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Severe anterograde amnesia with onset in childhood as a result of anoxic encephalopathy.

Our patient (M.S.) had an abrupt onset of amnesia due to a respiratory arrest at the age of 8 years and has been followed by one of us (A.L.R.) for 19 years. A specially designed MRI study indicated that the neuroanatomical localization of his lesion is restricted to the hippocampal formation bilaterally. Comparison of M.S.'s present IQ and academic scores with earlier scores revealed that his literacy skills, certain basic language functions and vocabulary development were arrested by his memory disorder. In contrast, development of mathematical skill was less curtailed, and verbal and nonverbal logical abilities developed to adult levels. Neuropsychological examination at the age of 27 years elicited a pattern of memory deficits similar to those found in a case (H.M.) of known mesial temporal lobe damage in adulthood. The neuropsychological pattern revealed those aspects of cognitive development that do, and those that do not, require intact memory. The limitations to intellectual development imposed by severe amnesia in childhood are not pervasive, but rather, are limited to specific types of abilities.

Adult↗

Situational versus pervasive hyperactivity in a community sample.

Groups of home and school situational hyperactive primary schoolboys identified from the community were compared with pervasive hyperactive and non-hyperactive controls on a wide range of measures. The hyperactive groups tended to persist in the same category over a half-year period. Both situational hyperactive groups had lower measured activity levels than the pervasive hyperactive group and only the latter differed from non-hyperactive controls. Home hyperactivity was characterized by poor family relationships and was not distinguishable from non-hyperactive home-antisocial controls. School hyperactive boys had specific correlates of low intelligence, motor clumsiness, poor reading and academic abilities. Pervasive hyperactive subjects differed from both situational groups in showing a higher percentage of delayed language development. While home hyperactivity has dubious identity, the distinct pattern of external correlates in school and pervasive hyperactivity speak for the need to regard these as separate entities.

Attention Deficit Disorder with Hyperactivity↗

The Coffin-Siris syndrome: data on mental development, language, behavior and social skills in 12 children.

In this report we present data on cognitive development, language, behavior and social skills in 12 children and adolescents, nine girls and three boys, aged between 2.5 and 19 years, with Coffin-Siris syndrome (CS). 1. Mental retardation was mild in three patients and moderate in the nine others. 2. Speech onset was severely retarded with little interest in language. In the older group (seven patients aged 7 to 19 years), language comprehension was appropriate to the mental level. 3. Gross-motor functioning and autonomy, with the lowest score on "Task-orientation", were equal to the mental development. 4. Most frequently, aggressive disturbed behavior was observed, especially in the youngest children, while mixed disturbed behavior was observed in the oldest patients. Almost half of the CS patients (5/11) presented symptoms of pervasive developmental disorder, with 2/11 scoring in the pathological range. Obsessive interests, strong dependence on patterns and rituals and unusual fears were characteristic behavioral problems also when they got older.

Adolescent↗

Developmental and acquired dyslexias.

Marshall (1984) highlighted potential parallels between children with developmental disorders of reading and adults who had acquired reading disorders. He advocated the use of a cognitive neuropsychological framework in the investigation of children with developmental abnormalities of cognition, including those with developmental dyslexias. Developmental phonological dyslexia has been extensively described and is a pervasive disorder. The relationship between reading difficulty and phonological difficulties evident in explicit oral phonological tasks continues to be a focus for debate. Clear cases of developmental deep dyslexia have now been described and the syndrome has also been described as characterising early reading development in Williams syndrome (WS), where there are also semantic errors in other domains, including naming and receptive vocabulary and there may be a generalised difficulty with the activation of fine grain semantic specifications. In the domain of number, highly selective reading disorders characterised by high rates of semantic errors have been documented, indicating that semantic reading errors can be domain-specific. They can occur to number words despite intact ability to read Arabic numbers and they can occur to Arabic numbers and number words despite intact ability to read words in other domains. Current models of reading written words do not allow for such material-specific dissociation. Developmental surface dyslexia has also been described in a range of countries, languages and orthographies. Descriptions of cases for whom there is no phonological impairment in reading have generated contrary evidence for theories suggesting that phonological impairment underlies all developmental dyslexia. As reading develops in Williams Syndrome, phonological reading skills may improve with over-reliance on these leading to surface dyslexia. Surface dyslexia has also been reported in cases of developmental amnesia in which there are semantic memory impairments. Hyperlexia can take several forms including broad hyperdevelopment with elevated phonological reading abilities, lexico-semantic reading abilities and reading comprehension as in Turner's syndrome (TS). This advantage has early onset in school-starters. These specific modular effects do not have pervasive impact across systems but demonstrate the limitations of functional plasticity in developmental and genetic disorders. The framework Marshall (1984) outlined has provided a foundation for the development of systematic investigation of developmental disorders.

Adolescent↗

Neuropsychological and neurophysiological indices of auditory processing impairment in children with multiple complex developmental disorder.

OBJECTIVE: To evaluate whether children with borderline disorder (also referred to as multiple complex developmental disorder) (BD/MCDD) and comorbid attention-deficit hyperactivity disorder (ADHD) demonstrate evidence of abnormal attention and/or auditory processing impairments as indexed by both behavioral and physiological measures. METHOD: Three groups of children were compared in two different experiments on behavioral rating scales (Conners Parent Rating Scale and Child Behavior Checklist), behavioral accuracy to auditory and visual target detection tasks, selected neuropsychological tests, and brain physiology (event-related potentials) collected during auditory and visual target detection tasks. RESULTS: The results demonstrate that children with BD/MCDD differ from children with ADHD in the (1) prevalence of internalizing and externalizing behaviors, (2) neuropsychological deficits related to auditory processing, and (3) event-related potential brain physiology associated with auditory cognitive target attention tasks. CONCLUSION: Some of the pervasive pathology described in children with BD/MCDD may be due to biological vulnerabilities, particularly problems with auditory processing. Auditory processing impairments in such children deserves special attention with respect to both understanding their behavioral symptoms and developing a comprehensive treatment plan.

Adolescent↗

Long-term consistency in speech/language profiles: I. Developmental and academic outcomes.

OBJECTIVE: This study examined the 7-year developmental and academic outcome of speech/language-impaired and control children selected from a community sample. METHOD: Speech/language and psychiatric measures were administered to the children at ages 5 and 12.5 years. Using children's age 5 speech/language test results, a cluster analysis was performed to ascertain whether specific linguistic subgroups would emerge. The long-term consistency of these subgroups was explored. The association between time 1 speech/language clusters and linguistic, cognitive and academic measures at time 2 were examined. RESULTS: Four groups were identified in the cluster analysis: high overall, poor articulation, poor comprehension, and low overall. Children with pervasive language problems continued to perform poorly on linguistic, cognitive, and academic measures, while those with comprehension problems fared slightly better but still had more difficulties than those with normal language. The poor articulation cluster had few articulation errors at follow-up. CONCLUSIONS: Empirically supported speech/language classifications identified as early as age 5 continued to be relevant into late childhood. Pervasive speech/language impairment in early childhood was associated with increased risk of poor linguistic and academic outcome at follow-up, while isolated articulatory problems improved over time. These findings reveal the urgent need for early intervention among children with pervasive speech/language impairment.

Child↗

Pervasive disintegrative disorder: are Rett syndrome and Heller dementia infantilis subtypes?

Children with developmental regression and emerging symptoms of autism have been given a variety of classifications. The authors compare two boys with Heller dementia with six girls with Rett syndrome. They all differed from children with classic autism in that they had normal prenatal and perinatal periods, followed by marked developmental regression, after which they acquired few or no skills. The boys differed from the girls in terms of estimated prevalence, age at onset, stereotypic breathing patterns, midline hand stereotypies, hand and gait apraxia and speech development. It is suggested that these children should be distinguished from those with classic autism, and should be classified as 'pervasive disintegrative disorder, Heller type' and 'pervasive disintegrative disorder, Rett type'.

Adolescent↗

PRN sedation-patterns of prescribing and administration in a child and adolescent mental health inpatient service.

OBJECTIVE: To identify utilisation rates of prn (pro re nata) sedation in children and adolescents receiving inpatient psychiatric treatment, and to compare correlates of prn prescribing and administration. METHOD: A retrospective chart review examined 122 medical charts from a child and youth mental health inpatient service. RESULTS: 71.3% of patients were prescribed prn sedation and 50.8% were administered prn sedation. Patients received an average of 8.0 doses of prn sedation, with 9.8% receiving 10 or more doses. Chlorpromazine and diazepam were the most commonly utilised agents. Prescribing of prn sedation was only related to use of regular medications (p < 0.01), and non-parent carers (p < 0.01). In contrast, administration of prn sedation was associated with multiple diagnoses (p < 0.01), pervasive development disorder (p < 0.01), mental retardation (p < 0.01) ADHD (p < 0.01), longer hospital admission (p < 0.01), use of atypical antipsychotics (p < 0.01) and polypharmacy (p < 0.01). CONCLUSIONS: Despite lack of data to inform practice, prn sedation is widely utilised, especially in complex patients. Future research in this area needs to incorporate nurses and examine whether patients benefit from prn sedation, which drugs and dosing patterns optimise safety and efficacy, and what is the role of prn sedation in the context of other medication.

Adolescent↗

Genetic evaluation of pervasive developmental disorders: the terminal 22q13 deletion syndrome may represent a recognizable phenotype.

The evaluation of mental retardation is always a challenge to clinicians. The recognition of specific physical or behavioral characteristics can vastly improve diagnostic yield. Several genetic disorders have been identified to have certain behavioral characteristics, such as Williams syndrome, Smith-Magenis syndrome, and the velocardiofacial syndrome (VCFS). The deletion affecting the chromosome 22q in the most distal band (22q13) appears to define yet another neurobehavioral phenotype. In addition to our report, there are about 17 other cases published of this particular deletion syndrome. We describe three children who share features of developmental delay and pervasive behaviors in addition to normal to advanced growth patterns. Results of cytogenetic analysis suggest that the 3 patients share a deletion affecting the terminal 22q13 region. Two were found to have a cryptic deletion, in the third it was detected by conventional cytogenetics. The cryptic deletions were demonstrated using fluorescent in situ hybridization (FISH), where the control probe for the DiGeorge/VCFS region was deleted. While there remain gaps in our understanding of this particular deletion syndrome, we propose that patients with normal or advanced growth, significantly delayed speech, deviant development and pervasive behaviors, with minor facial dysmorphism, be screened for this deletion.

Autistic Disorder↗

Impact of prenatal chlorpyrifos exposure on neurodevelopment in the first 3 years of life among inner-city children.

OBJECTIVE: The purpose of this study was to investigate the impact of prenatal exposure to chlorpyrifos on 3-year neurodevelopment and behavior in a sample of inner-city minority children. METHODS: As part of an ongoing prospective cohort study in an inner-city minority population, neurotoxicant effects of prenatal exposure to chlorpyrifos were evaluated in 254 children through the first 3 years of life. This report examined cognitive and motor development at 12, 24, and 36 months (measured with the Bayley Scales of Infant Development II) and child behavior at 36 months (measured with the Child Behavior Checklist) as a function of chlorpyrifos levels in umbilical cord plasma. RESULTS: Highly exposed children (chlorpyrifos levels of >6.17 pg/g plasma) scored, on average, 6.5 points lower on the Bayley Psychomotor Development Index and 3.3 points lower on the Bayley Mental Development Index at 3 years of age compared with those with lower levels of exposure. Children exposed to higher, compared with lower, chlorpyrifos levels were also significantly more likely to experience Psychomotor Development Index and Mental Development Index delays, attention problems, attention-deficit/hyperactivity disorder problems, and pervasive developmental disorder problems at 3 years of age. CONCLUSIONS: The adjusted mean 36-month Psychomotor Development Index and Mental Development Index scores of the highly and lower exposed groups differed by only 7.1 and 3.0 points, respectively, but the proportion of delayed children in the high-exposure group, compared with the low-exposure group, was 5 times greater for the Psychomotor Development Index and 2.4 times greater for the Mental Development Index, increasing the number of children possibly needing early intervention services.

Adult↗

The developmental association between eating disorders symptoms and symptoms of depression and anxiety in juvenile twin girls.

OBJECTIVE: We investigated the role of genetic and environmental factors in the developmental association among symptoms of eating disorders, depression, and anxiety syndromes in 8-13-year-old and 14-17-year-old twin girls. METHODS: Multivariate genetic models were fitted to child-reported longitudinal symptom data gathered from clinical interview on 408 MZ and 198 DZ female twin pairs from the Virginia Twin Study of Adolescent Behavioural Development (VTSABD). RESULTS: Model-fitting revealed distinct etiological patterns underlying the association among symptoms of eating disorders, depression, overanxious disorder (OAD), and separation anxiety disorder (SAD) during the course of development: 1) a common genetic factor influencing liability to all symptoms - of early and later OAD, depression, SAD, and eating symptoms; 2) a distinct genetic factor specifically indexing liability to early eating disorders symptoms; 3) a shared environmental factor specifically influencing early depression and early eating disorders symptoms; and 4) a common environmental factor affecting liability to symptoms of later eating disorders and both early and later separation anxiety. CONCLUSIONS: These results suggest a pervasive genetic effect that influences liability to symptoms of over-anxiety, separation anxiety, depression, and eating disorder throughout development, a shared environmental influence on later adolescent eating problems and persistent separation anxiety, genetic influences specific to early eating disorders symptoms, and a shared environmental factor influencing symptoms of early eating and depression.

Adolescent↗

Principles and practices of neurodevelopmental assessment in children: lessons learned from the Centers for Children's Environmental Health and Disease Prevention Research.

Principles and practices of pediatric neurotoxicology are reviewed here with the purpose of guiding the design and execution of the planned National Children's Study. The developing human central nervous system is the target organ most vulnerable to environmental chemicals. An investigation of the effects of environmental exposures on child development is a complex endeavor that requires consideration of numerous critical factors pertinent to a study's concept, design, and execution. These include the timing of neurodevelopmental assessment, matters of biologic plausibility, site, child and population factors, data quality assurance and control, the selection of appropriate domains and measures of neurobehavior, and data safety and monitoring. Here we summarize instruments for the assessment of the neonate, infant, and child that are being employed in the Centers for Children's Environmental Health and Disease Prevention Research, sponsored by the National Institute of Environmental Health Sciences and the U.S. Environmental Protection Agency, discuss neural and neurobiologic measures of development, and consider the promises of gene-environment studies. The vulnerability of the human central nervous system to environmental chemicals has been well established, but the contribution these exposures may make to problems such as attention deficit disorder, conduct problems, pervasive developmental disorder, or autism spectrum disorder remain uncertain. Large-scale studies such as the National Children's Study may provide some important clues. The human neurodevelopmental phenotype will be most clearly represented in models that include environmental chemical exposures, the social milieu, and complex human genetic characteristics that we are just beginning to understand.

Central Nervous System↗

Pregnancy outcome post renal transplantation.

BACKGROUND: The success in performing organ transplantations and prevention of rejection has resulted not only in a substantial increase in life expectancy, but also improvement in the patients' quality of life. Thus, women who underwent organ transplantation are now reaching puberty and the age of reproduction. This has presented new challenges regarding the teratogenicity and the long-term effect of immunosuppressive medications used by these patients. Previous studies have shown that pregnancies after renal transplantation are associated with an increased risk for both the mother and the fetus. There is, however, very little information available on neonatal and long-term pediatric follow-up of babies born to mothers who have undergone renal transplantation and have been exposed to immunosuppressive medications, compared to controls. We report the experience of our center, the largest in Canada, regarding the prenatal and long-term postnatal outcome of pregnancies after renal transplantation. METHODS: This is a retrospective case series reporting the outcome of 44 consecutive pregnancies followed by the Toronto Renal Transplant Program. Follow-up data were gathered on the 32 live born children by either a return visit to the clinic or by telephone interview. Medical, as well as developmental information, was gathered on all children and the study group was compared to controls, matched for maternal age (+/-2 years) and smoking status, obtained through the Motherisk Program. RESULTS: Of the 44 pregnancies followed by us, there were 32 live-born children delivered by 26 mothers and 12 stillborn/abortuses. Twenty-six pregnancies were treated with cyclosporine, azathioprine and prednisone, 13 with azathioprine and prednisone and five with cyclosporine and prednisone. The mean gestational age at delivery in the study group was 36.5 +/- 2.7 weeks compared to 40.2 +/- 1.6 weeks in the control group (P < 0.001). The mean birthweight in the study group was 2.54 +/- 0.67 kg, compared to 3.59 +/- 0.53 kg in the control group (P < 0.0001). In the study group there was one child with multiple anomalies and four stillbirths compared to zero in the control group. There were also six spontaneous abortions and two therapeutic abortions in the study group. On follow-up (from 3 months to 11 years of age) there was one child with insulin-dependent diabetes mellitus, two children with asthma and one child with recurrent otitis media. Developmental follow-up revealed one child with moderate to severe sensorineural hearing loss, one child with a learning disability and one child with pervasive developmental disorder. In none of these cases were there signs of perinatal asphyxia. CONCLUSION: There are significantly more stillbirths, preterm deliveries and increased incidence of low birth weight in the transplant group. Most pregnancies in the study group went well, however, and their offspring had normal postnatal growth and development. Further studies with long-term pediatric follow-up are needed to delineate their outcome and rule out possible long term effects of the immunosuppressive medication on their growth, development, reproduction and general health.

Abortion, Spontaneous↗

Communication and its disorders.

This article defines communication and describes its various disorders. Some of these disorders are associated with other DSM-IV conditions, such as mental retardation or pervasive developmental disorder. Others are specific to the language-learning process. The interactions between communication and psychiatric disorders are discussed. Suggestions for integrating treating approaches among communication disorders and mental health professionals are presented.

Child↗

The relevance of infant psychiatry to the understanding of adult psychopathology.

This paper examines the relevance recent findings in Infant Psychiatry may have for the understanding of adult psychopathology. For example, evidence of both continuity and discontinuity within development is cited as one way in which the continuity of specific psychiatric conditions from childhood to adulthood can be understood. Another example are the aims of Developmental Psychopathologists who examine the mechanisms which underlie developmental transformations and who try to differentiate variations seen in normal individuals from disorders. The paper ends by citing the advantages Infancy as a developmental period has in providing reference points for the understanding of cohesion within development. These are: 1. The rapid rate of change seen in children during this age period. This allows the observation of many developmental transformation and reorganizations. 2. The degree to which infants document how much we can shape our own environment. 3. The pervasive importance interpersonal relationships have for any human activity. 4. The need to understand development and hence change within the context of the environment. 5. The persuasive evidence which suggests that psychological disorders are generally based on real rather than imagined adverse events.

Adolescent↗

Human figure drawing test: validity in assessing intelligence in children aged 3-10 years.

BACKGROUND: Human-figure-drawing abilities are related with cognitive development in children. As cognitive skills progress, drawing abilities also improve in details and sex differentiation. The Goodenough-Harris (G-H) drawing test was developed to score human figure drawing with separate norms for males and females. OBJECTIVES: To evaluate whether the Goodenough-Harris human drawing test is valid to classify intelligence in children aged 3-10 years. MATERIAL AND METHOD: Record files of 528 children aged between 3-10 years who had attended the Child and Adolescent Unit at Queen Sirikit National Institute of Child Health (Children's Hospital), Bangkok, Thailand from January 1999 to December 2001 were retrospectively reviewed. Inclusion criteria included: 1) performing the human figure drawing test and standard intelligence tests on the same day and 2) diagnoses were addressed. RESULTS: The ages of the children ranged from 3 10/12 years to 10 11/12 years with a mean age of 7 9/12 years, 49.5 per cent were males and 50.5 per cent were female. The study group was diagnosed as pervasive development disorder 17 per cent, attention deficit hyperactivity disorder 13 per cent, and mental retardation 11.3 per cent. Overall correlation of full scale intellectual quotient (FSIQ) from the standard intelligence test and standard scores on the Goodenough-Harris system was 0.813 (p < 0.01). The overall validity of the human figure drawing test in classified correct intellectual level was 60.8 per cent but in children with an intellectual quotient (IQ) less than 70, the correct classification was 69.2 per cent. After stratification by age, it was found that the human figure drawing test had validity in predicting IQ below 70 in 88.7 per cent and 68.8 per cent of children aged < 6 years and aged > 6 years respectively. CONCLUSION: The human figure drawing test can be used as an additional measure of assessing intelligence in young children but it should not be substituted for standard tests. The test is not complicated, therefore, trained personnel can use it in combination with other screening tests for cognitive development in children.

Art↗

The emergence of repair strategies in infants and toddlers.

This article describes the emergence of repair behaviors in young children as a reflection of three significant developmental achievements: the emergence of communicative intentionality, the development of socioemotional perspective taking, and the acquisition of effective communicative means. Because research in the emergence of preverbal communicative repairs is limited, a cross-sectional study was conducted on the ontogeny of repair strategies using the normative samples from the Communication and Symbolic Behavior Scales (Wetherby & Prizant, 1993). Patterns of early repair behaviors of typically developing children, as well as those of small groups of children with hearing impairments and pervasive developmental disorders, are presented. Findings of this study suggest that the ability to repair communicative breakdowns develops at the same time as intentional communication and that gestures are integral to preconversational repairs. Assessment and intervention implications for clinical practice are discussed.

Child Language↗

Autism and developmental receptive language disorder--a follow-up comparison in early adult life. II: Social, behavioural, and psychiatric outcomes.

This paper focuses on general social functioning in two groups of young men, one with autism and one with developmental receptive language disorders, who were first assessed at the ages of 7-8 years. At that time, although matched for nonverbal IQ (mean 92-93) and expressive language, the Language group showed significantly fewer social and behavioural problems. At follow-up, when aged on average, 23 to 24 years, the Autism group continued to show significantly more impairments in terms of stereotyped behaviour patterns, social relationships, jobs, and independence. However, problems in all these areas were also common in the Language group. Many still lived with their parents, few had close friends or permanent jobs, and ratings of social interaction indicated abnormalities in a number of different areas. On a composite measure of social competence only 10% of the Language group was assessed as having severe social difficulties compared to 74% of the Autism group. Nevertheless, 65% were rated as having moderate social problems and only 25% were rated as being of near/normal social functioning. Two individuals in the Language group, but none in the Autism group, had also developed a florid paranoid psychosis in late adolescence. As in the follow-up of cognitive and linguistic functioning (see Mawhood et al., 2000, this volume, pp. 547-559), discriminant function analysis, which had clearly distinguished between the groups as children, now showed much greater overlap between them. Regression analysis indicated that although early language ability appeared to be related to outcome in the Autism group, there was little association between any measures of childhood functioning and prognosis in the Language group. Theoretically, these findings have implications for our understanding of the nature of autism and other pervasive language disorders, and of the relationship between them. Practically, they demonstrate the very persistent problems experienced by individuals with developmental language disorders, and their need for much greater help and support than is presently available.

Activities of Daily Living↗