Adults with Asperger syndrome with and without a cognitive profile associated with “non-verbal learning disability.” A brief report

Asperger syndrome (AS) and non-verbal learning disability (NLD) are both characterized by impairments in motor coordination, visuo-perceptual abilities, pragmatics and comprehension of language and social understanding. NLD is also defined as a learning disorder affecting functions in the right cerebral hemisphere. The present study investigates if individuals with AS and a cognitive profile consistent with NLD (i.e. verbal IQ > performance IQ) would also have other problems inherent in NLD, visual memory and attention, reading/writing ability and arithmetic in the presence of preserved verbal memory and attention. Forty-four individuals with AS were assessed with a battery of neuropsychological tests. Reading/writing and arithmetic abilities were investigated. Education and global social adaptive levels were studied. Very few AS participants, even though with NLD cognitive profile showed problems with any of the neurocognitive abilities or academic achievements. However, all had poor global social adaptive functioning and few had paid employment, regardless of their cognitive profile. The present study suggests that AS and NLD are two different conditions even though some individuals in both groups have the verbal IQ > performance IQ profile that has been proposed to be typical of both AS and NLD.     

Neuropsychological profiles of adults with Klinefelter syndrome.

Children and adolescents with Klinefelter syndrome (XXY) have been reported to show deficits in language processing including VIQ < PIQ and a learning disability in reading and spelling. However, whether this is characteristic of adults with Klinefelter syndrome has not been established. Thirty-five men with Klinefelter syndrome, aged 16 to 61, and 22 controls were evaluated with a comprehensive neuropsychological battery. The Klinefelter patients scored significantly below controls in language skills, verbal processing speed, verbal and nonverbal executive abilities, and motor dexterity. Within the Klinefelter sample, three cognitive subgroups were identified: VIQ 7 or more points below PIQ (n = 10), VIQ within 6 points of PIQ (n = 12), and PIQ 7 or more points below VIQ (n = 12). The deficits detected in language, verbal processing speed, and verbal executive skills were found to be isolated to the VIQ < PIQ subgroup, while the abnormalities in motor dexterity and nonverbal executive skills were confined to the PIQ < VIQ subgroup. Older age was significantly correlated with increases in VIQ relative to PIQ in the patient group, which suggests the intriguing possibility that the PIQ < VIQ subgroup primarily emerges in young adulthood, perhaps in response to the reported hormonal abnormalities detected in Klinefelter syndrome patients during puberty.     

Academic and Cognitive Abilities in Children of Parents With Bipolar Disorder: A Test of the Nonverbal Learning Disability Model

It has been hypothesized that children who are at genetic risk to develop bipolar disorder demonstrate deficiencies consistent with the syndrome of nonverbal learning disabilities (NLD); however, this hypothesis has never been tested directly. In the present study, a group of at-risk children (AR group; N = 28) was compared to a demographically matched control group of children of healthy parents (HC group; N = 24) for evidence of a constellation of features associated with NLD. Some characteristic features of NLD were evident, including significant Verbal IQ (VIQ) &gt; Performance IQ (PIQ) discrepancies and psychomotor deficits. However, academic deficiencies in mechanical arithmetic relative to reading and spelling abilities were not demonstrated. These findings replicate and extend the current literature on the cognitive functioning of children of parents with Bipolar disorder (BPD). The results, however, do not support the presence of NLD in these children.     

Non-verbal learning disabilities in children with infantile hydrocephalus, aged 4-7 years: a population-based, controlled study

The aim of this population-based, controlled study was to investigate non-verbal learning disabilities (NLD) in children with infantile hydrocephalus (IH). For this purpose, the results from subtests measuring either assets or deficits within Rourke's model of NLD were analyzed. Children with myelomeningocele (MMC), intracranial tumors, or IQ < 70 were excluded. Of the 52 IH children included in the study, 46 were shunt-treated, whereas 6 were not shunted. The 44 controls were matched according to age, gender, and geographic variables. The Neuropsychological Assessment of Children (NEPSY) was administered to 52 children (age 4-7) with IH; (F = 17, M = 35), and to 44 controls (F = 17, M = 27). The tests used were classified along each of the dimensions "assets" or "deficits", according to Rourke's model of the elements and the dynamics of the NLD syndrome. Differences between sum scores for the subtests classified as "assets", versus "deficits" were significantly higher in the IH group as compared with the controls (p < 0.005). These findings are compatible with a higher frequency of NLD in the IH group, in which neurological confounding factors have been excluded. In addition, the model of the elements and the dynamics of the NLD syndrome may be useful when analyzing neuropsychological test results obtained with traditional and comprehensive test batteries.     

Comparison Among Children with Children with Autism Spectrum Disorder, Nonverbal Learning Disorder and Typically Developing Children on Measures of Executive Functioning

It has been suggested that children with nonverbal learning disabilities (NLD) or Asperger’s Syndrome (AS) may show difficulties with executive functioning. There were 3 groups in this study who completed a neuropsychological battery of visual-spatial, executive functioning, and reasoning tasks; AS (n = 37), NLD (n = 31), and controls (n = 40). Results indicated that children in both clinical groups scored within average limits on measures of spatial reasoning and verbal ability. Fluid reasoning was also found to be within average ranges for all groups. The AS group experienced significant problems with cognitive flexibility compared to the other two groups. In contrast the NLD group showed fewer difficulties with cognitive flexibility but more problems with visual sequencing. These findings suggest that performance on executive function measures for children with AS or NLD is remarkably similar with subtle differences present.     

Academic and cognitive abilities in children of parents with bipolar disorder: a test of the nonverbal learning disability model

It has been hypothesized that children who are at genetic risk to develop bipolar disorder demonstrate deficiencies consistent with the syndrome of nonverbal learning disabilities (NLD); however, this hypothesis has never been tested directly. In the present study, a group of at-risk children (AR group; N = 28) was compared to a demographically matched control group of children of healthy parents (HC group; N = 24) for evidence of a constellation of features associated with NLD. Some characteristic features of NLD were evident, including significant Verbal IQ (VIQ) > Performance IQ (PIQ) discrepancies and psychomotor deficits. However, academic deficiencies in mechanical arithmetic relative to reading and spelling abilities were not demonstrated. These findings replicate and extend the current literature on the cognitive functioning of children of parents with Bipolar disorder (BPD). The results, however, do not support the presence of NLD in these children.     

Deficits in visual short-term memory binding in children at risk of non-verbal learning disabilities

It has been hypothesized that learning disabled children meet short-term memory (STM) problems especially when they must bind different types of information, however the hypothesis has not been systematically tested. This study assessed visual STM for shapes and colors and the binding of shapes and colors, comparing a group of children (aged between 8 and 10 years) at risk of non-verbal learning disabilities (NLD) with a control group of children matched for general verbal abilities, age, gender, and socioeconomic level. Results revealed that groups did not differ in retention of either shapes or colors, but children at risk of NLD were poorer than controls in memory for shape-color bindings.     

Review of the NIMH Israeli Kibbutz-City Study and the Jerusalem Infant Development Study

The National Institute of Mental Health (NIMH) Israeli Kibbutz-City Study has followed the development of offspring of schizophrenic parents from middle childhood through early adulthood. During childhood, a subgroup of offspring of schizophrenic patients showed clear neurobehavioral deficits often accompanied by poor social competence. Early followup data suggest that this subgroup of high-risk children is at greatest risk for adult schizophrenia spectrum illness. The Jerusalem Infant Development Study has followed a similar population of children at risk for schizophrenia from before birth through middle childhood. A subgroup of dysfunctioning in the high-risk children showed sensorimotor dysfunctioning in the first year of life, which was followed by perceptual, motor, and attentional dysfunctioning in childhood--identical to that found in the NIMH cohort. Results from both studies support the hypothesis that schizophrenic illness involves constitutional factors whose expression can be observed as early as infancy. Results also illustrate the importance of using data-analytic approaches that (1) look for subgroups within high-risk groups rather than only group differences between high- and low-risk groups, and (2) examine profiles of behavior rather than only single variables.     

Do individuals with high functioning autism have the IQ profile associated with nonverbal learning disability?

Previously researchers have noted a high level of occurrence of the IQ profile associated with nonverbal learning disability (NLD) in Asperger syndrome (ASP) but not in high functioning autism (HFA). We examined the IQ profile scores of a large sample of children (n = 69) and adults (n = 77) with HFA, stringently diagnosed according to ADOS, ADI-R, and DSM-IV criteria, and a corresponding sample of typical child (n = 72) and adult controls (n = 107). At least one of the three primary components of the Wechsler pattern seen in NLD were found in 17–26% of the children and 20–32% of the adults with HFA. All three components occurred in slightly more than 5% of the children and adults with autism. Overall, the VIQ > PIQ profile seen in NLD occurred in 18% of the sample of individuals stringently diagnosed with HFA. Therefore, obtaining this IQ profile is not a valid clinical discriminator between NLD and HFA.     

Memory Tests in Subgroups of Adult Attention Deficit Hyperactivity Disorder Reveals Simultaneous Capacity Deficit

Neuropsychological tests were used to evaluate different memory systems in the three subgroups of adult Attention Deficit Hyperactivity Disorder (ADHD) (n = 105) using analysis of means, factor analysis, and GLM analysis with covariance of gender, estimated IQ, and level of anxiety and depression measured with the Hospital anxiety and depression scale. A higher IQ level was found in the neuropsychological background tests for the predominantly inattentive subtype (ADD). In the memory tests the dual-task memory/simultaneous capacity tests “Brown-Peterson” Consonant Trigram and Benton Visual Retention Test (BVRT) were the most sensitive and were severely reduced in all three subgroups, but only the BVRT revealed a difference between the three ADHD groups. In learning and delayed recall measured with Rey Auditory Verbal learning test and modified Diagnosticum für Cerebralschädigung (mDCS), the Attention Deficit Disorder subgroup had the best learning and delayed capacity of the three groups. A good agreement was found between the interviewed DSM-IV-TR criteria, Conners CAARS S:S scale, and Wender WURS 25-item scales. Despite the difference in number of ADHD criteria for the three ADHD subgroups, the results in the neuropsychological memory tests indicate a severe reduction in all three subgroups of adult ADHD in simultaneous capacity.     

Memory tests in subgroups of adult attention deficit hyperactivity disorder reveals simultaneous capacity deficit

Neuropsychological tests were used to evaluate different memory systems in the three subgroups of adult Attention Deficit Hyperactivity Disorder (ADHD) (n=105) using analysis of means, factor analysis, and GLM analysis with covariance of gender, estimated IQ, and level of anxiety and depression measured with the Hospital anxiety and depression scale. A higher IQ level was found in the neuropsychological background tests for the predominantly inattentive subtype (ADD). In the memory tests the dual-task memory/simultaneous capacity tests "Brown-Peterson" Consonant Trigram and Benton Visual Retention Test (BVRT) were the most sensitive and were severely reduced in all three subgroups, but only the BVRT revealed a difference between the three ADHD groups. In learning and delayed recall measured with Rey Auditory Verbal learning test and modified Diagnosticum für Cerebralsch?digung (mDCS), the Attention Deficit Disorder subgroup had the best learning and delayed capacity of the three groups. A good agreement was found between the interviewed DSM-IV-TR criteria, Conners CAARS S:S scale, and Wender WURS 25-item scales. Despite the difference in number of ADHD criteria for the three ADHD subgroups, the results in the neuropsychological memory tests indicate a severe reduction in all three subgroups of adult ADHD in simultaneous capacity.     

癫痫对儿童学习障碍发生的影响

目的 观察癫痫患儿共患学习障碍(LD)的现状以及分型并探讨癫痫的各种因素与LD发病的相关性.方法 采用修订版学习障碍儿童筛查量表对110例癫痫儿童(癫痫组)进行测评,与120例对照组进行比较分析.结果 癫痫组LD检出率为36.4%,对照组为10.0%,两组相比较癫痫组明显高于对照组(P＜0.05).癫痫组儿童LD中非言语型学习障碍(NLD)占70.0%.回归分析可见癫痫控制与否、发病年龄和性别是癫痫患儿共患LD的相关因素.结论 癫痫儿童LD的发生率高,以NLD为常见;癫痫控制与否、发病年龄和性别与其共患LD有关.     

Do children with myelomeningocele and hydrocephalus display nonverbal learning disabilities? An empirical approach to classification.

As a group, children with myelomeningocele (MM) and early-onset hydrocephalus demonstrate many of the features of the syndrome of nonverbal learning disabilities (NLD). However, the frequency with which individual children display a pattern of neuropsychological functioning consistent with the NLD syndrome is unknown. We addressed this question by comparing the prevalence of NLD in 32 children with MM and shunted hydrocephalus to that in a group of 27 healthy siblings. Participants, who were between 8 and 15 years of age, completed a neuropsychological test battery that included 11 measures of possible assets and 17 measures of possible deficits that define the NLD syndrome. As a group, children with MM and shunted hydrocephalus displayed many of the specific assets and deficits. However, they also displayed significantly more variability in their patterns of assets and deficits than siblings, reflecting the substantial individual differences that characterize children with MM. About 50% of the children with myelomeningocele displayed a pattern of assets and deficits consistent with the NLD syndrome. Classification as NLD was weakly related to cumulative medical risk, as well as to left-handedness. The findings suggest a need for caution in making generalizations regarding the applicability of the NLD model to children with MM and early-onset hydrocephalus.     

The lateralizing value of IQ in mesiotemporal epilepsy: Differences between patients with unitemporal and bitemporal epileptiform discharges.

PURPOSE: We investigated the lateralizing ability of intelligence scores in mesial temporal lobe epilepsy (MTLE) patients according to the distribution of interictal epileptiform discharges (IEDs). METHODS: This study enrolled 82 MTLE patients. All patients had preoperative neuropsychological evaluations, including Korean Wechsler Adult Intelligence Scale. Patients were categorized as having uni- or bitemporal IEDs based on IEDs distribution (cutoff point, 90%). RESULTS: In patients with unitemporal IEDs, performance IQ (PIQ) was significantly lower in the right than in the left subgroup (89.6 vs. 99.4, p<0.05). Verbal IQ (VIQ)-PIQ discrepancy scores differed significantly between the left and right subgroups, being negative in the left and positive in the right subgroup. Based on multivariate analyses, two variables, right MTLE (p=0.042) and the unitemporal distribution of IEDs (p=0.030), were independently related to the VIQ-PIQ discrepancy of more than 10 points. About 47.4% of those with unitemporal IEDs had VIQ-PIQ discrepancies of greater than 10 points and the rate for correct lateralization was 77.8%. In patients with bitemporal IEDs, however, none of the intelligence scores showed evidence of correct lateralization. In patients with bitemporal IEDs, Full-scale IQ and PIQ were significantly lower in the left subgroup, and there was a significant difference in VIQ-PIQ discrepancy scores with the wrong direction. CONCLUSIONS: We found that intelligence scores had some lateralizing ability, but only in MTLE patients with unitemporal IEDs.     

Early Childhood Predictors of the Social Competence of Adults with Autism

Longitudinal research into adult outcomes in autism remains limited. Unlike previous longitudinal examinations of adult outcome in autism, the twenty participants in this study were evaluated across multiple assessments between early childhood (M = 3.9 years) and adulthood (M = 26.6 years). In early childhood, responsiveness to joint attention (RJA), language, and intelligence were assessed. In adulthood, the parents of participants responded to interviews assessing the adaptive functioning, autistic symptomology and global functioning of their children. RJA and early childhood language predicted a composite measure of adult social functioning and independence. Early childhood language skills and intelligence predicted adult adaptive behaviors. RJA predicted adult non-verbal communication, social skills and symptoms. Adaptive behaviors changed with development, but symptoms of autism did not. Additional factors associated with adult outcomes are discussed.     

Inter-rater reliability of the russell revision of the wechsler memory scale: An attempt to clarify some ambiguities in scoring

Abstract

A neuropsychological battery comprised of 10 linguistic and visual perceptual tests, chosen because of their use in previous research, was administered to 75 learning disabled readers (LDR) and 42 normal readers (NR) matched for age and IQ. Standard scores, derived from a comparison of each LDR child's score with the NR group's performance on each test, were cluster analyzed to identify subgroups within the LDR group. Five subgroups were identified. Multivariate analysis of variance and discriminant analysis indicated that all LDR subgroups were significantly different from one another with respect to subgroup members' performance on the set of neuropsychological variables. In addition, some significant differences were found among the five subgroups on measures of oral reading, word-attack skills, and reading comprehension.     

One hundred males with Asperger syndrome: a clinical study of background and associated factors

The objective of this study was to investigate the background and associated factors in a representative group of young males with Asperger syndrome (AS) presenting at a specialized autism clinic. One hundred males aged 5 years 6 months to 24 years 6 months, with a mean age of 11 years 4 months (SD 3y 10mo), who had a clinical diagnosis of AS were included in the study. An in-depth review of their medical records and neuropsychological test data was performed. There was a high rate (51%) of non-verbal learning disability (defined as Verbal IQ more than 15 points higher than Performance IQ), but otherwise there was little or no support for the notion of right-hemisphere brain dysfunction being at the core of the syndrome. There was a very high rate of close relatives with autism spectrum problems, but also high rates of prenatal and perinatal problems, including prematurity and postmaturity. In comparison with general population data, those with AS very often had a combination of genetic and prenatal and perinatal risk factors. Non-verbal learning disability test results applied in about half the group. There was a subgroup of individuals with AS who had macrocephalus. However, there was no support for an association of AS with low body mass index.     

Early neuropsychological signs of childhood adrenoleukodystrophy (ALD)

Objective: To identify the very early signs of childhood cerebral adrenoleukodystrophy (CALD) in asymptomatic boys. Methods: Subjects were 8 neurologically and radiologically asymptomatic CALD patients. They were chosen from 22 ALD patients before hematopoietic stem cell transplantation (HSCT) treatment. Wechsler Intelligence Scale tests and other neuropsychological tests were performed. Their IQ data were compared with those of symptomatic ALD children with apparent brain lesions. Results: All asymptomatic CALD showed normal FIQ. PIQ was significantly lower than VIQ in 2 patients. Occipital lesioned patients often showed normal VIQ with low PIQ. Gestalt Closure in The Kaufman Assessment Battery for Children and Constancy of Shape in the Frostig Developmental Test of Visual Perception seemed to be effective for detecting visual cognitive dysfunction in asymptomatic CALD whose difference of VIQ and PIQ was within the normal range. Conclusions: Follow-up of asymptomatic ALD boys with neuropsychological tests along with previously reported neurophysiological tests may help in determining the timing of therapeutic intervention. Neuropsychological abnormalities seemed to precede the appearance of clinical and MRI alterations.     

Asperger syndrome: tests of right hemisphere functioning and interhemispheric communication

The primary aim of this investigation was to assess to what extent Rourke's (1989, 1995) nonverbal learning disabilities syndrome (NLD) model resembles the pattern of assets and deficits seen in people with Asperger syndrome (AS). NLD can be characterized by a cluster of deficits primarily affecting nonverbal aspects of functioning, in the presence of proficiency in single word reading and a superior verbal memory. The neurological underpinnings of this syndrome may be dysfunction of white matter affecting right hemisphere functioning and interhemispheric communication. To explore this hypothesis, eight participants with AS (ages 10 to 41 years) were assessed in the following areas: the pragmatics of language and communication, verbal and visual memory, visual-spatial abilities, and bimanual motor skills. Results confirmed the close similarity in the neuropsychologic profiles of NLD and AS.     

Effects of anomalous language representation on neuropsychological performance in temporal lobe epilepsy.

OBJECTIVE: To examine the effects of anomalous language representation (i.e., mixed- and right-cerebral dominant) on neuropsychological performance. BACKGROUND: Right cerebral language dominance resulting from early cerebral injury is associated with relatively preserved language function with decreased visuospatial ability. However, previous reports of this phenomenon have examined patients with relatively large cerebral injuries (e.g., infantile hemiplegia) or limited sample sizes. METHODS: A total of 561 patients with complex partial seizures of left temporal lobe origin were studied. Patients were classified into left (n = 455), bilateral (n = 58), and right (n = 48) language dominant groups based on Wada testing. RESULTS: Right language dominant patients performed more poorly on multiple tests of visuospatial function, including Performance IQ (PIQ), than did left language patients. No significant group differences were detected for measures of language or general verbal function. The effects of bilateral language on PIQ differed according to handedness. Lowered PIQ was present in the bilateral nondextral group but not for bilateral dextral patients, and this pattern was observed with other visuospatial measures. CONCLUSIONS: In patients with relatively small lesions restricted to the left mesial temporal lobe, a shift in language dominance to the right hemisphere is associated with decreased visuospatial functions but preserved verbal abilities. Nondextral patients with bilateral language representation also displayed decreased visuospatial performance, although dextral patients with bilateral language did not.