OBJECTIVE:While posterior cerebral artery (PCA) involvement in moyamoya disease (MMD) is associated with poor educational and social outcomes, its specific impact on neurocognitive function remains unclear. The aim of this study was to clarify the neurocognitive profile of pediatric patients with MMD with PCA involvement. METHODS:This cross-sectional study included pediatric patients with MMD who underwent baseline neuropsychological assessment at a single institution between June 2016 and December 2023. Neurocognitive function was evaluated using the Das Naglieri Cognitive Assessment (CAS) system and the Wechsler Intelligence Scale for Children, Fourth Edition (WISC-IV). Profiles were compared between patients with and without PCA involvement using multivariate ANOVA (MANOVA), followed by multiple linear regression analyses. Regional cerebral blood flow was assessed using 3D stereotactic surface projection analysis and the stereotactic extraction estimation method. RESULTS:Fifty-two patients, including 11 with PCA involvement and 41 without, were analyzed. MANOVA revealed significant overall between-group differences across the 4 CAS domains and the 4 WISC-IV index scores. Post hoc comparisons showed that patients with PCA involvement had significantly lower scores in the CAS planning domain (p < 0.001) and WISC-IV processing speed index (p = 0.002), both of which involve timed tasks requiring visual scanning, number matching, connecting ordered sequences, and symbol coding. PCA involvement remained significantly associated with these deficits after adjustment for confounders. Patients with PCA involvement exhibited more severe hypoperfusion in the temporo-parieto-occipital regions than those without PCA involvement. CONCLUSIONS:Patients with MMD with PCA involvement exhibited a distinct neurocognitive profile characterized by weakness in visual processing speed and visual search, accompanied by hypoperfusion in the temporo-parieto-occipital regions. Early identification of these deficits could facilitate targeted educational and rehabilitative support, and long-term postoperative evaluation is required.

OBJECTIVE:Selective intraindividual weakness in successive processing, which is a unique verbal working memory scale included in the Das-Naglieri Cognitive Assessment System (CAS), is considered one of the intrinsic neurocognitive characteristics in pediatric moyamoya disease (MMD). The aim of the study was to elucidate the association between cerebral blood flow (CBF) and weakness in successive processing, and to identify regions related to the weakness. METHODS:The present cross-sectional study included children who had been diagnosed with MMD and were assessed using neuropsychological tests before surgery between June 2016 and December 2023. According to the CAS manual, intraindividual difference was calculated by subtracting the mean of the 4 standard scores from each standard score. Each patient was classified as either manifesting "intraindividual weakness in successive processing" (intraindividual difference of successive processing < 0) or not (intraindividual difference of successive processing ≥ 0), and CBF acquired with resting-state SPECT was compared between groups. Three-dimensional stereotactic surface projection (3D-SSP) was also used for topographical comparison of CBF. RESULTS:Of 51 children (mean age ± SD at admission 8.0 ± 2.6 years) who underwent preoperative neuropsychological tests, 43 were included in the CBF analysis. Both standard scores and intraindividual difference of the CAS significantly varied across 4 domains (p = 0.006 and p < 0.001, respectively), and those of successive processing were the lowest. Of the children analyzed, 35 (68.7%) were classified as having intraindividual weakness in successive processing. Multiple logistic regression analysis revealed the severest ischemic grade was significantly associated with intraindividual weakness in successive processing (OR 5.49 [95% CI 1.12-27.06]). Three-dimensional SSP analysis demonstrated a significant CBF decrease in the left dorsolateral and medial prefrontal cortexes in the children showing intraindividual weakness in successive processing compared with those who did not. CONCLUSIONS:Intraindividual weakness in successive processing typical of MMD might be associated with reduced CBF in the dorsolateral and medial prefrontal cortex, predominantly in the left hemisphere. Further studies in this area could contribute to the improvement of long-term social outcomes for patients with MMD.

This study describes the content validity process, focusing on children, to create and validate a tool for assessing motivation toward out-of-school physical activity (PA) among children with autism spectrum disorder (ASD) aged 6 to 17 years. Additionally, it establishes the eligible verbal communication age range necessary for its application. The initial development and content validity process with external experts is described elsewhere (An et al., 2025 under review). The MAT-PA was iteratively refined during the content validity process with twenty children (two with version 0.1, ten with version 0.2, and eight with version 0.3). Modifications were made based on feedback from children who completed the entire interview. The Vineland Adaptive Behavior Scales, Second Edition (VABS-2), assessed the age-equivalent for daily verbal communication skills required for the MAT-PA. Feedback from the twelve children who completed the entire interview process (two with version 0.1, six with version 0.2, and four with version 0.3) provided evidence supporting the tool's content validity. Challenges with attention spans and verbal abilities limited full participation from the remaining eight children. Parent-reported VABS-2 scores indicated that the MAT-PA is suitable for children with ASD who have verbal communication skills equivalent to 3-year-olds (receptive) and 6-year-olds (expressive). The MAT-PA is the first tool specifically designed to explore the psychological needs and behavioral regulation of children with ASD, providing evidence of content validity. Future work should focus on improving the tool's reliability for trial integration, exploring its applicability across diverse contexts, and leveraging technology to boost scalability and impact.

Background Successive processing, a form of working memory function detected with the Das Naglieri Cognitive Assessment System, is selectively impaired in pediatric moyamoya disease. We aimed to test whether successive processing in children with moyamoya disease was improved after bypass surgery under the control of confounding.Methods The present retrospective cohort study included children with moyamoya disease who underwent direct or combined bypass surgery. Neuropsychological tests including the Das Naglieri Cognitive Assessment System were administered at 2 time points, before and after surgery, approximately 1 year apart. The least squares (LS) mean standard score and LS mean difference between time points were calculated using a mixed model for repeated measures, which included 5 clinical factors along with the time point. Models including an interaction term were also generated to assess the effect of each clinical factor. Cognitive intra-individual variability across 4 domains of the Das Naglieri Cognitive Assessment System was assessed with an analysis of variance at each time point.Results Of 60 patients who underwent surgery, 42 fulfilled the inclusion criteria. The median duration between assessments was 15 months. The LS mean standard scores of successive processing increased after surgery (LS mean, 95.8 versus 100.2; LS mean difference, 4.4 [95% CI, 1.5-7.3]; P = 0.004). The increase was more pronounced in those with a younger age at onset of neurological symptoms, shorter delay before surgery, preexisting infarct, posterior cerebral artery involvement, and more severe ischemic stage before surgery. Intraindividual variability, shown as the lowest score of successive processing at baseline, resolved after surgery (F = 3.56, P = 0.016 versus F = 1.21, P = 0.31). Successive processing was the domain most likely to be improved after surgery.Conclusion The present results suggest that successive processing is improved after bypass surgery. Larger and longer follow-up studies are required to confirm the influencing factors and long-term effects.

Although cognitive impairment is well-documented in children with moyamoya disease (MMD), selective decline in specific neurocognitive domains remains controversial. The purpose of this study was to characterize the neurocognitive profile of children with MMD using the Das Naglieri Cognitive Assessment System (CAS) and the Wechsler Intelligence Scale for Children, Fourth Edition (WISC-IV). We analyzed the neurocognitive data of 30 children (median age, 7 years) with MMD who were assessed with the CAS and the WISC-IV before surgery. We focused on the comparison of standard scores and intraindividual differences across domains. The CAS scores significantly varied across four measures (standard scores, p < 0.001; intraindividual differences, p < 0.001). Post-hoc analyses revealed that the standard scores and intraindividual differences for successive processing were significantly lower than those for planning and attention. The WISC-IV scores did not significantly vary among the four measures, although the working memory index was the lowest among the four measures. The within-individual weakness in successive processing, a form of working memory function, may be a distinct characteristic of children with MMD. The CAS may be more sensitive than the WISC-IV for detecting this selective neurocognitive weakness in children with MMD.

もやもや病は厚生労働省の定める指定難病であり, わが国における若年者脳卒中の原因として上位を占める。京都大学医学部附属病院では, 2016 年にもやもや病支援センターを設置し, 筆者らは特に小児もやもや病患者の高次脳機能評価およびリハビリテーションや復学支援を積極的に実施している。小児では標準化された神経心理学的検査が成人と比較して少ないことから, 小児もやもや病患者の高次脳機能障害の特徴は明らかになっていない。本稿では, 当院の小児もやもや病患者の高次脳機能の特徴に関する予備的研究および事例を紹介し, 臨床適用の提言をする。

OBJECTIVE:Cognitive impairments are among the nonmotor symptoms in patients with Parkinson disease. Understanding the cognitive impairments in patients with Parkinson disease may be critical for developing effective rehabilitation interventions. The aims of this study were to assess cognitive function in patients with Parkinson disease using the Wechsler Adult Intelligence Scale Third Edition, and the Wechsler Memory Scale Revised and to investigate how cognitive impairments relate to progression of disease in patients with Parkinson disease according to the Hoehn and Yahr stages.DESIGN:Seventy-eight patients with Parkinson disease participated in the present study. Our study consisted of patients in the following Hoehn and Yahr groups: 1 (no disability, n = 11), 2 (mild, n = 34), 3 (moderate, n = 26), and 4 and 5 (severe, n = 7). Cognitive function was assessed using the Wechsler Adult Intelligence Scale Third Edition, and the Wechsler Memory Scale Revised.RESULTS:The verbal memory was significantly higher in group 1 (106.4 ± 12.0) than in the other groups (2: 90.5 ± 14.0, 3: 89.9 ± 16.9, 4 and 5: 89.6 ± 11.4). Visual memory and delayed recall were similar to the results seen with verbal memory; however, the differences between groups were not statistically significant. The full-scale IQ was not significantly different (1: 107.3 ± 8.1, 2: 96.9 ± 18.2, 3: 96.7 ± 14.8, 4 and 5: 91.7 ± 9.5).CONCLUSIONS:These results suggest that a comprehensive assessment focused on memory impairments is important for applying the appropriate interventions in patients with early-stage Parkinson disease.

Cognitive impairment is among the most frequent non-motor symptoms in Parkinson's disease (PD). To clear the characteristics of cognitive impairment in patient with PD may be critical for the planning of effective rehabilitation interventions. However, a few studies have sought to investigate relationship between cognitive impairment and motor functions. The purpose of this study was to compare cognitive impairment of PD patients in relation to the motor impairment severity according to Hoehn and Yahr (HY) stages. The subjects of this study were 78 patients with PD (33 males and 45 females). The Cognitive function was assessed using the Wechsler Adult Intelligent Scale-III (WAIS-III) and Wechsler Memory Scale-Revised (WMS-R). The HY scale was used to evaluate the motor impairment severity as I (no disability, n = 11), II (mild, n = 34), III (moderate, n = 26), or IV–V (severe, n = 7). The Kruskal–Wallis test was used to compare the cognitive impairment the between HY groups. The difference in performance intelligence quotient (PIQ) of WAIS-III (I: 105.6 ± 8.6, II: 94.9 ± 17.7, III: 91.5 ± 14.3, IV–V: 82.9 ± 8.2) and general memory quotient (GMQ) of WMS-R (I: 105.5 ± 14.4, II: 91.8 ± 15.6, III: 90.0 ± 18.2, IV–V: 86.1 ± 13.0) between groups were significant, scores of IV-V stage were lowest. There were statistically significant differences in processing speed index and verbal memory quotient among the groups. The difference in other measurements was not significant the between groups. Our present findings indicated that the cognitive impairment was observed in deterioration of the motor impairment in PD patients. Specifically, in the factor scale level, the low processing speed index and the low verbal memory quotient reduced each of the PIQ and GMQ in the lowest HY group patients. Therefore, we suggest that a careful assessment of cognitive impairments, especially processing speed and verbal memory, is important in identifying appropriate interventions for PD with severe stage.

Irlen syndrome is a proposed perceptual processing disorder characterized by visual distortions while reading. Patients with this syndrome may experience light sensitivity, visual stress, and other related problems such as dyslexia. Tinted lenses and colored overlays have been designed to help individuals with the symptoms of Irlen syndrome. However, there is still debate over the effectiveness of these interventions and whether this syndrome actually exists. In this report, we describe a case involving an 8-year-old girl with dyslexia who experienced severe visual hypersensitivity and whose symptoms completely resolved after wearing tinted lenses. While it is possible that she experienced a psychogenic visual disturbance that was relieved because of the placebo effect, the clinical course of her symptoms matched the findings previously described by Irlen. The patient was unable to read without tinted lenses. With tinted lenses, she could read at the appropriate age level, suggesting that her difficulty was due to a problem in optical information processing. The concepts underlying Irlen syndrome are vaguely defined, and several groups insist that the visual stress associated with this syndrome might be responsible for dyslexia as well as other disorders. These ambiguous criteria may be responsible for the criticism over the validity of this condition. Although this was only an anecdotal case, our patient exhibited the core functional deficit described in Irlen syndrome and showed a dramatic improvement with tinted lenses; therefore, this case may facilitate investigations into the mechanism underlying Irlen syndrome, if it actually exists. Although further studies are required to confirm the validity of this syndrome and the treatment approach, Irlen syndrome should be recognized as a disorder since its symptoms can be easily relieved by wearing tinted lenses or color filters.