Magnus Landgren

ADHD, DAMP AND OTHER NEURODEVELOPMENTAL/PSYCHIATRIC DISORDERS IN 6‐YEAR‐OLD CHILDREN: EPIDEMIOLOGY AND CO‐MORBIDITY

A total population of 589 6‐year‐old children were screened for neurodevelopmental/neuro‐psychiatric disorders by questionnaires to parents and preschool teachers, and by examination of motor abilities at the Child Health Center. Fifty screen‐positive and fifty screen‐negative children were assigned for complete neuropsychiatric assessment comprising a detailed history, psychiatric and neurodevelopmental assessment, neuropsychological examination and speech/languâge evaluation. Comprehensive diagnoses were made on the basis of all the available information. In the total population, 63 children (10.7%) with disorders were identified, 10 of whom had a diagnosis established before the study. The prevalence rates for deficits in attention, motor control and perception (DAMP) were 5.3 to 6.9%, for attention deficit hyperactivity disorder (ADHD) they were 2.4 to 4.0% and for mental retardation, 2.5%. Co‐morbidity was established for ADHD on the one hand and DAMP, mental retardation and Tourette syndrome on the other. The findings suggest the need for a school entrant screening examination for the types of problems examined in this study.

Prenatal Alcohol Exposure and Neurodevelopmental Disorders in Children Adopted From Eastern Europe

OBJECTIVES: The purposes of this investigation were to determine the frequencies of and associations between different neurodevelopmental disorders and to study the potential lasting effects of alcohol on children adopted from eastern Europe. METHODS: In a population-based, prospective, observational, multidisciplinary, cross-sectional, cohort study of 71 children adopted from eastern Europe, children were assessed 5 years after adoption, from pediatric, neuropsychological, and ophthalmologic perspectives. RESULTS: Fetal alcohol spectrum disorders, that is, fetal alcohol syndrome (FAS), partial FAS, and alcohol-related neurodevelopmental disorders, were identified for 52% of children; FAS was found for 30%, partial FAS for 14%, and alcohol-related neurodevelopmental disorders for 9%. Alcohol-related birth defects were found for 11% of children, all of whom also were diagnosed as having FAS. Mental retardation or significant cognitive impairment was found for 23% of children, autism for 9%, attention-deficit/hyperactivity disorder for 51%, and developmental coordination disorder for 34%. CONCLUSIONS: Fetal alcohol spectrum disorders and neurodevelopmental disorders were common in this long-term follow-up study of children adopted from orphanages in eastern Europe. Maternal alcohol consumption during pregnancy has long-lasting adverse effects, causing structural, behavioral, and cognitive damage despite a radically improved environment.

Attention deficit disorder with developmental coordination disorders

AIMS To analyse the contribution of certain social, familial, prenatal, perinatal, and developmental background factors in the pathogenesis of deficits in attention, motor control, and perception (DAMP). METHODS A population based case-control study was carried out with 113 children aged 6 years, 62 diagnosed with DAMP and 51 controls without DAMP. The children’s health and medical records were studied and their history with regard to background factors was taken at an interview with the mother using a standardised schedule. Familial factors, possible non-optimal factors during pregnancy (including smoking), developmental factors (including early language development), and medical and psychosocial data were scored in accordance with the reduced optimality method. RESULTS Low socioeconomic class was common in the group with DAMP. Familial language disorder and familial motor clumsiness were found at higher rates in the DAMP group. Neuropathogenic risk factors in utero were also more common in the children with DAMP. Maternal smoking during pregnancy appeared to be an important risk factor. Language problems were present in two thirds of the children with DAMP. Sleep problems and gastrointestinal disorders, but not atopy or otitis media, were significantly more common in the DAMP group. CONCLUSIONS Prenatal familial and neuropathogenic risk factors contribute to the development of DAMP. Primary prevention, such as improved maternal health care and early detection or treatment, or both, of associated language problems appear to be essential.

EPA supplementation improves teacher-rated behaviour and oppositional symptoms in children with ADHD.

Aim:  Measure efficacy of eicosapentaenoic acid (EPA) in children with attention deficit hyperactivity disorder (ADHD).

Delineation and Diagnostic Criteria of Oral-Facial-Digital Syndrome Type VI

Oral-Facial-Digital Syndrome type VI (OFD VI) represents a rare phenotypic subtype of Joubert syndrome and related disorders (JSRD). In the original report polydactyly, oral findings, intellectual disability, and absence of the cerebellar vermis at post-mortem characterized the syndrome. Subsequently, the molar tooth sign (MTS) has been found in patients with OFD VI, prompting the inclusion of OFD VI in JSRD. We studied the clinical, neurodevelopmental, neuroimaging, and genetic findings in a cohort of 16 patients with OFD VI. We derived the following inclusion criteria from the literature: 1) MTS and one oral finding and polydactyly, or 2) MTS and more than one typical oral finding. The OFD VI neuroimaging pattern was found to be more severe than in other JSRD subgroups and includes severe hypoplasia of the cerebellar vermis, hypoplastic and dysplastic cerebellar hemispheres, marked enlargement of the posterior fossa, increased retrocerebellar collection of cerebrospinal fluid, abnormal brainstem, and frequently supratentorial abnormalities that occasionally include characteristic hypothalamic hamartomas. Additionally, two new JSRD neuroimaging findings (ascending superior cerebellar peduncles and fused thalami) have been identified. Tongue hamartomas, additional frenula, upper lip notch, and mesoaxial polydactyly are specific findings in OFD VI, while cleft lip/palate and other types of polydactyly of hands and feet are not specific. Involvement of other organs may include ocular findings, particularly colobomas. The majority of the patients have absent motor development and profound cognitive impairment. In OFD VI, normal cognitive functions are possible, but exceptional. Sequencing of known JSRD genes in most patients failed to detect pathogenetic mutations, therefore the genetic basis of OFD VI remains unknown. Compared with other JSRD subgroups, the neurological findings and impairment of motor development and cognitive functions in OFD VI are significantly worse, suggesting a correlation with the more severe neuroimaging findings. Based on the literature and this study we suggest as diagnostic criteria for OFD VI: MTS and one or more of the following: 1) tongue hamartoma(s) and/or additional frenula and/or upper lip notch; 2) mesoaxial polydactyly of one or more hands or feet; 3) hypothalamic hamartoma.

Parametric control of fingertip forces during precision grip lifts in children with DCD (developmental coordination disorder) and DAMP (deficits in attention motor control and perception)

Twenty boys with developmental coordination disorder (DCD), 11 of whom had associated attention deficit disorder (ADD), were compared with an age-matched control group of 12 boys to examine mechanisms that adapt the grip force at the digit-object interface in a precision grip task. An experimental grip object equipped with pressure transducers registered the grip forces (normal to the surface) and the load force (tangential to the surface) generated by the fingertips. The surface of the object was changed to vary the frictional properties. Both study groups exhibited disturbances of the basic coordination of forces in the initial phase of the movement, manifested by longer time latencies and higher force levels than the control group. All subjects were able to adapt the force output in response to the friction at the digit-object interface. Higher grip forces and safety margins were documented for the DCD group in comparison to the controls. Furthermore, there was greater variation in the parametric control of the grip force in the DCD group. The results suggest that the control of the grip force is similar in children with DCD, regardless of whether they have associated ADD or not, but it is impaired in comparison to that of controls.

Deficits in attention, motor control and perception (DAMP): a simplified school entry examination.

Objective: To suggest an empirically based school entry screening examination for the detection of deficits in attention, motor control and perception (DAMP) in 6‐y‐old children. Material and methods: A population‐based cohort of 113 children, 6‐7 y of age (62 with and 51 without DAMP), compared on measures of attention, motor functions, language and cognition. Results: Attention deficits were convincingly identified by both parents and paediatrician. The motor function tests clearly distinguished between the two groups. Linguistic and meta‐linguistic tests demonstrated greater phonological processing difficulties in the DAMP group. The cognitive test revealed an overall lower IQ but no consistent characteristic pattern in the DAMP group.

Visual function and ocular features in children and adolescents with attention deficit hyperactivity disorder, with and without treatment with stimulants.

AimsTo investigate visual function and ocular features in children with attention deficit hyperactivity disorder (AD/HD) and establish whether treatment with stimulants is reflected in functioning of the visual system.MethodsDetailed ophthalmologic evaluations without and with stimulants were performed in 42 children (37 boys) with AD/HD, mean age 12 years, and compared with a reference group (ref; n=50; mean age 11.9 years; 44 boys). For a comparison between two groups, Mann–Whitneys U-test was used for ordered and continuous variables; for dichotomous variables, Fishers exact test was used. For paired comparison (with and without treatment), sign test was used.ResultsIn all, 83% had visual acuity of >0.8 (<0.1 logMAR) without treatment, 90% with stimulants (ref 98%; P=0.032 and n.s., respectively). Heterophoria was found in 29% without, and in 27% with, stimulants (ref 10%; P=0.038 and n.s., respectively) and subnormal stereovision (>60 s of arc) in 26% (ref 6%; P=0.016) without stimulants, and in 27%, with (P=0.014). Abnormal convergence (>6 cm or absent) was noted in 24% (ref 6%; P=0.031) without treatment and in 17%, with (n.s.). Astigmatism (≥1.0 D) was observed in 24% (ref 6%; P=0.03), and signs of visuoperceptual problems in 21% (ref 2%; P=0.007). We found smaller optic discs (n=8/38) and neuroretinal rim areas (n=7/38) (P<0.0001) and decreased tortuosity of retinal arteries (n=6/34) (P=0.0002) than that of controls.ConclusionsChildren with AD/HD had a high frequency of ophthalmologic findings, which were not significantly improved with stimulants. They presented subtle morphological changes of the optic nerve and retinal vasculature, indicating an early disturbance of the development of these structures.

Health before and after adoption from Eastern Europe

DESIGN A population-based study of pre-adoption, arrival and post-adoption health. AIM To report prenatal and postnatal background factors, morbidity, growth and development in adoptees from Eastern Europe. SUBJECTS AND METHODS All 99 children born in Eastern Europe between 1990 and 1995 and adopted to western Sweden during 1993-1997 were invited to participate in the study. Altogether, 76 (77%) participated. Medical records from the birth countries, from the examination at arrival and from medical reports made during a mean post-adoption period of 5 years were evaluated. RESULTS Low birth weight (< or = 2500 g) occurred in 48%. Congenital malformations were found in 22%. The biological mothers of 33% of the children had been considered alcoholics, and 16% of the childrens mothers had been diagnosed with a psychiatric disability. A high incidence of infectious diseases, neurodevelopment disorders and growth retardation had been noted during the pre-adoption period. Upon arrival in Sweden 75% were diagnosed with a medical condition, most often an infection. After a 5-year post-adoption period, small head circumference was associated with alcohol exposure during pregnancy and 46% had at least one neurodevelopment or behavioural disorder. CONCLUSION Adverse prenatal and perinatal factors, congenital malformations and post-adoption neurodevelopment disorders were common. Adoptees and adopters have complex needs for health support and information.

Golclenhar Syndrome and Autistic Behaviour

Two girls with concomitant Goldenhar syndrome (oculo‐auriculovertebral spectrum disorder) and autistic disorder are described. One was diagnosed as having Goldenhar syndrome in the first few weeks of life and as having autistic disorder in her fifth year; the other was diagnosed as having Goldenhar syndrome when she was referred for evaluation of autistic symptoms at seven years of age. The type of physical abnormalities encountered in Goldenhar syndrome suggests damage to neural structures in the second or late stages of the first trimester. The two cases described in this report suggest that autistic disorder sometimes can result from neural damage during the second trimester.