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To compare the scores of the Bayley Scales of Infant Development second edition (BSID-II) and the third edition, Bayley-III, in children with suspected developmental delay and to determine the cutoff score for developmental delay in the Bayley-III.
Children younger than 42 months (n=62) with suspected developmental delay who visited our department between 2014 and 2015 were assessed with both the BSID-II and Bayley-III tests.
The mean Bayley-III Cognitive Language Composite (CLC) score was 5.8 points higher than the mean BSID-II Mental Developmental Index (MDI) score, and the mean Bayley-III Motor Composite (MC) score was 7.9 points higher than the mean BSID-II Psychomotor Developmental Index (PDI) score. In receiver operating characteristic (ROC) analysis of a BSID-II MDI score <70, Bayley-III CLC scores showed a cutoff of 78.0 (96.6% sensitivity and 93.9% specificity). In ROC analysis of a BSID-II PDI score <70, the Bayley-III MC score showed a cutoff of 80.
There was a strong correlation between the BSID-II and Bayley-III in children with suspected developmental delay. The Bayley-III identified fewer children with developmental delay. The recommended cutoff value for developmental delay increased from a BSID-II score of 70 to a Bayley-III CLC score of 78 and Bayley-III MC score of 80.
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To investigate the neurodevelopmental outcomes in children with developmental disorder according to visual evoked potential (VEP) results.
We retrospectively analyzed children who visited our Department of Pediatric Rehabilitation Medicine with a chief complaint of developmental disability from January 2001 to July 2015. Of the 549 medical records reviewed, 322 children younger than 42 months who underwent both Bayley Scales of Infant and Toddler Development second edition (BSID-II) and VEP studies were enrolled. We compared the development of 182 children with normal VEP latency and 140 children with delayed VEP latency results using the BSID-II results. The Mann-Whitney U-test was used to analyze the differences between the two groups.
There were no significant differences in baseline characteristics between the two groups. The delayed VEP latency group showed a significant delay in BSID-II index scores and developmental quotients compared with the normal VEP latency group. In addition, a comparative analysis of developmental quotients of mental and psychomotor domains according to age (younger than 12 months, 12–23 months, and 24–42 months) revealed significantly lower values in children with delayed VEP latency compared to children with normal VEP latency, younger than 12 months and from 12 to 23 months.
Children with delayed VEP latency showed more developmental delay than children with normal VEP latency. It is suggested that VEP can be easily applied to children with suspected developmental delay when physicians have concerns about visual impairment. Furthermore, it is proposed that VEP results could provide an insight into children's development and serve as early indicators for consultation with an ophthalmologist for the existing problem.
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Patients with a duplication from 7q36 to the terminus or a deletion of 9p24 have been reported, whereas those harboring both mutations have not. Here, we report a patient with simultaneous de novo 7q36.1-q36.3 duplication and 9p24.3 deletion. A 6-year-old boy presented with speech developmental delay, microcephaly, and dysmorphic features, including a long face and small nose. Chromosome and array comparative genomic hybridization analyses revealed 46,XY,dup(7)(q36.1-q36.3) and del(9)(p24.3). The sizes of the duplication and deletion were 9.9 Mb and 1.9 Mb, respectively. The duplication of chromosome 7 contained 68 known genes, of which 3 are related with entries in the Developmental Disorders Genotype-to-Phenotype (DDG2P) database. The deletion of chromosome 9 contained 6 known genes, of which 2 are in the DDG2P database. We investigated the genotype and phenotype in this patient, and reviewed the relevant literatures for possible clinical presentation in these variations.
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To identify the usefulness of both the Bayley Scales of Infant and Toddler Development, 3rd edition (Bayley-III) and Denver Developmental Screening Test II (DDST-II) in preterm babies with neurodevelopmental impairment, considering the detection rate as regulation of criteria.
Retrospective medical chart reviews which included the Bayley-III and DDST-II, were conducted for 69 preterm babies. Detection rate of neurodevelopmental impairment in preterm babies were investigated by modulating scaled score of the Bayley-III. The detection rate of DDST-II was identified by regarding more than 1 caution as an abnormality. Then detection rates of each corrected age group were verified using conventional criteria.
When applying conventional criteria, 22 infants and 35 infants were detected as preterm babies with neurodevelopmental impairment, as per the Bayley-III and DDST-II evaluation, respectively. Detection rates increased by applying abnormal criteria that specified as less than 11 points in the Bayley-III scaled score. In DDST-II, detection rates rose from 50% to 68.6% using modified criteria. The detection rates were highest when performed after 12 months corrected age, being 100% in DDST II. The detection rate also increased when applying the modified criteria in both the Bayley-III and DDST-II.
Accurate neurologic examination is more important for detection of preterm babies with neurodevelopmental impairment. We suggest further studies for the accurate modification of the detection criteria in DDST-II and the Bayley-III for preterm babies.
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Joubert syndrome (JS) is a rare genetic disorder characterized by a congenital malformation of the hindbrain, and accompanied by axonal decussation abnormalities affecting the corticospinal tract and the superior cerebellar peduncles. To the best of our knowledge, there are no reports of normal pyramidal decussation in JS. Here, we describe the case of an 18-year-old boy presenting midline-crossing corticospinal projections, which were considered normal corticospinal tract trajectories. Diffusion tensor imaging and motor evoked potential study analysis demonstrated the exclusive presence of decussating corticospinal projections in the patient. Based on these results, we suggest that JS might be associated with several, diverse corticospinal motor tract organization patterns.
To evaluate the effectiveness of intensive neurodevelopmental treatment (NDT) on gross motor function for the children having developmental delay (DD), with or without cerebral palsy (CP).
Forty-two children had intensive NDT three times weekly, 60 minutes a day, for 3 months, immediately followed by conventional NDT once or twice a week, 30 minutes a day, for another 3 months. We assessed Gross Motor Function Measure (GMFM) over three time points: before conventional NDT, before and after intensive NDT, and after 3 months of additional conventional NDT.
The GMFM score in DD children significantly improved after intensive NDT, and the improvement maintained after 3 months of conventional NDT (p<0.05). The children were further divided into two groups: DD with CP and DD without CP. Both groups showed significant improvement and maintained the improvements, after intensive NDT (p<0.05). Also, there was no significant difference in treatment efficacy between the two groups. When we calculate the absence rate for comparing the compliance between intensive and conventional NDT, the absence rate was lower during the intensive NDT.
Intensive NDT showed significantly improved gross motor function and higher compliance than conventional NDT. Additionally, all improvements were maintained through subsequent short-term conventional NDT. Thus, we recommend the intensive NDT program by day-hospital centers for children with DD, irrespective of accompanying CP.
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Cerebral Palsy: Current Opinions on Definition, Epidemiology, Risk Factors, Classification and Treatment Options
Genetic screening is being widely applied to trace the origin of global developmental delay or intellectual disability. The 5q14.3 microdeletion has recently been uncovered as a clinical syndrome presenting with severe intellectual disability, limited walking ability, febrile convulsions, absence of speech, and minor brain malformations.
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Arnold-Chiari malformation type III (CM III) is an extremely rare anomaly with poor prognosis. An encephalocele with brain anomalies as seen in CM II, and herniation of posterior fossa contents like the cerebellum are found in CM III. The female infant was a twin, born at 33 weeks, weighing 1.7 kg with a huge hydrocele on the craniocervical junction. After operations were performed, she was referred to the department of rehabilitation medicine for poor motor development, swallowing dysfunction, and poor eye fixation at 22 months. The child was managed with neurodevelopmental treatment, oromotor facilitation, and light perception training. After 14 months, improvement of gross motor function was observed, including more stable head control, rolling, and improvement of visual perception. CM III has been known as a condition with poor prognosis. However, with the improvement in operative techniques and intensive rehabilitations, the prognosis is more promising than ever before. Therefore, more attention must be paid to the rehabilitation issues concerning patients with CM III.
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