Clinical profile of reading ability and reading and writing achievement of children with borderline full-scale intellectual quotient: a prospective study.

BACKGROUND: Poor reading ability is one of the common causes of low academic performance. In previous studies, children with dyslexia were found to demonstrate poor academic achievement due to poor reading ability. However, the relationship between academic achievement and reading ability in children with a borderline full-scale intellectual quotient (FSIQ) is unknown. This study aimed to clarify the clinical characteristics of children with borderline FSIQ and poor reading ability, and differentiate these characteristics from those of children with higher FSIQ and poor reading ability. METHODS: A total of 126 children (aged 6-15 years) identified as having low academic performance were enrolled. The reading ability of children was assessed through their performance on the hiragana (Japanese syllabary) reading task, while their reading and writing achievement was assessed through their reading and writing score on the Kaufman Assessment Battery for Children, Second Edition. Children were categorized into two groups based on their FSIQ score (FSIQ > 85 and 85 ≥ FSIQ ≥ 70). Reading ability in children was evaluated by referring to the linear relationship between FSIQ and the standard deviation value of reading tasks in typically developing children. A one-way analysis of variance (ANOVA) was performed to examine clinical characteristics between higher and lower FSIQ groups. Associations between reading and writing achievement, reading ability, and ages of children were assessed using Pearson's product-moment correlation coefficients for the higher and lower FSIQ groups. RESULTS: Poorer reading and writing achievement was associated with poorer reading ability in the higher FSIQ group. Conversely, poorer reading and writing achievement and poor reading ability were associated with older age in the lower FSIQ group. CONCLUSIONS: Poor reading and writing achievement were associated with older age, not with poor reading ability in the lower FSIQ group. Children with lower FSIQ need appropriate educational interventions based on independent assessments to further their academic achievement and reading ability. Moreover, they need more frequent evaluations of their academic achievement than do children with higher FSIQ and poor reading ability since they are more likely to be at a lower academic achievement level at an older age.

Association between lack of functional connectivity of the frontal brain region and poor response inhibition in children with frontal lobe epilepsy.

PURPOSE: We investigated the relationship between electroencephalographic (EEG) functional connectivity and executive function in children with frontal lobe epilepsy (FLE). METHODS: We enrolled 24 children with FLE (mean age, 11.0 years; 13 boys) and 22 sex-, age-, and intelligence-matched typically developing children (TDC) to undergo 19-channel EEG during light sleep. We estimated functional connectivity using the phase lag index (PLI) that captures the synchronization of EEG. We also performed continuous performance tests (CPTs) on the children and obtained questionnaire responses on attention deficit hyperactivity disorder and oppositional defiant disorder (ODD). RESULTS: The average gamma PLI was lower in the FLE group than in the TDC group, especially between long-distance frontoparietal pairs, between interhemispheric frontal pairs, and between interhemispheric parietotemporal pairs. Gamma PLIs with long-distance frontoparietal and interhemispheric frontal pairs were positively associated with inattention, ODD scores, omission error, and reaction time in the FLE group but not in the TDC group. Conversely, they were negatively associated with age, hyperactivity score, and commission error. CONCLUSIONS: A lack of functional connectivity of the frontal brain regions in children with FLE was associated with poor response inhibition.

Adaptive behavior and its related factors in children with focal epilepsy.

OBJECTIVE: We aimed to clarify the strengths and weaknesses in adaptive behavior in children with focal epilepsy and show children-associated factors related to adaptive behavior. MATERIALS AND METHODS: Sixty-three children with focal epilepsy aged 5-18 years with intellectual quotient (IQ) ranging from 67 to 135 were enrolled in this study. Adaptive behavior was evaluated using the Vineland Adaptive Behavior Scale, 2nd edition (VABS-II). The children performed continuous performance test and tests of reading, writing, and IQ; parents answered questionnaires regarding attention-deficit hyperactivity disorder and autism spectrum disorder (ASD). Participants were categorized into four groups based on IQ and adaptive behavior scores for statistical comparisons. RESULTS AND DISCUSSION: Children with low adaptive behavior were more likely to show a reduction in daily living skills, and those with both low adaptive behavior and IQ were more likely to show a reduction in daily living skills and communication. Lower adaptive behavior was related to more severe autistic symptoms, lower academic achievement in children with IQ > 85, and lower executive function in children with IQ ≤ 85. There was a qualitative difference of cognitive dysfunction in adaptive behavior between both groups. CONCLUSIONS: There were differences in VABS-II domain and subdomain characteristics between children with focal epilepsy and those with ASD; however, it was more difficult for children with more severe ASD and coexisting focal epilepsy to show age-equivalent adaptive behavior.

Prevalence estimates of neurodevelopmental disorders in Japan: A community sample questionnaire study.

AIM: Prevalence estimates of neurodevelopmental disorders have been calculated by questionnaire surveys scored by a single rater, which introduces inherent rater biases. The present study aimed to estimate the prevalence and comorbidity rates of four neurodevelopmental disorders based on both parent and teacher rating scales. METHODS: We performed a community sample survey recruiting 3852 children aged 6 to 9 years. Parents and teachers evaluated clinical conditions in children using questionnaire-style scales. These scales with cut-off values were used to estimate the prevalence and comorbidity rates of attention deficit hyperactive disorder, autism spectrum disorder, specific learning disorder (or developmental dyslexia), and developmental coordination disorder. RESULTS: The prevalence estimates were separately confirmed according to the raters. Some estimates were higher than those in previous studies conducted in other countries. We also found a large disagreement between the parent and teacher rating scores. Moreover, the degree of agreement between two raters varied depending on the severity of the child's clinical condition. CONCLUSION: These estimates are the first findings based on evaluating children by two different raters. The prevalence and comorbidity estimates are informative for researchers and clinicians of pediatric neurology. The disagreements between two different raters raise questions about previous estimates of neurodevelopmental disorders.

Additive dominant effect of a SOX10 mutation underlies a complex phenotype of PCWH.

Distinct classes of SOX10 mutations result in peripheral demyelinating neuropathy, central dysmyelinating leukodystrophy, Waardenburg syndrome, and Hirschsprung disease, collectively known as PCWH. Meanwhile, SOX10 haploinsufficiency caused by allelic loss-of-function mutations leads to a milder non-neurological disorder, Waardenburg-Hirschsprung disease. The cellular pathogenesis of more complex PCWH phenotypes in vivo has not been thoroughly understood. To determine the pathogenesis of PCWH, we have established a transgenic mouse model. A known PCWH-causing SOX10 mutation, c.1400del12, was introduced into mouse Sox10-expressing cells by means of bacterial artificial chromosome (BAC) transgenesis. By crossing the multiple transgenic lines, we examined the effects produced by various copy numbers of the mutant transgene. Within the nervous systems, transgenic mice revealed a delay in the incorporation of Schwann cells in the sciatic nerve and the terminal differentiation of oligodendrocytes in the spinal cord. Transgenic mice also showed defects in melanocytes presenting as neurosensory deafness and abnormal skin pigmentation, and a loss of the enteric nervous system. Phenotypes in each lineage were more severe in mice carrying higher copy numbers, suggesting a gene dosage effect for mutant SOX10. By uncoupling the effects of gain-of-function and haploinsufficiency in vivo, we have demonstrated that the effect of a PCWH-causing SOX10 mutation is solely pathogenic in each SOX10-expressing cellular lineage in a dosage-dependent manner. In both the peripheral and central nervous systems, the primary consequence of SOX10 mutations is hypomyelination. The complex neurological phenotypes in PCWH patients likely result from a combination of haploinsufficiency and additive dominant effect.

[Parenting resilience for rearing a child with autism spectrum disorder: a qualitative study].

OBJECTIVE: Resilience is defined as the dynamic process of positive adaptation despite the experience of adversity. The aims of this study were to apply the concept of resilience to the mothers of children with autism spectrum disorder (ASD), which we call "parenting resilience" for rearing a child with ASD, and to explain the construct of parenting resilience. METHODS: Interviews were conducted with 23 mothers of adults with ASD to collect data on rearing these children from infancy to adulthood. Data were analyzed using a modified grounded-theory approach. RESULTS: The analytic theme was the thought process from the problems associated with raising developmentally challenged children to the implementation of the appropriate coping method. We proposed a model comprising twelve concepts and five categories, i. e., "a sense of motherhood", "self-efficacy", "knowledge of the child's characteristics", "perceived social support", and "foresight". The model assumes that a sense of motherhood and self-efficacy motivate these mothers to cope with the problems associated with developmentally challenged children, and they derive the way of dealing with it from knowledge of the child's characteristics, perceived social support, and foresight. DISCUSSION: We suggest that the construct of parenting resilience for rearing a child with ASD is composed of the proposed categories and concepts.

Altered brain activity for phonological manipulation in dyslexic Japanese children.

Because of unique linguistic characteristics, the prevalence rate of developmental dyslexia is relatively low in the Japanese language. Paradoxically, Japanese children have serious difficulty analysing phonological processes when they have dyslexia. Neurobiological deficits in Japanese dyslexia remain unclear and need to be identified, and may lead to better understanding of the commonality and diversity in the disorder among different linguistic systems. The present study investigated brain activity that underlies deficits in phonological awareness in Japanese dyslexic children using functional magnetic resonance imaging. We developed and conducted a phonological manipulation task to extract phonological processing skills and to minimize the influence of auditory working memory on healthy adults, typically developing children, and dyslexic children. Current experiments revealed that several brain regions participated in manipulating the phonological information including left inferior and middle frontal gyrus, left superior temporal gyrus, and bilateral basal ganglia. Moreover, dyslexic children showed altered activity in two brain regions. They showed hyperactivity in the basal ganglia compared with the two other groups, which reflects inefficient phonological processing. Hypoactivity in the left superior temporal gyrus was also found, suggesting difficulty in composing and processing phonological information. The altered brain activity shares similarity with those of dyslexic children in countries speaking alphabetical languages, but disparity also occurs between these two populations. These are initial findings concerning the neurobiological impairments in dyslexic Japanese children.

[Relationship between magnocellular function and reading skills in children: a study using visual evoked potentials].

OBJECTIVE: Developmental dyslexia (DD) is a neurodevelopmental disorder that is characterized by difficulties with accurate and/or fluent word recognition and by poor spelling and decoding abilities. The magnocellular deficit theory is one of several hypotheses that have been proposed to explain the pathophysiology of DD. In this study, we investigated magnocellular system dysfunction in Japanese dyslexic children. METHODS: Subjects were 19 dyslexic children (DD group) and 19 aged-matched healthy children (TD group). They were aged between 7 and 16 years. Reversed patterns of black and white sinusoidal gratings generated at a low spatial frequency, high reversal frequency of 7.5 Hz, and low contrasts were used specifically to stimulate the magnocellular system. We recorded visual evoked potentials (VEP) from the occipital area and examined their relationship with reading and naming tasks, such as the time to read hiragana characters, rapid automatized naming of pictured objects, and phonological manipulation. RESULTS: Compared to the TD group, the DD group showed a significantly lower peak amplitude of VEPs through the complex demodulation method. Structural equation modeling showed that VEP peak amplitudes were related to the rapid automatized naming of pictured objects, and better rapid automatized naming resulted in higher reading skills. There was no correlation between VEP findings and the capacity for phonological manipulation. CONCLUSIONS: VEPs in response to the magnocellular system are useful for understanding the pathophysiology of DD. Single phonological deficit may not be sufficient to cause DD.

Abnormality in GABAergic postsynaptic transmission associated with anxiety in Bronx waltzer mice with an Srrm4 mutation.

The homozygous Bronx waltzer (bv) mouse, which shows hearing impairment, also exhibits anxiety accompanied by a reduction in cortical parvalbumin (PV)-positive GABAergic interneurons. Recently, a mutation in splicing factor Ser/Arg repetitive matrix 4 (Srrm4) was found in bv mice. However, the cellular consequences of the Srrm4 mutation for anxiety remain unknown. Here, we tested our hypothesis that bv mutant primarily affects interneurons through a cell-intrinsic pathology that leads to a reduction of interneurons and consequently causes anxiety. We found that the anxiety becomes apparent at 6 weeks of age in bv/bv mice. However, in situ hybridization revealed that Srrm4 is not expressed in interneurons, but rather dominates in pyramidal neurons. In addition, the PV-positive GABAergic interneurons were not reduced in number in the bv/bv cortex when anxiety became evident. However, electrophysiological abnormality of GABAergic transmission from interneurons was concomitantly present. Pharmacological blockage of GABAA receptors revealed increased excitability in bv/bv mice, although no gross change occurred in the expression of an Srrm4-downstream gene, Kcc2, which regulates chloride flux upon GABAergic transmission. These findings suggest that the bv-associated Srrm4 mutation mainly involves post-synaptic GABAergic transmission in the central nervous system, which may be associated with the anxiety phenotype in bv/bv mice.

Increased prefrontal oxygenation related to distractor-resistant working memory in children with attention-deficit/hyperactivity disorder (ADHD).

This study aimed at investigating the effect of distraction on working memory and its underlying neural mechanisms in children with attention-deficit/hyperactivity disorder (ADHD). To this end, we studied hemodynamic activity in the prefrontal cortex using near-infrared spectroscopy while 16 children with ADHD and 10 typically developing (TD) children performed a working memory task. This task had two conditions: one involved a distraction during the memory delay interval, whereas the other had no systematic distraction. The ADHD patients showed significantly poorer behavioral performance compared with the TD group, particularly under the distraction. The ADHD group exhibited significantly higher level of prefrontal activation than did TD children. The activity level was positively correlated with the severity of ADHD symptoms. These results suggest that the impairment in the inhibition of distraction is responsible for the working memory deficits observed in ADHD children. Inefficient processing in the prefrontal cortex appears to underlie such deficits.

[Development of a training program for Japanese dyslexic children and its short-term efficacy].

OBJECTIVE: The purpose of this study is to develop a computer training program of reading for the Japanese dyslexic children and to examine its short-term efficacy on their reading and writing abilities. METHODS: Fifteen dyslexic children underwent two sets of training programs, one for single-hiragana and non-word reading, and the other for the reading of real words, in which each hiragana was followed by the correctly read sound. Subjects were required to use a given program for five minutes a day for three weeks, switching to the other program after a three-week interval. Four kinds of reading test and one writing test were done at the beginning and end of each program period. RESULTS: The averages reading speeds increased, and the single-hiragana reading error average was lower after the training. Hiragana-writing errors also decreased, even though no writing procedure was involved in the programs. CONCLUSIONS: The results indicate the usefulness of these training programs as an early intervention of reading and writing for the Japanese dyslexic children.

Modulation effects of the intact motor skills on the relationship between social skills and motion perceptions in children with autism spectrum disorder: A pilot study.

BACKGROUND: An individual with autism spectrum disorder (ASD) has social skill, motor skill, and motion perception deficits. However, the relationship among them was not clarified. Therefore, this study aimed to evaluate the effects of motor skills on social skills and motion perception. METHODS: Five typically developed children and fourteen children with ASD participated in our study. The N200 component, a brain activity indicating motion perception, was induced in mid-temporal (MT/V5) brain area by watching a random dot kinematograph, and was recorded using a scalp electroencephalogram. Furthermore, the social responsiveness scale (SRS) indicating the social skill deficit, the developmental coordination disorder questionnaire (DCDQ) estimating the developmental coordination disorder (DCD), and the movement assessment battery for children second edition (MABC-2) indicating motor skills were recorded in the children with ASD. A hierarchical multiple regression analysis was conducted to examine the modulation effects of motor skills on the relationship between social skills and motion perception. The dependent variable was the N200 latency, and the independent variables were SRS, MABC-2, and combined MABC-2 and SRS. RESULTS: The N200 latency was more delayed in children with ASD relative that in typically developed children. Intact balance ability modulated the relationship between social skills and N200 latency in children with ASD. Within the high balance ability, when the social skills worsened, the N200 latency was shortened. CONCLUSIONS: This is the first report that intact motor skills could modulate the relationship between social skills and motion perception.

[Effectiveness of social skills training for children with developmental disorders: behavioral analysis using a two-dimensional motion capture system].

The current study sought to develop a new behavioral analysis methods to evaluate the effects of social skills training (SST). SST is known to be an effective method to improve the social skills of children with behavioral problems. However, current evaluation methods involve behavioral rating scales that are heavily dependent on evaluators' particular experiences they have had. To quantitatively examine the behavioral effects of SST, we examined subjects' head-movements related to social behavior, using a two-dimensional motion capture system (Kissei Comtec, Japan). Four children (three male, one female, 7-8 years of age) with pervasive developmental disorder (PDD) or attention deficit/hyperactivity disorder (AD/HD) participated in 16 sessions of SST. Before and after SST, head-coordinates on a two-dimensional plane were calculated using their behavior during a pair task, measured by four digital cameras. After SST, the number of communication behaviors was increased compared to before SST. In addition, children looked longer at another child within 30 degrees of the central visual field. Time-series analysis of the visual field during the detection of another child revealed significant auto-correlation from about -1.12 second. before to the beginning of communication behavior (p<0.05). The results suggested that our method can provide a quantitative index of characteristics related to skilled social behaviors. We conclude that a two-dimensional motion capture system would be useful for visualization of the interventional effects of SST, which would supplement assessments by the conventional observational strategies.

[Effects of a rich emotionally-satisfying childbirth experience of mothers on their later parental attitudes and behavior in school-age children].

This study examined the effects of a rich, emotionally-satisfying childbirth experience (CBE) of mothers on the behavior of school-age children using longitudinal data measured from immediately to 7 years and 6 months after birth. The results of structural equation modeling revealed the following: 1) giving birth in a midwifery center enhances emotional satisfaction with CBE, 2) a rich CBE of mothers was associated with parental warmth, 3) parental warmth during early childhood increased prosocial behavior and reduced behavioral problems in school-age children, and 4) temperamentally difficulty in early childhood were linked to later behavioral problems in school-age children. Thus, a rich CBE and parental warmth were suggested to be factors contributing to the good behavior of school-age children.

Detection of deviance in Japanese kanji compound words.

Reading fluency is based on the automatic visual recognition of words. As a manifestation of the automatic processing of words, an automatic deviance detection of visual word stimuli can be observed in the early stages of visual recognition. To clarify whether this phenomenon occurs with Japanese kanji compounds-since their lexicality is related to semantic association-we investigated the brain response by utilizing three types of deviants: differences in font type, lexically correct or incorrect Japanese kanji compound words and pseudo-kanji characters modified from correct and incorrect compounds. We employed magnetoencephalography (MEG) to evaluate the spatiotemporal profiles of the related brain regions. The study included 22 adult native Japanese speakers (16 females). The abovementioned three kinds of stimuli containing 20% deviants were presented during the MEG measurement. Activity in the occipital pole region of the brain was observed upon the detection of font-type deviance within 250 ms of stimulus onset. Although no significant activity upon detecting lexically correct/incorrect kanji compounds or pseudo-kanji character deviations was observed, the activity in the posterior transverse region of the collateral sulcus (pCoS)-which is a fusiform neighboring area-was larger when detecting lexically correct kanji compounds than when detecting pseudo-kanji characters. Taken together, these results support the notion that the automatic detection of deviance in kanji compounds may be limited to a low-level feature, such as the stimulus stroke thickness.

Effect of corpus callosotomy on attention deficit and behavioral problems in pediatric patients with intractable epilepsy.

To evaluate the effect of corpus callosotomy (CC) on attention deficit and behavioral problems in pediatric patients with intractable epilepsy, we retrospectively investigated sequential patients who had undergone CC to control seizures. Between August 2005 and April 2010, a total of 15 patients aged between 3.1 and 17.9 years underwent CC at our institute. All the patients experienced either drop attacks or head nodding, which were considered to be therapeutic targets of CC. A standardized instrument, the Child Behavior Checklist (CBCL), was used to assess behavioral and emotional problems before and after surgery. On postoperative EEGs, 8 (53%) showed improvement and 7 (47%) showed no change in epileptiform discharges. The Attention Problems scale and total score on the CBCL significantly improved in patients whose postoperative EEGs showed improvement. In addition to amelioration of target seizures, CC can improve attention impairments in association with improvement in the postoperative EEG.

Multiple band frequency analysis in a child of medial temporal lobe ganglioglioma.

We report a 1-year 6-month-old girl with ganglioglioma in the right medial temporal lobe who showed epileptic spasms in clusters. Spasms occasionally followed a dazed and fearful gaze. Interictal electroencephalography (EEG) showed diffuse bursts of slightly irregular high-voltage spikes and slow waves without hypsarrhythmia. The findings on ictal EEG, single-photon emission computed tomography, and F-18 fluorodeoxyglucose positron emission tomography indicated focus on the right medial temporal lobe. Ictal fast rhythmic activity analysis of scalp EEG by multiple band frequency analysis showed gamma rhythms at 65-80 Hz with a high spectral power around the tumor area. Epileptic spasms completely disappeared after tumor resection. These findings suggest that the cerebral cortex may be a source of epileptic spasms and indicate the possibility of usefulness of fast activity analysis in this condition.

[Developmental changes of rapid automatized naming and Hiragana reading of Japanese in elementary-school children].

Two hundred and seven Japanese elementary school children aged from 6 (Grade 1) to 12 (Grade 6) years old were tested for their abilities to name numbers and pictured objects along with reading Hiragana characters and words. These children all showed typical development and their classroom teachers judged that they were not having any problems with reading or writing. The children were randomly divided into two groups, the first group was assigned to two naming tasks;the rapid automatized naming (RAN) of "numbers" and "pictured objects," the second group was assigned to two rapid alternative stimulus (RAS) naming tasks using numbers and pictured objects. All children were asked to perform two reading tasks that were written in Hiragana script: single mora reading task and four syllable word reading task. The total articulation time for naming and reading and performance in terms of accuracy were measured for each task. Developmental changes in these variables were evaluated. The articulation time was significantly longer for the first graders, and it gradually shortened as they moved through to the upper grades in all tasks. The articulation time reached a plateau in the 5th grade for the number naming, while gradual change continued after drastic change in the lower grades for the pictured object naming. The articulation times for the single mora reading and RAN of numbers correlated strongly. The articulation time for the RAS naming was significantly longer compared to that for the RAN, though there were very few errors. The RAS naming showed the highest correlation with the four syllable word reading. This study demonstrated that the performance in rapid automatized naming of numbers and pictures were closely related with performance on reading tasks. Thus Japanese children with reading disorders such as developmental dyslexia should also be evaluated for rapid automatized naming.

The Relationships Among Autism Spectrum Disorder Traits, Loneliness, and Social Networking Service Use in College Students.

This study aimed to explore the relationships among autism spectrum disorder (ASD) traits, loneliness, and social networking services (SNS) use. We created a questionnaire to evaluate manners during LINE use, which included five factors: "low literacy," "inactive use," "low responsiveness," "lack of consideration," and "low group activity." Structural equation modeling revealed that difficulties in attention switching and low communication skills were associated with low literacy, low social skills were associated with inactive use, and low literacy and inactive use were associated with loneliness. We suggested that SNS use plays a role in maintaining and enhancing friendships, whereas college students with higher ASD traits tend to use inappropriate manners for SNS, which is associated with loneliness.

Effect of anti-attention-deficit hyperactivity disorder (ADHD) medication on clinical seizures and sleep EEG: A retrospective study of Japanese children with ADHD.

AIMS: Patients with attention-deficit hyperactivity disorder (ADHD) often exhibit basic or paroxysmal wave abnormalities on electroencephalography (EEG). Methylphenidate (MPH), an anti-ADHD stimulant, has been reported to lower the seizure threshold. However, there have been no reports comparing EEG changes before and after administration of the central nervous system (CNS) stimulant MPH, or atomoxetine (ATX) hydrochloride, a non-CNS stimulant. In this study, we investigated changes in sleep EEG before and after the administration of ADHD treatment drugs. METHOD: With the approval of the ethics committee, the medical records of 28 children with ADHD (23 men and 5 women) who gave consent were retrospectively investigated. The appearance of sudden abnormal waves during a 10-minute sleep EEG recording was measured in 0.1-second units, and the duration of these waves was calculated as the paroxysmal index (PI). RESULTS: Paroxysmal index did not differ significantly between patients who received MPH and those who received ATX. In addition, there were no exacerbations of clinical seizures. CONCLUSION: It was concluded that ADHD medications do not have an adverse effect on epileptic seizures or abnormal sleep EEGs.