Improvement in a post-stroke pediatric patient with hemiplegia: Use of a hand-arm bimanual intensive therapy with hybrid assistive limb.

BACKGROUND: Pediatric stroke is a rare medical condition that often leads to long-lasting motor and cognitive impairments. Although therapies for adults after a stroke are well described, treatments for motor deficits following a pediatric stroke are yet to be investigated. We report a case of pediatric stroke in the chronic phase, in which a combination of novel treatments resulted in a significant improvement in physical function. CASE REPORT: A seven-year-old girl with a left hemispheric cerebral infarction lost almost all right upper extremity motor function. Following onabotulinumtoxinA treatment, she underwent hand-arm bimanual intensive therapy augmented with a hybrid assistive limb for 90 h over 15 days. Evaluation after the training revealed significant improvements in physical function, daily activities, and occupational performance. CONCLUSIONS: This report highlights the importance of innovative combinations of techniques in the treatment of pediatric stroke.

Genetic Analysis of UGT1A1 Polymorphisms Using Preserved Dried Umbilical Cord for Assessing the Potential of Neonatal Jaundice as a Risk Factor for Autism Spectrum Disorder in Children.

Neonatal jaundice has been suggested as a perinatal risk factor for autism spectrum disorder (ASD). We examined UGT1A1 polymorphisms to assess the potential of neonatal jaundice as a risk factor for ASD in children by using DNA extracted from preserved umbilical cord. In total, 79 children with ASD were genotyped for UGT1A1*28 (c.-41-40dup), UGT1A1*6 (c.211 G > A), and UGT1A1*27 (c.686 C > A). The allele frequency of UGT1A1*6 (OR = 1.34, p = 0.26) and UGT1A1*28 (OR = 0.80, p = 0.54) and the prevalence of UGT1A1*28/*6 diplotypes did not differ significantly from those in the control population. No UGT1A1*27 allele was detected in the subjects. ASD symptom assessment scores were not associated with UGT1A1*28/*6/*27 genotypes or UGT1A1*28/*6 diplotypes. These results suggest that neonatal jaundice is not significantly associated with ASD.

Control by one drop of solvent: selective preparation of guest release/trap-triggered interconvertible molecular crystals.

Interconvertible molecular crystals 1close and 1open composed of 4-pyridyl-1,3-indanedione dimer 1 were selectively obtained. Thermal removal of solvent molecules in 1open afforded 1close. Further dipping of 1close in a specific solvent reproduced 1open. No crystallinity loss was observed even though both processes involved a drastic change of molecular packing arrangements.

[Reliability of the measurement of stature in subjects with severe motor and intellectual disabilities].

OBJECTIVE: The objective of this study was to examine the reliability of the measurement of stature in individuals with severe motor and intellectual disabilities. METHODS: Using a stratified sampling method 12 subjects (mean age of 28.7 +/- 11.5), were selected from 73 subjects with severe motor and intellectual disabilities (mean age of 37.1 +/- 15.0). Each subjects'stature was measured using two measurement methods. One measured the tibia length (TL-method) and, the other measured the whole body by measuring three sections. (Division-method). 3 examiners measured all subjects using the TL-method and Division-method two times repeatedly. In addition, one examiner measured all subjects within two weeks following the initial measurement. Intra-rater reliability was calculated from single and two times measurements using each method. The Inter-rater reliability was calculated using measurement results from 2 or 3 examiners in both single and two times measurements. The correlation between values measured by the TL-method and the Division-method was calculated using Spearman rank correlation coefficient. RESULTS: The TL-method and Division-method had good intra-rater reliability (ICC>0.90, 95%CI>0.80) and good inter-rater reliability (ICC>0.90, 95%CI>0.70) in both measurement methods. For both measurement methods, inter-rater reliability was more preferable when 3 examiners measured two times repeatedly (ICC>0.90, 95%CI>0.90). There was good correlation between values measured by TL-method and the Division-method (r=0.83). CONCLUSIONS: Both TL-method and Division-method had good reliability. However, the TL-method could be considered a more useful measurement method as it can be completed more easily and in a short period of time.

[A study of cognitive and behavioral development in pre-school and school children with high functioning pervasive developmental disorder].

It has been reported that school-aged children with high functioning pervasive developmental disorder (HFPDD) have numerous difficulties in their school class. We used three psychological tests to investigate whether there is a relationship between intelligence and cognitive, behavioral development in children with HFPDD. The three tests used were an intelligence test (WIPPSI, WISC-lll), the P-F (Picture Frustration) study, and behavioral assessment by their parents. In the P-F study, 60% of 23 children with HFPDD showed a GCR% (Group Conformity Rating) above or below the standard. There was no relationship between GCR% and IQ. In the behavioral assessment by their parents, over 50% of 40 children with HFPDD showed maladaptive behaviors. The high VIQ group showed more maladaptive behaviors than the low VIQ group. These findings suggest that school-aged children with H-FPDD need educational treatment for social deficits and maladaptive behaviors.

[Neuroradiological studies in Rett syndrome].

Rett syndrome is a neurodevelopmental disorder characterized by autistic behavior as well as cognitive and motor skill loss that occurs early in life and almost exclusively affects females. We studied the neuroradiological findings from MRI, SPECT, and proton magnetic resonance spectroscopy (1H-MRS) in 4 cases of Rett syndrome. Three of the 4 cases were diagnosed as Rett syndrome by gene analysis of MECP2. MRS demonstrated a decrease of N-acetylaspartate (NAA) in all 4 cases of Rett syndrome. In addition, MRI revealed frontal cortical atrophy and SPECT demonstrated low blood flow in the frontal lobe. We speculated that this decrease in NAA reflected neuronal loss, immaturity or hypofunction in these regions. The results of our study were in agreement with the previous studies on Rett syndrome by neuropathological methods.

Growth of head circumference in autistic infants during the first year of life.

This study analyzed the increase in head circumference (HC) of 85 autistic infants (64 boys and 21 girls) during their first year of life. The data were collected from their "mother-and-baby" notebooks. This notebook is a medical record of the baby's growth and development delivered to the parents of all babies born in Japan. This is a retrospective study which gathered the data from the notebooks after the diagnosis of autism. However, none of the babies were known to have autism at the time the records were made. The head circumference at birth of these autistic children was similar to that of the average found in a Japanese Government Study of 14,115 children. However, it showed a marked increase at 1 month after birth. The discrepancy reached a peak at 6 months, while the difference became smaller at 12 months. Body length (BL) and body weight (BW) began to increase at 3 months, although at a rate smaller than the head circumference increase.

Long-term follow-up of a patient with subacute sclerosing panencephalitis successfully treated with intrathecal interferon alpha.

To clarify long-term effects of intrathecal administration of interferon (IFN)-alpha in subacute sclerosing panencephalitis (SSPE), we followed up a patient with SSPE for 18 years, who had been treated successfully using intrathecal IFN-alpha with response-based dose adjustments. IFN-alpha therapy dramatically induced remission of disease and greatly improved quality of life for 7-8 years, but this was followed by severely deterioration with decorticate posturing and akinetic mutism. Thus, IFN-alpha-induced remission appears most likely to be temporary, even when an SSPE patient shows an excellent initial response. To improve long-term outcome for SSPE patients, more effective therapy is needed.

[Autistic disorders].

Autism is a syndrome that consists of disturbances in social interactions, communication and imagination, and its first-choice of therapy is education. Of special interest is the period of the onset and early diagnosis of autism, and its relation to the period of intervention. In retrospective studies from questionnaire and home videotape analysis, it has been reported that autistic children display significantly less social interaction, joint attention behavior and communicative behavior compared to typical children, before 12 to 18 months of age. Although there is less evidence of a marked reduction in autistic symptomatology, early intervention studies have been found to have positive effects with significant improvements being reported in social behavior, self care, and academic skill. However, early interventions are controversial. We discuss early diagnosis and interventions in children with autism in this paper.

[High functioning pervasive developmental disorder: coexistence with other clinical disorders].

We examined 50 children in high functioning pervasive developmental disorder (HFPDD) to study the comorbidity with other clinical disorders by questionnaires. Seventy-two percentage of them met the criteria for attention deficit hyperactivity disorder (AD/HD). The majority of the children had intelligence quotient less than 90. Learning problems, motor control problems, abnormal responses to sensory stimuli and anxiety disorder frequently coexisted in the children. Since clinical symptoms of HFPDD so diverse, it is possible that some children with HFPDD may be overlooked and diagnosed as having its coexisting disorders.

[An open trial of risperidone in autistic children].

Clinical effects of risperidone were evaluated in 9 young autistic children under informed consent of their parents. The patients were evaluated by the Children's Behavioral Checklist and Rutter's Autistic Behavioral Rating Scale. After the administration, two subjects (playing and adaptation to change) of the Children's Behavioral Checklist and four (anomalous autistic behavior, destructive behavior, developmental problem and activity level) of the Rutter's Autistic Behavioral Rating Scale were improved significantly. There were no serious side effects such as extrapyramidal symptoms except minor adverse effects including sedation, depression, increased appetite and constipation. Risperidone might be effective for serious behavioral disturbances in young autistic children.