Behavioral and electrophysiological indicators of auditory distractibility in children with ADHD and comorbid ODD.

Involuntary switching of attention to distracting sounds was studied by measuring effects of these events on auditory discrimination performance and event-related brain potentials (ERPs) in 6-11-year-old boys with Attention Deficit-Hyperactivity Disorder (ADHD) and comorbid Oppositional Defiant Disorder (ODD) and in age-matched controls. The children were instructed to differentiate between two animal calls by pressing one response button, for example, to a dog bark and another button to a cat mew. These task-relevant sounds were presented from one of two loudspeakers in front of the child, and there were occasional task-irrelevant changes in the sound location, that is, the loudspeaker. In addition, novel sounds (e.g., a sound of hammer, rain, or car horn) unrelated to the task were presented from a loudspeaker behind the child. The percentage of correct responses was lower for target sounds preceded by a novel sound than for targets not preceded by such sound in the ADHD group, but not in the control group. In both groups, a biphasic positive P3a response was observed in ERPs to the novel sounds. The later part of the P3a appeared to continue longer over the frontal scalp areas in the ADHD group than in the controls presumably because a reorienting negativity (RON) ERP response following the P3a was smaller in the ADHD group than in the control group. This suggests that the children with ADHD had problems in reorienting their attention to the current task after a distracting novel sound leading to deterioration of performance in this task. The present study also indicates that children with ADHD and comorbid ODD show same kind of distractibility as found in previous studies for children with ADHD without systematic comorbid ODD.

Brief report: validity of Finnish registry-based diagnoses of autism with the ADI-R.

AIMS: The aim of the study was to explore the validity of registry-based diagnoses of autism in Finland using the Autism Diagnostic Interview - Revised (ADI-R). This study was designed for the Finnish Prenatal Study of Autism and Autism Spectrum Disorders (FIPS-A), an ongoing research project where registry-based diagnoses will be used for epidemiological studies. METHODS: In this small pilot study, a clinical sample of 95 subjects diagnosed with childhood autism or pervasive developmental disorder/pervasive developmental disorder - not otherwise specified (PDD/PDD-NOS) or Asperger's syndrome according to the Finnish Hospital Discharge Register (FHDR) was gathered nationwide. A small control group consisting of siblings without any registered diagnoses of those being examined was also included in the study. Diagnoses were further re-evaluated by interviewing parents with the ADI-R. RESULTS: The mean scores of autistic subjects clearly exceeded cut-off limits for autism on all three ADI-R domains and 96% of the subjects with registered diagnosis of childhood autism fulfilled the criteria based on the instrument as well. CONCLUSION: These results suggest that the validity of Finnish registry-based diagnoses of childhood autism can be considered good. Our findings lay important groundwork for further population- based studies of the aetiology of autism.

Association of DISC1 with autism and Asperger syndrome.

The DISC1 gene at 1q42 has generated considerable interest in various psychiatric diseases, since a balanced translocation interrupting the gene was found to cosegregate with schizophrenia and related mental illnesses in a large Scottish pedigree. To date, linkage and association findings to this locus have been replicated in several study samples ascertained for psychotic disorders. However, the biological function of DISC1 in neuronal development would suggest a potential role for this gene also in other, early onset neuropsychiatric disorders. Here we have addressed the allelic diversity of the DISC1, DISC2 and TRAX genes, clustered in 1q42, in Finnish families ascertained for infantile autism (97 families, n(affected)=138) and Asperger syndrome (29 families, n(affected)=143). We established association between autism and a DISC1 intragenic microsatellite (D1S2709; P=0.004). In addition, evidence for association to Asperger syndrome was observed with an intragenic single nucleotide polymorphism (SNP) of DISC1 (rs1322784; P=0.0058), as well as with a three-SNP haplotype (P=0.0013) overlapping the HEP3 haplotype, that was previously observed to associate with schizophrenia in Finnish families. The strongest associations were obtained with broad diagnostic categories for both disorders and with affected males only, in agreement with the previous sex-dependent effects reported for DISC1. These results would further support the involvement of DISC1 gene also in the etiopathogenesis of early onset neuropsychiatric disorders.

Current incidence and management of children with traumatic head injuries: the Stockholm experience.

PURPOSE: A traumatic head injury is one of the most common causes of morbidity and mortality among children, however few population-based studies in this area have been reported. Therefore, the aim of this study was to evaluate the incidence and management of traumatic head injuries in children at a level-one trauma centre in Stockholm, Sweden. PARTICIPANTS: All children (n = 3168) who visited the emergency department with a history of head injury during 1 year were included. METHOD: The required information was collected retrospectively and the children's medical records were reviewed. RESULTS: The overall incidences of head injury were 865 per 100 000 children with the highest incidence (2379/10(5) children) occurring among children younger than 18 months of age. Twelve per cent (n = 396) were admitted to a hospital ward and CT scans were performed in 13% (n = 412) of all children. During this year, 0.3% required a neurosurgical intervention and only 1% of all children had documentation of a planned follow-up appointment. CONCLUSION: The findings indicate that clinical documentation as a part of the early management in children with a head injury is inconsistent and suffers from lack of valid criteria. Implementation of clinical guidelines during emergency care would help improve subsequent hospital care, as well as the planning of health care services for these children.

Atypical pattern of discriminating sound features in adults with Asperger syndrome as reflected by the mismatch negativity.

Asperger syndrome, which belongs to the autistic spectrum of disorders, is characterized by deficits of social interaction and abnormal perception, like hypo- or hypersensitivity in reacting to sounds and discriminating certain sound features. We determined auditory feature discrimination in adults with Asperger syndrome with the mismatch negativity (MMN), a neural response which is an index of cortical change detection. We recorded MMN for five different sound features (duration, frequency, intensity, location, and gap). Our results suggest hypersensitive auditory change detection in Asperger syndrome, as reflected in the enhanced MMN for deviant sounds with a gap or shorter duration, and speeded MMN elicitation for frequency changes.

Independent replication and initial fine mapping of 3p21-24 in Asperger syndrome.

BACKGROUND: Asperger syndrome is characterised by abnormalities in social interaction as well as repetitive and stereotyped behaviours and interests. The trait is thought to display complex inheritance, but in a subset of families the inheritance resembles the autosomal dominant model. Linkage to 3p14-24 has recently been reported in Asperger syndrome in Finnish families with a maximum multipoint NPL(all) of 3.32 at D3S2432. METHODS: We have replicated linkage findings to 3p21-24 in 12 new extended Asperger syndrome families. Linkage analyses were performed separately for the 12 new families, and linkage and association analyses were also performed jointly with data from the original genome-wide screen. RESULTS: Best two point and multipoint logarithm of the odds (LOD) scores in analyses of both data sets were obtained at D3S2432 (NPL(all) = 3.83) with both subsets of families contributing to linkage. Association analysis of the combined data set produced a trend towards association with D3S2432 and D3S1619. CONCLUSIONS: This study further validates 3q21-24 as a candidate region for Asperger syndrome.

Beneficial effect from a cognitive training programme on children with acquired brain injuries demonstrated in a controlled study.

PRIMARY OBJECTIVE: To test the effectiveness of a cognitive training programme in children and adolescents with attention and memory deficits after acquired brain injury (ABI). RESEARCH DESIGN: Randomized controlled study. PARTICIPANTS: Thirty-eight children with ABI, 9-16 years of age. METHODS AND PROCEDURES: The treatment group trained with the cognitive programme for 30 minutes per day interactively with a teacher or parent for a period of 17 weeks. Children in the control group had a freely chosen interactive activity 30 minutes daily for 17 weeks. Pre- and post-training assessments were made using a neuropsychological test battery. MAIN OUTCOME AND RESULTS: Significant improvements in the majority of neuropsychological tests of sustained and selective attention as well as in memory performance were shown in the treatment group as compared to controls. CONCLUSIONS: The immediate effect of the training programme improved complex attention and memory functions, indicating that this method may be a valuable treatment option for improving cognitive efficiency in children after ABI. On the basis of these results, the next step will be to evaluate long-term effects and further ecological validity.

Management and classification of children with head injury.

BACKGROUND: Traumatic brain injury is the most common cause of morbidity and mortality during childhood. AIM: This study was conducted to describe the primary management and classification of children admitted to the emergency department for head trauma. SERIES: All children (0-15 years) with a history of head injury who were admitted to the outpatient emergency department at a single paediatric trauma centre in the Stockholm region during 1 month were included. RESULTS: There were 242 children (137 girls and 105 boys; mean age 5.3+/-4.4 years) who fulfilled the inclusion criteria. Based on the Scandinavian Head Injury Classification, 73% were classified as minimal, 17% mild and 2% moderate head injury. This classification seemed to be more accurately reflective than the ICD-10-based classification in the management of the children with head trauma.

How do persons with intellectual disability manage in the open labour markets? A follow-up of the Northern Finland 1966 Birth Cohort.

BACKGROUND: The aim was to study how many of the individuals with intellectual disability (ID; IQ < or = 70) in an age cohort were not receiving a disability pension by the age of 34 years and what their life situation was like in terms of employment, education and morbidity. In 2000, the Northern Finland 1966 Birth Cohort (n=12,058 live-born) included 129 individuals with ID. METHOD: The outcome data on employment, education, pensions and morbidity were obtained from national registers. RESULTS: A total of 85.3% (n=110) of all the individuals with ID were on pension, and 66 of them had severe ID (IQ <50) and 44 had mild ID (IQ 50-70). Altogether 99 were drawing a pension because of ID, and 11 had a main diagnosis other than ID in the register of Social Insurance Institution. Nineteen individuals with mild ID were not on disability pension. The educational level of those without pension was low, and all whose occupation was known worked in low-level manual trades in the open labour market. During the past 8 years (1993-2000), their employment rate had been lower and unemployment rate correspondingly higher and unemployment periods longer than those of the reference group (IQ >85 or not measured). As to the morbidity, they had been hospitalized twice more often than those in the reference group and the mean of their hospitalization days was over fourfold. CONCLUSION: More attention should be paid to the vocational education and supported employment services of individuals with ID to help them to manage as independently as possible.

Electrophysiological evidence of enhanced distractibility in ADHD children.

Abnormal involuntary attention leading to enhanced distractibility may account for different behavioral and cognitive problems in children with attention deficit hyperactivity disorder (ADHD). This was investigated in the present experiment by recording event-related brain potentials (ERPs) to distracting novel sounds during performance of a visual discrimination task. The overall performance in the visual task was less accurate in the ADHD children than in the control children, and the ADHD children had a higher number of omitted responses following novel sounds. In both groups, the distracting novel sounds elicited a biphasic P3a ERP component and a subsequent frontal Late Negativity (LN). The early phase of P3a (180-240 ms) had significantly smaller amplitudes over the fronto-central left-hemisphere recording sites in the ADHD children than in the control group presumably due to an overlapping enhanced left-hemisphere dominant negative ERP component elicited in the ADHD group. Moreover, the late phase of P3a (300-350 ms) was significantly larger over the left parietal scalp areas in the ADHD children than in the controls. The LN had a smaller amplitude and shorter latency over the frontal scalp in the ADHD group than in the controls. In conclusion, the ERP and behavioral effects caused by the novel sounds reveal deficient control of involuntary attention in ADHD children that may underlie their abnormal distractibility.

Genome-wide scan for loci of Asperger syndrome.

Asperger syndrome (AS), characterised by inadequate social interaction, lack of empathy and a dependence of routines and rituals, is classified as belonging to the autism spectrum disorders (DSM-IV and ICD-10). Although the prevalence of AS has been estimated to range from 0.3 up to 48.4 per 10 000, the phenotype still remains relatively unrecognised by clinicians. Several reports, including the original description by Hans Asperger (1944), have suggested that AS has a strong genetic component. Here, we have performed a genome-wide scan on Finnish families ascertained for AS with a strictly defined phenotype. In the initial scan, Z(max)>1.5 was observed on nine chromosomal regions, 1q21-22, 3p14-24, 3q25-27, 4p14, 4q32, 6p25, 6q16, 13q31-33 and 18p11. In the fine mapping stage, the highest two-point LOD scores were observed on chromosomes 1q21-22 (D1S484, Z(max dom)=3.58), 3p14-24 (D3S2432, Z(max dom)=2.50) and 13q31-33 (D13S793, Z(max dom)=1.59). The loci on 1q21-22 and 3p14-24 overlap with previously published autism susceptibility loci, and the loci on 1q21-22 and 13q31-33 overlap with the reported schizophrenia susceptibility loci. The present study is the first genome-wide screen in AS and therefore replication data sets are needed to evaluate further the significance of the AS-loci identified here.

Attention and memory training in children with acquired brain injuries.

AIM: To test the feasibility of the Amsterdam memory and attention training for children (Amat-c) in Swedish children with acquired brain damage. METHODS: Amat-c consists of structured exercises in specific attention and memory techniques. Three Swedish children aged 9-16 y with acquired brain injuries and related memory and attention deficits trained with the Amat-c method for half an hour a day in school or at home interactively with a teacher or parent for a period of 20 wk. RESULTS: All children and their coaches completed the training without interruption. The results showed an improvement in several neuropsychological tests of sustained and selective attention as well as in memory performance. Questionnaires filled in by parents and teachers indicate that, using the Amat-c method, the children learned strategies that improved their school achievement and self-image. CONCLUSIONS: The Amat-c is a valuable treatment option for improving cognitive efficiency in children with acquired brain injuries. The results indicate improved performance in several psychometric measurements. On the basis of these results, the second step will be to modify the complexity and duration of the method, as well as to integrate a reward system before further evaluating the efficacy in a larger controlled study.

Follow-up of children with cerebral palsy after selective posterior rhizotomy with intensive physiotherapy or physiotherapy alone.

In all 21 children with spastic cerebral palsy (CP) underwent surgery involving selective posterior rhizotomy (SPR), followed by six months intensive physiotherapy (PT). Neurological and physiotherapeutic assessments were made one, three and five years after the operation. The children undergoing surgery were compared to 21 comparison children who took part in a regular physiotherapy programme during the same time period. At the preoperative assessment, the children undergoing surgery were similar to the comparative children in terms of age, sex, type of CP, spasticity of the legs and mean functional scores. The children were selected for SPR on the basis of more than half a year's arrest of motor development, which was the only significant difference to the comparative group. Motor function was measured using two different methods, the Illinois-St Louis Scale and the Gross Motor Functional Classification System (GMFC). Both groups experienced steady development during the five-year follow-up period and no significant differences were observed in the mean functional scores between the groups. We conclude that this comparative study, like most controlled studies, failed to demonstrate any additional effect of SPR on motor development of children with spastic CP. Nevertheless, SPR may contribute to a resumption of motor development in children with arrested motor development despite vigorous conservative therapy. SPR is therefore justified as treatment in selected cases.

A quantitative controlled MRI study of the brain in 28 persons with Asperger syndrome.

BACKGROUND: As structural brain abnormalities have been reported in infantile autism, the aim of this study was to determine whether such findings also exist in Asperger Syndrome (AS). METHODS: The diagnosis of Asperger Syndrome was based on the criteria in ICD-10 and DSM-IV. Brain magnetic resonance imaging (MRI) was performed with a 1.5 T imager. T2-weighted axial and coronal slices and T1-weighted three dimensional sagittal slices were obtained and visual and quantitative analysis were performed. SUBJECTS: There were 28 Asperger individuals, 17 children and adolescents (age 6-19 years, mean 12.4 years), 11 adults (age 20-60 years, mean 37. 9 years) and 28 healthy age and gender matched controls. RESULTS: Mild inconsistent alterations were detected in 13/28 of the individuals with Asperger Syndrome compared to 6/23 in the comparable controls. There were no differences between the right and left hemispheres, nor was there any abnormality in terms of myelination or migration. The anterior-posterior diameters of the mesencephalon were statistically significantly shorter in the Asperger syndrome individuals than in the controls. CONCLUSIONS: No consistent focal brain abnormalities for Asperger Syndrome were detected. The reduced diameters of the mesencephalon in the Asperger group support the hypothesis that the mesencephalon may be involved in the pathogenesis of Asperger Syndrome.

Cognitive performance and attachment patterns at four years of age in extremely low birth weight infants after early intervention.

This study aims at assessing the effects of an early occupational therapy intervention on the cognitive development and the development of attachment patterns in ELBW infants. The intervention, given weekly at home from six months to 12 months, aimed at supporting parent-child interaction and enhancing motor control and coordination. The study population consisted of 100 ELBW infants matched in pairs in accordance to their pre-perinatal risk scores and allocated successively to intervention or non-intervention groups. Cognitive development was assessed with the Bayley Scales at age two and with the WPPSI at age four. Attachment to primary caregiver was assessed with the Preschool Assessment of Attachment (PAA). Cognitive performance was within age norms in both groups at both ages. Intervention did not show any effect on cognitive performance at the age of two years. At the age of four years, cognitive level was overall, and most notably for verbal performance, higher in the intervention group than in the control group. There was an over-representation of the so-called atypical attachment patterns (those not fitting the normative A, B, or C categories) in the control group. The results are discussed in terms of finding more global ways to support the development of at risk pre-term children.

Cognitive development, temperament and behavior at 2 years as indicative of language development at 4 years in pre-term infants.

This study focuses on the early temperamental (TTQ = toddler temperament questionnaire), behavioral (IBR = infant behavior record), and cognitive precursors of impaired language functioning in preschool-age pre-terms infants. The study group consisted of 63 pre-term infants with a mean birth weight of 1246 +/- 437 g born in 1989-1991 in the University Central Hospital of Helsinki. Children with major disabilities (CP or mental retardation) were excluded. At the age of 4 years, 22% showed impaired language function. Logistic regression analysis showed that the Bayley MDI score was the best predictor in identifying an increased risk for language impairment. Behavioral characteristics were more strongly associated with subsequent language impairment than temperament.

Neurological development up to the age of four years of extremely low birthweight infants born in Southern Finland in 1991-94.

A total of 142 infants with birthweights of less than 1000 g were examined at the age of 4 y by a child neurologist, by a neuropsychologist and by an occupational therapist in order to establish the rate of neurological disorders. A total of 57 (40%) of the children had normal neurological development, 52 (37%) had minor neurological disorders, 27 (19%) were diagnosed with cerebral palsy, and 6 (4%) were mentally retarded (IQ <71) with no motor disability. The rate of cerebral palsy was identical with the figure at age 2 y, but the rate of minor neurological disorders increased from 25% to 37%. Compared with international rates, we found a higher rate of cerebral palsy, but the incidence of minor neurological disorders corresponded with figures published previously.

Management of the upper limb with botulinum toxin type A in children with spastic type cerebral palsy and acquired brain injury: clinical implications.

The aim of this article is to describe our clinical experience in treating muscle imbalance in 49 children with spastic upper extremity involvement. We discuss four cohorts of children treated with botulinum toxin type A (BTX-A), each with different treatment objectives. In the first group, 27 children were treated for functional improvement and, of these, 23 had a positive effect, while four had no objective benefit. In the second group, eight children were treated for purposes of presurgical planning; of these, four were referred for surgery, three continued with serial treatment and one child did not benefit from injection. The third group comprised six children who were treated to improve posture and care: in this group, four children demonstrated clear benefit and two children lost some function subsequent to injection. Finally, a fourth group of seven children were treated after acquired brain injury (three with severe tetraplegia, four with hemiplegia). In this group, all children experienced spasticity relaxation and two children with hemiplegia also gained functional benefit. In terms of adverse events, deterioration of upper extremity function was poorly tolerated but limited to the first 1--3 weeks postinjection. Grip strength or thumb grip were diminished if too high doses were used. Overall, our results with BTX-A were rewarding in children with no fixed contracture, good motor learning capacity and high motivation to train. Additionally, BTX-A treatment has proven valuable for counteracting spasticity in children with acquired brain injury. This treatment modality may not, however, be an appropriate treatment option for all children with severe upper extremity spasticity, due to the shorter duration of effect and the potential reduction in functional abilities seen in this cohort. In all cases, the selection of muscles to be treated needs careful clinical assessment. Dynamic EMG analysis should be performed whenever required to aid muscle selection, especially in children with spasticity combined with dystonia. Evaluation of M-responses suggests that for the forearm muscles, doses of BTX-A above 1.5 U/kg/muscle should not be used.

Teamwork in Swedish neuropaediatric habilitation.

Team collaboration in Swedish paediatric habilitation was analysed by means of a nation-wide sample of 202 professionals, representing all seven professions normally working in these teams. Provision of paediatric habilitation services as a part of the county secondary level health care is required by law, and has gradually been built up from the 1950s. The study focused on professional cooperation and interaction during different types of formal team meetings. The basic obstacles for the meetings were lack of time, poor meeting technique, and too large a group. The professionals appeared to interact on a fairly equal basis, able to challenge other's views and gaining a hearing for their own views, with the physiotherapists and the paediatricians perceived as being the most dominant team members. The perceived significance of the contribution from the different team members' dominance varied according to the age of the child and to changing needs. The prerequisites for interdisciplinary team working exist as far as meeting structure and a 'democratic' pattern of communication during meetings.

Botulinum toxin injection as an adjunct when planning hand surgery in children with spastic hemiplegia.

The usefulness of botulinum toxin A treatment when planning hand surgery in eight children with spastic hemiplegia was evaluated. The hand function of the children was assessed before and after treatment using a test battery consisting of quantitative and qualitative functional assessment. The results of preoperative botulinum treatment supported surgical intervention in four children and serial botulinum treatment in three children. In one child, the preoperative botulinum treatment provided no additional information. We conclude that preoperative botulinum A treatment in most children with spastic hemiplegia, for whom hand surgery is being considered, identifies the patients who would not benefit from the planned surgery or for whom the functional benefit would probably not outweigh the burden of surgical procedure and postoperative rehabilitation.