Five-year follow-up of prematurely born children with postnatally developing caudothalamic cysts.

AIM: To assess the long-term developmental outcome of very low birth weight children with postnatally developing caudothalamic cysts. METHODS: Five very low birth weight children with postnatal caudothalamic cysts were examined using cranial ultrasound and brain Magnetic Resonance Imaging as neonates, the Bayley Scales of Infant Development, 2nd edition, and the Hammersmith Infant Neurological Examination at 2 years of corrected age, and with the Wechsler Preschool and Primary Scale of Intelligence-Revised and the standardization version of NEPSY II at 5 years of age. The Magnetic Resonance Imaging of the brain was repeated at 5 years of age. The developmental outcome at 5 years of age was compared with that of 23 very low birth weight children with normal brain structure. RESULTS: A cognitive level below normal and/or neuropsychological impairments was seen in all the children with caudothalamic cysts as well as in those with normal brain structure. CONCLUSION: Very low birth weight children with postnatally developing caudothalamic cysts had cognitive and neuropsychological impairments similar to very low birth weight children without such cysts.

Introduction to the Special Issue on Normal Neuropsychological Development in the School-Age Years.

In neuropsychological research on normal development relatively little interest has been devoted to age-related changes in school age. This is surprising in light of the significance of the changes that take place in this period of life, the availability of normative data, and the ongoing research on neural development. The studies included in this issue take a closer look at normative data from school-age children of various ages in performances of attention, language, sensorimotor functions, perceptual functions, memory and learning, and functional asymmetries. A finding evident in many of the studies is that age effects seem to be more accentuated below 9 to 10 years than after that age. It is hoped that this special issue will draw attention to the scarcity of data in this realm and to the possibilities of utilizing existing databases for study on normal development in school age.

Effects of age on neurocognitive measures of children ages 5 to 12: a cross-sectional study on 800 children from the United States.

The standardization of the NEPSY-A Developmental Neuropsychological Assessment (Korkman, Kirk, & Kemp, 1998) provided an opportunity to study the effects of age across the age range 5 to 12 years. Test scores of 800 children on 20 subtests of NEPSY were analyzed. These measures are based on traditions of neuropsychological assessment and are thought to reflect attention and executive functions, language, sensorimotor functions, visuospatial functions, and memory and learning. The effects of age were very significant on all measures, confirming the developmental sensitivity of the NEPSY. The effects of age were more significant in the 5- to 8-year age range than in the 9- to 12-year range. Only performance on tasks of fluency and memory span for sentences showed significant age effects in the 10- to 12-year age range. This suggests that neurocognitive development is rapid in the 5- to 8-year age range and more moderate in the 9- to 12-year age range.

Differential development of attention and executive functions in 3- to 12-year-old Finnish children.

The developmental sequence of attention and executive functions (EFs) was studied by utilizing normative data from four hundred 3- through 12-year-old Finnish children. Data from 10 subtests measuring impulse control and inhibition of irrelevant responses, auditory and visual attention, visual search, planning, and verbal and visual fluency were included. The development proceeded sequentially, from motor inhibition and impulse control to functions of selective and sustained attention, and finally to EFs of fluency. Significant relations between gender and development and between parent education and development were found in several subtests. In a factor analysis, inhibition, auditory attention, visual attention, and the EF of fluency clustered into separate factors. The developmental staging and clustering of functions suggests that, although inhibition, attention, and EFs are highly interrelated cognitive functions, their developmental sequences are separate from one another. The development of basic inhibitory functions precedes the development of more complex functions of selective attention, and EFs continue to develop into adolescence.

Clinical significance of test refusal among young children.

The present study describes the incidence of test refusal at neuropsychological assessment, investigates its correlates, and its stability. The participants were 124 children aged 3.5 years whose development has been followed from birth in the Jyväskylä Longitudinal Study of Dyslexia (JLD). The frequency of test refusal on the Finnish version of the NEPSY was analyzed with respect to the children's concurrent and earlier cognitive and language skills, assessed using tests and parental ratings. Refusal during test-taking was found to be relatively common at this age, and high frequency of refusal at an earlier age was associated with similar tendency at a later age. High test refusal was associated with compromised neuropsychological and linguistic test scores. Missing data due to refusal were more common in neuropsychological tasks requiring verbal production. It is concluded that test refusals reflect a child's poor underlying skills and an attempt to avoid failure, rather than noncompliant or oppositional behavior.

Applying Luria's diagnostic principles in the neuropsychological assessment of children.

The first part of this article examines the theoretical justification for applying Luria's approach in the assessment of children. It is concluded that Luria's concepts of functional systems and the principle of specifying primary and secondary deficits may be applied to children. However, the selection of functional components to assess should be based on traditions of child neuropsychology rather than on Luria's assessment of adults. In addition, the tendency for comorbid disorders, mechanisms of neural adaptation to damage, and the prevalent types of brain abnormality in children render brain-behavior relationships more complex in children than in adults. The second part of the article describes how Luria's methods were adapted for use with children. An assessment, NEPSY, was developed by integrating Luria's views with contemporary child neuropsychological traditions. The NEPSY includes 27 homogeneous and psychometrically developed subtests, standardized in the United States and Finland for the age range of 3 to 12 years. The rationale of analyzing disorders of cognitive processes through a comprehensive and systematic assessment of their components, characteristic of Luria's approach, was preserved, but more specific principles of diagnosis were modified. Research findings obtained with a previously published, Finnish NEPSY version are presented.

Magnetoencephalography in presurgical evaluation of children with the Landau-Kleffner syndrome.

PURPOSE: Our aim was (a) to localize the primary epileptogenic cortex for possible multiple subpial transsection in four children with the Landau-Kleffner syndrome (LKS), and (b) to evaluate the impact of magnetoencephalography (MEG) in the localizing process. METHODS: We used EEG to detect the overall epileptiform activity and MEG for selective recording of fissural spikes. The cortical generators of MEG spikes were modeled with dipoles, and their activation order was determined. The voltage distribution, consistent with the earliest MEG sources, was then identified during the course of the patient's EEG spikes to determine the relative timing between stereotypic EEG and MEG spikes and to distinguish the earliest (primary) source area among the secondary ones. RESULTS: In all patients, the earliest spike activity originated in the intrasylvian cortex, spreading in one subject to the contralateral sylvian cortex within 20 ms. Secondary spikes occurred within 10-60 ms in ipsilateral perisylvian, temporooccipital, and parietooccipital areas. A single intrasylvian pacemaker initiated all epileptic activity in two patients, whereas the other two had independent left- and right-hemisphere circuits or focal spikes. MEG source dynamics predicted the results of the methohexital suppression test in two patients and was confirmed by surgery outcome in one patient, in whom all epileptic activity ceased after a small transsection of the sylvian pacemaker. CONCLUSIONS: (a) The intrasylvian cortex is a likely pacemaker of epileptic discharges in LKS, and (b) MEG provides useful presurgical information of the cortical spike dynamics in LKS patients.

Psychological findings in preterm children related to neurologic status and magnetic resonance imaging.

OBJECTIVE: Preterm children experience learning disabilities more often than full-term children, but detailed information on their neuropsychological and neurologic determinants is lacking. We therefore examined these problems more closely and also studied if clinical neurologic examination and/or magnetic resonance imaging (MRI) can be used as tools to screen the preterm children at risk for these problems. METHODS: In a population-based study, the psychological performance of 42 preterm children with a birth weight <1750 g and of their matched controls was assessed at 8 years of age and the findings were then related to clinical neurologic examination and MRI. Learning disabilities of these children, reported by the teachers, were also studied. RESULTS: The cognitive ability of the preterm children, although in the normal range, was significantly lower than that of the control children. They performed particularly poorly in tasks requiring spatial and visuoperceptual abilities, which were associated with the finding of periventricular leukomalacia in MRI, especially with posterior ventricular enlargement. The preterm children with minor neurodevelopmental dysfunction (MND) had the most problems in neuropsychological tests, whereas the clinically healthy preterm children and those with cerebral palsy had fewer problems. The problems of MND children emerged in the domain of attention. They also experienced the most problems at school. CONCLUSIONS: Visuospatial problems were associated with periventricular leukomalacia in MRI, but learning disabilities were most frequent among the preterm children with minor neurologic abnormalities. We recommend closer follow-up of preterm children with MND.

Neuropsychological characteristics of five children with the Landau-Kleffner syndrome: dissociation of auditory and phonological discrimination.

The Landau-Kleffner Syndrome (LKS) is characterized by acquired receptive aphasia and EEG abnormality with onset between the ages of 3 and 8 years. This study presents neuropsychological assessments in 5 children with LKS. The aims were (1) to specify the neuropsychological deficits characteristic of these children; and (2) to clarify the nature of the receptive aphasia by comparing nonverbal and verbal auditory discrimination. Receptive aphasia was present in all children. Retardation, poor motor coordination, hyperkinesia, and conduct problems were frequent but variable. All children exhibited a dissociation between the discrimination of environmental sounds and phonological auditory discrimination, the latter being more impaired than the former. This suggests that the primary deficit of the receptive aphasia is an impairment of auditory phonological discrimination rather than a generalized auditory agnosia.

Neuropsychological consequences of very low birth weight and asphyxia at term: follow-up until school-age.

This prospective, longitudinal study examined neuropsychological consequences of different conditions associated with risks of perinatal asphyxia. Four groups of children, 5 to 9 years of age, were studied: (1) very low birth weight (VLBW) children born small for gestational age (SGA) (n = 34); (2) VLBW children born appropriate for gestational age (AGA) (n = 43); (3) children with signs of birth asphyxia at term (birth asphyxia) (n = 36), and (4) control children (n = 45). Moderately and severely disabled children were excluded. The WISC-R and the NEPSY, a new neuropsychological assessment consisting of attention, language, motor, sensory, visuospatial, and memory subtests, were administered. The VLBW-SGA group had the poorest test results. The VLBW-AGA group was somewhat less impaired, whereas the birth asphyxia group performed at the control group level. Impairment, when present, tended to be diffuse in all groups, affecting psychometric intelligence, naming, visuo-motor performance, tactile finger discrimination, attention, and phonological analysis.

Evidence of altered dominance in children with congenital spastic hemiplegia.

The study aimed at investigating lateralization effects and signs of transfer and crowding in children with congenital lateralized brain damage with the aid of a dichotic listening test, a chimeric test, and verbal and nonverbal neuropsychological tests. Thirty-three children with spastic hemiplegia and 86 control children (age 5.0-12.0 yr) were assessed. Children with left-hemisphere damage (n = 17) were found to have a pathological left-ear advantage for verbal material, and children with right-hemisphere damage (n = 16) were found to have a pathological right visual half-field advantage for visual material. Children with left-hemisphere damage and a left-ear advantage on the dichotic test were also found to have a right visual half-field advantage on the chimeric test, which was regarded as a sign of reversed dominance. No verbal or nonverbal differences emerged between the left-hemisphere and the right-hemisphere damage groups, nor did differences emerge when the children were reclassified by considering children with left hemisphere damage and signs of reversed dominance as having damage to the nondominant hemisphere. It was concluded that although lateralized brain damage may alter the dominance for verbal and visual functions, there is still considerable inter-individual variability with respect to inter- and intrahemispheric neural adjustment to damage. The dichotic and the chimeric tests did not indicate the presence of brain damage accurately, but they indicated the lateralization of damage in children with stated abnormality with a high degree (91.3%) of accuracy.

A new classification of developmental language disorders (DLD).

Eighty children with DLD were examined with 18 language tests, mainly derived from a neuropsychological investigation called NEPSY (NEuroPSYchological Investigation for Children). The children were 6-0 to 7-9 years old and attended kindergarten. The test profiles of the first 40 children, Group 1, were utilized for the elaboration of a classification of DLD. The test profiles were grouped into five subgroups with the aid of a Q-type factor analysis. Then the classification was modified to suit clinical application by collapsing two pairs of subgroups. The resulting categories were called: the Global Subtype, the Specific Dyspraxia Subtype, and the Specific Comprehension Subtype. The classification was validated, first, by a follow-up study. It was predicted that spelling problems would occur in the Global and the Specific Comprehension Subtypes, but not in the Specific Dyspraxia Subtype. At follow-up, 3 years later, the hit rate was found to be 80.5%. In a second validation procedure, the classification was tried out on the 40 children examined later, Group 2. The coverage of the classification was 85%. Five outliers (12.5%) seemed to form a fourth category, called the Specific Dysnomia Subtype. An expressive subtype was not observed.

A comparison of neuropsychological test profiles of children with attention deficit-hyperactivity disorder and/or learning disorder.

The study compared 8-year-old children with pure attention deficit-hyperactivity disorder (ADHD) (n = 21), specific learning disorder (LD) (n = 12), and both (ADHD + LD) (n = 27) on a comprehensive set of neuropsychological measures. The tests were mainly derived from a new neuropsychological instrument, the Neuropsychological Assessment of Children. The children with ADHD were specifically impaired in the control and inhibition of impulses; the children with LD were impaired in phonological awareness, verbal memory span, and storytelling, as well as in verbal IQ. Children with both showed all of these deficiencies; they also had more pervasive attention problems and more visual-motor problems than the two other groups. All groups exhibited impaired performance in tasks of visual-motor precision and name retrieval. The latter finding may involve two different mechanisms, one related to linguistic impairment and possibly contributing to reading and spelling problems, and the other related to attentional problems.

Cognitive impairments at two years of age after prenatal alcohol exposure or perinatal asphyxia.

The purpose of this follow-up study was to assess and describe early cognitive impairments in two-year-old children exposed to alcohol (1) until the second trimester (n = 20), (2) until the third trimester (n = 20), (3) throughout pregnancy (n = 20), (4) children to mothers with preeclampsia (n = 37), (5) children surviving acute birth asphyxia (n = 14), and (6) a normal control group (n = 48). Alcohol exposure throughout pregnancy was found to be associated with impairments in language (mean SD score = -1.3) and visuo-motor development (mean SD score = -2.0). Preeclampsia was related to impairment in visuo-motor development (mean SD score = -1.2) and attention (mean SD score = -0.7). Alcohol exposure until the third trimester was associated with attention deficit alone (mean SD score = -0.9). Alcohol exposure until the second trimester and acute birth asphyxia were not associated with an increased risk of cognitive impairment. The study also showed that neuropsychological test profiles of language, visuo-motor functions and attention may be obtained with the aid of an adapted version of the Bayley Mental Scale and an evaluation of attention.

Mental development of 2-year-old children exposed to alcohol in utero.

In a prospective follow-up study, 60 children exposed to alcohol in utero were assessed by a psychologist (Bayley Mental scale) and a speech therapist (Reynell Verbal Comprehension scale) at a mean age of 27 months. Many mothers had been able to reduce their alcohol consumption during pregnancy, so the children could be divided into those exposed to heavy drinking during the first trimester only (group 1, n = 20), those exposed during the first and second trimesters (group 2, n = 20), and those exposed throughout pregnancy (group 3, n = 20). Forty-eight nonexposed children were examined to set the -2 SD limit for subnormal performance on the Bayley and Reynell tests. No definite effect of alcohol exposure on mental or language development was found in group 1. Children in group 3 scored significantly lower than children in group 1 both on the Bayley Mental scale and on the Reynell Verbal Comprehension scale; delay in language development was seen more often in group 2 than in group 1. The diagnosis of fetal alcohol syndrome was made in seven children (one in group 2 and six in group 3) and the diagnosis of fetal alcohol effects in 13 children (one in group 1, three in group 2, and nine in group 3). Efforts should be made to identify and find proper treatment for women who drink alcohol early in their pregnancies.

A pattern of test findings predicting attention problems at school.

The aim of this study was to identify different types of neuropsychological test profiles that would predict attention problems at school. Forty-six children with mild developmental disorders, among whom a high frequency of attention deficit disorder (ADD) was expected, were examined just before they started school. The tests were mainly drawn from a new assessment, called NEPSY, and corresponded to the various components of attention. The test profiles were grouped with the aid of a Q-type factor analysis into five subgroups. The test profiles of two of the subgroups were suggestive of attention deficits. The predictions were based on difficulties in tests aimed to evaluate impulse control, sustained attention, and selective attention. As such findings were found in both of the subgroups they were collapsed into one. Half a year later the collapsed subgroups were found to have a higher frequency of attention problems at school than the other three subgroups.

Landau-Kleffner syndrome: epileptic activity in the auditory cortex.

The Landau-Kleffner syndrome (LKS) is characterized by electroencephalographic spike discharges and verbal auditory agnosia in previously healthy children. We recorded magnetoencephalographic (MEG) spikes in a patient with LKS, and compared their sources with anatomical information from magnetic resonance imaging. All spikes originated close to the left auditory cortex. The evoked responses were contaminated by spikes in the left auditory area and suppressed in the right--the latter responses recovered when the spikes disappeared. We suggest that unilateral discharges at or near the auditory cortex disrupt auditory discrimination in the affected hemisphere, and lead to suppression of auditory information from the opposite hemisphere, thereby accounting for the two main criteria of LKS.

Prediction of perinatal brain damage by cord plasma vasopressin, erythropoietin, and hypoxanthine values.

For an assessment of whether cord plasma arginine vasopressin, erythropoietin, and hypoxanthine concentrations are predictors of perinatal brain damage, these concentrations were measured in 62 infants born after preeclampsia of pregnancy, 31 acutely asphyxiated infants, and 38 control infants. Follow-up at 2 years included neurologic examination and the determination of a Bayley mental score. Clear abnormality (death, cerebral palsy, or developmental delay) was found in four infants in the preeclampsia group and five in the asphyxia group; slight abnormality was found in 12 and 6 infants, respectively; and no abnormality was found in the remainder. Neither arginine vasopressin values nor hypoxanthine values predicted adverse outcome in either study group. A high erythropoietin level was found in infants born after preeclampsia regardless of outcome: normal outcome (geometric mean (GM), 102; 95% confidence interval [CI], 69 to 153 mU/ml), slightly abnormal outcome (GM, 100; 95% CI, 37 to 270 mU/ml) or clearly abnormal outcome (GM, 84; 95% CI, 19 to 378 mU/ml). However, asphyxiated infants with clearly abnormal outcome had higher erythropoietin values (GM, 67; 95% CI, 33 to 137 mU/ml; p less than 0.05) than the normal infants (GM, 37; 95% CI, 23 to 59 mU/ml). We conclude that a high erythropoietin level after normal pregnancy, but not after preeclampsia, indicates an increased risk for cerebral palsy or death.