Rudan, Vlasta; Sekulić, Slobodan; Shahini, Mimoza; de Mattos Silvares, Edwiges Ferreira; Szirovicza, Lajos; Valverde, José; Vera, Luis Anderssen; Villa, Maria Clara; Viola, Laura; Woo, Bernardine S C; Zhang, Eugene Yuqing

2012-12-01

To build on Achenbach, Rescorla, and Ivanova (2012) by (a) reporting new international findings for parent, teacher, and self-ratings on the Child Behavior Checklist, Youth Self-Report, and Teacher's Report Form; (b) testing the fit of syndrome models to new data from 17 societies, including previously underrepresented regions; (c) testing effects of society, gender, and age in 44 societies by integrating new and previous data; (d) testing cross-society correlations between mean item ratings; (e) describing the construction of multisociety norms; (f) illustrating clinical applications. Confirmatory factor analyses (CFAs) of parent, teacher, and self-ratings, performed separately for each society; tests of societal, gender, and age effects on dimensional syndrome scales, DSM-oriented scales, Internalizing, Externalizing, and Total Problems scales; tests of agreement between low, medium, and high ratings of problem items across societies. CFAs supported the tested syndrome models in all societies according to the primary fit index (Root Mean Square Error of Approximation [RMSEA]), but less consistently according to other indices; effect sizes were small-to-medium for societal differences in scale scores, but very small for gender, age, and interactions with society; items received similarly low, medium, or high ratings in different societies; problem scores from 44 societies fit three sets of multisociety norms. Statistically derived syndrome models fit parent, teacher, and self-ratings when tested individually in all 44 societies according to RMSEAs (but less consistently according to other indices). Small to medium differences in scale scores among societies supported the use of low-, medium-, and high-scoring norms in clinical assessment of individual children. Copyright © 2012 American Academy of Child and Adolescent Psychiatry. Published by Elsevier Inc. All rights reserved.

Residential Transitions among Adults with Intellectual Disability across 20 Years

PubMed Central

Woodman, Ashley C.; Mailick, Marsha R.; Anderson, Kristy A.; Esbensen, Anna J.

2014-01-01

The present study addresses critical gaps in the literature by examining residential transitions among 303 adults with intellectual disability over 10 years (Part 1) and 75 adults with Down syndrome over 20 years (Part 2). All adults lived at home at the start of the study, but many moved to a variety of settings. Several characteristics of the adults with intellectual disability differed across settings, most notably adaptive behavior and the number of residential transitions, while characteristics such as age, type of disability, and behavior problems were less predictive of residential placements. The number of moves over the course of the study varied widely, with critical links to earlier family dynamics, social relationships, and health and adaptive behavior. PMID:25354121

Treatment of Fragile X Syndrome with a Neuroactive Steroid

DTIC Science & Technology

2013-08-01

GABAA activity . We hypothesized that ganaxolone will significantly improve behavioral problems including anxiety, inattention and impulsivity...group I metabotropic glutamate receptors (mGluRs). In the hippocampal field CA1, activation of mGluR5 leads to long-term depression (LTD), which is seen...as a reduction in synaptic responses. Importantly, LTD triggered by mGluR activation (mGluR-LTD) requires the rapid translation of preexisting mRNA in

Paradoxical effect of baclofen on social behavior in the fragile X syndrome mouse model.

PubMed

Zeidler, Shimriet; Pop, Andreea S; Jaafar, Israa A; de Boer, Helen; Buijsen, Ronald A M; de Esch, Celine E F; Nieuwenhuizen-Bakker, Ingeborg; Hukema, Renate K; Willemsen, Rob

2018-06-01

Fragile X syndrome (FXS) is a common monogenetic cause of intellectual disability, autism spectrum features, and a broad range of other psychiatric and medical problems. FXS is caused by the lack of the fragile X mental retardation protein (FMRP), a translational regulator of specific mRNAs at the postsynaptic compartment. The absence of FMRP leads to aberrant synaptic plasticity, which is believed to be caused by an imbalance in excitatory and inhibitory network functioning of the synapse. Evidence from studies in mice demonstrates that GABA, the major inhibitory neurotransmitter in the brain, and its receptors, is involved in the pathogenesis of FXS. Moreover, several FXS phenotypes, including social behavior deficits, could be corrected in Fmr1 KO mice after acute treatment with GABA B agonists. As FXS would probably require a lifelong treatment, we investigated the effect of chronic treatment with the GABA B agonist baclofen on social behavior in Fmr1 KO mice on two behavioral paradigms for social behavior: the automated tube test and the three-chamber sociability test. Unexpectedly, chronic baclofen treatment resulted in worsening of the FXS phenotypes in these behavior tests. Strikingly, baclofen treatment also affected wild-type animals in both behavioral tests, inducing a phenotype similar to that of untreated Fmr1 KO mice. Altogether, the disappointing results of recent clinical trials with the R-baclofen enantiomer arbaclofen and our current results indicate that baclofen should be reconsidered and further evaluated before its application in targeted treatment for FXS. © 2018 The Authors. Brain and Behavior published by Wiley Periodicals, Inc.

YY1 Haploinsufficiency Causes an Intellectual Disability Syndrome Featuring Transcriptional and Chromatin Dysfunction.

PubMed

Gabriele, Michele; Vulto-van Silfhout, Anneke T; Germain, Pierre-Luc; Vitriolo, Alessandro; Kumar, Raman; Douglas, Evelyn; Haan, Eric; Kosaki, Kenjiro; Takenouchi, Toshiki; Rauch, Anita; Steindl, Katharina; Frengen, Eirik; Misceo, Doriana; Pedurupillay, Christeen Ramane J; Stromme, Petter; Rosenfeld, Jill A; Shao, Yunru; Craigen, William J; Schaaf, Christian P; Rodriguez-Buritica, David; Farach, Laura; Friedman, Jennifer; Thulin, Perla; McLean, Scott D; Nugent, Kimberly M; Morton, Jenny; Nicholl, Jillian; Andrieux, Joris; Stray-Pedersen, Asbjørg; Chambon, Pascal; Patrier, Sophie; Lynch, Sally A; Kjaergaard, Susanne; Tørring, Pernille M; Brasch-Andersen, Charlotte; Ronan, Anne; van Haeringen, Arie; Anderson, Peter J; Powis, Zöe; Brunner, Han G; Pfundt, Rolph; Schuurs-Hoeijmakers, Janneke H M; van Bon, Bregje W M; Lelieveld, Stefan; Gilissen, Christian; Nillesen, Willy M; Vissers, Lisenka E L M; Gecz, Jozef; Koolen, David A; Testa, Giuseppe; de Vries, Bert B A

2017-06-01

Yin and yang 1 (YY1) is a well-known zinc-finger transcription factor with crucial roles in normal development and malignancy. YY1 acts both as a repressor and as an activator of gene expression. We have identified 23 individuals with de novo mutations or deletions of YY1 and phenotypic features that define a syndrome of cognitive impairment, behavioral alterations, intrauterine growth restriction, feeding problems, and various congenital malformations. Our combined clinical and molecular data define "YY1 syndrome" as a haploinsufficiency syndrome. Through immunoprecipitation of YY1-bound chromatin from affected individuals' cells with antibodies recognizing both ends of the protein, we show that YY1 deletions and missense mutations lead to a global loss of YY1 binding with a preferential retention at high-occupancy sites. Finally, we uncover a widespread loss of H3K27 acetylation in particular on the YY1-bound enhancers, underscoring a crucial role for YY1 in enhancer regulation. Collectively, these results define a clinical syndrome caused by haploinsufficiency of YY1 through dysregulation of key transcriptional regulators. Copyright © 2017 The Authors. Published by Elsevier Inc. All rights reserved.

Social Behavior and Autism Traits in a Sex Chromosomal Disorder: Klinefelter (47XXY) Syndrome

ERIC Educational Resources Information Center

van Rijn, Sophie; Swaab, Hanna; Aleman, Andre; Kahn, Rene S.

2008-01-01

Although Klinefelter syndrome (47,XXY) has been associated with psychosocial difficulties, knowledge of the social behavioral phenotype is limited. We examined specific social abilities and autism traits in Klinefelter syndrome. Scores of 31 XXY men on the Scale for Interpersonal Behavior and the Autism Spectrum Questionnaire were compared to 24…

Oxytocin treatment in children with Prader-Willi syndrome: A double-blind, placebo-controlled, crossover study.

PubMed

Miller, Jennifer L; Tamura, Roy; Butler, Merlin G; Kimonis, Virginia; Sulsona, Carlos; Gold, June-Anne; Driscoll, Daniel J

2017-05-01

Prader-Willi syndrome (PWS) is a rare, complex multisystem genetic disorder which includes hypothalamic dysfunction, hyperphagia, cognitive and behavioral problems, increased anxiety, and compulsive behaviors. Individuals with PWS have a deficit of oxytocin producing neurons in the paraventricular nucleus of the hypothalamus. Oxytocin plays a role in regulation of feeding behaviors, social interactions, and emotional reactivity, which are all issues that significantly affect the quality of life for individuals with this syndrome. We performed a double-blind, placebo-controlled, crossover study in 24 children with PWS at three academic institutions using 5 days of intranasal oxytocin (IN-OT) or 5 days of intranasal placebo spray, followed by a 4 week washout period, and then patients returned for 5 days of treatment with the alternate source. Questionnaires, including the Aberrant Behavior Checklist, Social Responsiveness Scale, Repetitive Behavior Scale - Revised, and the Hyperphagia Questionnaire, as well as Clinical Global Impression scales were administered. Blood testing for sodium, potassium, and glucose levels on days 2, 4, and 6, and a 24 hr diet recall. All scales factor improvement from Day 3 to Day 6 favored oxytocin over placebo. No single factor showed a statistically significant difference (P < 0.05) between groups at Day 6. The drug effect appeared to be diminished at Day 14. There was no evidence of a difference between oxytocin and placebo in safety lab parameters, 60 min post dose vital signs, weight, or diet parameters. The results from this study suggest that low dose intranasal oxytocin is safe for individuals with PWS and may result in reduction in appetite drive, and improvements in socialization, anxiety, and repetitive behaviors. Further, long-term studies with a larger population of participants are necessary to confirm these findings. The results of this study are encouraging that oxytocin may be a safe and effective treatment for many of the issues that negatively impact individuals with PWS. © 2017 Wiley Periodicals, Inc.

Education and Children with Down Syndrome: Neuroscience, Development, and Intervention

ERIC Educational Resources Information Center

Fidler, Deborah J.; Nadel, Lynn

2007-01-01

Of the recent advances in education-related research in Down syndrome, the characterization of the Down syndrome behavioral phenotype has become a potentially critical tool for shaping education and intervention in this population. This article briefly reviews the literature on brain-behavior connections in Down syndrome and identifies aspects of…

Repetitive Behavior in Rubinstein-Taybi Syndrome: Parallels with Autism Spectrum Phenomenology

ERIC Educational Resources Information Center

Waite, Jane; Moss, Joanna; Beck, Sarah R.; Richards, Caroline; Nelson, Lisa; Arron, Kate; Burbidge, Cheryl; Berg, Katy; Oliver, Chris

2015-01-01

Syndrome specific repetitive behavior profiles have been described previously. A detailed profile is absent for Rubinstein-Taybi syndrome (RTS). The Repetitive Behaviour Questionnaire and Social Communication Questionnaire were completed for children and adults with RTS (N = 87), Fragile-X (N = 196) and Down (N = 132) syndromes, and individuals…

Comorbidity of functional urinary incontinence and encopresis: somatic and behavioral associations.

PubMed

Von Gontard, Alexander; Hollmann, Elke

2004-06-01

Functional urinary incontinence and encopresis are common comorbid disorders in childhood. We analyze the specific somatic and behavioral symptoms associated with functional enuresis/urinary incontinence and encopresis when they occur together. A total of 167 consecutive children 5 to 10 year olds, with day and/or night wetting were examined prospectively with ultrasound, uroflowmetry, electroencephalography, the Child Behavior Checklist, Culture Fair Intelligence Test and ICD-10 child psychiatric diagnoses. The main findings for the comorbid group (20 patients) with wetting and soiling were a significantly higher rate of daytime incontinence and micturition problems, thickened bladder walls and pathological electroencephalography. There were higher, although not significant, rates of previous urinary tract infections, antibiotic prophylaxis, residual volume and abnormal uroflow curves in this group. Behaviorally, hyperkinetic syndromes, and emotional and conduct disorders (according to ICD-10) were more common. Of the 20 patients 65% had a Child Behavior Checklist total score (greater than 90th percentile) in the clinical range. The externalizing, internalizing, delinquent and anxious/depressed problem scales were also significantly higher. This risk group requires detailed assessment and specific treatment. In addition to the symptomatic treatment of the wetting and soiling, many of these children are in need of specific behavioral, psychotherapeutic and pharmacological treatment.

Bridges and barriers to successful transitioning as perceived by adolescents and young adults with Asperger syndrome.

PubMed

Giarelli, Ellen; Ruttenberg, Jean; Segal, Andrea

2013-01-01

In this thematic content analysis we examined the expectations, and perceived facilitators of (referred to as bridges) and barriers to transition to community as reported by adolescents and young adults with Asperger syndrome. Participants were adolescents/young adults, ages 18-23 years were from the East Coast of the United States. Seventy percent of adolescents hoped for employment (n = 10). Thirty percent desired to find a partner and raise a family. Perceived barriers were: self-assessed behavioral problems, self-assessed associated features, other personal factors, and institutional factors. Bridges to facilitate transition were: accommodations in the community, cognitive abilities, personal qualities/strengths, and mentor's qualities. Copyright © 2013 Elsevier Inc. All rights reserved.

Leisure activities in Prader-Wili syndrome: implications for health, cognition and adaptive functioning.

PubMed

Dykens, Elisabeth M

2014-02-01

Although hyperphagia and compulsivity in Prader-Willi syndrome (PWS) are well described, recreation and adaptive skills are relatively unexplored. Parents of 123 participants with PWS (4-48 years) completed measures of their child's adaptive, recreation, and problem behaviors. Offspring received cognitive testing. Watching TV was the most frequent recreational activity, and was associated with compulsivity and skin picking. BMIs were negatively correlated with physical play, and highest in those who watched TV and played computer games. Computer games and physical activities were associated with higher IQ and adaptive scores. People with PWS and other disabilities need to watch less TV and be more engaged in physical activities, games, and leisure pursuits that are fun, and may bring cognitive or adaptive advantages.

The Relationship of Repetitive Behavior and Sensory Behavior to Parenting Stress in Mothers of Boys with Autism and Mothers of Boys with Fragile X Syndrome

ERIC Educational Resources Information Center

Richardson, Lolita Lisa

2010-01-01

This study investigated the relationship between repetitive behaviors and sensory behavior to the parenting stress of mothers of boys with fragile X syndrome and mothers of boys with autism. Participants consisted of two groups: 51 mothers with boys diagnosed with fragile X syndrome (M = 71.3, SD = 56.5) and 30 mothers with boys diagnosed with…

The developmental trajectory of disruptive behavior in Down syndrome, fragile X syndrome, Prader-Willi syndrome and Williams syndrome.

PubMed

Rice, Lauren J; Gray, Kylie M; Howlin, Patricia; Taffe, John; Tonge, Bruce J; Einfeld, Stewart L

2015-06-01

The aim of this study was to investigate the developmental trajectories of verbal aggression, physical aggression, and temper tantrums in four genetic syndrome groups. Participants were part of the Australian Child to Adult Development Study (ACAD), which collected information from a cohort of individuals with an intellectual disability at five time points over 18 years. Data were examined from a total of 248 people with one of the four following syndromes: Down syndrome, Fragile X syndrome, Prader-Willi syndrome, or Williams syndrome. Changes in behaviors were measured using validated items from the Developmental Behavior Checklist (DBC). The results indicate that, while verbal aggression shows no evidence of diminishing with age, physical aggression, and temper tantrums decline with age before 19 years for people with Down syndrome, Fragile X syndrome, and William syndrome; and after 19 years for people with Prader-Willi syndrome. These findings offer a somewhat more optimistic outlook for people with an intellectual disability than has previously been suggested. Research is needed to investigate the mechanisms predisposing people with PWS to persistence of temper tantrums and physical aggression into adulthood. © 2015 Wiley Periodicals, Inc.

Behavioral phenotype in a child with Prader-Willi syndrome and comorbid 47, XYY.

PubMed

Palkar, Pooja; Kabasakalian, Anahid; Taylor, Bonnie; Doernberg, Ellen; Ferretti, Casara Jean; Uzunova, Genoveva; Hollander, Eric

2016-08-01

We report a 12-year-old male with Prader-Willi syndrome (PWS) and 47, XYY syndrome. Genetic work up revealed 47, XYY karyotype. PWS diagnosis was made by polymerase chain reaction methylation and maternal uniparental disomy (mUPD) was determined to be the etiology. Review of distinct behavioral features, possible interplay between the two syndromes and considerations for diagnoses are presented. To our knowledge, this is the first report of behavioral features in PWS with comorbid 47, XYY.

A network view on psychiatric disorders: network clusters of symptoms as elementary syndromes of psychopathology.

PubMed

Goekoop, Rutger; Goekoop, Jaap G

2014-01-01

The vast number of psychopathological syndromes that can be observed in clinical practice can be described in terms of a limited number of elementary syndromes that are differentially expressed. Previous attempts to identify elementary syndromes have shown limitations that have slowed progress in the taxonomy of psychiatric disorders. To examine the ability of network community detection (NCD) to identify elementary syndromes of psychopathology and move beyond the limitations of current classification methods in psychiatry. 192 patients with unselected mental disorders were tested on the Comprehensive Psychopathological Rating Scale (CPRS). Principal component analysis (PCA) was performed on the bootstrapped correlation matrix of symptom scores to extract the principal component structure (PCS). An undirected and weighted network graph was constructed from the same matrix. Network community structure (NCS) was optimized using a previously published technique. In the optimal network structure, network clusters showed a 89% match with principal components of psychopathology. Some 6 network clusters were found, including "Depression", "Mania", "Anxiety", "Psychosis", "Retardation", and "Behavioral Disorganization". Network metrics were used to quantify the continuities between the elementary syndromes. We present the first comprehensive network graph of psychopathology that is free from the biases of previous classifications: a 'Psychopathology Web'. Clusters within this network represent elementary syndromes that are connected via a limited number of bridge symptoms. Many problems of previous classifications can be overcome by using a network approach to psychopathology.

Restless legs syndrome, rapid eye movement sleep behavior disorder, and hypersomnia in patients with two parkin mutations.

PubMed

Limousin, Nadège; Konofal, Eric; Karroum, Elias; Lohmann, Ebba; Theodorou, Ioannis; Dürr, Alexandra; Arnulf, Isabelle

2009-10-15

Parkin gene mutations cause a juvenile parkinsonism. Patients with these mutations may commonly exhibit REM sleep behaviour disorders, but other sleep problems (insomnia, sleepiness, restless legs syndrome) have not been studied. The aim of this study was to evaluate the sleep-wake phenotype in patients with two parkin mutations, compared with patients with idiopathic Parkinson's disease (iPD). Sleep interview and overnight video-polysomnography, followed by multiple sleep latency tests, were assessed in 11 consecutive patients with two parkin mutations (aged 35-60 years, from seven families) and 11 sex-matched patients with iPD (aged 51-65 years). Sleep complaints in the parkin group included insomnia (73% patients versus 45% in the iPD group), restless legs syndrome (45%, versus none in the iPD group, P = 0.04), and daytime sleepiness (45%, versus 54% in the iPD group). Of the parkin patients, 45% had REM sleep without atonia, but only 9% had a definite REM sleep behavior disorder. All sleep measures were similar in the parkin and iPD groups. Two parkin siblings had a central hypersomnia, characterized by mean daytime sleep latencies of 3 min, no sleep onset REM periods, and normal nighttime sleep. Although the patients with two parkin mutations were young, their sleep phenotype paralleled the clinical and polygraphic sleep recording abnormalities reported in iPD, except that restless legs syndrome was more prevalent and secondary narcolepsy was absent.

Pharmacological treatment of disruptive behavior in Smith-Magenis syndrome.

PubMed

Laje, Gonzalo; Bernert, Rebecca; Morse, Rebecca; Pao, Maryland; Smith, Ann C M

2010-11-15

Smith-Magenis syndrome (SMS) is a complex genetic syndrome caused by an interstitial deletion of chromosome 17p11.2. Children and adults with SMS appear to have unique neurobehavioral problems that include: sleep disturbance, self-injurious and maladaptive behaviors, stereotypies, and sensory integration disorders. We gathered retrospective psychotropic use information from parents or other caregivers of 62 individuals with SMS who were asked about use of psychotropic medication from a list of commonly used psychiatric medications. For those drugs identified, respondents were asked to rate the experience with the particular medication using a likert-type scale. Drugs were grouped into seven main categories: (1) stimulants; (2) antidepressants; (3) antipsychotics; (4) sleep aides; (5) mood stabilizers; (6) alpha 2 agonists; and (7) benzodiazepines. Relative frequencies, means and standard deviations pertaining to age and medication effect were derived for each medication category. Six of the seven medication categories examined showed no meaningful deviations from the "no change" score. The benzodiazepine group showed a mild detrimental effect. There were no gender differences in efficacy. Use of psychotropic medication started early in life (mean age 5 years), particularly with sleep aides. Although no medication category was identified as efficacious in SMS, all the categories reported herein may be considered as an option for brief symptomatic relief.

Angelman syndrome: current understanding and research prospects.

PubMed

Dan, Bernard

2009-11-01

Angelman syndrome is a neurogenetic disorder characterized by developmental delay, severe intellectual disability, absent speech, exuberant behavior with happy demeanor, motor impairment, and epilepsy, due to deficient UBE3A gene expression that may be caused by various abnormalities of chromosome 15. Recent findings in animal models demonstrated altered dendritic spine formation as well as both synaptic [including gamma-aminobutyric acid (GABA)(A) and N-methyl-D-aspartate (NMDA) transmission] and nonsynaptic (including gap junction) influences in various brain regions, including hippocampus and cerebellar cortex. Reversal of selected abnormalities in rescue genetically engineered animal models is encouraging, although it should not be misinterpreted as promising "cure" for affected patients. Much research is still required to fully understand the functional links between lack of UBE3A expression and clinical manifestations of Angelman syndrome. Studies of regulation of UBE3A expression, including imprinting-related methylation, may point to possibilities of therapeutic upregulation. Understanding relevant roles of the gene product might lead to targeted intervention. Further documentation of brain network dynamics, with particular emphasis on hippocampus, thalamocortical, and cerebellar networks is needed, including in a developmental perspective. There is also a need for further clinical research for improving management of problems such as epilepsy, behavior, communication, learning, motor impairment, and sleep disturbances.

Psychiatric and autistic comorbidity in fragile X syndrome across ages.

PubMed

Gabis, Lidia V; Baruch, Yael Kesner; Jokel, Ariela; Raz, Raanan

2011-08-01

Fragile X syndrome is caused by CGG trinucleotide repeat expansion within the fragile X mental retardation 1 gene, when repeat number exceeds 200. The typical psychiatric profile of fragile X syndrome patients includes cognitive and behavioral deficits, psychiatric comorbidity, and autistic characteristics. Specific psychiatric features have not yet been clarified, specifically in relationship to age and genetic characteristics. The objective of this study was to characterize psychiatric comorbidities in subjects with fragile X syndrome at different ages. Subjects with fragile X syndrome and their unaffected siblings were recruited and their parents filled out functional-behavioral and psychiatric comorbidities questionnaires. Adolescents with fragile X syndrome showed decreased prevalence of functional-behavioral deficits. Incidence and severity of most psychiatric comorbidities were lower in older subjects. Incidence of generalized anxiety disorder increased with age in the fragile X syndrome group. The typical profile of patients with fragile X syndrome changes with age. Unaffected siblings exhibit anxiety and motor tics.

The Prevalence and Phenomenology of Repetitive Behavior in Genetic Syndromes

ERIC Educational Resources Information Center

Moss, Joanna; Oliver, Chris; Arron, Kate; Burbidge, Cheryl; Berg, Katy

2009-01-01

We investigated the prevalence and phenomenology of repetitive behavior in genetic syndromes to detail profiles of behavior. The Repetitive Behaviour Questionnaire (RBQ) provides fine-grained identification of repetitive behaviors. The RBQ was employed to examine repetitive behavior in Angelman (N = 104), Cornelia de Lange (N = 101), Cri-du-Chat…

Why culture matters in health interventions: lessons from HIV/AIDS stigma and NCDs.

PubMed

Airhihenbuwa, Collins O; Ford, Chandra L; Iwelunmor, Juliet I

2014-02-01

Theories about health behavior are commonly used in public health and often frame problems as ascribed or related to individuals' actions or inaction. This framing suggests that poor health occurs because individuals are unable or unwilling to heed preventive messages or recommended treatment actions. The recent United Nations call for strategies to reduce the global disease burden of noncommunicable diseases like diabetes requires a reassessment of individual-based approaches to behavior change. We argue that public health and health behavior intervention should focus more on culture than behavior to achieve meaningful and sustainable change resulting in positive health outcomes. To change negative health behaviors, one must first identify and promote positive health behaviors within the cultural logic of its contexts. To illustrate these points, we discuss stigma associated with obesity and human immunodeficiency virus and acquired immune deficiency syndrome. We conclude that focusing on positive behaviors and sustaining cultural and personal transformations requires a culturally grounded approach to public health interventions, such as that provided by the PEN-3 model.

Adults with autism spectrum disorder as behavior technicians for young children with autism: Outcomes of a behavioral skills training program.

PubMed

Lerman, Dorothea C; Hawkins, Lynn; Hillman, Conrad; Shireman, Molly; Nissen, Melissa A

2015-01-01

Adults with autism spectrum disorder (ASD), who were interested in working as behavior technicians for young children with autism, participated in 2 experiments. Participants included 5 adults with Asperger syndrome or pervasive developmental disorder not otherwise specified, 19 to 23 years old, and 11 children with autism, 3 to 7 years old. In Experiment 1, training of the adults focused on the implementation of mand training via incidental teaching. Experiment 2 focused on teaching participants to use discrete-trial training (DTT) with children who exhibited problem behavior. Both experiments showed that behavioral skills training was effective for teaching the adult participants the behavioral procedures needed to teach children with autism. In addition, the children acquired skills as a result of training. Results of Experiment 2 further demonstrated that the DTT skills generalized across untrained targets and children. Social validity ratings suggested that some participants' teaching was indistinguishable from that of individuals without ASD. © Society for the Experimental Analysis of Behavior.

[Epidemiology of emotional and behavioral problems in children and adolescents reared in orphanages: a national comparative study].

PubMed

Simşek, Zeynep; Erol, Neşe; Oztop, Didem; Ozer Ozcan, Ozlem

2008-01-01

We examined the prevalence of emotional and behavioral problems, and associated factors in children and adolescents aged 6-18 years that were reared in orphanages. We aimed to compare these children and adolescents with a nationally representative age-matched sample that were raised by their own families and to identify mental health service needs in orphanages. This cross-sectional study included 674 children and adolescents aged 6-18 years that were selected from orphanages using stratified and probability cluster sampling. A socio-demographic information form, and the Child Behavior Checklist (CBCL), Teacher's Report Form (TRF), and Youth Self-Report Form (YSR) were used for data collection. According to the information provided by caregivers, teachers, and youths, the prevalence of problem behaviors ranged between 18.3% and 47% among those in institutional care versus between 9% and 11% among the national sample. Among those in institutional care, the prevalence of externalizing problems (21.4%-41.9%) was significantly higher than the prevalence of internalizing problems (6.2%-40.1%). At the syndrome level, the prevalence of social problems (5.7%-11.7%), thought disorders (7.2%-18.4%), and attention problems (7.7%-31.4%) among the youths in institutional care was higher than among the national sample (1.6%-5.8%). Age at first admission, receiving institutional care because of neglect and abuse, moves 2 or more times between institutions, recurrent physical illness, receiving poor quality care, lack of regular contact with parents or relatives, lack of regular contact with teachers and the institutional staff, poor problem-solving skills, fatalistic beliefs, tobacco and alcohol use, the feeling of stigmatization, and low-level competency were significantly associated with an increased risk of behavioral and emotional problems. In this representative study, only 2.4% of the children received any mental health care services. There is an urgent need to develop alternative care models and routine screening for mental health. The training of professionals and development of mental health services for children in institutional care should be a priority.

Insufficient sleep syndrome: An unrecognized but important clinical entity.

PubMed

Kohyama, Jun; Anzai, Yuki; Ono, Makoto; Kishino, Ai; Tamanuki, Keita; Takada, Kazuma; Inoue, Kento; Horiuchi, Maho; Hatai, Yoshiho

2018-04-01

A sleep clinic for adults and children was established in the Tokyo Bay Urayasu Ichikawa Medical Centre, in August 2012. Given that few sleep clinics are available in Japan specifically for children, this clinic provides the opportunity to provide data on child patients with sleep problems. Records of patients who visited the sleep clinic at the Tokyo Bay Urayasu Ichikawa Medical Centre aged ≤20 years at the first visit were retrospectively examined, along with the initial and final diagnoses. Of 2,157 patients who visited the sleep clinic at Tokyo Bay Urayasu Ichikawa Medical Centre between August 2012 and March 2017, 181 were ≤20 years old. In these 181 patients, the most frequent final diagnosis was insufficient sleep syndrome (ISS), n = 56, followed by circadian rhythm sleep-wake disorder, n = 28; insomnia, n = 28; and sleep-related movement disorder, n = 15. Insufficient sleep produces various brain dysfunctions in both adults and children, and is associated with behavioral, cognitive and physical problems, as well as with atypical early development. Insufficient sleep has also been reported to cause obesity. Insufficient sleep-induced obesity is often associated with the occurrence of metabolic syndrome. More effort is needed to ensure that children are receiving sufficient sleep. © 2018 Japan Pediatric Society.

Self-Injurious Behavior in Rett Syndrome: Interactions between Features of Rett Syndrome and Operant Conditioning.

ERIC Educational Resources Information Center

Oliver, Chris; And Others

1993-01-01

In this case study, interactions were examined between features of Rett syndrome and operant conditioning as determinants of self-injurious behavior (SIB). Analysis suggested different functions for two forms of SIB: automatic reinforcement by sensory stimulation and escape from social interactions. Features of Rett syndrome tended to maximize the…

Conceptualizing neurodevelopmental disorders through a mechanistic understanding of fragile X syndrome and Williams syndrome.

PubMed

Fung, Lawrence K; Quintin, Eve-Marie; Haas, Brian W; Reiss, Allan L

2012-04-01

The overarching goal of this review is to compare and contrast the cognitive-behavioral features of fragile X syndrome (FraX) and Williams syndrome and to review the putative neural and molecular underpinnings of these features. Information is presented in a framework that provides guiding principles for conceptualizing gene-brain-behavior associations in neurodevelopmental disorders. Abnormalities, in particular cognitive-behavioral domains with similarities in underlying neurodevelopmental correlates, occur in both FraX and Williams syndrome including aberrant frontostriatal pathways leading to executive function deficits, and magnocellular/dorsal visual stream, superior parietal lobe, inferior parietal lobe, and postcentral gyrus abnormalities contributing to deficits in visuospatial function. Compelling cognitive-behavioral and neurodevelopmental contrasts also exist in these two disorders, for example, aberrant amygdala and fusiform cortex structure and function occurring in the context of contrasting social behavioral phenotypes, and temporal cortical and cerebellar abnormalities potentially underlying differences in language function. Abnormal dendritic development is a shared neurodevelopmental morphologic feature between FraX and Williams syndrome. Commonalities in molecular machinery and processes across FraX and Williams syndrome occur as well - microRNAs involved in translational regulation of major synaptic proteins; scaffolding proteins in excitatory synapses; and proteins involved in axonal development. Although the genetic variations leading to FraX and Williams syndrome are different, important similarities and contrasts in the phenotype, neurocircuitry, molecular machinery, and cellular processes in these two disorders allow for a unique approach to conceptualizing gene-brain-behavior links occurring in neurodevelopmental disorders.

Behavioral differences among subjects with Prader-Willi syndrome and type I or type II deletion and maternal disomy.

PubMed

Butler, Merlin G; Bittel, Douglas C; Kibiryeva, Nataliya; Talebizadeh, Zohreh; Thompson, Travis

2004-03-01

To determine whether phenotypic differences exist among individuals with Prader-Willi syndrome with either type I or type II deletions of chromosome 15 or maternal disomy 15 leading to a better understanding of cause and pathophysiology of this classical genetic syndrome. We analyzed clinical, anthropometric, and behavioral data in 12 individuals (5 men, 7 women; mean age: 25.9 +/- 8.8 years) with PWS and a type I (TI) deletion, 14 individuals (6 men, 8 women; mean age: 19.6 +/- 6.5 years) with PWS and a type II (TII) deletion, and 21 individuals (10 men, 11 women; mean age: 23.6 +/- 9.2 years) with PWS and maternal disomy 15 (UPD). The deletion type was determined by genotyping of DNA markers between proximal chromosome 15 breakpoints BP1 and BP2. TI deletions are approximately 500 kb larger than TII deletions. Several validated psychological and behavioral tests were used to assess phenotypic characteristics of individuals with PWS representing the 3 genetic subtypes. Significant differences were found between the 2 deletion groups and those with UPD in multiple psychological and behavioral tests, but no differences were observed in other clinical or anthropometric data studied. Adaptive behavior scores were generally worse in individuals with PWS and the TI deletion, and specific obsessive-compulsive behaviors were more evident in the TI individuals compared with those with UPD. Individuals with PWS with TI deletions also had poorer reading and math skills as well as visual-motor integration. Our study indicates that individuals with TI deletion generally have more behavioral and psychological problems than individuals with the TII deletion or UPD. Four recently identified genes have been identified in the chromosome region between BP1 and BP2 with 1 of the genes (NIPA-1) expressed in mouse brain tissue but not thought to be imprinted. It may be important for brain development or function. These genes are deleted in individuals with TI deletion and are implicated in compulsive behavior and lower intellectual ability in individuals with TI versus TII.

Autobiographical Memory and Social Problem-Solving in Asperger Syndrome

ERIC Educational Resources Information Center

Goddard, Lorna; Howlin, Patricia; Dritschel, Barbara; Patel, Trishna

2007-01-01

Difficulties in social interaction are a central feature of Asperger syndrome. Effective social interaction involves the ability to solve interpersonal problems as and when they occur. Here we examined social problem-solving in a group of adults with Asperger syndrome and control group matched for age, gender and IQ. We also assessed…

Screening for Vision Problems, Including Usher's Syndrome, among Hearing Impaired Students.

ERIC Educational Resources Information Center

Fillman, Robyn D.; And Others

1987-01-01

A screening program for vision problems and Usher's Syndrome (a common cause of deaf-blindness) among 210 hearing-impaired students found 44 percent had significant vision problems and 1 percent had Usher's Syndrome. The program involved an interagency network of school, health care, and support personnel and utilized a dilated ophathalmological…

Personality Traits and Behavioral Syndromes in Differently Urbanized Populations of House Sparrows (Passer domesticus)

PubMed Central

Bókony, Veronika; Kulcsár, Anna; Tóth, Zoltán; Liker, András

2012-01-01

Urbanization creates novel environments for wild animals where selection pressures may differ drastically from those in natural habitats. Adaptation to urban life involves changes in various traits, including behavior. Behavioral traits often vary consistently among individuals, and these so-called personality traits can be correlated with each other, forming behavioral syndromes. Despite their adaptive significance and potential to act as constraints, little is known about the role of animal personality and behavioral syndromes in animals' adaptation to urban habitats. In this study we tested whether differently urbanized habitats select for different personalities and behavioral syndromes by altering the population mean, inter-individual variability, and correlations of personality traits. We captured house sparrows (Passer domesticus) from four different populations along the gradient of urbanization and assessed their behavior in standardized test situations. We found individual consistency in neophobia, risk taking, and activity, constituting three personality axes. On the one hand, urbanization did not consistently affect the mean and variance of these traits, although there were significant differences between some of the populations in food neophobia and risk taking (both in means and variances). On the other hand, both urban and rural birds exhibited a behavioral syndrome including object neophobia, risk taking and activity, whereas food neophobia was part of the syndrome only in rural birds. These results indicate that there are population differences in certain aspects of personality in house sparrows, some of which may be related to habitat urbanization. Our findings suggest that urbanization and/or other population-level habitat differences may not only influence the expression of personality traits but also alter their inter-individual variability and the relationships among them, changing the structure of behavioral syndromes. PMID:22574204

Longitudinal Trajectories of Aberrant Behavior in Fragile X Syndrome

PubMed Central

Hustyi, Kristin M.; Hall, Scott S.; Jo, Booil; Lightbody, Amy A.; Reiss, Allan L.

2016-01-01

The Aberrant Behavior Checklist—Community (ABC-C; Aman, Burrow, & Wolford, 1995) has been increasingly adopted as a primary tool for measuring behavioral change in clinical trials for individuals with fragile X syndrome (FXS). To our knowledge, however, no study has documented the longitudinal trajectory of aberrant behaviors in individuals with FXS using the ABC-C. As part of a larger longitudinal study, we examined scores obtained on the ABC-C subscales for 124 children and adolescents (64 males, 60 females) with FXS who had two or more assessments (average interval between assessments was approximately 4 years). Concomitant changes in age-equivalent scores on the Vineland Adaptive Behavior Scales (VABS) were also examined. As expected for an X-linked genetic disorder, males with FXS obtained significantly higher scores on all subscales of the ABC-C and significantly lower age-equivalent scores on the VABS than females with FXS. In both males and females with FXS, scores on the Irritability/Agitation and Hyperactivity/Noncompliance subscales of the ABC-C decreased significantly with age, with little to no change occurring over time on the Lethargy/Social Withdrawal, Stereotypic Behavior, and Inappropriate Speech subscales. The decrease in scores on the Hyperactivity/Noncompliance domain was significantly greater for males than for females. In both males and females, age-equivalent scores on the VABS increased significantly over this developmental period. These results establish a basis upon which to evaluate long-term outcomes from intervention-based research. However, longitudinal direct observational studies are needed to establish whether the severity of problem behavior actually decreases over time in this population. PMID:25129200

Diagnosis of severe developmental disorders in children under three years of age.

PubMed

Markiewicz, Katarzyna; Pachalska, Maria

2007-02-01

Autism, intellectual disability, and specific language impairment (SLI) constitute three important forms of developmental disability that are often mistaken for each other, especially in very young children (under age 4). Diagnostic problems are caused by the fact that a fundamental problem in cognition, language, or behavior has secondary effects on the remaining areas, which makes it difficult to separate cause from effect. A wrong or absent diagnosis can be a major hindrance in providing properly targeted therapy for developmentally disabled children. From a population of 667 children referred to a specialized outpatient clinic for developmentally disabled children, we identified 35 children in whom the fundamental diagnosis of autism, intellectual disability, or SLI was unambiguous, and then analyzed these children's scores on 7 subtests from the Munich Functional Developmental Diagnosis, in order to identify specific features of each of the three syndromes. The most reliable differentiating factor in our research group proved to be the MFDD subtest for self-reliance. A model was constructed to assist in analyzing the complex interactions of symptoms, which frequently overlap. Cognitive and communicative limitations resulting from underlying perceptual dysfunctions can lead to inappropriate adaptive behavior in children with developmental disorders, such as autism, intellectual disability, and specific language impairment. Each of these syndromes has a specific profile in respect to measures of cognitive function, social skills, and verbal communication.

Empirically based assessment and taxonomy of psychopathology for ages 1½-90+ years: Developmental, multi-informant, and multicultural findings.

PubMed

Achenbach, Thomas M; Ivanova, Masha Y; Rescorla, Leslie A

2017-11-01

Originating in the 1960s, the Achenbach System of Empirically Based Assessment (ASEBA) comprises a family of instruments for assessing problems and strengths for ages 1½-90+ years. To provide an overview of the ASEBA, related research, and future directions for empirically based assessment and taxonomy. Standardized, multi-informant ratings of transdiagnostic dimensions of behavioral, emotional, social, and thought problems are hierarchically scored on narrow-spectrum syndrome scales, broad-spectrum internalizing and externalizing scales, and a total problems (general psychopathology) scale. DSM-oriented and strengths scales are also scored. The instruments and scales have been iteratively developed from assessments of clinical and population samples of hundreds of thousands of individuals. Items, instruments, scales, and norms are tailored to different kinds of informants for ages 1½-5, 6-18, 18-59, and 60-90+ years. To take account of differences between informants' ratings, parallel instruments are completed by parents, teachers, youths, adult probands, and adult collaterals. Syndromes and Internalizing/Externalizing scales derived from factor analyses of each instrument capture variations in patterns of problems that reflect different informants' perspectives. Confirmatory factor analyses have supported the syndrome structures in dozens of societies. Software displays scale scores in relation to user-selected multicultural norms for the age and gender of the person being assessed, according to ratings by each type of informant. Multicultural norms are derived from population samples in 57 societies on every inhabited continent. Ongoing and future research includes multicultural assessment of elders; advancing transdiagnostic progress and outcomes assessment; and testing higher order structures of psychopathology. Copyright © 2017 Elsevier Inc. All rights reserved.

Behavioral Profiles of Children with Williams Syndrome from Spain and the United States: Cross-Cultural Similarities and Differences

ERIC Educational Resources Information Center

Pérez-García, Débora; Brun-Gasca, Carme; Pérez-Jurado, Luis A.; Mervis, Carolyn B.

2017-01-01

To identify similarities and differences in the behavioral profile of children with Williams syndrome from Spain (n = 53) and the United States (n = 145), we asked parents of 6- to 14-year-olds with Williams syndrome to complete the Child Behavior Checklist 6-18. The distribution of raw scores was significantly higher for the Spanish sample than…

Psychometric properties of the Children's Scale of Hostility and Aggression: Reactive/Proactive (C-SHARP).

PubMed

Farmer, Cristan A; Aman, Michael G

2010-01-01

Although often lacking "malice", aggression is fairly common in children with intellectual or developmental disability (I/DD). Despite this, there are no scales available that are appropriate for an in-depth analysis of aggressive behavior in this population. Such scales are needed for the study of aggressive behavior, which is a common target symptom in clinical trials. We assessed the reliability and validity of the Children's Scale of Hostility and Aggression: Reactive/Proactive (C-SHARP), a new aggression scale created for children with I/DD. Data are presented from a survey of 365 children with I/DD aged 3-21 years. Interrater reliability was very high for the Problem Scale, which characterizes type of aggression. Reliability was lower but largely acceptable for the Provocation Scale, which assesses motivation. Validity of the Problem Scale was supported by expected differences in children with autism, Down syndrome, comorbid disruptive behavior disorders (DBDs) and ADHD. The Provocation Scale, which categorizes behavior as proactive or reactive, showed expected differences in children with DBD, but was less effective in those with ADHD. The C-SHARP appears to have fundamentally sound psychometric characteristics, although more research is needed.

Behavioral features of CHARGE syndrome: parents' perspectives of three children with CHARGE syndrome.

PubMed

Lauger, Kimberly; Cornelius, Nancy; Keedy, Wendy

2005-03-15

As the population first diagnosed with CHARGE Syndrome has aged, information has emerged about specific behavioral features of this syndrome. Once the medical features are managed, it is the behavioral features that interfere with our children's ability to form reciprocal relationships, to gain formal communication skills, to make the educational gains they are cognitively capable of, and to ultimately care for themselves. In telling the informal case studies of our children, we hope their behavioral commonalities, in spite of their medical and educational differences, will be apparent as well as the complexity of the multiple types of behavior observed. We remain hopeful that answers can be found that will help our children and our families cope with this very debilitating feature of CHARGE. Copyright (c) 2005 Wiley-Liss, Inc.

First-year growth in children with Noonan syndrome: Associated with feeding problems?

PubMed

Croonen, Ellen A; Draaisma, Jos M T; van der Burgt, Ineke; Roeleveld, Nel; Noordam, Cees

2018-04-01

Children with Noonan syndrome show rapid decline of growth in the first year of life and feeding problems are present in over 50%. The aim of this study was to explore whether growth decelerates because of feeding problems or other Noonan syndrome-related factors. We performed a retrospective, longitudinal cohort study of clinically and genetically diagnosed subjects with Noonan syndrome (n = 143). Questionnaires about the phenotypic-genotypic profile and reported feeding problems were sent to eligible subjects. Data on first-year growth was obtained from growth charts. Ninety-one participants were excluded because of different criteria. A total of 52 subjects with Noonan syndrome were included. The largest decline in weight and length standard deviation score (SDS) occurred in the first 2.5 months after birth (-1.93 and -1.15, respectively), with feeding problems causing a decline of 0.57 SDS in the remaining months. At 1 year, children with feeding problems were on average 290 g lighter and 0.8 cm shorter than children without feeding problems. Weight gain was also negatively influenced by having a PTPN11 mutation (n = 39) and a higher gestational age, whereas children of parents with Noonan syndrome and with a higher birth weight gained more weight. Growth in length was reduced by having cardiac surgery and a higher gestational age, but positively influenced by birth length and maternal height. Growth in children with Noonan syndrome is impaired right after birth and only partially associated with feeding problems. In addition, several specific Noonan syndrome-related factors seem to influence growth in the first year. © 2018 Wiley Periodicals, Inc.

The Architecture of the Pollen Hoarding Syndrome in Honey Bees: Implications for Understanding Social Evolution, Behavioral Syndromes, and Selective Breeding.

PubMed

Rueppell, Olav

2014-05-01

Social evolution has influenced every aspect of contemporary honey bee biology, but the details are difficult to reconstruct. The reproductive ground plan hypothesis of social evolution proposes that central regulators of the gonotropic cycle of solitary insects have been coopted to coordinate social complexity in honey bees, such as the division of labor among workers. The predicted trait associations between reproductive physiology and social behavior have been identified in the context of the pollen hoarding syndrome, a larger suite of interrelated traits. The genetic architecture of this syndrome is characterized by a partially overlapping genetic architecture with several consistent, pleiotropic QTL. Despite these central QTL and an integrated hormonal regulation, separate aspects of the pollen hoarding syndrome may evolve independently due to peripheral QTL and additionally segregating genetic variance. The characterization of the pollen hoarding syndrome has also demonstrated that this syndrome involves many non-behavioral traits, which may be the case for numerous "behavioral" syndromes. Furthermore, the genetic architecture of the pollen hoarding syndrome has implications for breeding programs for improving honey health and other desirable traits: If these traits are comparable to the pollen hoarding syndrome, consistent pleiotropic QTL will enable marker assisted selection, while sufficient additional genetic variation may permit the dissociation of trade-offs for efficient multiple trait selection.

Prenatal exposure to very severe maternal obesity is associated with adverse neuropsychiatric outcomes in children.

PubMed

Mina, T H; Lahti, M; Drake, A J; Räikkönen, K; Minnis, H; Denison, F C; Norman, J E; Reynolds, R M

2017-01-01

Prenatal maternal obesity has been linked to adverse childhood neuropsychiatric outcomes, including increased symptoms of attention deficit hyperactivity disorder (ADHD), internalizing and externalizing problems, affective disorders and neurodevelopmental problems but few studies have studied neuropsychiatric outcomes among offspring born to very severely obese women or assessed potential familial confounding by maternal psychological distress. We evaluated neuropsychiatric symptoms in 112 children aged 3-5 years whose mothers had participated in a longitudinal study of obesity in pregnancy (50 very severe obesity, BMI ⩾40 kg/m2, obese class III and 62 lean, BMI 18.5-25 kg/m2). The mothers completed the Conners' Hyperactivity Scale, Early Symptomatic Syndrome Eliciting Neurodevelopmental Clinical Examination Questionnaire (ESSENCE-Q), Child's Sleep Habits Questionnaire (CSHQ), Strengths and Difficulties Questionnaire (SDQ), and Child Behavior Checklist (CBCL) to assess child neuropsychiatric symptoms. Covariates included child's sex, age, birthweight, gestational age, socioeconomic deprivation levels, maternal age, parity, smoking status during pregnancy, gestational diabetes and maternal concurrent symptoms of anxiety and depression assessed using State Anxiety of Spielberger State-Trait Anxiety Index (STAI) and General Health Questionnaire (GHQ), respectively. Children exposed to prenatal maternal very severe obesity had significantly higher scores in the Conners' Hyperactivity Scale; ESSENCE-Q; total sleep problems in CSHQ; hyperactivity, conduct problems and total difficulties scales of the SDQ; higher externalizing and total problems, anxious/depressed, aggressive behaviour and other problem syndrome scores and higher DSM-oriented affective, anxiety and ADHD problems in CBCL. Prenatal maternal very severe obesity remained a significant predictor of child neuropsychiatric problems across multiple scales independent of demographic factors, prenatal factors and maternal concurrent symptoms of anxiety and depression. Prenatal maternal very severe obesity is a strong predictor of increased neuropsychiatric problems in early childhood.

The Family Context of Autism Spectrum Disorders: Influence on the Behavioral Phenotype and Quality of Life

PubMed Central

Smith, Leann E.; Greenberg, Jan; Mailick, Marsha R.

2013-01-01

Synopsis In this review, we report the findings from our longitudinal program of research examining the bidirectional influences of the family environment on the behavioral phenotype of autism, and describe a newly developed family psychoeducation program, titled Transitioning Together, designed to reduce family stress, address behavior problems, and improve the overall quality of life of adolescents with autism and their families. In our search for characteristics of the family environment that influence the behavioral phenotype of adolescents and adults with autism, we focus on both positive dimensions of family life, such as warmth and positive remarks that may promote adaptive behavior in individuals with autism, as well as negative dimensions, such as high levels of criticism that may result in an escalation of behavior problems. We find that high levels of maternal warmth and positive remarks are associated with the abatement of behavior problems over time, while high levels of maternal criticism are associated with increasing levels of behavior problems in adolescents and adults with autism. These patterns of relationships have been replicated in a longitudinal study of families of children and adolescents with fragile X syndrome, and are consistent with other studies examining the impact of the family on the behavior of children with developmental disabilities. These findings suggest that the family environment is an important target for interventions not only to reduce family stress but also to improve the behavioral functioning of children, adolescents or adults with ASD. Building upon a well-developed intervention for families of individuals with psychiatric conditions, we report on the development of Transitioning Together, a psychoeducation program targeted to families with adolescents with autism who are approaching high school exit, a difficult transition stage for individuals with autism that is often marked by negative changes in behavior problems. The 8-week Transitioning Together program involves education on a variety of topics relevant to ASD and guided practice in helping families better manage problem behavior in adolescents with autism. Preliminary data suggest that the program can improve the parent-child relationship and increase parental expressions of warmth. A case study is presented that illustrates how Transitioning Together helps reduce family stress and improve the overall quality of the family environment. We conclude the article with a discussion of directions for future research on best practices in working with families of children, adolescents and adults with autism. PMID:24231173

Modulation of Fgfr1a signaling in zebrafish reveals a genetic basis for the aggression-boldness syndrome.

PubMed

Norton, William H J; Stumpenhorst, Katharina; Faus-Kessler, Theresa; Folchert, Anja; Rohner, Nicolas; Harris, Matthew P; Callebert, Jacques; Bally-Cuif, Laure

2011-09-28

Behavioral syndromes are suites of two or more behaviors that correlate across environmental contexts. The aggression-boldness syndrome links aggression, boldness, and exploratory activity in a novel environment. Although aggression-boldness has been described in many animals, the mechanism linking its behavioral components is not known. Here we show that mutation of the gene encoding fibroblast growth factor receptor 1a (fgfr1a) simultaneously increases aggression, boldness, and exploration in adult zebrafish. We demonstrate that altered Fgf signaling also results in reduced brain histamine levels in mutants. Pharmacological increase of histamine signaling is sufficient to rescue the behavioral phenotype of fgfr1a mutants. Together, we show that a single genetic locus can underlie the aggression-boldness behavioral syndrome. We also identify one of the neurotransmitter pathways that may mediate clustering of these behaviors.

Behavior and neuropsychiatric manifestations in Angelman syndrome

PubMed Central

Pelc, Karine; Cheron, Guy; Dan, Bernard

2008-01-01

Angelman syndrome has been suggested as a disease model of neurogenetic developmental condition with a specific behavioral phenotype. It is due to lack of expression of the UBE3A gene, an imprinted gene located on chromosome 15q. Here we review the main features of this phenotype, characterized by happy demeanor with prominent smiling, poorly specific laughing and general exuberance, associated with hypermotor behavior, stereotypies, and reduced behavioral adaptive skills despite proactive social contact. All these phenotypic characteristics are currently difficult to quantify and have been subject to some differences in interpretation. For example, prevalence of autistic disorder is still debated. Many of these features may occur in other syndromic or nonsyndromic forms of severe intellectual disability, but their combination, with particularly prominent laughter and smiling may be specific of Angelman syndrome. Management of problematic behaviors is primarily based on behavioral approaches, though psychoactive medication (eg, neuroleptics or antidepressants) may be required. PMID:18830393

Unhealthy Lifestyle Behaviors in Korean People with Metabolic Syndrome.

PubMed

Moon, Seongmi

2017-01-01

This study identified factors associated with unhealthy lifestyle behaviors in people with metabolic syndrome in South Korea. The sample consisted of 1,207 subjects with metabolic syndrome from the Sixth Korea National Health and Nutrition Examination Survey conducted in 2014. High-risk alcohol consumption, smoking, aerobic physical activity, leisure physical activity, excessive carbohydrate intake, and fat intake were measured. A secondary data analysis was performed using chi-square tests and logistic regression. Gender was associated with all unhealthy behaviors. The number of metabolic syndrome components, a poor perceived health status, and attempts to control weight were associated with physical inactivity. Those findings may be helpful to develop a tailored lifestyle modification programs for people with metabolic syndrome.

Conceptualizing neurodevelopmental disorders through a mechanistic understanding of fragile X syndrome and Williams syndrome

PubMed Central

Fung, Lawrence K.; Quintin, Eve-Marie; Haas, Brian W.

2013-01-01

Purpose of review The overarching goal of this review is to compare and contrast the cognitive-behavioral features of fragile X syndrome (FraX) and Williams syndrome and to review the putative neural and molecular underpinnings of these features. Information is presented in a framework that provides guiding principles for conceptualizing gene-brain-behavior associations in neurodevelopmental disorders. Recent findings Abnormalities, in particular cognitive-behavioral domains with similarities in underlying neurodevelopmental correlates, occur in both FraX and Williams syndrome including aberrant frontostriatal pathways leading to executive function deficits, and magnocellular/dorsal visual stream, superior parietal lobe, inferior parietal lobe, and postcentral gyrus abnormalities contributing to deficits in visuospatial function. Compelling cognitive–behavioral and neurodevelopmental contrasts also exist in these two disorders, for example, aberrant amygdala and fusiform cortex structure and function occurring in the context of contrasting social behavioral phenotypes, and temporal cortical and cerebellar abnormalities potentially underlying differences in language function. Abnormal dendritic development is a shared neurodevelopmental morphologic feature between FraX and Williams syndrome. Commonalities in molecular machinery and processes across FraX and Williams syndrome occur as well – microRNAs involved in translational regulation of major synaptic proteins; scaffolding proteins in excitatory synapses; and proteins involved in axonal development. Summary Although the genetic variations leading to FraX and Williams syndrome are different, important similarities and contrasts in the phenotype, neurocircuitry, molecular machinery, and cellular processes in these two disorders allow for a unique approach to conceptualizing gene–brain–behavior links occurring in neurodevelopmental disorders. PMID:22395002

An interesting case of systemic lupus erythematosus in a patient with Moebius syndrome.

PubMed

Tenorio, Jefferson da Rocha; Figueiredo, Marília Andrade; Espindula, Aline; Gallottini, Marina; Ortega, Karem López

2018-03-01

Moebius' syndrome (MS) is characterized by a nonprogressive facial palsy associated with impairment in eye abduction, which can be uni- or bilateral. Some authors raise the possibility that patients with MS may suffer from social stigmatism due to their facial dysmorphism and that constant teasing and bullying perpetrated by people in the same social circle are adjuvants in the development of low self-esteem, behavioral problems, and even psychiatric disorders. Psychological stress, anxiety, and depression are factors contributing to both development and impairment of autoimmune diseases, such as systemic lupus erythematosus (SLE). The objective of this work is to report the case of a patient with MS who developed SLE. In the present case report, we have emphasized the importance of both clinical dental examination and surgeon-dentist in the early diagnosis of systemic diseases by considering that these conditions can affect both syndromic and normoreactive patients. © 2018 Special Care Dentistry Association and Wiley Periodicals, Inc.

Improving self-esteem in women diagnosed with Turner syndrome: results of a pilot intervention.

PubMed

Chadwick, Paul M; Smyth, Arlene; Liao, Lih-Mei

2014-06-01

To evaluate a brief intervention to improve the self esteem of women diagnosed with Turner syndrome (TS). Prospective observational study. Turner Syndrome Support Society, UK. 30 women aged 18-60 years. A 1-day psychology workshop targeting problems of self-esteem in women diagnosed with TS. The workshop drew on cognitive-behavioral therapy and narrative therapy skills and emphasized increased self-awareness of interpersonal difficulties and improved capacity for self-management. Rosenberg Self-esteem Scale (RSS); Hospital Anxiety and Depression Scale (HADS); bespoke user experiences questionnaire. All 30 women provided baseline data, 27/30 provided immediate post-intervention data and 22/30 provided follow-up data at 3 months. The intervention improved RSS and HADS scores at 3 months. Generic skills-based psychological interventions have the potential to be adapted to provide brief and low-cost interventions to improve self-esteem and reduce psychological distress in women diagnosed with TS. Copyright © 2014 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.

Behavioral and Emotional Disturbance in Individuals with Williams Syndrome.

ERIC Educational Resources Information Center

Einfeld, Stewart L.; Tonge, Bruce J.; Florio, Tony

1997-01-01

Comparison of behavioral and emotional disturbance in 70 children and adolescents with Williams Syndrome (characterized by mental retardation and short stature) and a control group, found Williams Syndrome subjects were more likely to be diagnosed with a psychiatric disorder characterized by anxiety, hyperactivity, preoccupations, and…

Parental Alienation Syndrome in Italian legal judgments: an exploratory study.

PubMed

Lavadera, Anna Lubrano; Ferracuti, Stefano; Togliatti, Marisa Malagoli

2012-01-01

The present study highlights the characteristics of separated families in Italy for whom Parental Alienation Syndrome (PAS) has been diagnosed during court custody evaluations. The study analyzed the psychological reports of 12 court-appointed expert evaluations of families for whom PAS had been diagnosed. Twelve evaluations that did not receive the PAS diagnosis served as a control group. A specific coding system was used for data analysis. The results indicated that the alienating parents were always the parents who had custody of the children. Children who were diagnosed with PAS were predominantly the only child in the family, had identity problems and manifested manipulative behavior. The consultant in these cases suggested individual psychotherapy for the children and recommended foster care to the Social Services agency. Copyright © 2012 Elsevier Ltd. All rights reserved.

Social competence intervention for youth with Asperger Syndrome and high-functioning autism: an initial investigation.

PubMed

Stichter, Janine P; Herzog, Melissa J; Visovsky, Karen; Schmidt, Carla; Randolph, Jena; Schultz, Tia; Gage, Nicholas

2010-09-01

Individuals with high functioning autism (HFA) or Asperger Syndrome (AS) exhibit difficulties in the knowledge or correct performance of social skills. This subgroup's social difficulties appear to be associated with deficits in three social cognition processes: theory of mind, emotion recognition and executive functioning. The current study outlines the development and initial administration of the group-based Social Competence Intervention (SCI), which targeted these deficits using cognitive behavioral principles. Across 27 students age 11-14 with a HFA/AS diagnosis, results indicated significant improvement on parent reports of social skills and executive functioning. Participants evidenced significant growth on direct assessments measuring facial expression recognition, theory of mind and problem solving. SCI appears promising, however, larger samples and application in naturalistic settings are warranted.

THE EPIDEMIOLOGY OF FETAL ALCOHOL SYNDROME AND PARTIAL FAS IN A SOUTH AFRICAN COMMUNITY

PubMed Central

May, Philip A.; Gossage, J. Phillip; Marais, Anna-Susan; Adnams, Colleen M.; Hoyme, H. Eugene; Jones, Kenneth L.; Robinson, Luther K.; Khaole, Nathaniel C.O.; Snell, Cudore; Kalberg, Wendy O.; Hendricks, Loretta; Brooke, Lesley; Stellavato, Chandra; Viljoen, Denis L.

2007-01-01

OBJECTIVES The prevalence and characteristics of fetal alcohol syndrome (FAS) and partial fetal alcohol syndrome (PFAS) were determined in a third primary school cohort in a community in South Africa (S.A.). METHODS An active case ascertainment, two-tier screening methodology, and the revised Institute of Medicine diagnostic criteria were employed among 818 first grade pupils. Characteristics of children with FAS and PFAS are contrasted with a randomly-selected control group. Data were collected and analyzed for children in the study regarding: 1.) physical growth and development, including dysmorphology, 2.) intelligence and behavioral characteristics, and 3.) their mother’s social, behavioral, and physical characteristics. RESULTS The rate of FAS and PFAS in this area continues as the highest reported in any overall community and is much higher than rates elsewhere. In this cohort it is 68.0 to 89.2 per 1,000. Severe episodic drinking on weekends among mothers of children with FAS and PFAS accounts for 96% of all alcohol consumed. Various measures of maternal drinking are significantly correlated with negative outcomes of children in the areas of non-verbal intelligence (-0.26), verbal intelligence (-0.28), problem behavior (0.31), and overall dysmorphology score (0.59). Significantly more FAS and PFAS exists among children of rural residents (OR = 3.79). CONCLUSIONS A high rate of FAS and PFAS was again documented in this community, and it has increased. Given population similarities, we suspect that other communities in the Western Cape Province of South Africa also have high rates. Programs for prevention are needed. PMID:17127017

[Behaviour problems of children with Down syndrome in preschool-age - Results from the Heidelberg Down syndrome study].

PubMed

Sarimski, Klaus

2018-05-01

We report on the frequency and the correlations of behaviour problems among children with Down syndrome in preschool-age. As part of a longitudinal study 48 mothers of children with Down syndrome completed the German version of the “Strengths and Difficulties Questionnaire” (SDQ-D) and the Parenting Stress Inventory (PSI). The mothers were asked to fill out the questionnaires when the children had a mean age of five years. The results were compared to norms from children with typical development. Thirty per cent of the children with Down syndrome were rated as abnormal. Specifically, mean scores indicating problems with children of the same age and hyperactivity were elevated. A regression analysis predicting the total problem score of the SDQ-D revealed maternal educational level, optimistic attitude, and subjective parental stress at the age of one year and the degree of behavioural abnormalities at the age of three years as significant influential factors. Early intervention for Down syndrome children should include supporting parenting competence and coping skills in order to prevent behaviour problems.

Lifestyle and Behavioral Management of Polycystic Ovary Syndrome.

PubMed

Brennan, Leah; Teede, Helena; Skouteris, Helen; Linardon, Jake; Hill, Briony; Moran, Lisa

2017-08-01

Polycystic ovary syndrome (PCOS) is a common condition with serious physiological and psychological health consequences. It affects women across their reproductive lifespan and is associated with pregnancy complications, including gestational diabetes, preeclampsia, and large gestational-age babies. PCOS is associated with excess weight gain, which, in turn, exacerbates the health burden of PCOS. Therefore, weight management, including a modest weight loss, maintenance of weight loss, prevention of weight gain, and prevention of excess gestational weight gain, is a first-line treatment for women with PCOS during and independent of pregnancy. Despite evidence-based guidelines, international position statements, and Cochrane reviews promoting lifestyle interventions for PCOS, the optimal complexity, intensity, and behavioral components of lifestyle interventions for women with PCOS are not well understood. The focus of this narrative review is the evidence supporting the use of behavioral strategies in weight management interventions for reproductive-aged women to apply to PCOS. Behavioral theories, behavior change strategies, and psychological correlates of weight management have been thoroughly explored in weight loss interventions in the general population, reproductive-aged women, and peri-natal women. This article uses this parallel body of research to inform suggestions regarding lifestyle interventions in women with PCOS. Outcomes of weight management programs in women with PCOS are likely to be improved with the inclusion of behavioral and psychological strategies, including goal setting, self-monitoring, cognitive restructuring, problem solving, and relapse prevention. Strategies targeting improved motivation, social support, and psychological well-being are also important. These can be applied to the clinical management of women with PCOS at different reproductive life stages.

Premenstrual syndrome: A mini review.

PubMed

Ryu, Aeli; Kim, Tae-Hee

2015-12-01

Premenstrual syndrome (PMS) is characterized by recurrent, moderate-to-severe affective, physical, and behavioral symptoms that develop during the luteal menstrual cycle and disappear within a few days of menstruation. Premenstrual dysphoric disorder (PMDD) is a severe and disabling condition that can affect personal relationships and occupational activities. PMS occurs in 30-40% of reproductive-age females; PMDD affects 3-8% of this population. Although the etiology of PMS is unclear, several theories suggest increased sensitivity to normal hormonal changes and neurotransmitter abnormalities. The diagnostic method of PMS is the Daily Record of Severity of Problems, which women with PMS can use to self-report several symptoms and their severity. Although combined oral contraceptives and serotonergic antidepressants are effective drugs, each is a different option for treating PMS/PMDD. Serotonergic antidepressants are the drugs of choice for improving both physical and mood symptoms. Combined oral contraceptives appear to primarily improve physical symptoms. Clinicians should consider each patient's situation individually. Other treatment options include lifestyle modification, cognitive behavioral therapy, and herbal medicine (e.g., chasteberry). Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

Effects of Topiramate in Adults with Prader-Willi Syndrome

ERIC Educational Resources Information Center

Shapira, Nathan A.; Lessig, Mary C.; Lewis, Mark H.; Goodman, Wayne K.; Driscoll, Daniel J.

2004-01-01

Prader-Willi syndrome is a multisystem neurogenetic obesity disorder with behavioral manifestations, including hyperphagia, compulsive behavior, self-injury, and mild to moderate mental retardation. In an 8-week open-label study, we evaluated adjunctive therapy with the anticonvulsant topiramate in 8 adults with Prader-Willi syndrome. Appetite was…

A Network View on Psychiatric Disorders: Network Clusters of Symptoms as Elementary Syndromes of Psychopathology

PubMed Central

Goekoop, Rutger; Goekoop, Jaap G.

2014-01-01

Introduction The vast number of psychopathological syndromes that can be observed in clinical practice can be described in terms of a limited number of elementary syndromes that are differentially expressed. Previous attempts to identify elementary syndromes have shown limitations that have slowed progress in the taxonomy of psychiatric disorders. Aim To examine the ability of network community detection (NCD) to identify elementary syndromes of psychopathology and move beyond the limitations of current classification methods in psychiatry. Methods 192 patients with unselected mental disorders were tested on the Comprehensive Psychopathological Rating Scale (CPRS). Principal component analysis (PCA) was performed on the bootstrapped correlation matrix of symptom scores to extract the principal component structure (PCS). An undirected and weighted network graph was constructed from the same matrix. Network community structure (NCS) was optimized using a previously published technique. Results In the optimal network structure, network clusters showed a 89% match with principal components of psychopathology. Some 6 network clusters were found, including "DEPRESSION", "MANIA", “ANXIETY”, "PSYCHOSIS", "RETARDATION", and "BEHAVIORAL DISORGANIZATION". Network metrics were used to quantify the continuities between the elementary syndromes. Conclusion We present the first comprehensive network graph of psychopathology that is free from the biases of previous classifications: a ‘Psychopathology Web’. Clusters within this network represent elementary syndromes that are connected via a limited number of bridge symptoms. Many problems of previous classifications can be overcome by using a network approach to psychopathology. PMID:25427156

A Chinese patient with pusher syndrome and unilateral spatial neglect syndrome.

PubMed

Chen, Xiao-Wei; Lin, Cheng-He; Zheng, Hua; Lin, Zhen-Lan

2014-07-01

To observe clinical manifestations, behavioral characteristics, and effects of rehabilitation on a patient with pusher syndrome and unilateral spatial neglect caused by right thalamic hemorrhage. Assessment of pusher syndrome was made by the Scale for Contraversive pushing (SCP), and unilateral spatial neglect syndrome was diagnosed using line cancellation, letter and star cancellation, line bisection tests and copy and continuation of graphic sequence test. Behavioral therapy, occupational therapy, reading training and traditional Chinese medicine methods were adopted for treatment of pusher syndrome and unilateral spatial neglect. The patient showed typical pusher syndrome and unilateral spatial neglect symptoms. The pusher syndrome and unilateral spatial neglect symptoms were significantly improved following rehabilitation treatments. Pusher syndrome and unilateral spatial neglect syndrome occurred simultaneously after right thalamic hemorrhage. Early rehabilitation therapy can reduce the symptoms of pusher syndrome and unilateral spatial neglect syndrome and improve motor function.

Accounting for the Down syndrome advantage?

PubMed

Esbensen, Anna J; Seltzer, Marsha Mailick

2011-01-01

The authors examined factors that could explain the higher levels of psychosocial well being observed in past research in mothers of individuals with Down syndrome compared with mothers of individuals with other types of intellectual disabilities. The authors studied 155 mothers of adults with Down syndrome, contrasting factors that might validly account for the ?Down syndrome advantage? (behavioral phenotype) with those that have been portrayed in past research as artifactual (maternal age, social supports). The behavioral phenotype predicted less pessimism, more life satisfaction, and a better quality of the mother?child relationship. However, younger maternal age and fewer social supports, as well as the behavioral phenotype, predicted higher levels of caregiving burden. Implications for future research on families of individuals with Down syndrome are discussed.

[Reference value for metabolic syndrome checkup and some problems].

PubMed

Miyake, Noriko

2009-09-01

Metabolic syndrome is one of the most important risk factors of atherosclerotic disease, and visceral obesity is regarded as a principle component of metabolic syndrome. Medical checkups for metabolic syndrome were started in 2008 for the purpose of promoting lifestyle modification through health guidance. The original diagnosis of metabolic syndrome in Japan was presented by the Examination Committee of Criteria for Metabolic Syndrome in April 2005. This guideline defines the waist circumference measurement as an essential component, accompanied by at least two of the following three risk factors: dyslipidemia, a raised blood pressure, and glucose intolerance, and these risk factors were based on multiple representative Japanese cohort studies. However, there are some problems with these standards. For example, it is often the case that accurate evaluation is difficult because variable factors such as meals influence the serum triglyceride level. This influences the reliability of the results of cohort studies. In this symposium, problems with this guideline were presented along with an introduction to the cohort study on which the concept of the syndrome was based. I compiled a cohort study related to metabolic syndrome, and pointed out some problems from the viewpoint of clinical laboratory medicine.

Social Behavior and Cortisol Reactivity in Children with Fragile X Syndrome

ERIC Educational Resources Information Center

Hessl, David; Glaser, Bronwyn; Dyer-Friedman, Jennifer; Reiss, Allan L.

2006-01-01

Objective: To examine the association between limbic-hypothalamic-pituitary-adrenal (L-HPA) axis reactivity and social behavior in children with fragile X syndrome (FXS). Method: Salivary cortisol changes and concurrent anxiety-related behaviors consistent with the behavioral phenotype of FXS were measured in 90 children with the fragile X full…

Self-Injurious Behavior, Self-Restraint, and Compulsive Behaviors in Cornelia de Lange Syndrome.

ERIC Educational Resources Information Center

Hyman, Philippa; Oliver, Chris; Hall, Scott

2002-01-01

Analysis of questionnaires completed by caregivers of 77 individuals with Cornelia de Lange syndrome in the United Kingdom found a significant association between self-injurious behaviors and self-restraint, and those displaying both behaviors displayed significantly more compulsions than did those not exhibiting them. Findings extend the…

Modelling fragile X syndrome in the laboratory setting: A behavioral perspective.

PubMed

Melancia, Francesca; Trezza, Viviana

2018-04-25

Fragile X syndrome is the most common form of inherited mental retardation and the most frequent monogenic cause of syndromic autism spectrum disorders. The syndrome is caused by the loss of the Fragile X Mental Retardation Protein (FMRP), a key RNA-binding protein involved in synaptic plasticity and neuronal morphology. Patients show intellectual disability, social deficits, repetitive behaviors and impairments in social communication. The aim of this review is to outline the importance of behavioral phenotyping of animal models of FXS from a developmental perspective, by showing how the behavioral characteristics of FXS at the clinical level can be translated into effective, developmentally-specific and clinically meaningful behavioral readouts in the laboratory setting. After introducing the behavioral features, diagnostic criteria and off-label pharmacotherapy of FXS, we outline how FXS-relevant behavioral features can be modelled in laboratory animals in the course of development: we review the progress to date, discuss how behavioral phenotyping in animal models of FXS is essential to identify potential treatments, and discuss caveats and future directions in this research field. Copyright © 2018. Published by Elsevier B.V.

Prader-Willi Syndrome: Clinical Genetics and Diagnostic Aspects with Treatment Approaches.

PubMed

Butler, Merlin G; Manzardo, Ann M; Forster, Janice L

2016-01-01

Prader-Willi syndrome (PWS) is a neuro-developmental genetic disorder due to lack of expression of genes inherited from the paternal chromosome 15q11-q13 region with three main genetic subtypes. These include paternal 15q11-q13 deletion (about 70% of cases), maternal uniparental disomy 15 or both 15s from the mother (20-30% of cases), and defects in the imprinting center (1-3%) which controls the expression of imprinted genes in this chromosome region. Clinical manifestations include infantile hypotonia with a poor suck resulting in failure to thrive, short stature, small hands/feet and hypogonadism/hypogenitalism due to growth and other hormone deficiencies, hyperphagia and excessive weight gain with obesity and cognitive and behavioral problems including obsessive compulsions, tantrums and self-injury. The phenotype is likely related to hypothalamic dysfunction. Hyperphagia and obesity with related complications are major causes of morbidity and mortality in PWS requiring accurate diagnosis, appropriate medical management and treatment; the major objective of our report. An extensive review of the literature was undertaken including genetics, clinical and behavioral aspects, and updated health-related information addressing the importance of early diagnosis and treatment of individuals with Prader-Willi syndrome. A searchable, bulleted and formatted list of topics related to this obesity syndrome was provided utilizing a Table of Contents approach for the clinical practitioner. Physicians and other health care providers can use this review with clinical, genetic and treatment summaries divided into sections that are pertinent in the context of clinical practice. Finally, frequently asked questions by clinicians, families and other interested participants will be addressed.

Women's health issues with fibromyalgia syndrome.

PubMed

Shaver, Joan L F; Wilbur, Joellen; Robinson, F Patrick; Wang, Edward; Buntin, Mary S

2006-11-01

Fibromyalgia syndrome (FMS) involves multiple sensory, somatic, and cognitive symptoms that are bound to affect or be affected by physical and mental health status and behavioral components of daily life. From a telephone survey of 442 women with and 205 women without FMS as volunteers, data were compared on (1) general health status, (2) reproductive and sleep-related diagnoses, and (3) lifestyle health behaviors. All multiple or logistics regression analyses for group differences were controlled for age, body mass index (BMI), race, employment status, marital status, having a college degree, low household income, and having ever been diagnosed with depression, with a Bonferroni p value correction for multiple indicators. Accordingly, FMS negatively impacted both perceived physical and mental health status, although relatively more so for physical (p < 0.017). Women with FMS were more likely to have had reproductive health or sleep-related diagnoses, including premenstrual syndrome, dysmenorrhea, breast cysts, bladder cystitis, sleep apnea, restless leg syndrome, and abnormal leg movements (p < 0.0125). They were calculated to use less than half as many calories per week as control women (689 +/- 1293 vs. 1499 +/- 1584 kcal/week, p < 0.05) and had more sleep pattern difficulties (p < 0.0125), more negative changes in sexual function (greater odds for 5 of 10 indicators at p < 0.005), and lower alcohol use (odds ratio = 0.39, p < 0.05). Patients with FMS deserve careful assessment for reproductive conditions and sleep-related functional disorders. Besides more research into mechanisms underlying symptoms, intervention testing specifically to alleviate sleep problems, low physical activity levels, and sexual dysfunction should be paramount.

TPH2 G/T polymorphism is associated with hyperphagia, IQ, and internalizing problems in Prader-Willi syndrome

PubMed Central

Dykens, Elisabeth M.; Roof, Elizabeth; Bittel, Douglas; Butler, Merlin G.

2010-01-01

Background Prader-Willi syndrome (PWS) is a genetic, neurodevelopmental disorder characterized by intellectual disabilities, growth hormone dysregulation, hyperphagia, increased risks of morbid obesity, compulsive behaviors, and irritability. As aberrant serotonergic functioning is strongly implicated in PWS, we examined associations between the PWS phenotype and polymorphisms in tryptophan hydroxylase 2 (TPH2), the rate-limiting enzyme in the biosynthesis of serotonin in the brain. Methods 92 individuals with PWS aged 4 to 50 years (M = 21.97) were genotyped for the TPH2 G703-T polymorphism. IQ testing was conducted in offspring, and parents completed questionnaires that tapped their child’s compulsivity, hyperphagia, and other behavior problems. Results As expected, the frequency of G/T or T/T polymorphisms in participants with PWS (39%) was similar to rates found in the general population (38%). Compared to those with a homozygous (G/G) genotype, individuals with a T allele had significantly higher hyperphagic behavior, drive, and severity scores, and they also had a younger age of onset of hyperphagia. Those with a T allele also had higher IQ scores than their counterparts. Females with a T allele had significantly higher internalizing symptoms, primarily anxiety and depression, than all others. Conclusions TPH2 G/T polymorphisms, and presumed loss of enzyme function, were associated with specific aspects of the PWS phenotype. Aberrant serotonergic functioning is strongly implicated in hyperphagia in PWS, and females with TPH2 T alleles may be at higher risk for affective or mood disorders. Findings hold promise for examining other serotonin-altering genes in PWS, and for future serotonin-altering treatment trials. PMID:21418060

TPH2 G/T polymorphism is associated with hyperphagia, IQ, and internalizing problems in Prader-Willi syndrome.

PubMed

Dykens, Elisabeth M; Roof, Elizabeth; Bittel, Douglas; Butler, Merlin G

2011-05-01

Prader-Willi syndrome (PWS) is a genetic, neurodevelopmental disorder characterized by intellectual disabilities, growth hormone dysregulation, hyperphagia, increased risks of morbid obesity, compulsive behaviors, and irritability. As aberrant serotonergic functioning is strongly implicated in PWS, we examined associations between the PWS phenotype and polymorphisms in tryptophan hydroxylase 2 (TPH2), the rate-limiting enzyme in the biosynthesis of serotonin in the brain. Ninety-two individuals with PWS aged 4 to 50 years (M = 21.97) were genotyped for the TPH2 G703-T polymorphism. IQ testing was conducted in offspring, and parents completed questionnaires that tapped their child's compulsivity, hyperphagia, and other behavior problems. As expected, the frequency of G/T or T/T polymorphisms in participants with PWS (39%) was similar to rates found in the general population (38%). Compared to those with a homozygous (G/G) genotype, individuals with a T allele had significantly higher hyperphagic behavior, drive, and severity scores, and they also had a younger age of onset of hyperphagia. Those with a T allele also had higher IQ scores than their counterparts. Females with a T allele had significantly higher internalizing symptoms, primarily anxiety and depression, than all others. TPH2 G/T polymorphisms, and presumed loss of enzyme function, were associated with specific aspects of the PWS phenotype. Aberrant serotonergic functioning is strongly implicated in hyperphagia in PWS, and females with TPH2 T alleles may be at higher risk for affective or mood disorders. Findings hold promise for examining other serotonin-altering genes in PWS, and for future serotonin-altering treatment trials. © 2011 The Authors. Journal of Child Psychology and Psychiatry © 2011 Association for Child and Adolescent Mental Health.

Small gray matter volume in orbitofrontal cortex in Prader-Willi syndrome: a voxel-based MRI study.

PubMed

Ogura, Kaeko; Fujii, Toshikatsu; Abe, Nobuhito; Hosokai, Yoshiyuki; Shinohara, Mayumi; Takahashi, Shoki; Mori, Etsuro

2011-07-01

Prader-Willi syndrome (PWS) is a genetically determined neurodevelopmental disorder presenting with behavioral symptoms including hyperphagia, disinhibition, and compulsive behavior. The behavioral problems in individuals with PWS are strikingly similar to those in patients with frontal pathologies, particularly those affecting the orbitofrontal cortex (OFC). However, neuroanatomical abnormalities in the frontal lobe have not been established in PWS. The aim of this study was to look, using volumetric analysis, for morphological changes in the frontal lobe, especially the OFC, of the brains of individuals with PWS. Twelve adults with PWS and 13 age- and gender-matched control subjects participated in structural magnetic resonance imaging (MRI) scans. The whole-brain images were segmented and normalized to a standard stereotactic space. Regional gray matter volumes were compared between the PWS group and the control group using voxel-based morphometry. The PWS subjects showed small gray-matter volume in several regions, including the OFC, caudate nucleus, inferior temporal gyrus, precentral gyrus, supplementary motor area, postcentral gyrus, and cerebellum. The small gray-matter volume in the OFC remained significant in a separate analysis that included total gray matter volume as a covariate. These preliminary findings suggest that the neurobehavioral symptoms in individuals with PWS are related to structural brain abnormalities in these areas. Copyright © 2010 Wiley-Liss, Inc.

Cognition and behavior in pre-pubertal children with Prader-Willi syndrome and associations with sleep-related breathing disorders.

PubMed

Festen, Dederieke A M; Wevers, Maaike; de Weerd, Al W; van den Bossche, Renilde A S; Duivenvoorden, Hugo J; Hokken-Koelega, Anita C S

2008-12-01

Prader-Willi syndrome (PWS) is characterized by hypotonia, hypogonadism, obesity, and short stature. Neurobehavioral abnormalities, cognitive impairment, and sleep-related breathing disorders (SRBD) are common. In the general population associations between neurobehavioral and cognitive abnormalities and SRBD have been found. We investigated cognition, behavior, and SRBD in children with PWS. Thirty-one pre-pubertal PWS children were evaluated (5 with paternal deletion, 14 with maternal disomy, 4 with imprinting-center mutation, and in 8 the defect was not specified). Cognition was assessed by Wechsler scale subtests, and behavior by parent-questionnaires. Polysomnography was performed. Cognition, behavior, and associations with SRBD were evaluated. All cognitive subtests were significantly below O SDS, with the lowest median (interquartile range) scores for the Block design subtest (-2.7 SDS (-3.0 to -0.3)). In 60%, verbal subtests were less affected than performance subtests. Parents reported problem behavior related to "emotions/behavior not adapted to the social situation" and "insensitivity to social information." All children had SRBD, with an Apnea Hypopnea Index of 4.1/hr (2.6-7.9). One performance subtest score was significantly higher in children with better sleep efficiency, and daytime sleepiness was associated with more autistic-like social impairment. In contrast to our expectations, behavior was worse in children with better sleep-related breathing. In pre-pubertal PWS children, cognition is impaired. Neurobehavioral abnormalities are common, particularly autistic-like social impairment. Sleep efficiency was associated with better performance on one of the performance subtests, and neurobehavioral abnormalities were associated with daytime sleepiness. In contrast, we could not confirm a positive association of neurobehavioral abnormalities with SRBD in PWS. Copyright (c) 2008 Wiley-Liss, Inc.

Regional cerebral glucose metabolic abnormality in Prader-Willi syndrome: A 18F-FDG PET study under sedation.

PubMed

Kim, Sang Eun; Jin, Dong-Kyu; Cho, Sang Soo; Kim, Ji-Hae; Hong, Sungdo David; Paik, Kyung Hoon; Oh, Yoo Joung; Kim, An Hee; Kwon, Eun Kyung; Choe, Yon Ho

2006-07-01

Prader-Willi syndrome (PWS) is a genetic disorder caused by the nonexpression of paternal genes in the PWS region of chromosome 15q11-13 and is the most common cause of human syndromic obesity. We investigated regional brain metabolic impairment in children with PWS by 18F-FDG PET. Sixteen children with PWS (9 males, 7 females; mean age +/- SD, 4.2 +/- 1.1 y) and 7 healthy children (4 males, 3 females; mean age +/- SD, 4.0 +/- 1.7 y) underwent brain 18F-FDG PET in the resting state. The images of PWS children were compared using statistical parametric mapping analysis with those of healthy children in a voxelwise manner. Group comparison showed that children with PWS had decreased glucose metabolism in the right superior temporal gyrus and left cerebellar vermis, regions that are associated with taste perception/food reward and cognitive and emotional function, respectively. Metabolism was increased in the right orbitofrontal, bilateral middle frontal, right inferior frontal, left superior frontal, and bilateral anterior cingulate gyri, right temporal pole, and left uncus, regions that are involved in cognitive functions related to eating or obsessive-compulsive behavior. Interestingly, no significant metabolic abnormality was found in the hypothalamus, the brain region believed to be most involved in energy intake and expenditure. This study describes the neural substrate underlying the abnormal eating behavior and psychobehavioral problems of PWS.

Experimental Functional Analysis of Aggression in Children with Angelman Syndrome

ERIC Educational Resources Information Center

Strachan, Rachel; Shaw, Rebecca; Burrow, Caroline; Horsler, Kate; Allen, Debbie; Oliver, Chris

2009-01-01

Background: Kinship theory suggests that genomic imprinting could account for phenotypic behaviors that increase (in the case of Angelman syndrome) or decrease (for Prader-Willi syndrome) the drive to access social resources (adult contact) depending on the imprinting parent-of-origin. Difficult to manage behaviors, such as aggression that is…

BORDERLINE PERSONALITY DISORDER IN THE MEDICAL SETTING: Suggestive Behaviors, Syndromes, and Diagnoses.

PubMed

Sansone, Randy A; Sansone, Lori A

2015-01-01

Borderline personality disorder is a personality dysfunction that is characterized by disinhibition and impulsivity, which oftentimes manifest as self-regulation difficulties. Patients with this disorder have always been present in medical settings, but have been described as "difficult patients" rather than patients with borderline personality disorder. According to empirical findings, a number of behaviors and medical syndromes/diagnoses are suggestive of borderline personality disorder. Suggestive behaviors in the medical setting may include aggressive or disruptive behaviors, the intentional sabotage of medical care, and excessive healthcare utilization. Suggestive medical syndromes and diagnoses in the medical setting may include alcohol and substance misuse (including the abuse of prescription medications), multiple somatic complaints, chronic pain, obesity, sexual impulsivity, and hair pulling. While not all-inclusive or diagnostic, these behaviors and syndromes/diagnoses may invite further clinical evaluation of the patient for borderline personality disorder.

Associations between depressive syndromes and HIV risk behaviors among San Francisco men who have sex with men.

PubMed

Chen, Yea-Hung; Raymond, Henry Fisher

2017-12-01

HIV prevention plans for men who have sex with men (MSM) are often multifaceted. They involve reduction of sexual risk behaviors, such as condomless intercourse, but also often include pharmaceutical approaches, such as early treatment of HIV-infected individuals with antiretroviral therapy (ART). Effectiveness is possibly threatened by individual-level factors, such as depression. In this study of 322 San Francisco MSM (240 HIV-uninfected individuals and 82 HIV-infected individuals, according to self-report), we examine associations between depressive syndromes and HIV risk behaviors (sexual risk behaviors and ART non-adherence). Our study failed to find evidence that depressive syndromes lead to increases in ART non-adherence (risk difference, RD: 27.9; 95% confidence interval, CI: -3.5, 59.3). However, the study does suggest an association between depressive syndromes and concurrence of non-adherence and potentially HIV-discordant condomless receptive anal intercourse (RD: 36.0; 95% CI: 5.2, 66.8). Among HIV-uninfected MSM, our study suggests negative associations between depressive syndromes and sexual risk behaviors. We recommend screening and treatment of depression among HIV-infected MSM.

Prader-Willi Syndrome: Genetics and Behavior.

ERIC Educational Resources Information Center

Thompson, Travis; Butler, Merlin G.; MacLean, William E., Jr.; Joseph, Beth

1996-01-01

Reviews the behavioral, cognitive, and other psychological features of Prader-Willi Syndrome (PWS), exploring their relationships to known genetic mechanisms. PWS is a genetic developmental disability characterized by a group of specific behavioral features, including an insatiable appetite. The article briefly touches on PWS-related research at…

Münchhausen by proxy syndrome in clinical child neuropsychology: a case presenting with neuropsychological symptoms.

PubMed

Heubrock, D

2001-12-01

Münchhausen by proxy syndrome (MBPS) is a rare but dramatic variant of child abuse. In MBPS adults, mostly the mother, invent, manipulate, or produce the child's illness, and as a consequence the child has to undergo numerous diagnostic or treatment procedures. Typically, valid information about the etiology of the child's illness is withheld by the parents, and reversible symptoms vanish, when the child and the responsible adults are separated. Although valid statistical data about the epidemiology of MBPS are not available, MBPS should be considered more often than normally recognized. Neurological and neuropsychological presentations including developmental delays and learning problems appear to be common among MBPS cases so that clinical child neuropsychologists should be aware of this problem and consider MBPS at least in some of the mysterious cases that come to their attention. The present study describes a case of MBPS in which neurological and neuropsychological symptoms predominate. It presents a MBPS variant that is characterized by developmental delays and learning problems induced by unnecessary isolation at home, hospitalization, and treatment procedures. In the present case MBPS was at first suspected following neuropsychological assessment, since some of the main features of non-authenticity of symptom presentation gave cause for suspecting deceptive behavior on the mother's (and possibly also on the maternal grandmother's) side.

Acute exposure to 17α-ethinylestradiol alters boldness behavioral syndrome in female Siamese fighting fish.

PubMed

Dzieweczynski, Teresa L; Campbell, Brennah A; Marks, Jodi M; Logan, Brittney

2014-09-01

The role of anthropogenic sources in generating, maintaining, and influencing behavioral syndromes has recently been identified as an important area of future research. Endocrine disrupting chemicals are prevalent and persistent in aquatic ecosystems worldwide. These chemicals are known to have marked effects on the morphology and behavior of exposed individuals and, as such, may serve as a potential influence on behavioral syndromes. However, both the effects of exposure on behaviors beyond courtship and aggression and how exposure might affect behavioral variation at the individual level are understudied. To address this question, we examined boldness behavior in female Siamese fighting fish in three different assays (Novel Environment, Empty Tank, Shoaling) both before and after they were exposed to the estrogen mimic, 17α-ethinylestradiol (EE2). EE2 influences courtship, aggression, and boldness in males of this species but its effects have not been examined in females, to our knowledge. Females were tested multiple times in each assay before and after exposure so that behavioral consistency could be examined. A behavioral syndrome for boldness and activity level occurred across the three assays. The reductions in boldness and loss of the behavioral syndrome that resulted from EE2 exposure were surprising and suggest that the effects of EE2 exposure on female behavior and physiology should be examined more frequently. This study is one of the first to examine the effects of EE2 in females as well as on correlated behaviors and emphasizes the importance of examining the effects of endocrine disrupting chemicals on individual behavioral variation and consistency. Copyright © 2014 Elsevier Inc. All rights reserved.

Down Syndrome Health Screening--The Fife Model

ERIC Educational Resources Information Center

Jones, Jill; Hathaway, Dorothy; Gilhooley, Mary; Leech, Amanda; MacLeod, Susan

2010-01-01

People with Down syndrome have a greater risk of developing a range of health problems, including cardiac problems, thyroid disorders, sensory impairments, reduced muscle tone (hypotonia) and Alzheimer's disease. Despite this increased risk, regular screening is not typically offered to individuals with Down syndrome. A multidisciplinary health…

Characterisation of Sleep Problems in Children with Williams Syndrome

ERIC Educational Resources Information Center

Annaz, Dagmara; Hill, Catherine M.; Ashworth, Anna; Holley, Simone; Karmiloff-Smith, Annette

2011-01-01

Sleep is critical to optimal daytime functioning, learning and general health. In children with established developmental disorders sleep difficulties may compound existing learning difficulties. The purpose of the present study was to evaluate the prevalence and syndrome specificity of sleep problems in Williams syndrome (WS), a…

Normal Cognition and Behavior in a Smith-Lemli-Opitz Syndrome Patient Who Presented With Hirschsprung Disease

PubMed Central

Mueller, C.; Patel, S.; Irons, M.; Antshel, K.; Salen, G.; Tint, G.S.; Bay, C.

2005-01-01

Smith-Lemli-Opitz syndrome (SLOS) is an autosomal recessive disorder of cholesterol biosynthesis. It is caused by mutations in the gene encoding the enzyme 7-dehydrocholesterol Δ7-reductase (DHCR7), which catalyzes the final step in cholesterol biosynthesis, usually resulting in cholesterol deficiency. We report a 3.5-year-old girl who has cognition in the low average range and normal behavior, but in whom molecular studies identified two missense mutations in DHCR7: V326L and F284L. She was born at term following an uncomplicated pregnancy and delivery, and presented at 12 days of age with poor feeding, abdominal distention, and jaundice. Colonic biopsy was consistent with Hirschsprung disease. On physical examination she had mild ptosis, a long philtrum, mild micrognathia, a short, upturned nose, and subtle 2,3 syndactyly. Her 7-dehydrocholesterol (7-DHC) level was markedly elevated at 8.7 mg/dl (normal 0.10 ± 0.05), and her cholesterol level was normal at 61 mg/dl (normal for newborn period 50–80 mg/dl). Karyotype analysis was normal, 46,XX. Breast milk feeding was initiated and continued for 18 months. Cholesterol supplementation was implemented at 100 mg/kg/day at 3 months, which resulted in increased cholesterol levels and reduced dehydrocholesterol levels. Neuropsychological testing has shown functioning in the low average range, between the 14th and 18th centiles when compared to peers. This is markedly higher than most children with SLOS. She has no behavioral problems. MRI and MRS testing of the brain revealed no structural abnormalities. This is in contrast to a recently reported case by Prasad et al. [2002: Am J Med Genet 108:64–68] with a mild phenotype, behavioral problems, and abnormal MRI, who is compound heterozygote for both a null and missense mutation. Our case suggests that patients with severe feeding disorders with or without Hirschprung disease and postnatal onset microcephaly may warrant screening for SLOS. PMID:14556255

Sleep Problems in Individuals with Angelman Syndrome

ERIC Educational Resources Information Center

Didden, Robert; Korzilius, Hubert; Smits, Marcel G.; Curfs, Leopold M. G.

2004-01-01

Prevalence of severe sleep problems and its association with other variables were investigated with 109 individuals who have Angelman syndrome. Severe settling problems, frequent night waking, and early waking were found in 2%, 37%, and 10% of the individuals, respectively. Sleep problems were persistent in this sample. No statistically…

Understanding AIDS-Risk Behavior Among Adolescents in Psychiatric Care: Links to Psychopathology and Peer Relationships

PubMed Central

DONENBERG, GERI R.; EMERSON, ERIN; BRYANT, FRED B.; WILSON, HELEN; WEBER-SHIFRIN, ERYN

2005-01-01

Objective: Severely mentally ill youths are at elevated risk for human immunodeficiency virus infection, but little is known about acquired immunodeficiency syndrome (AIDS) risk behavior in adolescents who seek outpatient mental health services or about the links between psychiatric problems and particular high-risk behaviors. This pilot study used structural equation modeling to conduct a path analysis to explore the direct and indirect effects of adolescent psychopathology on risky sex, drug/alcohol use, and needle use. Method: Ethnically diverse youths (N = 86) and their caregivers who sought outpatient psychiatric services in Chicago completed questionnaires of adolescent psychopathology. Youths reported their relationship attitudes, peer influence, sexual behavior, and drug/alcohol use. Results: Different AIDS-risk behaviors were associated with distinct forms of adolescent psychopathology (e.g., delinquency was linked to drug/alcohol use, whereas aggression was related to risky sexual behavior), and peer influence mediated these linkages. Some patterns were similar for caregiver- and adolescent-reported problems (e.g., peer influence mediated the relation between delinquency and drug/alcohol use), but others were different (e.g., caregiver-reported delinquency was associated with risky sex, whereas adolescent-reported delinquency was not). Conclusions: Findings underscore the complexity of factors (types of informants and dimensions of psychopathology) that underlie AIDS risk in troubled youths, and they offer specific directions for designing and implementing uniquely tailored AIDS prevention programs, for example, by targeting delinquent behavior and including high-risk peers and important family members in interventions. PMID:11392341

Behavior in the Lesch--Nyhan Syndrome

ERIC Educational Resources Information Center

Nyhan, William L.

1976-01-01

The Lesch-Nyhan syndrome (which only affects males) is a heritable disorder of the metabolism of uric acid in which behavioral manifestations are prominent and among the most provocative. (Author/SBH)

Public Health Literature Review of Fragile X Syndrome

PubMed Central

Raspa, Melissa; Wheeler, Anne C.; Riley, Catharine

2017-01-01

OBJECTIVES The purpose of this systematic literature review is to describe what is known about fragile X syndrome (FXS) and to identify research gaps. The results can be used to help inform future public health research and provide pediatricians with up-to-date information about the implications of the condition for individuals and their families. METHODS An electronic literature search was conducted, guided by a variety of key words. The search focused on 4 areas of both clinical and public health importance: (1) the full mutation phenotype, (2) developmental trajectories across the life span, (3) available interventions and treatments, and (4) impact on the family. A total of 661 articles were examined and 203 were included in the review. RESULTS The information is presented in the following categories: developmental profile (cognition, language, functional skills, and transition to adulthood), social-emotional profile (cooccurring psychiatric conditions and behavior problems), medical profile (physical features, seizures, sleep, health problems, and physiologic features), treatment and interventions (educational/behavioral, allied health services, and pharmacologic), and impact on the family (family environment and financial impact). Research gaps also are presented. CONCLUSIONS The identification and treatment of FXS remains an important public health and clinical concern. The information presented in this article provides a more robust understanding of FXS and the impact of this complex condition for pediatricians. Despite a wealth of information about the condition, much work remains to fully support affected individuals and their families. PMID:28814537

An evaluation of antisocial behaviour in children after traumatic brain injury: the prospect of improving the quality of life in rehabilitation.

PubMed

Tomaszewski, Wiesław; Buliński, Leszek; Mirski, Andrzej; Rasmus, Anna; Kowalczyk, Jakub; Bazan, Maria; Pąchalska, Maria

2014-01-01

The aim of the article is to present the consequences of traumatic brain injury in children, associated with general cognition and behavioural disorders, mainly of the antisocial type. A total of 20 school-age children took part in the study, including six girls and 14 boys. The average age of the children was 13.35 years (standard deviation SD = 1.95). The research instruments included an analysis of documentation, a structured clinical interview, MMSE and Frontal Behavioral Inventory (FBInv) with additional set of five supplementary questions directed for detection of antisocial behavior. The research was conducted from the beginning of January 2009 until the end of May 2009. As hypothesized, the functioning of the children with traumatic brain injury is severely disrupted, because of the presence of cognitive impairment, however, dementia is not manifested. In a significant number of the children with traumatic brain injury we found not only the frontal syndrome, but also the occurrence of antisocial behaviour. The most commonly reported behavioural problems were: disorganization commonly referred to as laziness, hypersensitivity, and anxiety. The most common types of anti-social behaviour were: impulsivity, physical and verbal aggression, and also an outburst of anger. The children with traumatic brain injury suffer from a cognitive disorders and behavioural problems, especially impulsivity, physical and verbal aggression, increased anxiety, and disorganization. The occurrence of frontal syndrome is related to the development of antisocial behaviour.

Public Health Literature Review of Fragile X Syndrome.

PubMed

Raspa, Melissa; Wheeler, Anne C; Riley, Catharine

2017-06-01

The purpose of this systematic literature review is to describe what is known about fragile X syndrome (FXS) and to identify research gaps. The results can be used to help inform future public health research and provide pediatricians with up-to-date information about the implications of the condition for individuals and their families. An electronic literature search was conducted, guided by a variety of key words. The search focused on 4 areas of both clinical and public health importance: (1) the full mutation phenotype, (2) developmental trajectories across the life span, (3) available interventions and treatments, and (4) impact on the family. A total of 661 articles were examined and 203 were included in the review. The information is presented in the following categories: developmental profile (cognition, language, functional skills, and transition to adulthood), social-emotional profile (cooccurring psychiatric conditions and behavior problems), medical profile (physical features, seizures, sleep, health problems, and physiologic features), treatment and interventions (educational/behavioral, allied health services, and pharmacologic), and impact on the family (family environment and financial impact). Research gaps also are presented. The identification and treatment of FXS remains an important public health and clinical concern. The information presented in this article provides a more robust understanding of FXS and the impact of this complex condition for pediatricians. Despite a wealth of information about the condition, much work remains to fully support affected individuals and their families. Copyright © 2017 by the American Academy of Pediatrics.

Associations between causal attributions and personal stigmatizing attitudes in untreated persons with current mental health problems.

PubMed

Stolzenburg, Susanne; Freitag, Simone; Schmidt, Silke; Schomerus, Georg

2018-02-01

Past research has shown that among the general public, certain causal explanations like biomedical causes are associated with stronger desire for social distance from persons with mental illness. Aim of this study was to find out how different causal attributions of persons with untreated mental health problems regarding their own complaints are associated with stigmatizing attitudes, anticipated self-stigma when seeking help and perceived stigma-stress. Altogether, 207 untreated persons with a current depressive syndrome were interviewed. Biomedical causes, but also belief in childhood trauma or unhealthy behavior as a cause of the problem, were associated with stronger personal stigma and with more stigma-stress. Similarities and differences to findings among the general population and implications for future research are discussed. Copyright © 2017 Elsevier B.V. All rights reserved.

Noonan syndrome

MedlinePlus

... ray , or echocardiogram Hearing tests Growth hormone levels Genetic testing can help diagnose this syndrome. ... Problems with the structure of the heart Short height Social problems due to physical symptoms

Adolescents with Asperger Syndrome Can Use a Mindfulness-Based Strategy to Control Their Aggressive Behavior

ERIC Educational Resources Information Center

Singh, Nirbhay N.; Lancioni, Giulio E.; Singh, Angela D. A.; Winton, Alan S. W.; Singh, Ashvind N. A.; Singh, Judy

2011-01-01

Children and adolescents with Asperger syndrome occasionally exhibit aggressive behavior against peers and parents. In a multiple baseline design across subjects, three adolescents with Asperger syndrome were taught to use a mindfulness-based procedure called "Meditation on the Soles of the Feet" to control their physical aggression in the family…

Behavioral Assessment of Social Anxiety in Females with Turner or Fragile X Syndrome.

ERIC Educational Resources Information Center

Lesniak-Karpiak, Katarzyna; Mazzocco, Michele M. M.; Ross, Judith L.

2003-01-01

This study compared 29 females with Turner syndrome and 21 females with fragile X syndrome (ages 6-22) on a videotaped role-play interaction with 34 females in a comparison group. Three of eight behavioral measures of social skills differentiated the participant groups. Fragile-X subjects required more time to initiate interactions and Turner…

Clinical relevance of cannabis tolerance and dependence.

PubMed

Jones, R T; Benowitz, N L; Herning, R I

1981-01-01

Psychoactive drugs are often widely used before tolerance and dependence is fully appreciated. Tolerance to cannabis-induced cardiovascular and autonomic changes, decreased intraocular pressure, sleep and sleep EEG, mood and behavioral changes is acquired and, to a great degree, lost rapidly with optimal conditions. Mechanisms appear more functional than metabolic. Acquisition rate depends on dose and dose schedule. Dependence, manifested by withdrawal symptoms after as little as 7 days of THC administration, is characterized by irritability, restlessness, insomnia, anorexia, nausea, sweating, salivation, increased body temperature, altered sleep and waking EEG, tremor, and weight loss. Mild and transient in the 120 subjects studied, the syndrome was similar to sedative drug withdrawal. Tolerance to drug side effects can be useful. Tolerance to therapeutic effects or target symptoms poses problems. Clinical significance of dependence is difficult to assess since drug-seeking behavior has many determinants. Cannabis-induced super sensitivity should be considered wherever chronic drug administration is anticipated in conditions like epilepsy, glaucoma or chronic pain. Cannabis pharmacology suggests ways of minimizing tolerance and dependence problems.

Caregiver Burden in Frontotemporal Degeneration and Corticobasal Syndrome

PubMed Central

Armstrong, Nicole; Schupf, Nicole; Grafman, Jordan; Huey, Edward D.

2015-01-01

Background and Aims Caregiver stress is often a serious problem when caring for a patient with frontal lobe dysfunction. Methods A total of 102 caregivers of both patients with frontotemporal degeneration and corticobasal syndrome completed the Frontal Systems Behavior Scale (FrSBe) and the Zarit Burden Interview (ZBI). To analyze the association between apathy or disinhibition (or both) and caregiver burden, the effects of the total FrSBe and the apathy and disinhibition subscales of the FrSBE on the total ZBI score were assessed with logistic regressions and t tests. Results Total FrSBE score and the apathy FrSBE subscore predicted caregiver burden. Apathy occurred without disinhibition, and the two occurred together, but disinhibition without apathy was very rare. Conclusions Disinhibition without apathy occurred very rarely. Apathy was more associated with caregiver burden than disinhibition. PMID:24022248

STOP Abusive Behavior Syndrome: Developing a Community Response.

ERIC Educational Resources Information Center

Holzman, Lois; Rivera, Mary

This paper discusses the social-therapeutic approach to preventing abusive behavior, and describes the implementation of specific STOP Abusive Behavior Syndrome (ABS) projects in New York City, New York. The projects' goal is to empower people to continually develop throughout their lifespans. Basic tenants include the following: (1) emotions are…

Behavioral Phenotype in Adults with Prader-Willi Syndrome

ERIC Educational Resources Information Center

Sinnema, Margje; Einfeld, Stewart L.; Schrander-Stumpel, Constance T. R. M.; Maaskant, Marian A.; Boer, Harm; Curfs, Leopold M. G.

2011-01-01

Prader-Willi syndrome (PWS) is characterized by temper tantrums, impulsivity, mood fluctuations, difficulty with change in routine, skinpicking, stubbornness and aggression. Many studies on behavior in PWS are limited by sample size, age range, a lack of genetically confirmed diagnosis of PWS and inconsistent assessment of behavior. The aim of…

Genetics Home Reference: FG syndrome

MedlinePlus

... inheritance is that fathers cannot pass X-linked traits to their sons. Related Information What does it ... Opitz JO. Behavior phenotype of FG syndrome: cognition, personality, and behavior in eleven affected boys. Am J ...

The Early Interventionist and the Medical Problems of the Child with Down Syndrome.

ERIC Educational Resources Information Center

Roizen, Nancy J.

2003-01-01

This article describes medical problems that occur more frequently in young children with Down syndrome. These include common cardiac, hearing, and vision problems and less common thyroid and celiac diseases. Guidelines for preventing dental and obesity problems are followed by suggestions to early interventionists for watching for leukemia, neck…

Toxic shock syndrome

MedlinePlus

Staphylococcal toxic shock syndrome; Toxic shock-like syndrome; TSLS ... Toxic shock syndrome is caused by a toxin produced by some types of staphylococcus bacteria. A similar problem, called toxic shock- ...

Syndrome specificity and mother-child interactions: Examining positive and negative parenting across contexts and time

PubMed Central

Blacher, Jan; Baker, Bruce L.; Kaladjian, Araksia

2012-01-01

This study examined the extent to which child syndromes and observation context related to mothers’ parenting behaviors. Longitudinal observations were conducted of parenting behavior across ages 3, 4, and 5 years during structured and unstructured activities. The 183 participants included mothers of children with autism spectrum disorders, cerebral palsy, Down syndrome, undifferentiated developmental delay, or typical cognitive development. Negative parenting behaviors were higher in structured activities and higher in mothers of children in all developmentally delayed groups. Positive parenting was higher in unstructured activities and especially high for mothers of children with Down syndrome. Despite differences found through direct observation of parenting children in different diagnostic groups, they are not as strong as syndrome-group differences found through more commonly used self-report questionnaires assessing domains like parenting stress. PMID:22829243

Metabolic consequences of stress during childhood and adolescence.

PubMed

Pervanidou, Panagiota; Chrousos, George P

2012-05-01

Stress, that is, the state of threatened or perceived as threatened homeostasis, is associated with activation of the stress system, mainly comprised by the hypothalamic-pituitary-adrenal axis and the arousal/sympathetic nervous systems. The stress system normally functions in a circadian manner and interacts with other systems to regulate a variety of behavioral, endocrine, metabolic, immune, and cardiovascular functions. However, the experience of acute intense physical or emotional stress, as well as of chronic stress, may lead to the development of or may exacerbate several psychologic and somatic conditions, including anxiety disorders, depression, obesity, and the metabolic syndrome. In chronically stressed individuals, both behavioral and neuroendocrine mechanisms promote obesity and metabolic abnormalities: unhealthy lifestyles in conjunction with dysregulation of the stress system and increased secretion of cortisol, catecholamines, and interleukin-6, with concurrently elevated insulin concentrations, lead to development of central obesity, insulin resistance, and the metabolic syndrome. Fetal life, childhood, and adolescence are particularly vulnerable periods of life to the effects of intense acute or chronic stress. Similarly, these life stages are crucial for the later development of behavioral, metabolic, and immune abnormalities. Developing brain structures and functions related to stress regulation, such as the amygdala, the hippocampus, and the mesocorticolimbic system, are more vulnerable to the effects of stress compared with mature structures in adults. Moreover, chronic alterations in cortisol secretion in children may affect the timing of puberty, final stature, and body composition, as well as cause early-onset obesity, metabolic syndrome, and type 2 diabetes mellitus. The understanding of stress mechanisms leading to metabolic abnormalities in early life may lead to more effective prevention and intervention strategies of obesity-related health problems. Copyright © 2012 Elsevier Inc. All rights reserved.

Fibromyalgia

MedlinePlus

... following: Anxiety. Depression. Irritable bowel syndrome. Restless legs syndrome. Increased sensitivity to odors, bright lights, loud noises, or medicines. Headaches, migraines, or jaw pain. Dry eyes or mouth. Dizziness and problems with balance. Problems ...

Social Anxiety in Cornelia de Lange Syndrome

ERIC Educational Resources Information Center

Richards, Caroline; Moss, Jo; O'Farrell, Laura; Kaur, Gurmeash; Oliver, Chris

2009-01-01

In this study we assessed the behavioral presentation of social anxiety in Cornelia de Lange syndrome (CdLS) using a contrast group of Cri du Chat syndrome (CdCS). Behaviors indicative of social anxiety were recorded in twelve children with CdLS (mean age = 11.00; SD = 5.15) and twelve children with CdCS (8.20; SD = 2.86) during social…

Klinefelter's syndrome and Prader-Willi syndrome: a rare combination.

PubMed

Verhoeven, W M A; de Vries, B B A; Duffels, S J H; Egger, J I M; Noordam, C; Tuinier, S

2007-01-01

In this paper a review is presented of the rare combination of Klinefelter's syndrome and Prader-Willi syndrome (PWS) and a second case of this combination with a uniparental disomy (UPD) etiology of PWS is described. Patients outlined in all other 8 reports and the present case have a PWS phenotype. Virtually no information is available on the behavioral and psychopathological phenotype in this combination. The latter may be explained by the observation that psychiatric syndromes are especially prevalent in PWS patients with a UPD. It is concluded that instability in mood and behavior in this and other syndromes should be preferentially treated with mood stabilizing agents. Copyright (c) 2007 S. Karger AG, Basel.

Reversed gender ratio of autism spectrum disorder in Smith-Magenis syndrome.

PubMed

Nag, Heidi Elisabeth; Nordgren, Ann; Anderlid, Britt-Marie; Nærland, Terje

2018-01-01

A substantial amount of research shows a higher rate of autistic type of problems in males compared to females. The 4:1 male to female ratio is one of the most consistent findings in autism spectrum disorder (ASD).Lately, the interest in studying ASD in genetic disorders has increased, and research has shown a higher prevalence of ASD in some genetic disorders than in the general population.Smith-Magenis syndrome (SMS) is a rare and complex genetic syndrome caused by an interstitial deletion of chromosome 17p11.2 or a mutation on the retinoic acid induced 1 gene. The disorder is characterised by intellectual disability, multiple congenital anomalies, obesity, neurobehavioural abnormalities and a disrupted circadian sleep-wake pattern. Parents of 28 persons with SMS between 5 and 50 years old participated in this study. A total of 12 of the persons with SMS were above the age of 18 at the time of the study. A total of 11 came from Sweden and 17 were from Norway.We collected information regarding the number of autism spectrum symptoms using the Social Communication Questionnaire (SCQ) and the Social Responsiveness Scale (SRS). Adaptive behaviour was also measured using the Vineland Adaptive Behavior Scale II. The level of intellectual disability was derived from a review of the medical chart. We found significant gender differences in ASD symptomatology using the SCQ and SRS questionnaires. We found approximately three females per male above the SCQ cutoff. The same differences were not found in the intellectual level and adaptive behaviour or for behavioural and emotional problems.Gender had an independent contribution in a regression model predicting the total SCQ score, and neither the Vineland Adaptive Behavior Scale II nor the Developmental Behaviour Checklist had an independent contribution to the SCQ scores. We found a clear reversed gender difference in ASD symptomatology in persons with SMS. This may be relevant in the search for female protective factors assumed to explain the male bias in ASD.

Links between sleep and daytime behaviour problems in children with Down syndrome.

PubMed

Esbensen, A J; Hoffman, E K; Beebe, D W; Byars, K C; Epstein, J

2018-02-01

In the general population, sleep problems have an impact on daytime performance. Despite sleep problems being common among children with Down syndrome, the impact of sleep problems on daytime behaviours in school-age children with Down syndrome is an understudied topic. Our study examined the relationship between parent-reported and actigraphy-measured sleep duration and sleep quality with parent and teacher reports of daytime behaviour problems among school-age children with Down syndrome. Thirty school-age children with Down syndrome wore an actigraph watch for a week at home at night. Their parent completed ratings of the child's sleep during that same week. Their parent and teacher completed a battery of measures to assess daytime behaviour. Parent reports of restless sleep behaviours on the Children's Sleep Habits Questionnaire, but not actigraph-measured sleep efficiency, was predictive of parent and teacher behavioural concerns on the Nisonger Child Behaviour Rating Form and the Vanderbilt ADHD Rating Scales. Actigraph-measured sleep period and parent-reported sleep duration on the Children's Sleep Habits Questionnaire was predictive of daytime parent-reported inattention. Actigraph-measured sleep period was predictive of parent-reported hyperactivity/impulsivity. The study findings suggest that sleep problems have complex relationships to both parent-reported and teacher-reported daytime behaviour concerns in children with Down syndrome. These findings have implications for understanding the factors impacting behavioural concerns and their treatment in school-age children with Down syndrome. © 2017 MENCAP and International Association of the Scientific Study of Intellectual and Developmental Disabilities and John Wiley & Sons Ltd.

Randomized open-label trial of dextromethorphan in Rett syndrome.

PubMed

Smith-Hicks, Constance L; Gupta, Siddharth; Ewen, Joshua B; Hong, Manisha; Kratz, Lisa; Kelley, Richard; Tierney, Elaine; Vaurio, Rebecca; Bibat, Genila; Sanyal, Abanti; Yenokyan, Gayane; Brereton, Nga; Johnston, Michael V; Naidu, Sakkubai

2017-10-17

To determine safety and perform a preliminary assessment of dose-dependent efficacy of dextromethorphan in normalizing electrographic spikes, clinical seizures, and behavioral and cognitive functions in girls with Rett syndrome. We used a prospective randomized, open-label trial in fast metabolizers of dextromethorphan to examine the effect of dextromethorphan on core clinical features of Rett syndrome. Interictal spike activity and clinical seizures were determined using EEG and parent reporting. Cognitive data were obtained using the Mullen Scales of Early Learning and Vineland Adaptive Behavior Scales, while behavioral data were obtained from parent-completed checklists, the Aberrant Behavior Checklist-Community Version, and the Screen for Social Interaction. Anthropometric data were obtained according to the National Health and Nutrition Examination Survey. The Rett Syndrome Severity Scale provided a clinical global impression of the effect of dextromethorphan on clinical severity. Dextromethorphan is safe for use in 3- to 15-year-old girls with Rett syndrome. Thirty-five girls were treated with 1 of 3 doses of dextromethorphan over a period of 6 months. Statistically significant dose-dependent improvements were seen in clinical seizures, receptive language, and behavioral hyperactivity. There was no significant improvement in global clinical severity as measured by the Rett Syndrome Severity Scale. Dextromethorphan is a potent noncompetitive antagonist of the NMDA receptor channel that is safe for use in young girls with Rett syndrome. Preliminary evidence suggests that dextromethorphan may improve some core features of Rett syndrome. This study provides Class IV evidence that dextromethorphan at various doses does not change EEG spike counts over 6 months, though precision was limited to exclude an important effect. © 2017 American Academy of Neurology.

Conversational behaviour of children with Asperger syndrome and conduct disorder.

PubMed

Adams, Catherine; Green, Jonathan; Gilchrist, Anne; Cox, Anthony

2002-07-01

Social communication problems in individuals who have Asperger syndrome constitute one of the most significant problems in the syndrome. This study makes a systematic analysis of the difficulties demonstrated with the use of language (pragmatics) in adolescents who have Asperger syndrome. Recent advances in discourse analysis were applied to conversational samples from a group of children with Asperger syndrome and a matched control group of children with severe conduct disorder. Two types of conversation were sampled from each group, differing in emotional content. The results showed that in these contexts children with Asperger syndrome were no more verbose as a group than controls, though they showed a tendency to talk more in more emotion-based conversations. Children with Asperger syndrome, as a group, performed similarly to control subjects in ability to respond to questions and comments. However, they were more likely to show responses which were problematic in both types of conversation. In addition, individuals with Asperger syndrome showed more problems in general conversation than during more emotionally and socially loaded topics. The group with Asperger syndrome was found to contain a small number of individuals with extreme verbosity but this was not a reliable characteristic of the group as a whole.

Socio-Behavioral Characteristics of Children with Rubinstein-Taybi Syndrome

ERIC Educational Resources Information Center

Galera, Cedric; Taupiac, Emmanuelle; Fraisse, Sonia; Naudion, Sophie; Toussaint, Eva; Rooryck-Thambo, Caroline; Delrue, Marie-Ange; Arveiler, Benoit; Lacombe, Didier; Bouvard, Manuel-Pierre

2009-01-01

Research regarding the behavioral aspects of children with Rubinstein-Taybi syndrome (RTS) has suggested some possible behavioral patterns including autistic features. Caregivers of 39 children (mean age = 8.4 years) with RTS (49% showing abnormality in "CREBBP" gene) and 39 children (mean age = 8.6 years) matched on developmental level,…

Maladaptive Behaviors Related to Dementia Status in Adults with Down Syndrome

ERIC Educational Resources Information Center

Urv, Tiina K.; Zigman, Warren B.; Silverman, Wayne

2008-01-01

Changes in maladaptive behaviors related to specific stages of dementia were investigated in 251 adults 45 years of age and older with Down syndrome. Findings indicate clear differences in maladaptive behaviors at various stages of dementia. Generally, individuals with no signs or symptoms of dementia displayed fewer and less severe maladaptive…

Effects of Environmental Events on Smiling and Laughing Behavior in Angelman Syndrome.

ERIC Educational Resources Information Center

Oliver, Chris; Demetriades, Louisa; Hall, Scott

2002-01-01

A study examined the variability of smiling and laughing behaviors of three children (ages 7-17) with Angelman syndrome. Results found laughing and smiling increased during social situations and occurred at low levels during non-social situations. The behaviors, therefore, did not occur totally inappropriately, as has been suggested. (Contains…

Maladaptive and Repetitive Behaviors in Children with Down Syndrome and Autism Spectrum Disorders: Implications for Screening

ERIC Educational Resources Information Center

Hepburn, Susan L.; MacLean, William E.

2009-01-01

Researchers and clinicians are recognizing that a subgroup of children with Down syndrome (DS) also present with clinically significant impairments in social-communication and restricted patterns of behavior, such as those that characterize autism spectrum disorders (ASD). Efforts to better describe the behavioral presentation of children with…

Insomnia of childhood.

PubMed

Lipton, Jonathan; Becker, Ronald E; Kothare, Sanjeev V

2008-12-01

Insomnia is a major public health problem and is the most common sleep disturbance in both adults and children. The causes of sleeplessness are age-dependent and have potentially enormous effects on cognitive development, behavior, family dynamics, and the metabolic health of children. Here we review the epidemiology, cause, pathophysiology, and clinical approach to pediatric insomnia. Normal sleep is crucial for brain function, behavior, and normal metabolism. Consistently, sleep loss has been linked to behavioral and attention problems, impaired learning and memory, obesity, and psychiatric disorders. The neurological mechanisms that govern sleep initiation and maintenance are poorly understood. The types of insomnia are age-dependent and can occur as primary disorders, or in the context of another primary sleep disorder such as restless legs syndrome, or secondary to another underlying medical condition. Children with chronic diseases and especially children with neurodevelopmental disorders are at particular risk of insomnia. Pediatric insomnia is common and is a source of potential psychophysiological stress to both children and their caregivers. The causes of insomnia are various. Pediatricians should have a working knowledge of the causes of sleeplessness in order to promptly curtail the chronic effects of sleep loss and effectively screen for underlying, potentially treatable disorders.

Low self-control and co-occurrence of gambling with substance use and delinquency among Chinese adolescents.

PubMed

Cheung, Nicole W T

2014-03-01

Relatively little is known about gambling co-morbidity in Asian youth populations. The role of trait self-control in co-morbidity also remains under-explored in the gambling literature. This study examined the association between gambling, substance use and delinquency among Chinese adolescents, and the extent to which these forms of risk behavior are commonly predicted by low self-control. Data from a cross-sectional questionnaire survey of a stratified, random sample of 4,734 high school students aged 12-23 years in Hong Kong were analyzed. The prevalence of gambling pathology, frequency and attitudes showed statistically significant, positive and consistent relationships with tobacco use, alcohol use, and delinquent acts at the p < .001 level. Further analyses revealed that low self-control significantly (p < .001) predicts at-risk/probable pathological gambling, frequent gambling, strong permissiveness toward gambling, heavy use of tobacco and alcohol, and delinquent involvement, even after controlling for the potential shared correlates of socioeconomic characteristics, parental monitoring and peer delinquency. Hence, the concept that gambling problems and strong receptivity to gambling are likely to be part of a general problem behavior syndrome is evinced cross-culturally among young people in a Chinese context. It may also be cost-effective to increase intervention efforts to improve the self-control deficit in adolescents, as this should reduce their gambling and concurrent problem behavior.

Haploinsufficiency of HDAC4 Causes Brachydactyly Mental Retardation Syndrome, with Brachydactyly Type E, Developmental Delays, and Behavioral Problems

PubMed Central

Williams, Stephen R.; Aldred, Micheala A.; Der Kaloustian, Vazken M.; Halal, Fahed; Gowans, Gordon; McLeod, D. Ross; Zondag, Sara; Toriello, Helga V.; Magenis, R. Ellen; Elsea, Sarah H.

2010-01-01

Brachydactyly mental retardation syndrome (BDMR) is associated with a deletion involving chromosome 2q37. BDMR presents with a range of features, including intellectual disabilities, developmental delays, behavioral abnormalities, sleep disturbance, craniofacial and skeletal abnormalities (including brachydactyly type E), and autism spectrum disorder. To date, only large deletions of 2q37 have been reported, making delineation of a critical region and subsequent identification of candidate genes difficult. We present clinical and molecular analysis of six individuals with overlapping deletions involving 2q37.3 that refine the critical region, reducing the candidate genes from >20 to a single gene, histone deacetylase 4 (HDAC4). Driven by the distinct hand and foot anomalies and similar cognitive features, we identified other cases with clinical findings consistent with BDMR but without a 2q37 deletion, and sequencing of HDAC4 identified de novo mutations, including one intragenic deletion probably disrupting normal splicing and one intragenic insertion that results in a frameshift and premature stop codon. HDAC4 is a histone deacetylase that regulates genes important in bone, muscle, neurological, and cardiac development. Reportedly, Hdac4−/− mice have severe bone malformations resulting from premature ossification of developing bones. Data presented here show that deletion or mutation of HDAC4 results in reduced expression of RAI1, which causes Smith-Magenis syndrome when haploinsufficient, providing a link to the overlapping findings in these disorders. Considering the known molecular function of HDAC4 and the mouse knockout phenotype, taken together with deletion or mutation of HDAC4 in multiple subjects with BDMR, we conclude that haploinsufficiency of HDAC4 results in brachydactyly mental retardation syndrome. PMID:20691407

Haploinsufficiency of HDAC4 causes brachydactyly mental retardation syndrome, with brachydactyly type E, developmental delays, and behavioral problems.

PubMed

Williams, Stephen R; Aldred, Micheala A; Der Kaloustian, Vazken M; Halal, Fahed; Gowans, Gordon; McLeod, D Ross; Zondag, Sara; Toriello, Helga V; Magenis, R Ellen; Elsea, Sarah H

2010-08-13

Brachydactyly mental retardation syndrome (BDMR) is associated with a deletion involving chromosome 2q37. BDMR presents with a range of features, including intellectual disabilities, developmental delays, behavioral abnormalities, sleep disturbance, craniofacial and skeletal abnormalities (including brachydactyly type E), and autism spectrum disorder. To date, only large deletions of 2q37 have been reported, making delineation of a critical region and subsequent identification of candidate genes difficult. We present clinical and molecular analysis of six individuals with overlapping deletions involving 2q37.3 that refine the critical region, reducing the candidate genes from >20 to a single gene, histone deacetylase 4 (HDAC4). Driven by the distinct hand and foot anomalies and similar cognitive features, we identified other cases with clinical findings consistent with BDMR but without a 2q37 deletion, and sequencing of HDAC4 identified de novo mutations, including one intragenic deletion probably disrupting normal splicing and one intragenic insertion that results in a frameshift and premature stop codon. HDAC4 is a histone deacetylase that regulates genes important in bone, muscle, neurological, and cardiac development. Reportedly, Hdac4(-/-) mice have severe bone malformations resulting from premature ossification of developing bones. Data presented here show that deletion or mutation of HDAC4 results in reduced expression of RAI1, which causes Smith-Magenis syndrome when haploinsufficient, providing a link to the overlapping findings in these disorders. Considering the known molecular function of HDAC4 and the mouse knockout phenotype, taken together with deletion or mutation of HDAC4 in multiple subjects with BDMR, we conclude that haploinsufficiency of HDAC4 results in brachydactyly mental retardation syndrome.

Assessing the impact of caring for a child with Dravet syndrome: Results of a caregiver survey.

PubMed

Campbell, Jonathan D; Whittington, Melanie D; Kim, Chong H; VanderVeen, Gina R; Knupp, Kelly G; Gammaitoni, Arnold

2018-03-01

The objective of this study was to describe and quantify the impact of caring for a child with Dravet syndrome (DS) on caregivers. We surveyed DS caregivers at a single institution with a large population of patient with DS. Survey domains included time spent/difficulty performing caregiving tasks (Oberst Caregiving Burden Scale, OCBS); caregiver health-related quality of life (EuroQoL 5D-5L, EQ-5D); and work/activity impairment (Work Productivity and Activity Impairment questionnaire, WPAI). Modified National Health Interview Survey (NHIS) questions were included to assess logistical challenges associated with coordinating medical care. Thirty-four primary caregivers responded, and 30/34 respondents completed the survey. From OCBS, providing transportation, personal care, and additional household tasks required the greatest caregiver time commitment; arranging for child care, communication, and managing behavioral problems presented the greatest difficulty. EuroQoL 5D-5L domains with the greatest impact on caregivers (0=none, 5=unable/extreme) were anxiety/depression (70% of respondents≥slight problems, 34%≥moderate) and discomfort/pain (57% of respondents≥slight problems, 23%≥moderate). The mean EQ-5D general health visual analogue scale (VAS) score (0=death; 100=perfect health) was 67 (range, 11-94). Respondents who scored <65 were two- to fourfold more likely to report ≥moderate time spent and difficulty managing child behavior problems and assisting with walking, suggesting that children with DS with high degrees of motor or neurodevelopmental problems have an especially high impact on caregiver health. On the WPAI, 26% of caregivers missed >1day of work in the previous week, with 43% reporting substantial impact (≥6, scale=1-10) on work productivity; 65% reported switching jobs, quitting jobs, or losing a job due to caregiving responsibilities. National Health Interview Survey responses indicated logistical burdens beyond the home; 50% of caregivers made ≥10 outpatient visits in the past year with their child with DS. Caring for patients with DS exerts physical, emotional, and time burdens on caregivers. Supportive services for DS families are identified to highlight an unmet need for DS treatments. Copyright © 2018 The Authors. Published by Elsevier Inc. All rights reserved.

Guide to Understanding Pfeiffer Syndrome

MedlinePlus

... sockets (orbits). Approximately 50% of children with Pfeiffer syndrome have some form of hearing loss secondary to an abnormally small ear canal and middle ear. Dental problems are also common. Visual problems ...

Young People with Intellectual Disability Transitioning to Adulthood: Do Behaviour Trajectories Differ in Those with and without Down Syndrome?

PubMed

Foley, Kitty-Rose; Taffe, John; Bourke, Jenny; Einfeld, Stewart L; Tonge, Bruce J; Trollor, Julian; Leonard, Helen

2016-01-01

Young people with intellectual disability exhibit substantial and persistent problem behaviours compared with their non-disabled peers. The aim of this study was to compare changes in emotional and behavioural problems for young people with intellectual disability with and without Down syndrome as they transition into adulthood in two different Australian cohorts. Emotional and behavioural problems were measured over three time points using the Developmental Behaviour Checklist (DBC) for those with Down syndrome (n = 323 at wave one) and compared to those with intellectual disability of another cause (n = 466 at wave one). Outcome scores were modelled using random effects regression as linear functions of age, Down syndrome status, ability to speak and gender. DBC scores of those with Down syndrome were lower than those of people without Down syndrome indicating fewer behavioural problems on all scales except communication disturbance. For both groups disruptive, communication disturbance, anxiety and self-absorbed DBC subscales all declined on average over time. There were two important differences between changes in behaviours for these two cohorts. Depressive symptoms did not significantly decline for those with Down syndrome compared to those without Down syndrome. The trajectory of the social relating behaviours subscale differed between these two cohorts, where those with Down syndrome remained relatively steady and, for those with intellectual disability from another cause, the behaviours increased over time. These results have implications for needed supports and opportunities for engagement in society to buffer against these emotional and behavioural challenges.

Problem gambling symptomatology and alcohol misuse among adolescents: A parallel-process latent growth curve model.

PubMed

Mutti-Packer, Seema; Hodgins, David C; El-Guebaly, Nady; Casey, David M; Currie, Shawn R; Williams, Robert J; Smith, Garry J; Schopflocher, Don P

2017-06-01

The objective of the current study was to examine the possible temporal associations between alcohol misuse and problem gambling symptomatology from adolescence through to young adulthood. Parallel-process latent growth curve modeling was used to examine the trajectories of alcohol misuse and symptoms of problem gambling over time. Data were from a sample of adolescents recruited for the Leisure, Lifestyle, and Lifecycle Project in Alberta, Canada (n = 436), which included 4 assessments over 5 years. There was an average decline in problem gambling symptoms followed by an accelerating upward trend as the sample reached the legal age to gamble. There was significant variation in the rate of change in problem gambling symptoms over time; not all respondents followed the same trajectory. There was an average increase in alcohol misuse over time, with significant variability in baseline levels of use and the rate of change over time. The unconditional parallel process model indicated that higher baseline levels of alcohol misuse were associated with higher baseline levels of problem gambling symptoms. In addition, higher baseline levels of alcohol misuse were associated with steeper declines in problem gambling symptoms over time. However, these between-process correlations did not retain significance when covariates were added to the model, indicating that one behavior was not a risk factor for the other. The lack of mutual influence in the problem gambling symptomatology and alcohol misuse processes suggest that there are common risk factors underlying these two behaviors, supporting the notion of a syndrome model of addiction. (PsycINFO Database Record (c) 2017 APA, all rights reserved).

Attention skills and executive functioning in children with Noonan syndrome and their unaffected siblings.

PubMed

Pierpont, Elizabeth I; Tworog-Dube, Erica; Roberts, Amy E

2015-04-01

Emerging research indicates that gene mutations within the RAS-MAPK signaling cascade, which cause Noonan syndrome and related disorders, affect neurophysiologic activity in brain regions underlying attention and executive functions. The present study examined whether children with Noonan syndrome are at heightened risk for symptoms of attention-deficit-hyperactivity disorder (ADHD) and executive dysfunction relative to an unaffected sibling comparison group, and investigated three key aspects of behavioral attention: auditory attention, sustained attention, and response inhibition. Children and adolescents with Noonan syndrome (n=32, 17 males, 15 females, mean age 11y 3mo, SD 3y) and their unaffected siblings (n=16, eight males, eight females, mean age 11y, SD 3y 6mo) were administered standardized tests of intellectual functioning and clinic-based measures of behavioral attention. Parent ratings of ADHD symptoms, executive functioning, and behavior were also obtained. Children with Noonan syndrome demonstrated higher rates of past ADHD diagnosis, as well as reduced performance compared with unaffected siblings on behavioral attention measures. Parent-rated functional impairments in attention, social skills, working memory, and self-monitoring were more prevalent in the Noonan syndrome group. The relationship between attention regulation skills (sustained attention and inhibitory control) and intellectual test performance was significantly stronger in the Noonan syndrome group than the comparison group. Clinical screening/evaluation for ADHD and executive dysfunction in Noonan syndrome is recommended to facilitate appropriate intervention and to address functional impact on daily life activities. © 2014 Mac Keith Press.

Application of the transtheoretical model: exercise behavior in Korean adults with metabolic syndrome.

PubMed

Kim, Chun-Ja; Kim, Bom-Taeck; Chae, Sun-Mi

2010-01-01

Although regular exercise has been recommended to reduce the risk of cardiovascular disease (CVD) among people with metabolic syndrome, little information is available about psychobehavioral strategies in this population. The purpose of this study was to identify the stages, processes of change, decisional balance, and self-efficacy of exercise behavior and to determine the significant predictors explaining regular exercise behavior in adults with metabolic syndrome. This descriptive, cross-sectional survey design enrolled a convenience sample of 210 people with metabolic syndrome at a university hospital in South Korea. Descriptive statistics were used to analyze demographic characteristics, metabolic syndrome risk factors, and transtheoretical model-related variables. A multivariate logistic regression analysis was used to determine the most important predictors of regular exercise stages. Action and maintenance stages comprised 51.9% of regular exercise stages, whereas 48.1% of non-regular exercise stages were precontemplation, contemplation, and preparation stages. Adults with regular exercise stages displayed increased high-density lipoprotein cholesterol level, were more likely to use consciousness raising, self-reevaluation, and self-liberation strategies, and were less likely to evaluate the merits/disadvantages of exercise, compared with those in non-regular exercise stages. In this study of regular exercise behavior and transtheoretical model-related variables, consciousness raising, self-reevaluation, and self-liberation were associated with a positive effect on regular exercise behavior in adults with metabolic syndrome. Our findings could be used to develop strategies and interventions to maintain regular exercise behavior directed at Korean adults with metabolic syndrome to reduce CVD risk. Further prospective intervention studies are needed to investigate the effect of regular exercise program on the prevention and/or reduction of CVD risk among this population. Health care providers, especially nurses, are optimally positioned to help their clients initiate and maintain regular exercise behavior in clinical and community settings.

Medical Issues among Children and Teenagers with Down Syndrome in Hong Kong

ERIC Educational Resources Information Center

Yam, Winnie Ka-Ling; Tse, Philomena Wan Ting; Yu, Chak Man; Chow, Chun Bong; But, Wai Man; Li, Kit Yu; Lee, Lai Ping; Fung, Eva Lai Wah; Mak, Pauline Pui Yee; Lau, Joseph Tak Fai

2008-01-01

We examined the prevalence of medical problems in children and teenagers with Down syndrome in Hong Kong. Methods: Children with Down syndrome receiving care from seven regional hospitals were included and their hospital records were reviewed. A total of 407 patients, aged between 0.06 and 17.16 years were included. Cardiovascular problems were…

Mental Health Problems in Adults with Down Syndrome and Their Association with Life Circumstances

ERIC Educational Resources Information Center

Mallardo, Mariarosa; Cuskelly, Monica; White, Paul; Jobling, Anne

2014-01-01

This study focused on current life circumstances, previous life events, and engagement with productive and enjoyable activities. It examined the association of these variables with mental health problems and mood in a cohort of young adults with Down syndrome. Participants were 49 adults with Down syndrome (age range 20-31 years) and their…

The Architecture of the Pollen Hoarding Syndrome in Honey Bees: Implications for Understanding Social Evolution, Behavioral Syndromes, and Selective Breeding

PubMed Central

Rueppell, Olav

2014-01-01

Social evolution has influenced every aspect of contemporary honey bee biology, but the details are difficult to reconstruct. The reproductive ground plan hypothesis of social evolution proposes that central regulators of the gonotropic cycle of solitary insects have been coopted to coordinate social complexity in honey bees, such as the division of labor among workers. The predicted trait associations between reproductive physiology and social behavior have been identified in the context of the pollen hoarding syndrome, a larger suite of interrelated traits. The genetic architecture of this syndrome is characterized by a partially overlapping genetic architecture with several consistent, pleiotropic QTL. Despite these central QTL and an integrated hormonal regulation, separate aspects of the pollen hoarding syndrome may evolve independently due to peripheral QTL and additionally segregating genetic variance. The characterization of the pollen hoarding syndrome has also demonstrated that this syndrome involves many non-behavioral traits, which may be the case for numerous “behavioral” syndromes. Furthermore, the genetic architecture of the pollen hoarding syndrome has implications for breeding programs for improving honey health and other desirable traits: If these traits are comparable to the pollen hoarding syndrome, consistent pleiotropic QTL will enable marker assisted selection, while sufficient additional genetic variation may permit the dissociation of trade-offs for efficient multiple trait selection. PMID:25506100

Growth Problems

MedlinePlus

... hormones needed to grow and develop. For example, Turner syndrome is a genetic condition (due to a problem ... a missing or abnormal X chromosome. Girls with Turner syndrome tend to be short and don't usually ...

Brown Syndrome

MedlinePlus

... Does Brown syndrome cause eye problems besides abnormal eye movements? In the more severely affected cases of Brown ... acquired and congenital cases. In congenital cases, the eye movement problem is usually constant and unlikely to resolve ...

Social Behavior and Characteristics of Autism Spectrum Disorder in Angelman, Cornelia de Lange, and Cri du Chat Syndromes

ERIC Educational Resources Information Center

Moss, Joanna; Howlin, Patricia; Hastings, Richard Patrick; Beaumont, Sarah; Griffith, Gemma M.; Petty, Jane; Tunnicliffe, Penny; Yates, Rachel; Villa, Darrelle; Oliver, Chris

2013-01-01

We evaluated autism spectrum disorder (ASD) characteristics and social behavior in Angelman (AS; "n" ?=? 19; mean age ?=?10.35 years), Cornelia de Lange (CdLS; "n" ?=? 15; mean age ?=?12.40 years), and Cri du Chat (CdCS, also known as 5 p-syndrome; "n" ?=? 19; mean age ?=? 8.80 years) syndromes. The proportion of…

Grouping and trajectories of the neuropsychiatric symptoms in patients with Alzheimer's disease, part I: symptom clusters.

PubMed

Garre-Olmo, Josep; López-Pousa, Secundino; Vilalta-Franch, Joan; de Gracia Blanco, Manuel; Vilarrasa, Antoni Bulbena

2010-01-01

Behavioral and psychological symptoms of dementia (BPSD) are frequently observed in Alzheimer's disease (AD) and affect more than 80% of patients over the course of AD. The goal of this study was to establish a model for grouping the symptoms of BPSD into clinical syndromes. Over a 24-month period, an observational study was conducted using a population of ambulatory patients with AD of mild to moderate severity. The Neuropsychiatric Inventory (NPI) was administered to the patients' caregivers every 6 months. BPSD were grouped using exploratory factor analysis (EFA) and confirmatory factor analysis (CFA) of the NPI scores of each assessment. The sample population consisted of 491 patients (70.9% women) with an average age of 75.2 years (SD=6.6). The five EFA suggested that there was a stable three-factor structure. According to the results of the EFA, three models of symptom grouping were adjusted using CFA methodology. The CFA model that satisfactorily grouped the NPI scores into three factors included a psychotic syndrome (hallucinations, delusions), an affective syndrome (depression, anxiety, irritability, agitation) and a behavior syndrome (euphoria, disinhibition, apathy, aberrant motor behavior). Based on our findings, we propose a model for grouping the BDSD in which there are core nuclear syndromes (psychotic and affective) as well as an unspecified behavior syndrome comprising satellite symptoms that may be related to the presence of the nuclear syndromes.

Aging and Intellectual Disability: Insights from Mouse Models of Down Syndrome

ERIC Educational Resources Information Center

Ruparelia, Aarti; Pearn, Matthew L.; Mobley, William C.

2013-01-01

Down syndrome (DS) is one of many causes of intellectual disability (ID), others including but not limited to, fetal alcohol syndrome, Fragile X syndrome, Rett syndrome, Williams syndrome, hypoxia, and infection. Down syndrome is characterized by a number of neurobiological problems resulting in learning and memory deficits and early onset…

Marfan Syndrome

MedlinePlus

Marfan syndrome is a disorder that affects connective tissue. Connective tissues are proteins that support skin, bones, blood vessels, ... A problem with the fibrillin gene causes Marfan syndrome. Marfan syndrome can be mild to severe, and ...

Learning about Fragile X Syndrome

MedlinePlus

... Physical problems that have been seen include eye, orthopedic, heart and skin problems. Girls who have the ... to their sons. Top of page NHGRI Clinical Research on Fragile X Syndrome Currently, NHGRI is not ...

Excessive masturbation after epilepsy surgery.

PubMed

Ozmen, Mine; Erdogan, Ayten; Duvenci, Sirin; Ozyurt, Emin; Ozkara, Cigdem

2004-02-01

Sexual behavior changes as well as depression, anxiety, and organic mood/personality disorders have been reported in temporal lobe epilepsy (TLE) patients before and after epilepsy surgery. The authors describe a 14-year-old girl with symptoms of excessive masturbation in inappropriate places, social withdrawal, irritability, aggressive behavior, and crying spells after selective amygdalohippocampectomy for medically intractable TLE with hippocampal sclerosis. Since the family members felt extremely embarrassed, they were upset and angry with the patient which, in turn, increased her depressive symptoms. Both her excessive masturbation behavior and depressive symptoms remitted within 2 months of psychoeducative intervention and treatment with citalopram 20mg/day. Excessive masturbation is proposed to be related to the psychosocial changes due to seizure-free status after surgery as well as other possible mechanisms such as Kluver-Bucy syndrome features and neurophysiologic changes associated with the cessation of epileptic discharges. This case demonstrates that psychiatric problems and sexual changes encountered after epilepsy surgery are possibly multifactorial and in adolescence hypersexuality may be manifested as excessive masturbation behavior.

Guide to Understanding Moebius Syndrome

MedlinePlus

... due to upper body weakness • Strabismus (crossed eyes) • Dry eyes and irritability • Dental problems • High palate • Cleft palate • Hand and feet problems including club foot and missing or fused fingers (syndactyly) • Hearing problems • Poland’s syndrome (chest wall and upper limb anomalies) Although they ...

Brief Report: Parental Descriptions of Sleep Problems in Children with Autism, Down Syndrome, and Prader-Willi Syndrome

ERIC Educational Resources Information Center

Cotton, Sue; Richdale, Amanda

2006-01-01

Children with an intellectual disability (ID) are at high risk of developing sleep problems. The extent to which the prevalence and nature of sleep problems in these children is dependent on the disorder underlying their intellectual impairments remains unclear. This study examined and compared parental descriptions of sleep problems in children…

Successful Treatment of Olfactory Reference Syndrome with Cognitive Behavioral Therapy: A Case Study

ERIC Educational Resources Information Center

Martin-Pichora, Andrea L.; Antony, Martin M.

2011-01-01

Olfactory reference syndrome (ORS) is characterized by a preoccupation with the belief that one's body emits a foul odor. Cognitive behavioral therapy (CBT) was used to treat a woman in her 50s who presented in our outpatient anxiety disorders specialty clinic with ORS, accompanied by embarrassment, shame, distress, avoidance behavior, and social…

The Longitudinal Effects of Parenting on Adaptive Behavior in Children with Fragile X Syndrome

ERIC Educational Resources Information Center

Warren, Steven F.; Brady, Nancy; Fleming, Kandace K.; Hahn, Laura J.

2017-01-01

Several studies have reported declines in adaptive behavior amongst children with fragile X syndrome (FXS) starting in middle childhood. We examined the effects of maternal responsivity on adaptive behavior in 55 children with FXS visited 5-6 times in their homes from early through middle childhood. Our analyses indicated that sustained maternal…

Mental and Behavioral Symptoms of Person's with Asperger's Syndrome: Relationships with Social Isolation and Handicaps

ERIC Educational Resources Information Center

Tani, Masayuki; Kanai, Chieko; Ota, Haruhisa; Yamada, Takashi; Watanabe, Hiromi; Yokoi, Hideki; Takayama, Yuko; Ono, Taisei; Hashimoto, Ryuichiro; Kato, Nobumasa; Iwanami, Akira

2012-01-01

People with Asperger's syndrome (AS) experience mental comorbidities, and behavioral symptoms that can deepen social isolation and handicaps. We compared the frequency of mental and behavioral symptoms, motor abnormality, and life history between adults with AS and those with no mental disorders but with disturbance of social functions and…

Genetics Home Reference: renal coloboma syndrome

MedlinePlus

... the back of the eye ( the retina ). The vision problems caused by these abnormalities can vary depending ... visual problems, while others may have severely impaired vision. Less common features of renal coloboma syndrome include ...

Genetics Home Reference: Sjögren-Larsson syndrome

MedlinePlus

... view the expand/collapse boxes. Description Sjögren-Larsson syndrome is a condition characterized by dry, scaly skin (ichthyosis); neurological problems; and eye problems. These symptoms are apparent by early childhood ...

Nonverbal intelligence in young children with dysregulation: the Generation R Study.

PubMed

Basten, Maartje; van der Ende, Jan; Tiemeier, Henning; Althoff, Robert R; Rijlaarsdam, Jolien; Jaddoe, Vincent W V; Hofman, Albert; Hudziak, James J; Verhulst, Frank C; White, Tonya

2014-11-01

Children meeting the Child Behavior Checklist Dysregulation Profile (CBCL-DP) suffer from high levels of co-occurring internalizing and externalizing problems. Little is known about the cognitive abilities of these children with CBCL-DP. We examined the relationship between CBCL-DP and nonverbal intelligence. Parents of 6,131 children from a population-based birth cohort, aged 5 through 7 years, reported problem behavior on the CBCL/1.5-5. The CBCL-DP was derived using latent profile analysis on the CBCL/1.5-5 syndrome scales. Nonverbal intelligence was assessed using the Snijders Oomen Nonverbal Intelligence Test 2.5-7-Revised. We examined the relationship between CBCL-DP and nonverbal intelligence using linear regression. Analyses were adjusted for parental intelligence, parental psychiatric symptoms, socio-economic status, and perinatal factors. In a subsample with diagnostic interview data, we tested if the results were independent of the presence of attention deficit hyperactivity disorder (ADHD) or autism spectrum disorders (ASD). The results showed that children meeting the CBCL-DP (n = 110, 1.8%) had a 11.0 point lower nonverbal intelligence level than children without problems and 7.2-7.3 points lower nonverbal intelligence level than children meeting other profiles of problem behavior (all p values <0.001). After adjustment for covariates, children with CBCL-DP scored 8.3 points lower than children without problems (p < 0.001). The presence of ADHD or ASD did not account for the lower nonverbal intelligence in children with CBCL-DP. In conclusion, we found that children with CBCL-DP have a considerable lower nonverbal intelligence score. The CBCL-DP and nonverbal intelligence may share a common neurodevelopmental etiology.

Measuring feeding difficulties in toddlers with Down syndrome.

PubMed

van Dijk, Marijn; Lipke-Steenbeek, Wilma

2018-07-01

Early feeding problems occur frequently across the population, but have a higher incidence in children with Down syndrome (DS). Early identification can possibly be improved with the help of a valid screening instrument based on caregiver reports. In a previous study, we investigated the concurrent validity of the Dutch version of the Montreal Children's Hospital Feeding Scale (MCH-FS, SEP in Dutch) in a sample of typically developing toddlers, and we found a correlation between the score on the instrument and observed behavior during a regular meal. The current pilot study was a replication in a sample of children with DS (aged 1; 0-3; 0) and their primary caregivers (n = 32). The results showed that children in the sample did not score higher on the SEP than children in their respective norm groups. In addition, when caregivers reported more symptoms of feeding problems on the SEP, children showed more food refusal and negative affect during the observed meal. This suggests that the screening instrument is particularly associated with negative mealtime interactions. This is in contrast with earlier results, which mainly indicated a relation with eating skills. Copyright © 2018 Elsevier Ltd. All rights reserved.

Attentional Disengagement in Adults with Williams syndrome

PubMed Central

Lense, Miriam D.; Key, Alexandra P.; Dykens, Elisabeth M.

2011-01-01

Williams syndrome (WS) is a neurodevelopmental disorder characterized by a distinctive behavioral and cognitive profile, including widespread problems with attention. However, the specific nature of their attentional difficulties, such as inappropriate attentional allocation and/or poor attentional disengagement abilities, has yet to be elucidated. Furthermore, it is unknown if there is an underlying difficulty with the temporal dynamics of attention in WS or if their attentional difficulties are task-dependent, because previous studies have examined attention in established areas of deficit and atypicality (specifically, visuospatial and face processing). In this study, we examined attentional processing in 14 adults with WS (20-59 years) and 17 typically developing controls (19-39 years) using an attentional blink (AB) paradigm. The AB is the decreased ability to detect a second target when it is presented in close proximity to an initial target. Overall, adults with WS had an AB that was prolonged in duration, but no different in magnitude, compared with typically developing control participants. AB performance was not explained by IQ, working memory, or processing speed in either group. Thus, results suggest that the attention problems in WS are primarily due to general attentional disengagement difficulties rather than inappropriate attentional allocation. PMID:21885176

Persuasive user experiences of a health Behavior Change Support System: A 12-month study for prevention of metabolic syndrome.

PubMed

Karppinen, Pasi; Oinas-Kukkonen, Harri; Alahäivälä, Tuomas; Jokelainen, Terhi; Keränen, Anna-Maria; Salonurmi, Tuire; Savolainen, Markku

2016-12-01

Obesity has become a severe health problem in the world. Even a moderate 5% weight loss can significantly reduce the prevalence of metabolic syndrome, which can be vital for preventing comorbidities caused by the obesity. Health Behavior Change Support Systems (hBCSS) emphasize an autogenous approach, where an individual uses the system to influence one's own attitude or behavior to achieve his or her own goal. Regardless of promising results, such health interventions technology has often been considered merely as a tool for delivering content that has no effect or value of its own. More research on actual system features is required. The objective of this study is to describe how users perceive persuasive software features designed and implemented into a support system. The research medium in this study is a web-based information system designed as a lifestyle intervention for participants who are at risk of developing a metabolic syndrome or who are already suffering from it. The system was designed closely following the principles of the Persuasive Systems Design (PSD) model and the Behavior Change Support Systems (BCSS) framework. A total of 43 system users were interviewed for this study during and after a 52 week intervention period. In addition, the system's login data and subjects' Body Mass Index (BMI) measures were used to interpret the results. This study explains in detail how the users perceived using the system and its persuasive features. Self-monitoring, reminders, and tunneling were perceived as especially beneficial persuasive features. The need for social support appeared to grow along the duration of the intervention. Unobtrusiveness was found to be very important in all stages of the intervention rather than only at the beginning. Persuasive software features have power to affect individuals' health behaviors. Through their systematicity the PSD model and the BCSS framework provide effective support for the design and development of technological health interventions. Designers of such systems may choose, for instance, to implement more self-monitoring tools to help individuals to adjust their personal goals with the system's offerings better. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

Genetics Home Reference: cold-induced sweating syndrome

MedlinePlus

... Health Conditions Cold-induced sweating syndrome Cold-induced sweating syndrome Printable PDF Open All Close All Enable ... view the expand/collapse boxes. Description Cold-induced sweating syndrome is characterized by problems with regulating body ...

Study of Thai Language Oral Reading Problems for Students with Down Syndrome: Grade Range 1

ERIC Educational Resources Information Center

Kaewchote, Nantawan; Chongchaikit, Maturos

2011-01-01

The purpose of the study was to explore the Thai Language Oral Reading Problems of students with Down syndrome, Grade Range1 at Watnonsaparam School, Saraburi Thailand in favor of Web Quest Lessons Development Enhancing Oral Reading Skills of Down syndrome Students. The research instruments were the 2 observation forms on Thai Language Reading…

The brain finger protein gene (ZNF179), a member of the RING finger family, maps within the Smith-Magenis syndrome region at 17p11.2

DOE Office of Scientific and Technical Information (OSTI.GOV)

Kimura, Toshiyuki; Arakawa, Yoshiki; Inazawa, Johji

1997-03-31

Smith-Magenis syndrome (SAIS) is caused by a microdeletion of 17p11.2 and comprises developmental and growth delay, facial abnormalities, unusual behavior and sleep problems. This phenotype may be due to haploinsufficiency of several contiguous genes. The human brain finger protein gene (ZNF179), a member of the RING finger protein family, has been isolated and mapped to l7p11.2. FISH analyses of metaphase or interphase chromosomes of 6 patients with SMS show that ZNF179 was deleted in one of the 2 homologs (17p11.2), indicating a possible association of the defect of this gene with the pathogenesis of SMS. Furthermore, using a prophase FISHmore » ordering system, we sublocalized ZNF179 proximally to LLGL which lies on the critical region for SMS. 27 refs., 2 figs.« less

Vulvar vestibulitis syndrome and vaginismus. A case report.

PubMed

Wijma, B; Jansson, M; Nilsson, S; Hallböök, O; Wijma, K

2000-03-01

Recent reports have argued for a revision of the criteria used for the establishment of a diagnosis of vulvar vestibulitis syndrome (VVS). On theoretical grounds it might be hypothesized that women with VVS also suffer from vaginismus. A young woman presented with a history, symptoms and objective findings typical of vaginismus, yet she suffered from continuous, burning pain and itching in the vestibule. Earlier in the course of the problem she had received a diagnosis VVS. The patient was treated with behavioral therapy developed for vaginismus. Notations made during the course of therapy supported the assumption that the pain and itching were conditioned responses to penetration in the same way that a vaginal muscular reflex is. Differential diagnostic difficulties exist in the field of VVS and vaginismus. Psychophysiologic theories are needed as the basis for research to clarify the connections between different diagnostic entities associated with coital burning pain and itching in the vestibule.

[Behavioral characteristics of children with Prader-Willi syndrome in preschool and school age: an exploratory study on ritualistic behavior].

PubMed

Sarimski, Klaus; Ebner, Sarah; Wördemann, Claudia

2012-01-01

Parents of 64 children and youths with Prader-Willi syndrome (PWS) describe their children's behaviour on the "Temperament and Atypical Behavior Scale" (TABS) and the German version of the "Developmental Behavior Checklist" (VFE). In the younger age group, there are no specific behavioural abnormalities which characterize a behavioral phenotype. In the older age group the data reveal elevated levels of abnormal behaviors (communication disturbance, social relations and disruptive behaviors). Parents stress ritualistic behaviors as especially challenging. The results concerning form and age-dependency of abnormal behaviors are discussed in the context of prevention and treatment options.

Daytime napping associated with increased symptom severity in fibromyalgia syndrome.

PubMed

Theadom, Alice; Cropley, Mark; Kantermann, Thomas

2015-02-07

Previous qualitative research has revealed that people with fibromyalgia use daytime napping as a coping strategy for managing symptoms against clinical advice. Yet there is no evidence to suggest whether daytime napping is beneficial or detrimental for people with fibromyalgia. The purpose of this study was to explore how people use daytime naps and to determine the links between daytime napping and symptom severity in fibromyalgia syndrome. A community based sample of 1044 adults who had been diagnosed with fibromyalgia syndrome by a clinician completed an online questionnaire. Associations between napping behavior, sleep quality and fibromyalgia symptoms were explored using Spearman correlations, with possible predictors of napping behaviour entered into a logistic regression model. Differences between participants who napped on a daily basis and those who napped less regularly, as well as nap duration were explored. Daytime napping was significantly associated with increased pain, depression, anxiety, fatigue, memory difficulties and sleep problems. Sleep problems and fatigue explained the greatest amount of variance in napping behaviour, p < 0.010. Those who engaged in daytime naps for >30 minutes had higher memory difficulties (t = -3.45) and levels of depression (t = -2.50) than those who napped for shorter periods (<30 mins) (p < 0.010). Frequent use and longer duration of daytime napping was linked with greater symptom severity in people with fibromyalgia. Given the common use of daytime napping in people with fibromyalgia evidence based guidelines on the use of daytime napping in people with chronic pain are urgently needed.

Respiratory problems in children with Down syndrome.

PubMed

Pandit, Chetan; Fitzgerald, Dominic A

2012-03-01

Down syndrome is associated with a significant health burden, which is particularly apparent in young children who will frequently present with cardiac and respiratory problems. Respiratory presentations include problems related to structural abnormalities of the airways and lungs, glue ears, recurrent lower respiratory tract infections and obstructive sleep apnoea. These conditions are readily identifiable and able to be treated. An awareness of the breadth of respiratory problems and a plan to monitor patients with Down syndrome for their development has the potential to improve outcomes. © 2011 The Authors. Journal of Paediatrics and Child Health © 2011 Paediatrics and Child Health Division (Royal Australasian College of Physicians).

Research Thinking of Low-intensity laser For the Treatment of Menopausal Syndrome

NASA Astrophysics Data System (ADS)

Chen, G. Z.; Xu, Y. X.; Wang, X. Y.; Liu, S. H.; Li, L. J.

2011-02-01

Female climacteric syndrome is a clinical syndrome due to autonomic nerve dysfunction occurring in women during climacteric period, which may affect their physical and mental health. Therefore, how to pass climacteric period for women without any problems, avoid or reduce the occurrence of climacteric syndrome, prevent geriatric diseases and improve life quality is a key issue now for great attention. Looking for a convenient, effective, and safer method without toxic-side effects to control the disease is a modern medical problem. By analyzing the relationship between laser technology and traditional acupuncture and moxibustion, the advantage and the existing problems on acupuncture and moxibustion for the treatment of menopausal syndrome, the application of laser methods for the mechanism research on TCM diagnosis and treatment of menopausal syndrome was discussed. It's pointed out that the laser acupuncture is safe and effective to treat menopausal syndrome. Breakthrough will be achieved from the research of the selection of the acupoint prescription and mechanism of Acupuncture and Moxibustion for the treatment of menopausal syndrome by utilizing the advantage of interdisciplinary intersection. Laser technology will make the development of acupuncture and moxibustion science possess an unprecedented field.

Prader-Willi Syndrome

ERIC Educational Resources Information Center

Kundert, Deborah King

2008-01-01

Although known for its distinctive food-related behaviors, Prader-Willi syndrome is a multisystem disorder with genetic, developmental, and behavioral features. Two separate and distinct eating disorders are noted: initial feeding difficulties and failure to thrive, and later overeating. Additional outcomes observed with this disorder include…

Mathematics learning disabilities in girls with fragile X or Turner syndrome during late elementary school.

PubMed

Murphy, Melissa M; Mazzocco, Michèle M M

2008-01-01

The present study focuses on math and related skills among 32 girls with fragile X (n = 14) or Turner (n = 18) syndrome during late elementary school. Performance in each syndrome group was assessed relative to Full Scale IQ-matched comparison groups of girls from the general population (n = 32 and n = 89 for fragile X syndrome and Turner syndrome, respectively). Differences between girls with fragile X and their comparison group emerged on untimed arithmetic calculations, mastery of counting skills, and arithmetic problem verification accuracy. Relative to girls in the comparison group, girls with Turner syndrome did not differ on untimed arithmetic calculations or problem verification accuracy, but they had limited mastery of counting skills and longer response times to complete the problem verification task. Girls with fragile X or Turner syndrome also differed from their respective comparison groups on math-related abilities, including visual-spatial, working memory, and reading skills, and the associations between math and those related skills. Together, these findings support the notion that difficulty with math and related skills among girls with fragile X or Turner syndrome continues into late elementary school and that the profile of math and related skill difficulty distinguishes the two syndrome groups from each other.

Young People with Intellectual Disability Transitioning to Adulthood: Do Behaviour Trajectories Differ in Those with and without Down Syndrome?

PubMed Central

Foley, Kitty-Rose; Taffe, John; Bourke, Jenny; Einfeld, Stewart L.; Tonge, Bruce J.; Trollor, Julian; Leonard, Helen

2016-01-01

Background Young people with intellectual disability exhibit substantial and persistent problem behaviours compared with their non-disabled peers. The aim of this study was to compare changes in emotional and behavioural problems for young people with intellectual disability with and without Down syndrome as they transition into adulthood in two different Australian cohorts. Methods Emotional and behavioural problems were measured over three time points using the Developmental Behaviour Checklist (DBC) for those with Down syndrome (n = 323 at wave one) and compared to those with intellectual disability of another cause (n = 466 at wave one). Outcome scores were modelled using random effects regression as linear functions of age, Down syndrome status, ability to speak and gender. Results DBC scores of those with Down syndrome were lower than those of people without Down syndrome indicating fewer behavioural problems on all scales except communication disturbance. For both groups disruptive, communication disturbance, anxiety and self-absorbed DBC subscales all declined on average over time. There were two important differences between changes in behaviours for these two cohorts. Depressive symptoms did not significantly decline for those with Down syndrome compared to those without Down syndrome. The trajectory of the social relating behaviours subscale differed between these two cohorts, where those with Down syndrome remained relatively steady and, for those with intellectual disability from another cause, the behaviours increased over time. Conclusions These results have implications for needed supports and opportunities for engagement in society to buffer against these emotional and behavioural challenges. PMID:27391326

Parental Report of Sleep Problems in Down Syndrome

ERIC Educational Resources Information Center

Breslin, J. H.; Edgin, J. O.; Bootzin, R. R.; Goodwin, J. L.; Nadel, L.

2011-01-01

Background: Children with Down syndrome (DS) suffer from sleep problems, including sleep maintenance problems, as well as snoring, and other symptoms of disordered breathing. To examine sleep in DS, we gave parents a questionnaire assessing their child's sleep. Materials and methods: The parents of 35 children with DS (mean age = 12.65 years,…

Delayed Puberty

MedlinePlus

... which make sex hormones • Genetic problems such as Turner syndrome in girls or Klinefelter syndrome in boys • Some ... FS_MH_Klinefelter_Syndrome_EN-6-12.pdf ——Turner Syndrome: www.hormone.org/Resources/upload/ FS_GD_Turner_ ...

Oral Health Characteristics of Preschool Children with Autistic Syndrome Disorder.

PubMed

Sarnat, Haim; Samuel, Eli; Ashkenazi-Alfasi, Naomi; Peretz, Benjamin

2016-01-01

to assess the dental status of young children with Autistic Syndrome Disorder (ASD) in order to allow better understanding of the dentist's role in treating these children. The ASD group consisted of 47 children diagnosed as ASD from three special kindergartens from three towns in Israel. The control group was 44 normally developed children from 4 kindergartens from 2 neighboring towns. Parents were asked to complete a questionnaire requesting the following: socio-demographic information, general medical condition, dental information (previous visit to a dentist, feeding habits, oral hygiene behavior, oral habits and the Vinland Adaptive Behavioral Scales (VABS). While in the control group there was no report of no brushing at all, among the ASD children 25% did not brush at all. Use of pacifier, fussy eating and eating problems were significantly more prevalent among the ASD group. In addition, the ASD children significantly preferred more salty, spicy and sweet foods than the control. There were more cariesfree children among the ASD group. In the ASD group, the ability to perform everyday life functions was slightly more than half of what is expected for age. autistic children have a relative age of one half, meaning they function at half the level of normally developed children at their chronological age, more eating problems and more persistent oral habits yet no correlation to dental health could be shown. Caries experience of autistic children was lower than in the control group, maintaining good oral hygiene is difficult for autistic children yet their gingival health was found to be good.

Children with problematic sexualized behaviors in the child welfare system.

PubMed

Baker, Amy J L; Gries, Len; Schneiderman, Mel; Parker, Rob; Archer, Marc; Friedrich, Bill

2008-01-01

This study assessed the utility of the Child Sexual Behavior Inventory (CSBI) in a child welfare sample. In this study, 97 children from ages 10 to 12 from either foster boarding homes or a residential treatment center participated. Researchers interviewed foster parents or primary therapists about children's sexual behavior, traumatic events, clinical symptoms, and their attitudes toward the child. Findings revealed that problematic sexualized behaviors were more prevalent in the residential treatment center (RTC) sample than they were in a normative sample. The pattern of associations between sexual behavior problems, traumatic events, and clinical syndromes in both the RTC and the foster boarding home (FBH) samples was similar to what has been found in samples in which biological custodial parents were the respondents. Analyses comparing youth who met the criterion for having problematic sexualized behaviors and youth who did not meet the criterion revealed that the two groups differed on clinical symptoms, prior traumatic events, and negative reports by caregivers. Results confirm the utility of the CSBI measure for this population and highlight several important clinical and programmatic concerns for addressing problematic sexual behavior in children in the child welfare system.

Tourette syndrome: the self under siege.

PubMed

Leckman, James F; Bloch, Michael H; Scahill, Lawrence; King, Robert A

2006-08-01

Tourette syndrome is a neurodevelopmental disorder characterized by motor and vocal tics--rapid, repetitive, stereotyped movements or vocalizations. Tourette syndrome typically has a prepubertal onset, and boys are more commonly affected than girls. Symptoms usually begin with transient bouts of simple motor tics. By age 10 years, most children are aware of nearly irresistible somatosensory urges that precede the tics. These urges likely reflect a defect in sensorimotor gating because they intrude into the child's conscious awareness and become a source of distraction and distress. A momentary sense of relief typically follows the completion of a tic. Over the course of hours, tics occur in bouts, with a regular intertic interval. Tics increase during periods of emotional excitement and fatigue. Tics can become "complex" in nature and appear to be purposeful. Tics can be willfully suppressed for brief intervals and can be evoked by the mere mention of them. Tics typically diminish during periods of goal-directed behavior, especially those that involve both heightened attention and fine motor or vocal control, as occur in musical and athletic performances. Over the course of months, tics wax and wane. New tics appear, often in response to new sources of somatosensory irritation, such as the appearance of a persistent vocal tic (a cough) following a cold. Over the course of years, tic severity typically peaks between 8 and 12 years of age. By the end of the second decade of life, many individuals are virtually tic free. Less than 20% of cases continue to experience clinically impairing tics as adults. Tics rarely occur in isolation, and other coexisting conditions--such as behavioral disinhibition, hypersensitivity to a broad range of sensory stimuli, problems with visual motor integration, procedural learning difficulties, attention-deficit hyperactivity disorder (ADHD), obsessive-compulsive disorder, depression, anxiety, and emotional instability--are often a greater source of impairment than the tics themselves. Emerging behavioral treatments of Tourette syndrome are based in part on an understanding of the moment-to-moment experience of somatosensory urges and motor response. With identification of specific genes of major effect and advances in our understanding of the neural circuitry of sensorimotor gating, habit formation, and procedural memory--together with insights from postmortem brain studies, in vivo brain imaging, and electrophysiologic recordings--we might be on the threshold of a deeper understanding of the phenomenology and natural history of Tourette syndrome.

Prader-Willi Syndrome: Parent Perceptions of School, Professional, Social, and Informational Support, and Relations between Support, Child Behavior, and Stress

ERIC Educational Resources Information Center

Lowe-Greenlee, Barbara

2010-01-01

Prader-Willi syndrome (PWS) is a rare genetic disorder that adversely impacts child development and health conditions, and is often associated with significant behavioral challenges. In particular, children with PWS typically exhibit extremely high levels of maladaptive behavior (e.g., excessive food seeking, hording, and binging; temper tantrums;…

Less Structured Movement Patterns Predict Severity of Positive Syndrome, Excitement, and Disorganization

PubMed Central

Walther, Sebastian

2014-01-01

Disorganized behavior is a key symptom of schizophrenia. The objective assessment of disorganized behavior is particularly challenging. Actigraphy has enabled the objective assessment of motor behavior in various settings. Reduced motor activity was associated with negative syndrome scores, but simple motor activity analyses were not informative on other symptom dimensions. The analysis of movement patterns, however, could be more informative for assessing schizophrenia symptom dimensions. Here, we use time series analyses on actigraphic data of 100 schizophrenia spectrum disorder patients. Actigraphy recording intervals were set at 2 s. Data from 2 defined 60-min periods were analyzed, and partial autocorrelations of the actigraphy time series indicated predictability of movements in each individual. Increased positive syndrome scores were associated with reduced predictability of movements but not with the overall amount of movement. Negative syndrome scores were associated with low activity levels but unrelated with predictability of movement. The factors disorganization and excitement were related to movement predictability but emotional distress was not. Thus, the predictability of objectively assessed motor behavior may be a marker of positive symptoms and disorganized behavior. This behavior could become relevant for translational research. PMID:23502433

Considerations for Serving Adolescents with Usher's Syndrome.

ERIC Educational Resources Information Center

Fillman, Robyn D.; And Others

1989-01-01

Usher's syndrome is described, with emphasis on the visual symptomatology characteristic of retinitis pigmentosa. Also discussed are the services needed by individuals with Usher's Syndrome, the syndrome's psychosocial aspects, ways to prevent self-defeating behavior, orientation and mobility, and classroom adaptations. (JDD)

Prevalence and onset of comorbidities in the CDKL5 disorder differ from Rett syndrome.

PubMed

Mangatt, Meghana; Wong, Kingsley; Anderson, Barbara; Epstein, Amy; Hodgetts, Stuart; Leonard, Helen; Downs, Jenny

2016-04-14

Initially described as an early onset seizure variant of Rett syndrome, the CDKL5 disorder is now considered as an independent entity. However, little is currently known about the full spectrum of comorbidities that affect these patients and available literature is limited to small case series. This study aimed to use a large international sample to examine the prevalence in this disorder of comorbidities of epilepsy, gastrointestinal problems including feeding difficulties, sleep and respiratory problems and scoliosis and their relationships with age and genotype. Prevalence and onset were also compared with those occurring in Rett syndrome. Data for the CDKL5 disorder and Rett syndrome were sourced from the International CDKL5 Disorder Database (ICDD), InterRett and the Australian Rett syndrome Database (ARSD). Logistic regression (multivariate and univariate) was used to analyse the relationships between age group, mutation type and the prevalence of various comorbidities. Binary longitudinal data from the ARSD and the equivalent cross-sectional data from ICDD were examined using generalized linear models with generalized estimating equations. The Kaplan-Meier method was used to estimate the failure function for the two disorders and the log-rank test was used to compare the two functions. The likelihood of experiencing epilepsy, GI problems, respiratory problems, and scoliosis in the CDKL5 disorder increased with age and males were more vulnerable to respiratory and sleep problems than females. We did not identify any statistically significant relationships between mutation group and prevalence of comorbidities. Epilepsy, GI problems and sleep abnormalities were more common in the CDKL5 disorder than in Rett syndrome whilst scoliosis and respiratory problems were less prevalent. This study captured a much clearer picture of the CDKL5 disorder than previously possible using the largest sample available to date. There were differences in the presentation of clinical features occurring in the CDKL5 disorder and in Rett syndrome, reinforcing the concept that CDKL5 is an independent disorder with its own distinctive characteristics.

Assessing the feasibility, acceptability, and potential effectiveness of a behavioral-automaticity focused lifestyle intervention for African Americans with metabolic syndrome: The Pick two to Stick to protocol.

PubMed

Fritz, Heather; Brody, Aaron; Levy, Philip

2017-09-01

Metabolic syndrome (MetS) significantly increases the risk of developing diabetes and cardiovascular disease. Being physically active and eating a healthy diet can reduce MetS risk factors. Too frequently, however, studies report that the effects of interventions targeting those factors are not maintained once interventions are withdrawn. A potential solution to the problem is targeting behavioral automaticity (habit-development) to aid in initiation and maintenance of health-behavior changes. The Pick two to Stick To (P2S2), is an 8-week, theory-based hybrid (face-to-face/telecoaching) habit focused lifestyle intervention designed to increase healthful physical activity and dietary behavioral automaticity. The purpose of this article is to describe the rationale and protocol for evaluating the P2S2 program's feasibility, acceptability and potential effectiveness. Using a prospective, non-comparative design, the P2S2 program will be implemented by trained occupational therapy 'coaches' to 40 African Americans aged 40 and above with MetS recruited from the emergency department. Semi-structured interviews with participants, bi-weekly research meetings with study staff, and observations of intervention delivery will provide data for a process evaluation. Estimates of effectiveness include weight, blood pressure, waist circumference, BMI, and behavioral automaticity measures that will be collected at baseline and week 20. The P2S2 program could facilitate the development of healthful dietary and physical activity habits in an underserved population. Whether interventions aimed at changing habits can feasibly influence this automaticity, particularly for high-risk, low resource communities where other barriers exist, is not known. This pilot study, therefore, will fill an important gap, providing insight to inform subsequent trials.

Treatment of tics and tourette syndrome.

PubMed

Singer, Harvey S

2010-11-01

Tics come in a variety of types and frequencies; have a waxing and waning course; are exacerbated by stress, anxiety, and fatigue; and often resolve or improve in the teenage or early adult years. Tourette syndrome requires the presence of chronic, fluctuating motor and phonic tics. In addition to tics, individuals with Tourette syndrome often have a variety of comorbid conditions such as attention deficit hyperactivity disorder (ADHD), obsessive-compulsive disorder, depression and anxiety, episodic outbursts, and academic difficulties. These conditions often are a greater source of difficulty than the tics themselves. All patients with tics should be evaluated to assure proper diagnosis and to identify any associated psychopathology or academic difficulty. The treatment of tics begins with education of the patient and family, including discussions about the fundamentals of tics: their characteristics, etiology, outcomes, and available treatments. Therapy should be individualized based on the extent of impairment, available support, ability to cope, and the presence of other problems. Indications for the treatment of tics include psychosocial problems (loss of self-esteem, comments from peers, excessive worries about tics, diminished participation in activities), functional difficulties, classroom disruption, and physical discomfort. A variety of behavioral approaches can be used. Recent studies have emphasized the value of comprehensive behavioral intervention for tics (CBIT). Because habit reversal is the major component of CBIT, a cooperative patient, the presence of a premonitory urge, and a committed family are essential ingredients for success. If tic-suppressing medication is required, a two-tier approach and monotherapy are recommended. First-tier medications, notably the α-adrenergic agonists, are recommended for individuals with milder tics, especially persons with both tics and ADHD. Second-tier medications include various typical and atypical neuroleptics. Their sequence of prescription is often based on physician experience; I favor pimozide and fluphenazine. Atypical antipsychotics, such as risperidone and aripiprazole, have some advantages based on their side-effect profile and are particularly beneficial in individuals with significant co-existing behavioral issues. As will become readily apparent, however, few medications have been adequately assessed. Deep brain stimulation is an emerging therapy, but further data are required to optimize the location of electrode placement and stimulation and to determine precise indications for its implementation. Stimulant medication is effective in treating ADHD in children with tics; studies reducing concerns about its use are discussed.

Behavioural profile and maternal stress in Greek young children with Williams syndrome.

PubMed

Papaeliou, C; Polemikos, N; Fryssira, E; Kodakos, A; Kaila, M; Yiota, X; Benaveli, E; Michaelides, C; Stroggilos, V; Vrettopoulou, M

2012-11-01

Williams syndrome (WS) is a genetic disorder causing intellectual disability. Children with WS often exhibit various kinds of maladaptive behaviours that affect their social functioning. In order to determine whether these behaviours are syndrome-specific, it would be necessary to compare children with WS with children with other syndromes as well as to provide data on the socio-emotional profile in WS from a variety of cultures. The present study investigated the behavioural profile and its relation to maternal stress in Greek young children with WS in comparison with young children with Down syndrome and typically developing (TD) children. Participants were 60 mothers, 20 in each syndrome group and 20 in the control group. The three groups were matched for mental age. The behavioural profile of the participants was investigated through the Child Behaviour Checklist (1.5-5 years) and maternal stress through the Parental Stress Index. In accordance with studies in other cultures, it was found that young children with WS received significantly higher rates in emotional problems and anxiety/depression, compared with both children with Down syndrome and TD children. Moreover, mothers of children with WS reported significantly higher scores in the Total Stress index compared with mothers of TD children. However, in contrast with previous studies, only 25% of children with WS fell into the clinical range in the total Child Behavior Checklist score. The consistency of the socio-emotional characteristics of children with WS across cultures and developmental stages implies a strong influence of the genetic phenotype. However, Greek mothers avoided to characterize these behaviours as pathological. Implications of these findings for clinical practice are also discussed. © 2011 Blackwell Publishing Ltd.

Social anxiety in Cornelia de Lange syndrome.

PubMed

Richards, Caroline; Moss, Jo; O'Farrell, Laura; Kaur, Gurmeash; Oliver, Chris

2009-08-01

In this study we assessed the behavioral presentation of social anxiety in Cornelia de Lange syndrome (CdLS) using a contrast group of Cri du Chat syndrome (CdCS). Behaviors indicative of social anxiety were recorded in twelve children with CdLS (mean age = 11.00; SD = 5.15) and twelve children with CdCS (8.20; SD = 2.86) during social interaction. Lag sequential analysis revealed that participants with CdLS were significantly more likely to evidence behavior indicative of anxiety in close temporal proximity to the point at which they maintained eye contact or spoke. Individuals with CdLS demonstrate a heightened probability of anxiety related behavior during social interaction but only at the point at which social demand is high.

What Are the Symptoms of Turner Syndrome?

MedlinePlus

... Pinterest Email Print What are the symptoms of Turner syndrome? Turner syndrome causes a variety of symptoms in girls and ... some people, symptoms are mild, but for others, Turner syndrome can cause serious health problems. In general, women ...

Individual Differences and Short-Term Military Factors Associated With Unhealthy Weight Control Behaviors Among Active Duty and Reserve Army Soldiers

DTIC Science & Technology

2009-03-01

syndrome eating disorders (Eisenberg & Neumark-Sztainer, 2008; Neumark-Sztainer et al., 2002; Rukavina & Pokrajac-Bulian, 2006). Although the...risk for developing full- syndrome eating disorders. Repeated periods of extreme weight loss behavior may reinforce that purging, fasting, or excessive...exercise are “good” ways to lose weight. In fact, many individuals with full syndrome eating disorders start by engaging in repeated periods of

TARGETED TREATMENTS IN AUTISM AND FRAGILE X SYNDROME

PubMed Central

Gürkan, C. Kağan; Hagerman, Randi J.

2012-01-01

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. PMID:23162607

Mecp2 truncation in male mice promotes affiliative social behavior

PubMed Central

Pearson, B.L.; Defensor, E.B.; Pobbe, R.L.H.; Yamamoto, L.H.L.; Bolivar, V.J.; Blanchard, D.C.; Blanchard, R.J.

2018-01-01

Mouse models of Rett syndrome, with targeted mutations in the Mecp2 gene, show a high degree of phenotypic consistency with the clinical syndrome. In addition to severe and age-specific regression in motor and cognitive abilities, a variety of studies have demonstrated that Mecp2 mutant mice display impaired social behavior. Conversely, other studies indicate complex enhancements of social behavior in Mecp2 mutant mice. Since social behavior is a complicated accumulation of constructs, we performed a series of classic and refined social behavior tasks and revealed a relatively consistent pattern of enhanced pro-social behavior in hypomorphic Mecp2308/Y mutant mice. Analyses of repetitive motor acts, and cognitive stereotypy did not reveal any profound differences due to genotype. Taken together, these results suggest that the mutations associated with Rett syndrome are not necessarily associated with autism-relevant social impairment in mice. However, this gene may be a valuable candidate for revealing basic mechanisms of affiliative behavior. PMID:21909962

Social cognition and self-other distinctions in neuropsychiatry: Insights from schizophrenia and Tourette syndrome.

PubMed

Eddy, Clare M

2018-03-02

Impairments in social cognition may reflect dysfunction of disorder specific or disorder general mechanisms. Although cross-disorder comparison may prove insightful, few studies have compared social cognition in different neuropsychiatric disorders. Parallel investigation of schizophrenia and Tourette syndrome (TS) is encouraged by similarities including the presence of problematic social behavior, echophenomena, emotional dysregulation and dopamine dysfunction. Focusing on tests of social cognition administered in both disorders, this review aims to summarize behavioral, neurophysiological and neuroimaging findings, before exploring how these may contribute to clinical symptoms. Studies investigating social cognition (imitation, emotion recognition, and understanding of beliefs or intentions) in patients with schizophrenia or TS were identified through Web of Science and PubMed searches. Although findings indicate that social cognitive deficits are more apparent in schizophrenia, adults with TS can exhibit similar task performance to patients with paranoia. In both disorders, behavioral and neuroimaging findings raise the possibility of increased internal simulation of others' actions and emotions, in combination with a relative under-application of mentalizing. More specifically, dysfunction in neurobiological substrates such as temporo-parietal junction and inferior frontal gyrus may underlie problems with self-other distinctions in both schizophrenia and TS. Difficulties in distinguishing between actions and mental states linked to the self and other may contribute to a range of psychiatric symptoms, including emotional dysregulation, paranoia, social anhedonia and socially disruptive urges. Comparing different patient populations could therefore reveal common neuro-cognitive risk factors for the development of problematic social behaviors, in addition to markers of resilience, coping strategies and potential neuro-compensation mechanisms. Copyright © 2017 Elsevier Inc. All rights reserved.

Cognitive, sensory, and psychosocial characteristics in patients with Bardet-Biedl syndrome.

PubMed

Brinckman, Danielle D; Keppler-Noreuil, Kim M; Blumhorst, Catherine; Biesecker, Leslie G; Sapp, Julie C; Johnston, Jennifer J; Wiggs, Edythe A

2013-12-01

Forty-two patients with a clinical diagnosis of Bardet-Biedl syndrome ages 2-61 years were given a neuropsychological test battery to evaluate cognitive, sensory, and behavioral functioning. These tests included the Wechsler scales of intelligence, Rey Auditory Verbal Learning Test, Boston Naming Test, D-KEFS Verbal Fluency Test, D-KEFS Color-Word Interference Test, D-KEFS Sorting Test, Wide Range Achievement Test: Math and Reading Subtests, Purdue Pegboard, The University of Pennsylvania Smell Identification Test, Social Communication Questionnaire, Social Responsiveness Scale, and Behavior Assessment System for Children, Second Edition, Parent Rating Scale. On the age appropriate Wechsler scale, the mean Verbal Comprehension was 81 (n = 36), Working Memory was 81 (n = 36), Perceptual Reasoning was 78 (n = 24) and Full Scale IQ was 75 (n = 26). Memory for a word list (Rey Auditory Verbal Learning Test) was in the average range with a mean of 89 (n = 19). Fine motor speed was slow on the Purdue with mean scores 3-4 standard deviations below norms. All subjects were microsmic on the University of Pennsylvania Smell Identification Test. Of these 42 patients, only 6 were able to complete all auditory and visual tests; 52% were unable to complete the visual tests due to impaired vision. A wide range of behavioral issues were endorsed on questionnaires given to parents. Most had social skill deficits but no pattern of either externalizing or internalizing problems. We identify a characteristic neuro-behavioral profile in our cohort comprised of reduced IQ, impaired fine-motor function, and decreased olfaction. © 2013 Wiley Periodicals, Inc.

Fetal Alcohol Syndrome: A Behavioral Teratology.

ERIC Educational Resources Information Center

Kavale, Kenneth A.; Karge, Belinda D.

1986-01-01

The review examines the literature on the behaviorally teratogenic aspects of Fetal Alcohol Syndrome, including: (1) prevalence of alcohol abuse among women, (2) acute and chronic effects of alcohol on the fetus, (3) genetic susceptibility, (4) neuropathology, (5) correlative conditions, and (6) animal studies. (Author/DB)

Emotional and behavioural problems in children with visual impairment, intellectual and multiple disabilities.

PubMed

Alimovic, S

2013-02-01

Children with multiple impairments have more complex developmental problems than children with a single impairment. We compared children, aged 4 to 11 years, with intellectual disability (ID) and visual impairment to children with single ID, single visual impairment and typical development on 'Child Behavior Check List/4-18' (CBCL/4-18), Parent Report. Children with ID and visual impairment had more emotional and behavioural problems than other groups of children: with single impairment and with typical development (F = 23.81; d.f.1/d.f.2 = 3/156; P < 0.001). All children with special needs had more emotional and behavioural problems than children with typical development. The highest difference was found in attention problems syndrome (F = 30.45; d.f.1/d.f.2 = 3/156; P < 0.001) where all groups of children with impairments had more problems. Children with visual impairment, with and without ID, had more somatic complaints than children with normal vision. Intellectual disability had greater influence on prevalence and kind of emotional and behavioural problems in children than visual impairment. © 2012 The Author. Journal of Intellectual Disability Research © 2012 Blackwell Publishing Ltd.

Impact of Sleep on Executive Functioning in School-Age Children with Down Syndrome

ERIC Educational Resources Information Center

Esbensen, A. J.; Hoffman, E. K.

2018-01-01

Background: Sleep problems have an impact on executive functioning in the general population. While children with Down syndrome (DS) are at high risk for sleep problems, the impact of these sleep problems on executive functioning in school-age children with DS is less well documented. Our study examined the relationship between parent-reported and…

Health and Sleep Problems in Cornelia de Lange Syndrome: A Case Control Study

ERIC Educational Resources Information Center

Hall, S. S.; Arron, K.; Sloneem, J.; Oliver, C.

2008-01-01

Background: Self-injury, sleep problems and health problems are commonly reported in Cornelia de Lange Syndrome (CdLS) but there are no comparisons with appropriately matched participants. The relationship between these areas and comparison to a control group is warranted. Method: 54 individuals with CdLS were compared with 46 participants with…

Sensory Impairment and Head Circumference in Fragile X Syndrome, Down Syndrome and Idiopathic Intellectual Disability.

ERIC Educational Resources Information Center

Turk, Jeremy; Patton, Michael

2000-01-01

Eighteen boys with fragile X syndrome were compared with 42 with idiopathic intellectual disability, and 45 with Down syndrome. Boys with Down syndrome had more sensory problems and smaller head circumferences than normal. Head circumferences of boys with fragile X syndrome and with idiopathic intellectual disability were larger than normal.…

Language and Literacy in Turner Syndrome

ERIC Educational Resources Information Center

Murphy, Melissa M.

2009-01-01

Language problems can be associated with specific genetic syndromes, such as Klinefelter syndrome and fragile X syndrome, even in the absence of intellectual and developmental disabilities. Turner syndrome, a relatively common genetic disorder, is caused by the complete or partial absence of 1 of the 2 X chromosomes typically present in women. The…

Update on autism: a review of 1300 reports published in 2008.

PubMed

Hughes, John R

2009-12-01

This publication, by reviewing 1300 studies published on autism in 2008, represents an update on this topic. Results include possible parental influences, maternal conditions, and studies on genes and chromosomes. Possible etiological factors involve the "extreme male brain," defects in the mirror neuron system, vaccines, underconnectivity, disorders of central coherence, and many other more specific etiologies. Assessments or tests for autism are also reviewed. Characteristics of autistic individuals include repetitive behavior, language disorders, sleep disturbances, social problems, joint attention disorders, seizures, allergic reactions, and various behavioral changes. Cognitive changes involve IQ, reasoning, and verbal and language disorders. The savant syndrome is a fascinating phenomenon, at times seen in autistic individuals. Neurophysiological and neuroanatomical changes are also reviewed, as are comorbid conditions. Finally, treatment involves various medications including risperidone, ziprasidone, and antipsychotic drugs, as well as different procedures such as magnetic stimulation, acupuncture, and hyperbaric oxygen therapy. As mentioned in the 2007 survey, nearly every conceivable problem that a child can have may be found in these unfortunate children and nearly every conceivable etiology has been mentioned to account for this serious disorder.

Behavioral Concepts in the Analysis of Chronic Pain Syndromes.

ERIC Educational Resources Information Center

Keefe, Francis J.; Gil, Karen M.

1986-01-01

Reviews behavioral and psychological concepts currently applied to the assessment and treatment of chronic pain syndromes, including operant conditioning and psychophysiologic concepts such as the stress-pain hypothesis, the pain-muscle spasm-pain cycle, and the neuromuscular pain model. Discusses relaxation and biofeedback training and concepts…

Understanding Williams Syndrome: Behavioral Patterns and Interventions.

ERIC Educational Resources Information Center

Semel, Eleanor; Rosner, Sue R.

This guide to Williams syndrome (WS), a congenital disorder characterized by developmental/cognitive limitations but relatively high verbal and social skills, explains the strengths, difficulties and variations found among individuals with the condition and offers guidelines for intervention in the unusual properties of the WS behavioral profile.…

Dual-Task Processing as a Measure of Executive Function: A Comparison between Adults with Williams and Down Syndromes

ERIC Educational Resources Information Center

Kittler, Phyllis M.; Krinsky-McHale, Sharon J.; Devenny, Darlynne A.

2008-01-01

Behavioral phenotypes of individuals with Williams syndrome and individuals with Down syndrome have been contrasted in relation to short-term memory. People with Down syndrome are stronger visuospatially and those with Williams syndrome are stronger verbally. We examined short-term memory, then explored whether dual-task processing further…

[Neuropsychiatric coaching of an adult with Asperger syndrome].

PubMed

Sihvonen, Janne

2011-01-01

Asperger syndrome is a lifelong neurodevelopmental condition. The major features of the syndrome include problems in social interaction and communication, narrow interests and stereotyped behaviour. Cognitive abilities are usually within normal. The syndrome potentially leads to a diminished level of life management in adulthood. Neuropsychiatric coaching is a solution-focused and practically oriented process of interventions for clients with neurodevelopmental problems. The methods include forms of evaluation and self reflection, structuring, guidance and visualization aids. Coaching does not exclude simultaneous therapeutic elements. The effectiveness has not yet been established by research, but the experiences reported have been encouraging. Neuropsychiatric coaching is recommended for adults with Asperger syndrome to rehabilitate life management skills.

Fluency disorders in genetic syndromes.

PubMed

Van Borsel, John; Tetnowski, John A

2007-01-01

The characteristics of various genetic syndromes have included "stuttering" as a primary symptom associated with that syndrome. Specifically, Down syndrome, fragile X syndrome, Prader-Willi syndrome, Tourette syndrome, Neurofibromatosis type I, and Turner syndrome all list "stuttering" as a characteristic of that syndrome. An extensive review of these syndromes indicated clients diagnosed with these syndromes do show evidence of nonfluency patterns, but not all would be considered stuttering. Many of the syndromes are marked by degrees of mental retardation that probably contribute to a higher than average prevalence of stuttering, as well as a higher than average prevalence of other fluency disorders (when compared to the population at large). An in-depth analysis of the available data indicates that some of these genetic syndromes show patterns of stuttering that may be indicative of only that syndrome (or similar syndromes) that can be differentially diagnosed from developmental stuttering. Among these patterns are the word-final nonfluencies noted in Prader-Willi syndrome; the presence of stuttering in the absence of secondary behaviors noted in Prader-Willi syndrome and; the presence of palilalia, word-final and word-medial nonfluencies, and word-medial and word-final nonfluencies in Tourette syndrome. Implications for future research are discussed in light of these findings. The reader will be able to: (1) describe the various different genetic syndromes that are associated with fluency disorders; (2) describe the types of nonfluencies that are associated with the major types of genetic syndromes that have fluency disorders; (3) describe the behaviors that may assist in differentially diagnosing different types of speech characteristics associated with various genetic syndromes.

Problem Behaviour and Psychosocial Functioning in Young Children with Williams Syndrome: Parent and Teacher Perspectives

ERIC Educational Resources Information Center

Klein-Tasman, B. P.; Lee, K.

2017-01-01

Background: There is sparse literature about problem behaviour in young children with Williams syndrome (WS) and little consideration of the perspectives of multiple respondents. Methods: Problem behaviour of 35 children with WS ages 2 to 6 was examined based on both parent and teacher report using the Achenbach preschool forms. Results: The most…

Behaviour Problems, Maternal Internalising Symptoms and Family Relations in Families of Adolescents and Adults with Fragile X Syndrome

ERIC Educational Resources Information Center

Baker, J. K.; Seltzer, M. M.; Greenberg, J. S.

2012-01-01

Background: Studies have linked the behaviour problems of children with fragile X syndrome (FXS) to maternal well-being, but less is known about how behaviour problems relate to important family factors such as marital satisfaction and family cohesion. Methods: Married mothers of 115 adolescents and adults with FXS completed questionnaires and…

Healthcare and Guidelines: A Population-Based Survey of Recorded Medical Problems and Health Surveillance for People with Down syndrome

ERIC Educational Resources Information Center

Maatta, Tuomo; Maatta, Joonas; Tervo-Maatta, Tuula; Taanila, Anja; Kaski, Markus; Iivanainen, Matti

2011-01-01

Background: Medical problems are described in a population of persons with Down syndrome. Health surveillance is compared to the recommendations of national guidelines. Method: Case records from the specialised and primary healthcare and disability services were analysed. Results: A wide spectrum of age-specific medical and surgical problems was…

Self-Management Program for Heart Healthy Behavior Among Middle- and Old-Aged Korean Women at Risk for Metabolic Syndrome.

PubMed

Shin, Nah-Mee; Choi, JiWon; Cho, InHae; Park, Byung-Jun

The prevalence of metabolic syndrome (MetS) has been increasing among Koreans, and middle-aged and older women are at risk of metabolic syndrome. Effective strategies to promote lifestyle modification need to be developed. We examined the effects of a self-management program on improving the cardiovascular health status and promoting healthy behaviors among overweight or obese Korean women at risk of metabolic syndrome. A pretest and posttest intervention design was used. Sixty women participated in a group teaching session. They also received a pedometer and a diary for self-monitoring. On the basis of blood test results, women's metabolic syndrome status was identified. Thirty women with metabolic syndrome received additional tailored counseling and weekly follow-up calls for 4 weeks, whereas 30 women without metabolic syndrome did not receive any tailored counseling or follow-up calls. Twenty-three women in the MetS group and 22 women in the non-MetS group completed the posttest. Overall, women significantly improved their cardiovascular health status including systolic blood pressure, diastolic blood pressure, body mass index, low-density lipoprotein, triglycerides, number of metabolic syndrome risk factors, and 10-year risk estimates from pretest to posttest. Seventy-eight percent of the MetS group (n = 18) no longer had metabolic syndrome, whereas 5% of the non-MetS group (n = 1) became to have metabolic syndrome. Women significantly increased frequency and duration of walking per week and significantly decreased the time spent sitting. Promoting self-management for healthy behaviors might be effective for obese or overweight women to prevent metabolic syndrome and cardiovascular diseases, if it is tailored to their health needs.

Scatter Plot Analysis of Excessive Daytime Sleepiness and Severe Disruptive Behavior in Adults with Prader-Willi Syndrome: A Pilot Study

ERIC Educational Resources Information Center

Maas, Anneke P. H. M.; Didden, Robert; Bouts, Lex; Smits, Marcel G.; Curfs, Leopold M. G.

2009-01-01

Individuals with Prader-Willi syndrome (PWS) are at risk for excessive daytime sleepiness (EDS) and disruptive behavior. This pilot study explores temporal characteristics of EDS and severe disruptive behavior across time of day and day of week in seven individuals with PWS (aged between 33 and 49 years) of whom five were matched to controls.…

Asperger Syndrome: A Primer for Behavioral Interventionists

ERIC Educational Resources Information Center

Polirstok, Susan Rovet; Houghteling, Lawrence

2006-01-01

Children and adolescents with Asperger Syndrome are intellectually capable, rigid and often obsessive, adhere to stereotypic routines, demonstrate difficulties with pragmatic language and characteristically lack social skills. While the extent of these behaviors may wax and wane and vary by individual, they are in stark contrast with the high…

Functional Analysis of Inappropriate Social Interactions in Students with Asperger's Syndrome

ERIC Educational Resources Information Center

Roantree, Christina F.; Kennedy, Craig H.

2012-01-01

We analyzed the inappropriate social interactions of 3 students with Asperger's syndrome whose behavior was maintained by social positive reinforcement. We tested whether inappropriate social behavior was sensitive to social positive reinforcement contingencies and whether such contingencies could be reversed to increase the probability of…

The Neuroanatomy and Neuroendocrinology of Fragile X Syndrome

ERIC Educational Resources Information Center

Hessl, David; Rivera, Susan M.; Reiss, Allan L.

2004-01-01

Fragile X syndrome (FXS), caused by a single gene mutation on the X chromosome, offers a unique opportunity for investigation of gene-brain-behavior relationships. Recent advances in molecular genetics, human brain imaging, and behavioral studies have started to unravel the complex pathways leading to the cognitive, psychiatric, and physical…

Williams Syndrome: A Critical Review of the Cognitive, Behavioral, and Neuroanatomical Phenotype

ERIC Educational Resources Information Center

Martens, Marilee A.; Wilson, Sarah J.; Reutens, David C.

2008-01-01

This review critically examines the research findings which characterize the cognitive, behavioral, and neuroanatomical features of Williams syndrome (WS). This article analyzes 178 published studies in the WS literature covering the following areas: 1) General intelligence, 2) Language skills, 3) Visuospatial and face processing skills, 4)…

Behavior in Prader-Willi Syndrome: Relationship to Genetic Subtypes and Age

ERIC Educational Resources Information Center

Dykens, Elisabeth M.; Roof, Elizabeth

2008-01-01

Background: Some behavioral features of Prader-Willi syndrome (PWS) are associated with the major genetic subtypes of this disorder. While most agree that those with maternal uniparental disomy (UPD) have a distinctive cognitive and psychiatric profile, findings are more controversial regarding possible differences among persons who vary in…

Attention in Williams Syndrome and Down's Syndrome: Performance on the New Early Childhood Attention Battery

ERIC Educational Resources Information Center

Breckenridge, Kate; Braddick, Oliver; Anker, Shirley; Woodhouse, Margaret; Atkinson, Janette

2013-01-01

Attentional problems are commonly reported as a feature of the behavioural profile in both Williams syndrome (WS) and Down's syndrome (DS). Recent studies have begun to investigate these impairments empirically, acknowledging the need for an approach that considers cross-syndrome comparisons and developmental changes across the different component…

Elucidating X chromosome influences on Attention Deficit Hyperactivity Disorder and executive function.

PubMed

Green, Tamar; Bade Shrestha, Sharon; Chromik, Lindsay C; Rutledge, Keetan; Pennington, Bruce F; Hong, David S; Reiss, Allan L

2015-09-01

To identify distinct behavioral and cognitive profiles associated with ADHD in Turner syndrome (TS), relative to idiopathic ADHD and neurotypical controls, in order to elucidate X-linked influences contributing to ADHD. We used a multilevel-model approach to compare 49 girls with TS to 37 neurotypical females, aged 5-12, on established measures of behavior (BASC-2) and neurocognitive function (NEPSY). We further compared girls with TS to BASC-2 and NEPSY age-matched reference data obtained from children with idiopathic ADHD. Within the TS group, 51% scored at or above the "at-risk" range for ADHD-associated behaviors on the BASC-2 (TS/+ADHD). The BASC-2 behavioral profile in this TS/+ADHD-subgroup was comparable to a reference group of boys with ADHD with respect to attentional problems and hyperactivity. However, the TS/+ADHD-subgroup had significantly higher hyperactivity scores relative to a reference sample of girls with ADHD (p = 0.016). The behavioral profile in TS was associated with significantly lower attention and executive function scores on the NEPSY relative to neurotypical controls (p = 0.015); but was comparable to scores from a reference sample of children with idiopathic ADHD. Deficits in attention and executive function were not observed in girls with TS having low levels of ADHD-associated behavior (TS/-ADHD). ADHD-associated behavioral and cognitive problems in TS are prevalent and comparable in severity to those found in children with idiopathic ADHD. The ADHD phenotype in TS also appears relatively independent of cognitive features typically associated with TS, like visuospatial weaknesses. These findings suggest that X-linked haploinsufficiency and downstream biological effects contribute to increased risk for ADHD. Copyright © 2015 Elsevier Ltd. All rights reserved.

Sex differences in a shoaling-boldness behavioral syndrome, but no link with aggression.

PubMed

Way, Gregory P; Kiesel, Alexis L; Ruhl, Nathan; Snekser, Jennifer L; McRobert, Scott P

2015-04-01

A behavioral syndrome is observed in a population when specific behaviors overlap at the individual level in different contexts. Here, we explore boldness and aggression personality spectra, the repeatability of shoaling, and possible associated correlations between the behaviors in a population of lab-reared zebrafish (Danio rerio). Our findings describe a sex-specific boldness-shoaling behavioral syndrome, as a link between boldness and shoaling behaviors is detected. The results indicate that bold males are likely to have a stronger shoaling propensity than shy males for unfamiliar conspecifics. Conversely, bold females are more likely to shoal than shy females, but only when presented with heterospecific individuals. Additionally, aggression does not correlate with boldness or shoaling propensity for either sex. A positive relationship between boldness and shoaling that differs by sex is contrary to most of the present literature, but could help to explain population dynamics and may also have evolutionary implications. Copyright © 2015 Elsevier B.V. All rights reserved.

Rett Syndrome

MedlinePlus

Rett syndrome is a rare genetic disease that causes developmental and nervous system problems, mostly in girls. It's related to autism spectrum disorder. Babies with Rett syndrome seem to grow and develop normally at first. ...

Genetics Home Reference: fragile X syndrome

MedlinePlus

... Citation on PubMed Koukoui SD, Chaudhuri A. Neuroanatomical, molecular genetic, and behavioral correlates of fragile X syndrome. Brain ... GJ, Dictenberg J. The fragile X syndrome: from molecular genetics to neurobiology. Ment Retard Dev Disabil Res Rev. ...

Adaptive behavior of institutionalized individuals with Down syndrome.

PubMed

Silverstein, A B; Ageno, D; Alleman, A C; Derecho, K T; Gray, S B; White, J F

1985-03-01

Institutional residents with Down syndrome (N = 413) were matched with an equal number of residents in other diagnostic categories with respect to sex, age, length of hospitalization, and IQ. The two groups were compared on 62 items of the Client Development Evaluation Report, and significant differences were found on 19 of these items. The subjects with Down syndrome tended to show greater social competence (except in the clarity of their speech) and less maladaptive behavior. To account for these differences, we offer the speculative hypothesis that the favorable expectations of service providers for their clients with Down syndrome may come to serve as self-fulfilling prophecies.

Testing the 8-syndrome structure of the child behavior checklist in 30 societies.

PubMed

Ivanova, Masha Y; Dobrean, Anca; Dopfner, Manfred; Erol, Nese; Fombonne, Eric; Fonseca, Antonio Castro; Frigerio, Alessandra; Grietens, Hans; Hannesdottir, Helga; Kanbayashi, Yasuko; Lambert, Michael; Achenbach, Thomas M; Larsson, Bo; Leung, Patrick; Liu, Xianchen; Minaei, Asghar; Mulatu, Mesfin S; Novik, Torunn S; Oh, Kyung Ja; Roussos, Alexandra; Sawyer, Michael; Simsek, Zeynep; Dumenci, Levent; Steinhausen, Hans-Christoph; Metzke, Christa Winkler; Wolanczyk, Tomasz; Yang, Hao-Jan; Zilber, Nelly; Zukauskiene, Rita; Verhulst, Frank C; Rescorla, Leslie A; Almqvist, Fredrik; Weintraub, Sheila; Bilenberg, Niels; Bird, Hector; Chen, Wei J

2007-01-01

There is a growing need for multicultural collaboration in child mental health services, training, and research. To facilitate such collaboration, this study tested the 8-syndrome structure of the Child Behavior Checklist (CBCL) in 30 societies. Parents' CBCL ratings of 58,051 6- to 18-year-olds were subjected to confirmatory factor analyses, which were conducted separately for each society. Societies represented Asia; Africa; Australia; the Caribbean; Eastern, Western, Southern, and Northern Europe; the Middle East; and North America. Fit indices strongly supported the correlated 8-syndrome structure in each of 30 societies. The results support use of the syndromes in diverse societies.