A description of adaptive and maladaptive behaviour in children and adolescents with Cri-du-chat syndrome
adolescent; development; Rehabilitation; epidemiology; Neurology; child; Education & Educational Research; prevalence; Genetics & Heredity; individuals; Neurosciences &; Psychiatry; adults; behaviour; Cri-du-chat syndrome; behavioral problems; Cri-du-chat; trajectory; characteristics; maladaptive behaviors
Background Psychological tests can be useful to record adaptive and maladaptive behaviours of children with intellectual disability. The objective of this study was to describe the adaptive and maladaptive behaviour of children and adolescents with Cri-du-chat syndrome. Methods The sample consisted of 10 children and adolescents with Cri-du-chat syndrome (mean chronological age = 11.3 years, mean mental age = 18 months). The developmental quotient was calculated through the Psychoeducational Profile - Revised. An observational protocol was used to record adaptive and maladaptive behaviours. Results The number of maladaptive behaviours observed was different among participants. However, all of them had high rates of adaptive behaviours, such as rule-following. Conclusions These results, though preliminary, justify that we continue to think about the need for psychoeducational interventions aimed at stimulating the repertoire of adaptive behaviours, in people with Cri-du-chat syndrome.
Teixeira Mctv; Emerich D R; Orsati F T; Rimerio R C; Gatto K R; Chappaz I O; Kim C A
Journal of Intellectual Disability Research
2011
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1111/j.1365-2788.2010.01377.x" target="_blank" rel="noreferrer noopener">10.1111/j.1365-2788.2010.01377.x</a>
A Longitudinal Follow-Up Study of Affect in Children and Adults With Cornelia de Lange Syndrome
Rehabilitation; behavior; Education & Educational Research; Cornelia de Lange syndrome; behavioral-phenotype; mental-retardation; affect; autistic; behavioral phenotypes; delange-syndrome; follow-up; fragile-x-syndrome; genetic syndromes; genotype-phenotype correlations; longitudinal; pervasive developmental disorders; spectrum disorders; williams-syndrome; behavioral problems; De Lange syndrome; Cri-du-chat; trajectory; characteristics; mood; interest; pleasure
Studies of individuals with Cornelia de Lange syndrome (CdLS) have described changes in mood and behavior with age, although no empirical or longitudinal studies have been conducted. Caregivers of individuals with CdLS (N = 67), cri du chat syndrome (CdCS; N = 42), and Fragile X syndrome (FXS; N = 142) completed the Mood, Interest and Pleasure Questionnaire (MIPQ) at Time 1 and 2 years later (Time 2). Scores on the MIPQ were significantly lower in the CdLS group compared with the CdCS and FXS groups at Time 1 and Time 2. Lower MIPQ scores were characteristic of older adolescents (> 15 years) and adults with CdLS. However, there were no significant differences in MIPQ scores between Time 1 and Time 2. Age and insistence on sameness predicted MIPQ scores in CdLS.
Nelson L; Moss J; Oliver C
American Journal on Intellectual and Developmental Disabilities
2014
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1352/1944-7558-119.3.235" target="_blank" rel="noreferrer noopener">10.1352/1944-7558-119.3.235</a>
The prevalence and phenomenology of repetitive behavior in genetic syndromes
Middle Aged; Male; Young Adult; Surveys and Questionnaires; Child; Humans; Adult; Adolescent; Prevalence; Female; Child Preschool; Phenotype; Syndrome; London; Stereotyped Behavior; Angelman Syndrome/epidemiology/psychology; Cri-du-Chat Syndrome/epidemiology/psychology; De Lange Syndrome/epidemiology/psychology; Fragile X Syndrome/epidemiology/psychology; Intellectual Disability/epidemiology/genetics/psychology; Prader-Willi Syndrome/epidemiology/psychology; behavioral problems; tone and motor problems; cri-du-chat; De Lange syndrome; trajectory; characteristics; repetitive behavior
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 (N = 58), Fragile X (N = 191), Prader-Willi (N = 189), Lowe (N = 56) and Smith-Magenis (N = 42) syndromes and individuals with intellectual disability of heterogeneous aetiology (N = 56). Repetitive behavior was variable across syndromes. Fragile X syndrome scored highly on all subscales. Angelman syndrome demonstrated a significantly lowered probability for most behaviors. Prader-Willi, Cri-du-Chat and Smith-Magenis syndrome evidenced unique profiles of repetitive behavior. There is extreme heterogeneity of repetitive behavior across genetic syndromes, highlighting syndrome specific profiles.
Moss J; Oliver C; Arron K; Burbidge C; Berg K
Journal of Autism and Developmental Disorders
2009
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1007/s10803-008-0655-6" target="_blank" rel="noreferrer noopener">10.1007/s10803-008-0655-6</a>
Sleep in individuals with Cri du Chat syndrome: a comparative study
Middle Aged; Male; Infant Newborn; Severity of Illness Index; Statistics Nonparametric; Young Adult; Surveys and Questionnaires; Child; Humans; Adult; Adolescent; Prevalence; Female; Child Preschool; Infant; Cri-du-Chat Syndrome/ep [Epidemiology]; Sleep Wake Disorders/di [Diagnosis]; Sleep Wake Disorders/ep [Epidemiology]; Cri-du-Chat Syndrome/ge [Genetics]; Genetic Testing; sleep disturbance/disorders; Cri-du-chat; trajectory; characteristics
BACKGROUND: Sleep problems are common in individuals with intellectual disability. Little is known about sleep in children and adults with Cri du Chat syndrome (CDC).;METHOD: Sleep was investigated in 30 individuals with CDC using a sleep questionnaire. Sleep problems and sleep behaviours in individuals with CDC were compared with individuals with non-specific intellectual disabilities (NS) (n = 30) and Down's syndrome (DS) (n = 30).;RESULTS: Nine individuals with CDC (i.e. 30%) had a sleep problem, compared with seven individuals with NS (i.e. 23%) and three individuals with DS (i.e. 10%). Though there were few differences between diagnostic groups, night waking problems were most common in CDC. Individuals with CDC frequently showed behaviours related to disordered breathing and poor-quality sleep. Several behaviours related to sleep had a higher occurrence in CDC than in DS (P < 0.05) but not in NS.;CONCLUSIONS: It is concluded that individuals with CDC do not have an increased probability of sleep problems as compared with other individuals who share similar demographic characteristics. Hypotheses about causes of night waking problems in CDC are generated and suggestions for future research of sleep in individuals with CDC are given.
Maas A P; Didden R; Korzilius H; Braam W; Smits M G; Curfs L M
Journal of Intellectual Disability Research
2009
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1111/j.1365-2788.2009.01184.x" target="_blank" rel="noreferrer noopener">10.1111/j.1365-2788.2009.01184.x</a>
Correlates of maladaptive behavior in individuals with 5p- (cri du chat) syndrome
Male; Surveys and Questionnaires; Child; Humans; Adult; Adolescent; Female; Child Preschool; Analysis of Variance; Risk Factors; Psychiatric Status Rating Scales; Adjustment Disorders/etiology; Child Behavior Disorders/etiology; Cri-du-Chat Syndrome/genetics/psychology; Gene Deletion; Intellectual Disability/psychology; Mental Disorders/etiology; Translocation Genetic; behavioral problems; Cri-du-chat; trajectory; characteristics; self-injury; aggression; pain behaviors; mood; low mood; hyperactivity; impulsivity; repetitive language use
This study examined the range, distinctiveness, and correlates of maladaptive behavior in 146 subjects with 5p- (cri du chat) syndrome using the Aberrant Behavior Checklist as a standardized measure. Hyperactivity was the most significant and frequent problem in the sample. Subjects with 5p- syndrome also showed aggression, tantrums, self-injurious behavior, and stereotypies; some of these problems were more pronounced in individuals with lower cognitive-adaptive levels, as well as in those with histories of previous medication trials. Autistic-like features and social withdrawal were more characteristic of individuals with translocations as opposed to deletions, even when controlling for the lower adaptive level of the translocation group. These findings encourage further research on the behavior of individuals with 5p- syndrome.
Dykens E M; Clarke D J
Developmental Medicine and Child Neurology
1997
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1111/j.1469-8749.1997.tb07377.x" target="_blank" rel="noreferrer noopener">10.1111/j.1469-8749.1997.tb07377.x</a>
Developmental and behavioural characteristics of cri du chat syndrome
behavior; bowel incontinence; feeding difficulties; tone and motor problems; urinary incontinence; cri-du-chat; trajectory; characteristics; self-injury; repetitive movements; sound hypersensitivity; clumsiness; obsessive attachment to objects
Developmental and behavioural characteristics were assessed in 27 children with cri du chat syndrome using the Society for the Study of Behavioural Phenotypes questionnaire, which gave information on prenatal and perinatal conditions, neurological problems, and developmental and behavioural difficulties. The findings suggest that the behavioural profile of children with cri du chat syndrome incorporates self injurious behaviour, repetitive movements, hypersensitivity to sound, clumsiness, and obsessive attachments to objects. In terms of a developmental profile, children with cri du chat syndrome were able to communicate their needs, socially interact with others, and have some degree of mobility.
Cornish K M; Pigram J
Archives of Disease in Childhood
1996
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1136/adc.75.5.448" target="_blank" rel="noreferrer noopener">10.1136/adc.75.5.448</a>
Adaptive and maladaptive behaviour in children with Cri-du-chat syndrome
Psychology; Rehabilitation behavioral problems; Cri-du-chat; trajectory; characteristics; externalizing behaviors; poor concentration; hyperactivity; impulsiveness
Twenty children with cri-du-chat syndrome were assessed on the Vineland Adaptive Behavioural Scales. Strengths within rather than between the adaptive domains were identified with posthoc analyses revealing the main differences to lie within the domains of Communication and Daily-living Skills. In the former domain, Receptive Skills were significantly more developed than Expressive and Written skills; and in the latter domain, Personal skills were significantly more developed than Community-based Skills. The core behavioural problems were Hyperactivity, poor Concentration span and Impulsivity. The implications of the present findings are discussed with reference to the development of effective remedial programmes that take into account the strengths and weaknesses in the adaptive and maladaptive profile of children with cri-du-chat syndrome.
Cornish K M; Munir F; Bramble D
Journal of Applied Research in Intellectual Disabilities
1998
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1111/j.1468-3148.1998.tb00064.x" target="_blank" rel="noreferrer noopener">10.1111/j.1468-3148.1998.tb00064.x</a>
A survey of the prevalence of stereotypy, self-injury and aggression in children and young adults with Cri du Chat syndrome
Male; Comorbidity; Surveys and Questionnaires; Child; Humans; Adult; Adolescent; Prevalence; Female; Aggression/psychology; Cri-du-Chat Syndrome/epidemiology; Self-Injurious Behavior/epidemiology; Stereotypic Movement Disorder/epidemiology; behavior; tone and motor problems; cri-du-chat; trajectory; characteristics; stereotypy; self-injury; aggression
The aim of the present study was to determine the prevalence and frequency of stereotypy, self-injurious behaviour (SIB), and aggression in children and adults with Cri du Chat syndrome (CCS), and to investigate the relationship between SIB, aggressive behaviour and stereotypy in these individuals. Sixty-six families of children and adults diagnosed with CCS completed the Behaviour Problems Inventory. Additional information relating to gender, chronological age, type of school/post-school occupation and medication was also included in the survey. Stereotyped behaviour was reported for 82% of subjects, more than half the sample displaying it on a daily basis. The occurrence percentage of 15 topographies of SIB suggested that head banging, hitting the head against body parts, self-biting and rumination are the most frequently occurring behaviours in CCS. Aggressive behaviour was reported for 88%, with a statistically significant negative correlation between age and the number of aggressive behaviours reported. The present findings suggest that specific types of stereotypy and SIB are observed frequently in CCS.
Collins M S; Cornish K
Journal of Intellectual Disability Research
2002
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1046/j.1365-2788.2002.00361.x" target="_blank" rel="noreferrer noopener">10.1046/j.1365-2788.2002.00361.x</a>
Association Between Fatigue and Autistic Symptoms in Children With Cri du Chat Syndrome
alertness; sleep disturbance; cri-du-chat; trajectory; characteristics; fatigue; autistic symptoms
In the current study, the authors examined whether the fatigue level of children diagnosed with cri du chat syndrome was associated with the expression of autistic symptoms. Sixty-nine children with cri du chat syndrome were compared with 47 children with moderate to severe intellectual disabilities who did not differ on intellectual severity. Participants were assessed using the Infant Sleep Questionnaire (J. M. B. Morrell, 1999) for fatigue-level rating and the Childhood Autism Rating Scale (E. Schopler, R. J. Reichler, & B. R. Renner, 1988) for autism-level rating. In support of the authors' hypothesis, results indicated that children who exhibited high levels of fatigue were more likely to express high levels of autistic symptoms. Contrary to the authors' hypothesis, children in the comparison group who exhibited high levels of fatigue conferred the greatest vulnerability to the expression of autistic symptoms.
Claro A; Cornish K; Gruber R
American Journal on Intellectual and Developmental Disabilities
2011
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1352/1944-7558-116.4.278" target="_blank" rel="noreferrer noopener">10.1352/1944-7558-116.4.278</a>
Problem behaviors associated with deletion Prader-Willi, Smith-Magenis, and cri du chat syndromes
behavioral problems; Cri-du-chat; trajectory; characteristics
Problem behaviors of individuals who had one of three chromosome deletion disorders (5p- cri-du-chat, 15q- Prader-Willi, or 17p- Smith-Magenis) were investigated. The Aberrant Behavior Checklist was used. Results were contrasted with those of two groups of people with mental retardation who were described in other studies. The checklist rates many, but not all, potentially relevant behaviors. Eating abnormalities, known to be problematic in Prader-Willi syndrome, and sleep abnormalities, believed to be problematic in Smith-Magenis syndrome, were not included in the survey. All three disorders were associated with greater ratings of problem behaviors than the comparison groups on at least one subscale of the checklist. The results lend support to the partial specificity model of behaviors associated with genetically determined syndromes.
Clarke D J; Boer H
American Journal of Mental Retardation
1998
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1352/0895-8017(1998)103" target="_blank" rel="noreferrer noopener">10.1352/0895-8017(1998)103<0264:PBAWDP>2.0.CO;2</a>