Behavioral Profiles Of Children Research Articles

Psychological impact of motor impairment in tow forms of congenital muscular dystrophy

IntroductionCongenital muscular dystrophies (CMDs) represent a heterogeneous group of early-onset muscle disorders presenting primarily with hypotonia and delayed motor development. Several genes are known to be responsible for CMDs, including the LAMA2 gene, involved in merosin-deficient type 1A (MDC1A), and the FKRP gene involved in muscular dystrophy-dystroglycanopathy type B5 (MDDGB5). These two forms of CMD are autosomal recessive and are each characterized by the presence of a mutation with a founder effect in South Tunisia. Cognitive development associated with the founder mutation in the LAMA2 gene (c.8007delT) is often conserved, whereas in the founder mutation of the FKRP gene (c.1364 C>A), motor impairment is associated with intellectual disability (ID).ObjectivesTo compare the psychological impact of motor impairment in children presenting these two forms of CMD and their families.MethodsThe study consisted of a survey of parents of children with a confirmed diagnosis of MDC1A (5 from 3 unrelated families) or MDDGB5 (3 from 3 unrelated families). The correspondent founder mutation was already identified in the homozygous state by targeted sequencing. Participants’ parents completed the Parent Strengths and Difficulties Questionnaire (SDQ), a behavioral screening tool designed for children aged from 2 to 17 years. The SDQ assesses emotional symptoms, behavior problems, hyperactivity, and peer relationships; The SDQ Impact Supplement assesses the impact of all these children’s difficulties on their families.ResultsThe average age of the children was 4.95±3.92 with two children who were not assessable by the SDQ (age< 2 years). Unlike children with MDC1A, ID has been reported in all children with MDDGB5. The mean SDQ total score for children with MDC1A was 11, whereas the mean score for children with MDDGB5 was 14.875, reflecting greater difficulty for children with MDDGB5. The family impact score was higher in families with children with MDDGB5 than in children with MDC1A (10,5 vs 7), which may be due to the burden of management of the ID associated with the motor impairment. The more pronounced difficulties associated with MDDGB5 are likely to be related to the associated ID. Whereas in MDC1A, the difficulties observed are related to the direct impact of the motor impairment. The presence of cognitive disorders associated with a motor deficit aggravates behavioral adaptation and makes the management of these children more difficult.ConclusionsIn the absence of a comparable study in the literature, the present is conducting future studies on the behavioral profile of children with CMD to obtain a better understanding of their difficulties in everyday life and to develop interventions adapted to their familiesDisclosure of InterestNone Declared

Cognitive and Behavioral Profiles of Children with Aspartylglucosaminuria: A Case Series

AbstractAspartylglucosaminuria (AGU) is a rare lysosomal storage disorder with an autosomal recessive inheritance. It affects the aspartylglucosaminidase (AGA) gene and causes a deficiency in activity of the enzyme N-aspartyl-β-glucosaminidase with resultant accumulation of glycoasparagines in body tissues. Children with AGU present with developmental delays in cognitive and language skills. Currently, information on cognition and behavior of children with AGU is limited. This case series presents the neuropsychological and behavioral profiles of three children with AGU assessed at two time points. All the three children had full-scale intelligence quotient (IQ) scores in the intellectual disabled range (<70). IQ had declined since their previous cognitive assessment 1 to 2 years prior. Other cognitive skills were significantly below expected levels. Parents reported several behavioral problems and adaptive difficulties. The findings challenge current beliefs that cognition is impaired but stable in children with declines seen in a short time.

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

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.

Behavioural and Psychiatric Phenotypes in Men and Boys with X-Linked Ichthyosis: Evidence from a Worldwide Online Survey.

BackgroundX-linked ichthyosis (XLI) is a rare dermatological condition arising from deficiency for the enzyme steroid sulfatase (STS). Preliminary evidence in boys with XLI, and animal model studies, suggests that individuals lacking STS are at increased risk of developmental disorders and associated traits. However, the behavioural profile of children with XLI is poorly-characterised, and the behavioural profile of adults with XLI has not yet been documented at all.Materials and MethodsUsing an online survey, advertised worldwide, we collected detailed self- or parent-reported information on behaviour in adult (n = 58) and younger (≤18yrs, n = 24) males with XLI for comparison to data from their non-affected brothers, and age/gender-matched previously-published normative data. The survey comprised demographic and background information (including any prior clinical diagnoses) and validated questionnaires assaying phenotypes of particular interest (Adult ADHD Self-Report Scale v1.1, Barrett Impulsiveness Scale-11, adult and adolescent Autism Quotient, Kessler Psychological Distress Scales, and Disruptive Behaviour Disorder Rating Scale).ResultsIndividuals with XLI generally exhibited normal sensory function. Boys with XLI were at increased risk of developmental disorder, whilst adults with the condition were at increased risk of both developmental and mood disorders. Both adult and younger XLI groups scored significantly more highly than male general population norms on measures of inattention, impulsivity, autism-related traits, psychological distress and disruptive behavioural traits.ConclusionsThese findings indicate that both adult and younger males with XLI exhibit personality profiles that are distinct from those of males within the general population, and suggest that individuals with XLI may be at heightened risk of psychopathology. The data are consistent with the notion that STS is important in neurodevelopment and ongoing brain function, and with previous work suggesting high rates of developmental disorders in boys with XLI. Our results suggest that individuals with XLI may require medical care from multidisciplinary teams, and should help to inform genetic counselling for the condition.

Neurocognitive and behavioral profiles of children with Landau-Kleffner syndrome

ABSTRACTThis is a retrospective study of 14 cases of children with Landau-Kleffner syndrome (LKS), the most prominent feature of which is acquired aphasia. These children were followed at a tertiary care pediatric epilepsy center. From the research data base, all LKS cases with neuropsychological evaluation were extracted. Children ranged in age from 6 to 13 years (M = 9.12; SD = 2.19) at the time of assessment (1 to 10 years post-onset). The majority of the children were white males, and all but one continued to experience seizure activity. Global intellectual functioning ranged from 59 to 101 (M = 82.07; SD = 12.14). Across the 14 cases reviewed, the neuropsychological profiles are considered in the context of neurological and syndrome-related factors. For these cases, 86% demonstrated continued expressive, and 50% had receptive language problems with 57% exhibiting poor auditory processing. Furthermore, 50 to 57% had deficits in auditory working memory and verbal memory. Academically, the majority had poor reading fluency and comprehension; 50% exhibited difficulty with mathematics. Finally, 57% evidenced attentional or other behavioral problems. Better understanding of LKS can assist in targeted assessment and intervention planning.

Family Functioning, Comorbidities, and Behavioral Profiles of Children With ADHD and Disruptive Mood Dysregulation Disorder

Objective: Children with ADHD-combined type (ADHD-C), disruptive mood dysregulation disorder (DMDD), and healthy controls (HC) were compared with respect to the sociodemographic features, psychiatric comorbidities, behavioral patterns, and family functioning. Method: Research groups were identified using the Schedule for Affective Disorders and Schizophrenia for School-Age Children–Present and Lifetime Version (K-SADS-PL). Conners’ Rating Scale–Revised Long Form, Wechsler Intelligence Scale for Children–Revised (WISC-R) test, Sociodemographic Questionnaire, and Family Assessment Device (FAD) were administered to research groups. Results: DMDD group’s rate of psychiatric comorbidity was higher than the ADHD-C group’s rate. In most of the subscales of Conners, DMDD group’s average scores were higher than the other groups’ scores. In “Communication,” “Affective Responsiveness” subscales of FAD, DMDD group’s average scores were higher than the ADHD-C group’s scores. In “Affective Involvement,” “General Functioning” subscales of FAD, DMDD group’s average scores were higher than the other groups’ scores. Conclusion: Children with DMDD were distinguished from children with ADHD-C by their higher comorbidity rate, more impaired behavioral patterns, and family functioning.

Associations between Inadequate Parenting Practices and Behavioral Problems in Children and Adolescents with Attention Deficit Hyperactivity Disorder.

Children and adolescents with ADHD present behaviors such as impulsiveness, inattention, and difficulties with personal organization that represent an overload for parents. Moreover, it also increases their level of stress and leads them to resort to inadequate educational strategies. The present study verifies associations between inadequate parenting practices and behavioral profiles of children and adolescents with ADHD. The sample was composed of 22 children with ADHD (age range 6–16 years) and their mothers. Spearman correlation analyses were made with the scores of Parenting Style Inventory (PSI) and Child Behavior Checklist for ages 6–18 (CBCL/6–18). Results indicate statistically significant associations between behavioral problems and the use of punishment practices and negligence. When assessing a child with ADHD, it is important to verify the predominant types of parenting practices that can influence both immediate interventions and the prognosis of the disorder.

Cognitive and Behavioral Profiles of Children with Fetal Alcohol Spectrum Disorders

The last two decades have witnessed a rapid growth of published data on the cognitive and behavioral functioning of children with fetal alcohol spectrum disorders (FASD). The main of aim of this paper is to review cognitive and behavioral data on FASD using a causal modeling framework originally proposed by Morton and Frith [1, 2], according to which data pertinent to a neurodevelopmental disorder can be organized at three levels: neurobiology, cognition, and behavior. In this review, we confine ourselves to reviewing the studies of cognitive abilities and behavior in children with FASD. The studies of cognitive functions can be further divided into two groups: those of elementary functions (e.g., reflexive orienting responses) and those of complex functions (e.g., language, memory). There is evidence that children with FASD are slower at reflective orienting responses and exhibit deficits in associative learning. The pattern of results emerging from the studies of complex cognitive functions is that the FASD group shows performance decrements with increased task complexity. At the behavioral level, children with prenatal alcohol exposure are rated as showing deficient adaptive skills, particularly in the social domain. These children have also been observed to show deficits in executive functioning with notable behavioral and emotional regulatory problems. A broad range of risk factors including the alcohol’s teratogenicity seem to interactively contribute to these cognitive and behavioral problems.

Behavioral Profiles of Children and Adolescents with Specific Language Impairment and High Functioning Autism (P3.332)

Behavioral Profiles of Children and Adolescents with Specific Language Impairment and High Functioning Autism (P3.332)

Behavioral Profiles of Children and Adolescents with Specific Language Impairment and High Functioning Autism (I4-1.006)

Behavioral Profiles of Children and Adolescents with Specific Language Impairment and High Functioning Autism (I4-1.006)

The behavioural profile of children with attention-deficit/hyperactivity disorder and of their siblings

The behavioural profiles in N = 69 index children with attention-deficit/hyperactivity disorder (ADHD),N = 32 siblings with ADHD, N = 35 siblings without ADHD, and N = 36 normal controls were compared by the use of standardized parent and teacher rating scales. The four groups were matched by age and IQ. The behavioural profiles of the two ADHD groups were very similar not only in the behavioural domains of ADHD, but also in scales measuring emotional and conduct problems. Siblings without ADHD shared more similarities with normal controls except for more emotional problems. These general trends were stronger in the parent compared to the teacher ratings. These findings indicate that not only ADHD-related but also other behaviours show a strong family aggregation. The informant differences may reflect context dependent differences in child behaviour and contrast effects particularly in parental ratings.

The Strange Animal Situation Test

ABSTRACTMany tools assess the reactions of humans encountering familiar or unfamiliar partners or environments. Companion animals belong to our everyday environment and influence our lives. Whereas many standardized tools test companion animals' reactions to humans, few evaluate humans' reactions to companion animals. We present here a test with a guinea pig that can be applied to a wide range of people in the home environment. This standardized test and simple coding system enabled us to characterize individual behavioral profiles of children and compare them in relation to different factors (e.g., gender, age, pet ownership). We observed 59 children (32 girls, 27 boys), aged between 6 and 12 years old. Our results show that most children first looked at the guinea pig (72%), smiled when they saw it (49%), and then went directly towards it without looking at their parent (79%). Many children touched the animal without hesitation (86%). Moreover, this test reveals more than the mere interest of children in guinea pigs. Indeed, a cluster analysis differentiated four behavioral profiles that reflected aspects of the children's experience, gender, and lifestyles. When encountering the unfamiliar guinea pig, children could be “confident” (go straight to the animal and touch it; 64%), “anxious” (look at parent; 12%), “indirect” (hesitate and touch; 14%), or “careful” (emit vocal and/or verbal behaviors; 10%). The potential future application of this research is to compare behavioral profiles quantitatively over the long term, taking into account the development and experiences of people with typical development and those with atypical development (e.g., autistic disorders).

P09.7 The neuropsychological and behavioural profile of children with tectum glioma and hydrocephalus compared to children who were treated for a cerebellar pilocytic astrocytoma

P09.7 The neuropsychological and behavioural profile of children with tectum glioma and hydrocephalus compared to children who were treated for a cerebellar pilocytic astrocytoma

The Cognitive and Behavioral Characteristics of Children With Low Working Memory

This study explored the cognitive and behavioral profiles of children with working memory impairments. In an initial screening of 3,189 five- to eleven-year-olds, 308 were identified as having very low working memory scores. Cognitive skills (IQ, vocabulary, reading, and math), classroom behavior, and self-esteem were assessed. The majority of the children struggled in the learning measures and verbal ability. They also obtained atypically high ratings of cognitive problems/inattentive symptoms and were judged to have short attention spans, high levels of distractibility, problems in monitoring the quality of their work, and difficulties in generating new solutions to problems. These data provide rich new information on the cognitive and behavioral profiles that characterize children with low working memory.

Behavioral profiles of children with epilepsy: Parent and teacher reports of emotional, behavioral, and educational concerns on the BASC‐2

AbstractThe Behavior Assessment Scale for Children – Second Edition (BASC‐2) was administered to 108 parents and 37 teachers of children with epilepsy (mean age of 10.6 years; 51% female, 49% male). Results demonstrated high mean scores on the Atypicality, Attention Problems, Withdrawal, and Adaptive Skills scales and a high percentage of At‐Risk or Clinically Significant scores on the majority of scales. Ratings were correlated with level of functioning, age of onset, and number of antiepileptic medications. In addition, children with poor seizure control had higher ratings on the Depression, Somatization, and Withdrawal scales, and children with temporal lobe seizures had higher scores on the Depression scale. These results confirm a high rate of behavioral concerns in children with epilepsy. © 2008 Wiley Periodicals, Inc.

Behavioral profiles of children with infantile nephropathic cystinosis

Children with infantile nephropathic cystinosis have evidence of visuospatial and arithmetic deficits on a background of normal intellectual and verbal skills. This study aimed to define further their behavioral phenotype. The Achenbach Child Behavior Checklist was completed by parents of: 64 children and adolescents with cystinosis (33 females, 31 males; mean age 8y 8mo, range 4 to 16y, SD 2y 11mo); 101 healthy controls (47 females, 54 males; mean age 8y 4mo, range 4 to 16y, SD2y 11mo); 21 children and adolescents with cystic fibrosis (CF), termed chronic‐disease controls (9 females, 12 males; mean age 11y 3mo, age range 4 to 17y, SD 3y 5mo). Compared with healthy controls, individuals with cystinosis had evidence of a significantly higher incidence of behavioral problems, including social problems, somatic complaints, and attention problems. Compared with the chronic‐disease control group, the cystinosis group differed only on the Social Problems scale, with 22% of participants with cystinosis scoring in the‘at risk’range whereas no participant with CF received an elevated score on this scale. We conclude that children and adolescents with cystinosis have evidence of a significant incidence of social difficulties compared with individuals with another chronic illness and healthy participants. The combination of visuospatial problems, difficulty with arithmetic, attention problems, and social difficulties seen in the cystinosis group constitutes a behavioral phenotype of this genetic disorder. This cluster of cognitive and behavioral symptoms is also seen in the nonverbal learning disabilities syndrome, and suggests a possible early difference in brain development in children with cystinosis compared with children who do not share this genetic disorder.

Behavioral profiles of children with infantile nephropathic cystinosis

Children with infantile nephropathic cystinosis have evidence of visuospatial and arithmetic deficits on a background of normal intellectual and verbal skills. This study aimed to define further their behavioral phenotype. The Achenbach Child Behavior Checklist was completed by parents of: 64 children and adolescents with cystinosis (33 females, 31 males; mean age 8 y 8 mo, range 4 to 16y, SD 2 y 11 mo); 101 healthy controls (47 females, 54 males; mean age 8 y 4 mo, range 4 to 16 y, SD 2 y 11 mo); 21 children and adolescents with cystic fibrosis (CF), termed chronic-disease controls (9 females, 12 males; mean age 11 y 3 mo, age range 4 to 17 y, SD 3 y 5 mo). Compared with healthy controls, individuals with cystinosis had evidence of a significantly higher incidence of behavioral problems, including social problems, somatic complaints, and attention problems. Compared with the chronic-disease control group, the cystinosis group differed only on the Social Problems scale, with 22% of participants with cystinosis scoring in the 'at risk' range whereas no participant with CF received an elevated score on this scale. We conclude that children and adolescents with cystinosis have evidence of a significant incidence of social difficulties compared with individuals with another chronic illness and healthy participants. The combination of visuospatial problems, difficulty with arithmetic, attention problems, and social difficulties seen in the cystinosis group constitutes a behavioral phenotype of this genetic disorder. This cluster of cognitive and behavioral symptoms is also seen in the nonverbal learning disabilities syndrome, and suggests a possible early difference in brain development in children with cystinosis compared with children who do not share this genetic disorder.

Effects of internal displacement and resettlement on the mental health of Turkish children and adolescents

Aims. – To evaluate the effects of internal displacement and resettlement within Turkey on the emotional and behavioral profile of children, age 5–18 after controlling for possible confounding and demographic variables. Method. – We conducted a national population survey using a self-weighted, equal probability sample. We compared the CBCL, TRF and YSR responses regarding children with ( n = 1644) and without ( n = 1855) experience of internal displacement. We examined the effects of gender, age, paternal employment, resettlement, urban residence and physical illness. Results. – The children and adolescents with internal displacement had significantly higher internalizing, externalizing and total problem scores on the CBCL and YSR, and higher internalizing scores on the TRF. The effect of displacement was related to higher internalizing problems when factors like physical illness, child age, child gender and urban residence were accounted. The overall effect was small explaining only 0.1–1.5% of the total variance by parent reports, and not evident by teacher reports. Discussion. – To our knowledge the present study is the first to examine Turkish children and adolescents with and without experience of internal displacement. The results are consistent with previous immigration studies: child age, gender, presence of physical illness and urban residence were more important predictors of internalization and externalization problem scores irrespective of informant source.

Hypothalamic hamartoma and epilepsy in children: illustrative cases of possible evolutions

ABSTRACT The progresses of neuroimaging have allowed an earlier detection of hypothalamic hamartoma in children presenting with gelastic or dacrystic seizures. Associated symptoms can include other types of seizures, precocious puberty, and behavioral or cognitive deterioration. Combination of all these features is not constant and, when present, their evolution may be variable. When epilepsy proves intractable, surgery may be a solution but is not without risks. Therefore, it can only be justified on the basis of a considerable degree of certainty on the progressive character of the disorder, both in terms of epilepsy and global development. Even though epilepsy is a major and usually the most important problem, it is not always possible to predict its course and to be able to evaluate its potential effects on development. Available data suggests that deterioration is partly related to the epileptogenic activity. We reviewed data from 16 personal cases and discussed the possible evolutions of the epilepsy syndrome on the basis of 6 illustrative cases and a review of the literature. We point out that seizures may start early in life and evolve either towards a catastrophic encephalopathy or may be transiently severe and will progressively settle down. Intermediate situations also exist as well as cases presenting with a mild epilepsy. In almost all cases cognitive difficulties are present and may be associated with behavioral disturbances. They are of variable severity, usually in relation to the severity of the epilepsy and the evolution of the EEG abnormalities. Some of our cases also illustrate that, in young children whose seizures are limited to “a sensation of a pleasant feeling”, “a pressure to laugh” or “smiling”, early detection of the hamartoma may still be difficult and the epilepsy pattern may be misdiagnosed as an epilepsy temporal or frontal origin. Detailed analysis of the electro‐clinical evolution of representative cases highlights the variable expression of the epilepsy syndrome and renders difficult any dogmatic position on early surgery. However, recent data suggests that a surgical solution must be sought early. Prospective studies are needed to evaluate, not only outcome in terms of control the seizures without unacceptable side effects but also on the evolution of the cognitive and behavioral profile of children with HH and epilepsy are needed. [published with videosequences]

Community Violence Exposure and Associated Behavior Problems Among Children and Adolescents in Residential Treatment

Community violence exposure is increasingly recognized as playing an important role in the presenting behavioral profiles of children and adolescents, although little is known about the specific role played among children and adolescents served in mental health settings. This cross-sectional study reports findings on the lifetime rates of community violence exposure in a sample of 101 children and adolescents living in residential treatment, and the associations between such exposure and their presenting behavior problems. A total of 41% of the study participants reported they had been severely physically victimized, and 80% stated they had witnessed severe physical victimization. After controlling for background variables, hierarchical regression analyses indicated that sexual victimization and, to a lesser degree, personal physical victimization selectively predicted greater behavior problems, whereas witnessed physical violence predicted fewer behavior problems overall. These findings highlight a need to conduct multidimensional assessments of violence exposure among children and adolescents in residential and other mental health settings.