Neuropsychological Findings in Two Children Diagnosed with Hamartoses: Evidence of a NLD Phenotypic Profile

Hamartoses (HM) are defined as disorders involving nonneoplastic tissue overgrowth. Studies have examined the neuropsychological profiles of children with common HM, such as neurofibromatosis type 1. Limited information is known regarding neuropsychological profiles of rare HM such as Osteochondromatosis Syndrome (OS) and Klippel-Trenaunay Syndrome (KTS). The current investigation is, to our knowledge, the first attempt to define the cognitive phenotypes in two boys with OS and KTS. Results revealed significantly greater impairments involving sensorimotor and visuospatial skills, while verbal memory and language skills appeared relatively preserved. Significant neurobehavioral problems and marked social difficulties were evident. These findings suggest that these syndromes are on a Nonverbal Learning Disorder (NLD) continuum, with varying degrees of severity.     

A Case of Triple X Syndrome Manifesting with the Syndrome of Nonverbal Learning Disabilities

There has been a relative absence of studies that have examined comprehensively the neuropsychological profiles of females with Triple X Syndrome across a battery of tests and measures. A case is reported of a 9 1/2-year-old female with Triple X Syndrome whose neuropsychological test results were suggestive of greater right- versus left-cerebral hemisphere dysfunction. Overall, the patient's neuropsychological profile was indicative of the syndrome of Nonverbal Learning Disabilities (NLD) as proposed by Rourke (Rourke 1987, 1988, 1995; Rourke & Tsatsanis, 1996). The results are discussed in light of Rourke's NLD Syndrome and the related white matter model.     

Intellectual and adaptive functioning in Sturge-Weber Syndrome

The present study examined the intellectual and adaptive functioning in a sample of children and young adults with Sturge-Weber Syndrome (SWS). A total of 80 research participants from a SWS study database underwent full neurological evaluation as part of their participation or concurrent medical care. Twenty-nine of the participants received neuropsychological evaluations. Analyses indicated no significant demographic or neurological differences between those who did and did not receive neuropsychological evaluations. Overall, the neuropsychological evaluation sample displayed significantly lower functioning relative to published normative data across domains of intellectual and adaptive functioning. Thirty-two percent of the sample displayed impaired performance (standard score ≤ 75) in intellectual functioning and 58% displayed impaired performance in adaptive functioning. Hemiparesis status independently predicted overall adaptive functioning while seizure frequency independently predicted overall intellectual functioning. Younger participants displayed significantly higher (more intact) ratings in adaptive functioning compared to older participants, specifically in overall adaptive functioning, motor skills, and community living skills. A composite measure of neurological status (SWS-NRS) incorporating seizure and hemiparesis status effectively distinguished between individuals with impaired or nonimpaired adaptive and intellectual functioning and showed promise as a screening method for identifying individuals with more involved intellectual and/or adaptive needs.     

Neuropsychological Deficits in Childhood Epilepsy Syndromes

Seizure disorders are relatively common in childhood, and the International League Against Epilepsy (ILAE) provides a hierarchical classification system to define seizure types. At the final level of classification, specific epilepsy syndromes are defined that represent a complex of signs and symptoms unique to an epilepsy condition. The present review discusses the issues related to several of these epilepsy syndromes in childhood, including those classified as generalized idiopathic epilepsies (e.g., childhood absence epilepsy, juvenile absence epilepsy, juvenile myoclonic epilepsy), focal epilepsies (benign rolandic epilepsy, occipital epilepsy, temporal lobe epilepsy, frontal lobe epilepsy) and the "epileptic encephalopathies," including Dravet's Syndrome, West Syndrome, Lennox-Gastaut Syndrome, Myoclonic Astatic Epilepsy, and Landau-Kleffner Syndrome. For each syndrome, the epidemiology, clinical manifestations, treatments, and neuropsychological findings are discussed.     

22q11.2 deletion syndrome: genetics, neuroanatomy and cognitive/behavioral features keywords.

This paper presents a conceptual review of the genetic underpinnings of 22q11.2 Deletion Syndrome. The neuroanatomical, neuropsychological, behavioral, and psychiatric phenotype associated with 22q11.2 Deletion Syndrome is also explored, including variables that are thought to affect symptom expression. The history of the deletion syndrome is described, and future directions for continued research are discussed.     

22q11.2 Deletion Syndrome: Genetics,Neuroanatomy and Cognitive/Behavioral Features Keywords

This paper presents a conceptual review of the genetic underpinnings of 22q11.2 Deletion Syndrome. The neuroanatomical, neuropsychological, behavioral, and psychiatric phenotype associated with 22q11.2 Deletion Syndrome is also explored, including variables that are thought to affect symptom expression. The history of the deletion syndrome is described, and future directions for continued research are discussed.     

Word memory test performance in children.

One-hundred and thirty-five children between the ages of 7 and 18 years were evaluated clinically. Their diagnoses included Fetal Alcohol Syndrome (FAS) or Effects, Schizophrenia, Bipolar Mood Disorder, various neurological diseases, Attention Deficit Hyperactivity Disorder (ADHD), Conduct Disorder, Oppositional-Defiant Disorder and learning disabilities. As part of a comprehensive neuropsychological assessment, the children were given the Word Memory Test (WMT; Green, Allen, & Astner, 1996; Green & Astner, 1995), containing various subtests which measure, respectively, effort and verbal memory. Although age and verbal intelligence are known to affect scores on most ability tests, they were not found to be significant determinants of WMT effort scores. Younger children did not score any lower on the effort subtests than older children. The children scored as well as a group of parents seeking custody of their children and they scored higher than adult patients with mild head injuries. The computerized WMT requires some basic reading skills and some children with lower than a grade 3 reading level scored at a relatively low level on the effort subtests. The current data suggest that most children with at least a grade 3 reading level can pass the WMT using the adult criteria. It is concluded that the WMT is potentially useful in the evaluation of effort during pediatric neuropsychological evaluations. Further research is needed to replicate these findings and to develop child norms for the memory subtests.     

Word Memory Test Performance in Children

One-hundred and thirty-five children between the ages of 7 and 18 years were evaluated clinically. Their diagnoses included Fetal Alcohol Syndrome (FAS) or Effects, Schizophrenia, Bipolar Mood Disorder, various neurological diseases, Attention Deficit Hyperactivity Disorder (ADHD), Conduct Disorder, Oppositional-Defiant Disorder and learning disabilities. As part of a comprehensive neuropsychological assessment, the children were given the Word Memory Test (WMT; Green, Allen, & Astner, 1996; Green & Astner, 1995), containing various subtests which measure, respectively, effort and verbal memory. Although age and verbal intelligence are known to affect scores on most ability tests, they were not found to be significant determinants of WMT effort scores. Younger children did not score any lower on the effort subtests than older children. The children scored as well as a group of parents seeking custody of their children and they scored higher than adult patients with mild head injuries. The computerized WMT requires some basic reading skills and some children with lower than a grade 3 reading level scored at a relatively low level on the effort subtests. The current data suggest that most children with at least a grade 3 reading level can pass the WMT using the adult criteria. It is concluded that the WMT is potentially useful in the evaluation of effort during pediatric neuropsychological evaluations. Further research is needed to replicate these findings and to develop child norms for the memory subtests.     

Neuropsychological evaluation and rehabilitation in mental retardation

A discussion is provided of issues related to neuropsychological assessment of mentally retarded individuals. The first matters considered are test selection in relation to mental age and diagnostic issues. Various methods of evaluation are then considered, including assessment of orientation to sound and visual stimuli, language comprehension, right-left discrimination, and abstract and symbolic processes. Methods are also reviewed for detailed evaluation of language functions including testing for aphasia and for academic skills such as reading, writing, and calculation, as well as for spatial and constructional abilities. A section is devoted to use of neuropsychological assessment for rehabilitation of various disorders such as congenital aphasia, alexia, and spatial-constructional disorders.     

Neuropsychological, learning and psychosocial profile of primary school aged children with the velo-cardio-facial syndrome (22q11 deletion): evidence for a nonverbal learning disability?

In this exploratory study, the neuropsychological and learning profile of nine primary school age children with velo-cardio-facial syndrome (VCFS) was studied by systematic neuropsychological testing. In five out of nine children, the following profile was found: a VIQ-PIQ discrepancy (in favor of the VIQ), significantly better scores (.05 level) for reading (decoding) and spelling compared to arithmetic, deficient tactile-perceptual skills (difficulties mainly on the left side of the body), weak but not deficient visual-perceptual abilities, deficient visual-spatial skills, extremely poor psychomotor skills (gross motor skills more deficient than fine motor skills), problems with processing of new and complex material, poor visual attention, good auditory memory and relatively good language skills. These findings correspond to the pattern of neuropsychological assets and deficits that has been described for the syndrome of nonverbal learning disabilities (NLD) (Rourke, 1987, 1988, 1989, 1995). The psychosocial profile of all nine children with VCFS also correspond to that of children with NLD. Further studies on the relationship between cognitive function, behavior, psychiatric disorder and abnormalities in brain anatomy in young people with VCFS will be needed. In clinical practice, it is worthwhile exploring in greater depth the neuropsychological functions of children with VCFS to rule out NLD, since they may benefit from specific remediation following the learning principles of the NLD-treatment.     

Working Memory Interference Control Deficit in Children Referred by Teachers for ADHD Symptoms

In this exploratory study, the neuropsychological and learning profile of nine primary school age children with velo-cardio-facial syndrome (VCFS) was studied by systematic neuropsychological testing. In five out of nine children, the following profile was found: a VIQ-PIQ discrepancy (in favor of the VIQ), significantly better scores (.05 level) for reading (decoding) and spelling compared to arithmetic, deficient tactile-perceptual skills (difficulties mainly on the left side of the body), weak but not deficient visual-perceptual abilities, deficient visual-spatial skills, extremely poor psychomotor skills (gross motor skills more deficient than fine motor skills), problems with processing of new and complex material, poor visual attention, good auditory memory and relatively good language skills. These findings correspond to the pattern of neuropsychological assets and deficits that has been described for the syndrome of nonverbal learning disabilities (NLD) (Rourke, 1987, 1988, 1989, 1995). The psychosocial profile of all nine children with VCFS also correspond to that of children with NLD. Further studies on the relationship between cognitive function, behavior, psychiatric disorder and abnormalities in brain anatomy in young people with VCFS will be needed. In clinical practice, it is worthwhile exploring in greater depth the neuropsychological functions of children with VCFS to rule out NLD, since they may benefit from specific remediation following the learning principles of the NLD-treatment.     

The Neuropsychology of Heading and Head Trauma in Association Football (Soccer): A Review

Association Football (soccer) is the most popular and widespread sport in the world. A significant proportion of the injuries suffered in football are head injuries involving trauma to the brain. In normal play, head trauma frequently arises from collisions, but some researchers have claimed that it also may arise as a consequence of heading the ball. Although assessments based on biomechanical analyses are equivocal on the potential for brain injury due to football heading, a growing literature seems to support the claim that neuropsychological impairment results from general football play and football heading in particular. However, this review suggests a distinction is required between the neuropsychological effects of concussive and subconcussive head trauma and that all of the neuropsychological studies conducted so far suffer from methodological problems. At best, a few of these studies may be regarded as exploratory. The review concludes that presently, although there is exploratory evidence of subclinical neuropsychological impairment as a consequence of football-related concussions, there is no reliable and certainly no definitive evidence that such impairment occurs as a result of general football play or normal football heading. The neuropsychological consequences of football-related subconcussive effects await confirmatory investigation.     

Neuropsychological functioning and social functioning of survivors of pediatric brain tumors: evidence of nonverbal learning disability.

The purpose of the study was to examine if survivors of pediatric brain tumors exhibit a pattern of performance consistent with nonverbal learning disability (NVLD) and to explore the relationship between neuropsychological and social functioning in these children. A comprehensive neuropsychological battery and objective measures of psychosocial function designed to assess NVLD were administered to 15 survivors of brain tumors, ages 8-12 years. Despite the small sample size, a trend for better verbal skills compared to nonverbal skills was found using composite scores. Parents reported significant social deficits and a tendency for greater internalizing behavior problems as expected in NVLD. Additionally, there was a trend for a positive association between nonverbal scores and social function. Further research is needed to determine if the NVLD pattern observed is attributable to white matter damage of the right hemisphere. Routine neuropsychological and psychosocial assessment and intervention are indicated.     

Relationship of temporal lobe volumes to neuropsychological test performance in healthy children

Ecological validity of neuropsychological assessment includes the ability of tests to predict real-world functioning and/or covary with brain structures. Studies have examined the relationship between adaptive skills and test performance, with less focus on the association between regional brain volumes and neurobehavioral function in healthy children. The present study examined the relationship between temporal lobe gray matter volumes and performance on two neuropsychological tests hypothesized to measure temporal lobe functioning (visual perception-VP; peabody picture vocabulary test, third edition-PPVT-III) in 48 healthy children ages 5-18 years. After controlling for age and gender, left and right temporal and left occipital volumes were significant predictors of VP. Left and right frontal and temporal volumes were significant predictors of PPVT-III. Temporal volume emerged as the strongest lobar correlate with both tests. These results provide convergent and discriminant validity supporting VP as a measure of the "what" system; but suggest the PPVT-III as a complex measure of receptive vocabulary, potentially involving executive function demands.     

The growth of reading skills in children with Down Syndrome

There appears to be a close and probably causal relationship between early variations in phoneme skills and later reading skills in typically developing children, though the pattern in children with Down Syndrome is less clear. We present the results of a 2-year longitudinal study of 49 children with Down Syndrome (DS) and 61 typically developing (TD) control children with similar initial levels of reading skill. Phoneme awareness and vocabulary were strong concurrent predictors of initial levels of reading skill in both groups. However, longitudinally phoneme awareness was a predictor of the growth of reading skills in TD children but not in children with DS. There was a very high degree of longitudinal stability in reading skills in children with DS, and initial levels of reading skills seemed to be highly constrained by general language skills, as indexed by vocabulary knowledge, in this population. We conclude that reading development in children with DS shows similarities and differences to the pattern observed in TD children and that phoneme awareness appears to be a less powerful influence on the development of reading skills in children with DS.     

Neuropsychological Findings in Congenital and Acquired Childhood Hydrocephalus

Hydrocephalus is an increase in cerebrospinal fluid volume that can be caused by a variety of etiologies. The most common connatal and acquired causes of hydrocephalus are spina bifida, aqueduct stenosis, and preterm low birthweight infants with ventricular hemorrhage. In general, the literature suggests mild neuropsychological deficits associated with hydrocephalus, which are predominant in visuospatial and motor functions, and other nonlanguage skills. Although the precise nature of the neuropsychological deficits in hydrocephalus are not completely known, several factors such as etiology, raised intracranial pressure, ventricular size, and changes in gray and white matter tissue composition as well as shunt treatment complications have been shown to influence cognition. In fact, the presence of complications and other brain abnormalities in addition to hydrocephalus such as infections, trauma, intraventricular hemorrhage, low birthweight, and asphyxia are important determinants of the ultimate cognitive status, placing the child at a high risk of cognitive impairment.     

A Two-Year Longitudinal Study of Neuropsychological and Cognitive Performance in Relation to Behavioral Problems and Competencies in Elementary School Children

Despite interest in early neuropsychological status as a possible contributor to children's behavioral development, prospective longitudinal investigations of neuropsychological measures in relation to later behavioral outcomes in childhood are few. A 2-year longitudinal study in a nonselected childhood sample is reported. The study tested the influence of early neuropsychological performance (verbal fluency, mental inhibitory control, and visual spatial ability) on later childhood behavioral problems and social competency. Regular education children (n = 235) were assessed at three time points 1 year apart. To control for autocorrelation of outcome measures, Time 1 behavior was partialed while testing the effects of Time 1 neuropsychological scores on Time 3 outcome. To control for autocorrelation of neuropsychological scores, Time 2 scores were partialed while testing the predictive effect of Time 1 scores on Time 3 outcome. Both sets of regression models suggested modest but statistically significant effects for inhibitory control and verbal fluency, but not IQ, reading, or visual spatial ability, on behavioral outcome. Study results are consistent with a modest causal effect of selected neuropsychological skills on later behavioral adjustment. The findings support theories that implicate subtle neuropsychological dysfunction in the development of behavioral problems in childhood.     

Plasticity for recovery, plasticity for development: cognitive outcome in twins discordant for mid-childhood ischemic stroke.

Cognitive and language profiles in a set of likely monozygotic 13-year-old twins raised together and discordant for an extensive left hemisphere stroke are described 6 years post-stroke. Recovery differed across four skill profiles, created based on a model of plasticity. Full recovery of lost skills and full or partial development of new skills typically involved either semantic memory or right hemisphere lateralized functions such as visual perception skills. Limited development of new skills and full or partial recovery of lost skills took place in either active verbal memory or oral language skills that are left hemisphere lateralized. Language skills concerned with real-time lexical retrieval, or the use and understanding of morphology and grammar, phonological processing skills, and auditory working memory were least likely to recover and continue to develop.     

An Introduction to Sports Concussion for the Sport Psychology Consultant

This paper was designed to introduce the sport psychology consultant (SPC) to the relevant practical and theoretical information pertaining to sports concussion. The paper discusses four key areas related to sports concussion: 1) recognition, 2) management, 3) psychological issues following a concussion, and 4) prevention and education. Throughout the paper, the authors emphasize the role of the SPC in each of these four areas. An integrated review of the sport concussion literature and current research and guidelines regarding the definition, pathophysiology, symptoms, neuropsychological testing, and risk factors for concussion is presented. The SPC's roles in educating and helping athletes deal with post-concussion issues such as the pressure to return to play and fear of reinjury are examined. The authors highlight the need for baseline neuropsychological testing of athletes; and advocate a multidimensional team approach to sport concussion, involving the SPC as a key member of that team.     

The impact of sleep deprivation on decision making: a review

Few sleep deprivation (SD) studies involve realism or high-level decision making, factors relevant to managers, military commanders, and so forth, who are undergoing prolonged work during crises. Instead, research has favored simple tasks sensitive to SD mostly because of their dull monotony. In contrast, complex rule-based, convergent, and logical tasks are unaffected by short-term SD, seemingly because of heightened participant interest and compensatory effort. However, recent findings show that despite this effort, SD still impairs decision making involving the unexpected, innovation, revising plans, competing distraction, and effective communication. Decision-making models developed outside SD provide useful perspectives on these latter effects, as does a neuropsychological explanation of sleep function. SD presents particular difficulties for sleep-deprived decision makers who require these latter skills during emergency situations.