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Head circumference in autism and other pervasive developmental disorders.

Recent studies have found that an unexpectedly large proportion of autistic children have large heads. Anthropometric measures of consecutive clinic attenders with pervasive developmental disorder (PDD), other psychiatric or language disorders were analysed. Similar data were obtained from two schools for language disordered children. These data, combined with those from previous studies, indicate that about one-third of children with PDD have macrocephaly based on current percentile charts; this rate was significantly higher than in children with language disorder alone. The finding was not a consequence of recognizable medical disorders and suggests that PDD is sometimes associated with abnormal physical development.

Anthropometry↗

Risperidone in the treatment of disruptive behavioral symptoms in children with autistic and other pervasive developmental disorders.

OBJECTIVE: To investigate the efficacy and safety of risperidone for the treatment of disruptive behavioral symptoms in children with autism and other pervasive developmental disorders (PDD). METHODS: In this 8-week, randomized, double-blind, placebo-controlled trial, risperidone/placebo solution (0.01-0.06 mg/kg/day) was administered to 79 children who were aged 5 to 12 years and had PDD. Behavioral symptoms were assessed using the Aberrant Behavior Checklist (ABC), Nisonger Child Behavior Rating Form, and Clinical Global Impression-Change. Safety assessments included vital signs, electrocardiogram, extrapyramidal symptoms, adverse events, and laboratory tests. RESULTS: Subjects who were taking risperidone (mean dosage: 0.04 mg/kg/day; 1.17 mg/day) experienced a significantly greater mean decrease on the irritability subscale of the ABC (primary endpoint) compared with those who were taking placebo. By study endpoint, risperidone-treated subjects exhibited a 64% improvement over baseline in the irritability score almost double that of placebo-treated subjects (31%). Risperidone-treated subjects also exhibited significantly greater decreases on the other 4 subscales of the ABC; on the conduct problem, insecure/anxious, hyperactive, and overly sensitive subscales of the Nisonger Child Behavior Rating Form (parent version); and on the Visual Analog Scale of the most troublesome symptom. More risperidone-treated subjects (87%) showed global improvement in their condition compared with the placebo group (40%). Somnolence, the most frequently reported adverse event, was noted in 72.5% versus 7.7% of subjects (risperidone vs placebo) and seemed manageable with dose/dose-schedule modification. Risperidone-treated subjects experienced statistically significantly greater increases in weight (2.7 vs 1.0 kg), pulse rate, and systolic blood pressure. Extrapyramidal symptoms scores were comparable between groups. CONCLUSIONS: Risperidone was well tolerated and efficacious in treating behavioral symptoms associated with PDD in children.

Aggression↗

[Characteristics of juvenile court cases with pervasive developmental disorder].

Forty-eight juvenile delinquency cases, who were sent to a family court between April, 2002 and March, 2005, and diagnosed with Pervasive Developmental Disorder (PDD), were examined regarding the following characteristics: (1) demographic, cognitive and social profiles (sex, age, intellectual development, school register), (2) diagnostic subclasses of PDD, and (3) types (classification), causes, and backgrounds of delinquency. While the PDD group revealed no noticeable differences in the distributions of sex, age, school register status, and intellectual abilities, compared with a general delinquent juvenile population, the PDD group showed a much higher rate of sexual offences and antisocial behaviors based on the confusion due to limited interpersonal abilities. Forty-two (87.5%) were diagnosed with Asperger's disorder, with an IQ within the normal range. In more than half of the cases, offensive conducts occurred in their inappropriate a Hemprs to approach others, caused by the underdevelopment of social abilities. In other cases, the primary handicap and associated symptoms of PDD, as well as the obsessive pursuit of a scientific/experimental interest, played some role in the development of delinquency. In dealing with delinquent cases of juveniles with PDD, it seems very important to keep in mind the characteristics of PDD, and cooperate with professionals and organizations of other fields related to juvenile mental health, such as child psychiatrists, social welfare facilities, and institutions of the Ministry of Justice (juvenile detention home, juvenile training school, and probationary office).

Adolescent↗

Does pervasive developmental disorder protect children and adolescents against drug and alcohol use?

AIM: The aim of this paper is (1) to compare the rates of reported drug and/or alcohol use (DAU) in treatment seeking adolescents with Pervasive Developmental Disorders (PDD), and those with other psychiatric diagnoses (psychiatric controls) seen in tertiary child and adolescent mental health services and (2) to explore the relationship of psychopathology, environmental stressors, and social communication difficulties to DAU. METHOD: Data from the chart review of children and adolescents aged between 12 and 18 years with psychiatric diagnosis seen in tertiary child and adolescent mental health services between 1992 and 2001 (n=1484) was used to investigate the relationship between DAU, psychopathology, environmental stressors and items related to social communication. RESULTS: A total of 97 subjects (7%) met criteria for PDD. Subjects with PDD report significantly lower DAU than psychiatric controls, 3% vs. 17% respectively (P<0.000). Factors reflecting PDD such as speech and language difficulties, developmental difficulties, discordant peer relationships in adolescents are negatively associated with DAU, while conduct problems, affective symptoms, inadequate parental supervision or control have positive association with DAU. DAU was present in PDD only when comorbid with Attention Deficit Hyperactivity Disorder (ADHD). CONCLUSION: Adolescents with speech and language difficulties, developmental difficulties and discordant peer relationships, all reflecting PDD, are less likely to have DAU.

Adolescent↗

A genome-wide search for alleles and haplotypes associated with autism and related pervasive developmental disorders on the Faroe Islands.

The involvement of genetic factors in the etiology of autism has been clearly established. We undertook a genome-wide search for regions containing susceptibility genes for autism in 12 subjects with childhood autism and related pervasive developmental disorders (PDDs) and 44 controls from the relatively isolated population of the Faroe Islands. In total, 601 microsatellite markers distributed throughout the human genome with an average distance of 5.80 cM were genotyped, including 502 markers in the initial scan. The Faroese population structure and genetic relatedness of cases and controls were also evaluated. Based on a combined approach, including an assumption-free test as implemented in CLUMP, Fisher's exact test for specific alleles and haplotypes, and IBD(0) probability calculations, we found association between autism and microsatellite markers in regions on 2q, 3p, 6q, 15q, 16p, and 18q. The most significant finding was on 3p25.3 (P(T1)=0.00003 and P(T4)=0.00007), which was also supported by other genetic studies. Furthermore, no evidence of population substructure was found, and a higher degree of relatedness among cases could not be detected, decreasing the risk of inflated P-values. Our data suggest that markers in these regions are in linkage disequilibrium with genes involved in the etiology of autism, and we hypothesize susceptibility genes for autism and related PDDs to be localized within these regions.

Adolescent↗

The PDD Behavior Inventory: a rating scale for assessing response to intervention in children with pervasive developmental disorder.

The PDD Behavior Inventory (PDDBI) is a rating scale filled out by caregivers or teachers that was designed to assess children having a Pervasive Developmental Disorder (PDD; autism, Asperger disorder, PDD-NOS, or childhood disintegrative disorder). Both adaptive and maladaptive behaviors are assessed in the scale, making it useful for treatment studies in which decreases in maladaptive behaviors and improvements in adaptive social and language skills relevant to PDD are expected. The adaptive behaviors assessed include core features of the disorder such as joint attention skills, pretend play, and referential gesture. The maladaptive behaviors sample a wide variety of behaviors observed in both lower- and higher-functioning individuals and include stereotyped behaviors, fears, aggression, social interaction deficits, and aberrant language. The inventory was found to have a high degree of internal consistency. Inter-rater reliability was better for adaptive behaviors than for maladaptive behaviors. Factor analyses confirmed the structure of the PDDBI and indicated good construct validity. In a subsample of children between 3 and 6 years of age, raw scores for adaptive behaviors increased with age in the parent and teacher versions, as did measures of social pragmatic problems. It was concluded that the PDDBI is both reliable and valid and is useful in providing information not typically available in most instruments used to assess children with PDD.

Adolescent↗

The use of intonation to communicate in pervasive developmental disorders.

The objective of this paper was to employ a functional linguistic approach to explore pragmatic failure in the spontaneous speech of subjects with pervasive developmental disorders (PDD). Patterns of intonation use were compared among subjects with Asperger's syndrome (AS), high-functioning autism (HFA), and psychiatric out-patient controls (OPC) with a variety of non-specific social problems. Written transcripts and audio-recordings were used to measure rates of various intonation types relative to the amount of speech produced. The major finding of the study was that the HFA subjects less often tend to employ useful patterns of intonation for communication than the AS or OPC groups. This suggests that HFA either send random intonation signals to hearers or else demonstrate systematic misuse of the linguistic system. AS subjects differed little from the controls. The implications of these results for understanding the communicative failure of PDD subjects is discussed.

Adolescent↗

A retrospective analysis of quetiapine in the treatment of pervasive developmental disorders.

BACKGROUND: The purpose of this study was to examine the effectiveness and tolerability of quetiapine for aggression, hyperactivity, and self-injury in pervasive developmental disorders (PDDs). METHOD: The medical records of all patients with PDDs diagnosed according to DSM-IV criteria and treated with quetiapine were retrospectively reviewed. Patients who received quetiapine for at least 4 weeks and who were not concurrently treated with another antipsychotic or mood stabilizer were included. Improvement was measured with the Clinical Global Impressions-Improvement scale (CGI-I), with response determined by ratings of "much improved" or "very much improved." Data were collected from May 15, 2003 through November 30, 2003. RESULTS: Of 857 records reviewed, 20 patients (16 male, 4 female) (mean +/- SD age = 12.1 +/- 6.7 years; range, 5-28 years) received a quetiapine trial (mean +/- SD dosage = 248.7 +/- 198.4 mg/day; range, 25-600 mg/day) over a mean duration of 59.8 +/- 55.1 weeks (range, 4-180 weeks). Eight (40%) of 20 patients were judged "responders" to quetiapine; the mean CGI-I score for the entire group was 3.0 +/- 1.1 (minimally improved). A statistically significant improvement (p = .002) was found between a mean pretrial CGI-Severity of Illness scale (CGI-S) score of 5.1 +/- 0.6 (markedly ill) and a posttrial CGI-S score of 4.2 +/- 1.1 (moderately ill). Adverse effects occurred in 50% (N = 10) of patients and led to drug discontinuation in 15% (N = 3) of patients. CONCLUSION: Quetiapine was modestly effective for maladaptive behavior in patients with a PDD. Controlled studies are needed to further assess these preliminary findings.

Adolescent↗

[A clinical study of pyridoxine treatment for pervasive developmental disorders with hypersensitivity to sound].

In our earlier study we conducted a randomized, placebo-controlled, triple blind trial, and showed that pyridoxine treatment of pervasive developmental disorders with expressive verbal disorders and hypersensitivity to sound as significantly effective in improving VIQ scores. This study analyzed the long-term changes of the subjects. We confirmed that their VIQ scores improved over time. An analysis of the reports of their daily life provided by their parents and teachers showed that the children's hypersensitivity to sound was also improved. They became able to adjust to their daily life at home and school without any panic. Our results indicate there are PDD subgroups whose expressive language capabilities and hypersensitivity to sound can be improved by pyridoxine treatment.

Adolescent↗

Pervasive developmental disorders in preschool children.

CONTEXT: Prevalence rates of autism-spectrum disorders are uncertain, and speculation that their incidence is increasing continues to cause concern. OBJECTIVE: To estimate the prevalence of pervasive developmental disorders (PDDs) in a geographically defined population of preschool children. DESIGN, SETTING, AND PARTICIPANTS: Survey conducted July 1998 to June 1999 in Staffordshire, England. The area's 15 500 children aged 2.5 to 6.5 years were screened for developmental problems. Children with symptoms suggestive of a PDD were intensively assessed by a multidisciplinary team, which conducted standardized diagnostic interviews and administered psychometric tests. MAIN OUTCOME MEASURE: Prevalence estimates for subtypes of PDDs. RESULTS: A total of 97 children (79.4% male) were confirmed to have a PDD. The prevalence of PDDs was estimated to be 62.6 (95% confidence interval, 50.8-76.3) per 10 000 children. Prevalences were 16.8 per 10 000 for autistic disorder and 45.8 per 10 000 for other PDDs. The mean age at diagnosis was 41 months, and 81% were originally referred by health visitors (nurse specialists). Of the 97 children with a PDD, 25.8% had some degree of mental retardation and 9.3% had an associated medical condition. CONCLUSIONS: Our results suggest that rates of PDD are higher than previously reported. Methodological limitations in existing epidemiological investigations preclude interpretation of recent high rates as indicative of increased incidence of these disorders although this hypothesis requires further rigorous testing. Attention is nevertheless drawn to the important needs of a substantial minority of preschool children.

Autistic Disorder↗

Vestibular hyporeactivity in infants at risk for schizophrenia. Association with critical developmental disorders.

Vestibular responses to caloric stimulation were measured from birth to age 2 years in ten infants born to schizophrenic mothers. This is part of a study of evolving neurointegrative disorders that may be associated with a genetic risk for schizophrenia. Transiently decreased vestibular responses coincided with several developmental disorders that were related to psychopathology at 10 years. Absent to decreased responses were associated with (1) a "pandevelopmental retardation" involving physical growth as well as postural-motor and visual-motor development, (2) an "abnormally quiet" state in the first month, and (3) failures of bimanual integration between 4 and 6 months. The transitory nature of the decreased nystagmus rules out the possibility of an organic lesion of the vestibular system. Rather, it suggests that some covert decrease in arousal accompanied those periods when central nervous system integration was disrupted.

Arousal↗

[Developmental disorder in girls due to Rett syndrome].

Three girls with Rett syndrome are presented. Patients A and B had initially exhibited normal development, patient C showed severe developmental delay from birth on. In all three stereotypical hand movements arose which led to Rett syndrome being suspected. For patients A and B the clinical diagnosis was further supported by the identification of mutations in the MECP2-gene. In patient C, the mutation found turned out to be a neutral variant. Rett syndrome is a X-linked developmental disorder, which is particularly prevalent in girls. In 70-90% of clinically diagnosed RS patients a mutation is detected. MECP2-mutations result in a far wider range of phenotypes than classic RS. Mutations of this gene also occur in boys, with or without Rett-syndrome type phenotypes.

Child↗

Personality adjustment and social support of parents who care for children with pervasive developmental disorders.

The goal of this study was to examine the degree of personality adjustment and perceived social support of parents who care for children with pervasive developmental disorders. Data were collected with the California Q-Set (a personality measure) and the Norbeck Social Support Questionnaire. Personality adjustment of the parents was found to be significantly less than optimal, with a high degree of anxiety, minimal ego resilience, and use of defensive coping processes. Siblings of the child with the disorder and mothers of the parents were those most frequently identified as providing support to the caregiver, although friends, special education teachers, and daycare staff were the individuals whose support was most significantly related to greater amounts of perceived affection and affirmation, tangible aid, and concrete caregiving assistance. In fact, the more the caregivers depended upon their families for support, the less well adjusted were the caregivers on the personality measure. The implications of the findings for caregivers and their families are discussed.

Adaptation, Psychological↗

Mice as models of human developmental disorders: natural and artificial mutants.

Over the past year, the mouse has been used as a model to make significant contributions towards our understanding of human developmental disorders. The existence of both natural and artificial mouse mutants has not only facilitated the identification of mutations and provided candidate genes for human disorders but has also increased our knowledge regarding the cellular and molecular processes involved in normal mammalian embryogenesis.

Animals↗

Guanfacine treatment of hyperactivity and inattention in pervasive developmental disorders: a retrospective analysis of 80 cases.

OBJECTIVE: The aim of this study was to retrospectively review a large sample of children and adolescents with pervasive developmental disorders (PDDs) treated with open-label guanfacine in order to gather preliminary data as to its effectiveness and safety. METHOD: Eighty (80) subjects with PDDs (10 females, 70 males) (mean +/- SD age = 7.7 +/- 3.5 years, range 3-18 years) were treated with guanfacine within an academic specialty clinic. Charts were reviewed to determine the response of specific target symptoms, including hyperactivity, inattention, and impulsivity. The relationship between treatment response and age, diagnosis, level of cognitive impairment, and symptom severity was determined. Adverse effects were also evaluated. RESULTS: Guanfacine (mean daily dose = 2.6 +/- 1.7 mg, range 0.25-9 mg; mean duration of treatment = 334 +/- 374 days, range 7-1776 days) treatment was effective in 19 of 80 (23.8%) subjects. Subjects with PDD not otherwise specified (11 of 28 responders; 39.3%) and Asperger's disorder (2 of 6 responders; 33.3%) showed a greater rate of global response than those with autistic disorder (6 of 46 responders; 13.0 %). There was a trend for subjects without comorbid mental retardation (9 of 24 subjects; 37.5%) to respond at a greater rate than those with mental retardation (10 of 56 subjects; 17.9%). Symptom improvement was seen in hyperactivity, inattention, insomnia, and tics. Guanfacine was well tolerated, and did not lead to significant changes in blood pressure or heart rate. CONCLUSIONS: Guanfacine may have a role in the treatment of hyperactivity and inattention occurring in some persons with PDDs. Further studies are needed to determine its efficacy in this population.

Adolescent↗

Embryology, Teratology, and arrested developmental disorders in otolaryngology.

The normal embryonic development of the head and neck is reviewed, with emphasis on the branchial derivatives. To understand teratology and the arrested developmental disorders in otolaryngology, a knowledge of normal embryogenesis is important. A brief review of some important congenital anomalies and syndromes is presented.

Branchial Region↗

Visual-proprioceptive mapping in children with developmental coordination disorder.

Developmental coordination disorder (DCD) occurs in a small but significant proportion of children who present with impaired body-eye coordination and show poor acquisition of motor skills. This study investigated the visual-proprioceptive mapping ability of children with DCD from a small selected group, with particular reference to the use of vision in matching tasks. The children with DCD in this study were significantly poorer than control children on all matching tasks. They seemed to have particular difficulty in cross-modal judgements that required the use of visual information to guide proprioceptive judgements of limb position. A distinction is drawn between tasks that can be achieved purely through sensory matching and those that require body-centred spatial judgements, suggesting that it is the latter that posits a particular difficulty for children with DCD.

Case-Control Studies↗

Motor performance and handedness in children with developmental language disorder.

Developmental language disorder (DLD) is diagnosed when there is a failure of normal language development in a child with normal nonverbal intelligence. The discussion about additional or causal deficits is controversial. In this study a computer-based motor performance series with a tapping, aiming and pegboard movement task and an additional paper-pencil handedness test were applied to a group of children with DLD of the phonologic-syntactic subtype and with normal nonverbal intelligence to describe the additional motor problems. Furthermore we examined whether our DLD children showed a different handedness. Tapping and pegboard with both hands were significantly impaired in our DLD children. Overall our DLD children did not show a different handedness than the control group.

Age Factors↗