The Long-Term Outcome and Rehabilitative Approach of Intraventricular Hemorrhage at Preterm Birth

Technological advances in neonatology led to the improvement of the survival rate in preterm babies with very low birth weights. However, intraventricular hemorrhage (IVH) has been one of the major complications of prematurity. IVH is relevant to neurodevelopmental disorders, such as cerebral palsy, language and cognitive impairments, and neurosensory and psychiatric problems, especially when combined with brain parenchymal injuries. Additionally, severe IVH requiring shunt insertion is associated with a higher risk of adverse neurodevelopmental outcomes. Multidisciplinary and longitudinal rehabilitation should be provided for these children based on the patients’ life cycles. During the infantile period, it is essential to detect high-risk infants based on neuromotor examinations and provide early intervention as soon as possible. As babies grow up, close monitoring of language and cognitive development is needed. Moreover, providing continuous rehabilitation with task-specific and intensive repetitive training could improve functional outcomes in children with mild-to-moderate disabilities. After school age, maintaining the level of physical activity and managing complications are also needed.

Incidental Diagnosis of Pediatric Arytenoid Cartilage Dislocation During Videofluoroscopic Swallowing Study: A Case Report

Arytenoid cartilage dislocation is one of the most common mechanical causes of vocal fold immobility. The most common etiologies are intubation and external trauma, but its incidence is lower than 0.1%. Its symptoms include dysphonia, vocal fatigue, loss of vocal control, breathiness, odynophagia, dysphagia, dyspnea, and cough. Although there are some reports of arytenoid cartilage dislocation in adults, there are only few reports on its occurrence in children. It is particularly difficult to detect the symptoms of arytenoid cartilage dislocation in uncooperative pediatric patients with brain lesions without verbal output or voluntary expression. We report a case of arytenoid cartilage dislocation with incidental findings in a videofluoroscopic swallowing study performed to evaluate the swallowing function.

Architectural Changes in the Medial Gastrocnemius on Sonography after Nerve Ablation in Healthy Adults

Architectural changes in healthy muscle after denervation have not yet been reported. This study aimed to investigate architectural changes in the medial head of the gastrocnemius muscle (GCM) after aesthetic tibial nerve ablation in healthy adults using ultrasonography (US). The effects of tibial nerve ablation were verified by visual observation and surface electromyography analysis. US images of medial GCMs were taken by one trained physician using B-mode and real-time US with a linear-array probe before nerve ablation, at 1 week after nerve ablation and at 3 months after nerve ablation in an anatomic standing position with the feet about shoulder-width apart in 19 healthy adults (17 females and 2 males). Muscle thickness was significantly reduced on the left side at 1 week and 3 months after the procedure and on the right side at 3 months after the procedure (p<0.050). Although fascicle length was not significantly changed, pennation angle was significantly reduced on both sides at 3 months after the procedure (p<0.050). Muscle thickness and pennation angle of the muscle fascicle were significantly reduced, although fascicle length was not significantly changed, after tibial nerve ablation in the medial GCM of healthy adults.

Determinants of Hip and Femoral Deformities in Children With Spastic Cerebral Palsy

OBJECTIVE: To find factors affecting hip and femoral deformities in children with spastic cerebral palsy (CP) by comparing various clinical findings with imaging studies including plain radiography and computed tomography (CT) imaging. METHODS: Medical records of 709 children with spastic CP who underwent thorough baseline physical examination and functional assessment between 2 to 6 years old were retrospectively reviewed. Fifty-seven children (31 boys and 26 girls) who had both plain radiography of the hip and three-dimensional CT of the lower extremities at least 5 years after baseline examination were included in this study. RESULTS: The mean age at physical examination was 3.6 years (SD=1.6; range, 2–5.2 years) and the duration of follow-up imaging after baseline examination was 68.4 months (SD=22.0; range, 60–124 months). The migration percentage correlated with motor impairment and the severity of hip adductor spasticity (R1 angle of hip abduction with knee flexion). The femoral neck and shaft angle correlated with the ambulation ability and severity of hip adductor spasticity (R1 and R2 angles of hip abduction with both knee flexion and extension). CONCLUSION: Hip subluxation and coxa valga deformity correlated with both dynamic spasticity and shortening of hip adductor muscles. However, we found no correlation between femoral deformities such as femoral anteversion, coxa valga, and hip subluxation.

Cerebral Palsy due to Intracranial Hemorrhage Caused by Consumptive Coagulopathy in Protein C Deficiency: A Case Report

Protein C (PROC) is a potent anticoagulant inactivating coagulation factors Va and VIIIa. PROC deficiency is very rare condition inherited as an autosomal dominant or recessive trait, and associated with various thromboembolic and ischemic conditions. Moreover, severe form of PROC deficiency can cause fatal hemorrhagic complications due to consumptive coagulopathy. We reported two children with hemorrhagic stroke who were diagnosed as severe PROC deficiency caused by two different types of compound heterozygous PROC gene mutations. We described results of laboratory tests, genetic analysis, brain magnetic resonance images, and functional outcomes. Both children received prophylactic anticoagulation therapy and presented with purple-colored skin lesions during rehabilitation. Purpura fulminans caused by insufficient anticoagulation should be differentiated from hematoma caused by excessive anticoagulation therapy in these children.

Cerebral Palsy due to Intracranial Hemorrhage Caused by Consumptive Coagulopathy in Protein C Deficiency: A Case Report

Protein C (PROC) is a potent anticoagulant inactivating coagulation factors Va and VIIIa. PROC deficiency is very rare condition inherited as an autosomal dominant or recessive trait, and associated with various thromboembolic and ischemic conditions. Moreover, severe form of PROC deficiency can cause fatal hemorrhagic complications due to consumptive coagulopathy. We reported two children with hemorrhagic stroke who were diagnosed as severe PROC deficiency caused by two different types of compound heterozygous PROC gene mutations. We described results of laboratory tests, genetic analysis, brain magnetic resonance images, and functional outcomes. Both children received prophylactic anticoagulation therapy and presented with purple-colored skin lesions during rehabilitation. Purpura fulminans caused by insufficient anticoagulation should be differentiated from hematoma caused by excessive anticoagulation therapy in these children.

Immediate Effect of a Single Session of Whole Body Vibration on Spasticity in Children With Cerebral Palsy

OBJECTIVE: To investigate the immediate effect of a single session of whole body vibration (WBV) on lower extremity spasticity in children with cerebral palsy (CP). METHODS: Seventeen children with spastic CP were included. A single session of WBV was administered: 10-minute WBV, 1-minute rest, and 10-minute WBV. The effects of WBV were clinically assessed with the Modified Ashworth Scale (MAS) and Modified Tardieu Scale (MTS) before and immediately, 30 minutes, 1 hour, 2 hours, 3 hours, and 4 hours after WBV. RESULTS: Spasticity of the ankle plantarflexor, as assessed by MAS and MTS scores, was reduced after WBV. Post-hoc analysis demonstrated that, compared to baseline, the MAS significantly improved for a period of 1 hour after WBV, and the R1 and R2–R1 of the MTS significantly improved for a period of 2 hours after WBV. CONCLUSION: A single session of WBV improves spasticity of ankle plantarflexors for 1–2 hours in children with CP. Future studies are needed to test whether WBV is an effective preparation before physiotherapy and occupational therapy.

Correction: Immediate Effect of a Single Session of Whole Body Vibration on Spasticity in Children With Cerebral Palsy

The authors noticed that the original version of the paper contains typographical errors in Figs. 2 and 3.

Change in Pulmonary Function after Incentive Spirometer Exercise in Children with Spastic Cerebral Palsy: A Randomized Controlled Study

PURPOSE: The aim of this study was to investigate the effect of incentive spirometer exercise (ISE) on pulmonary function and maximal phonation time (MPT) in children with spastic cerebral palsy (CP). MATERIALS AND METHODS: Fifty children with CP were randomly assigned to two groups: the experimental group and the control group. Both groups underwent comprehensive rehabilitation therapy. The experimental group underwent additional ISE. The forced vital capacity (FVC), forced expiratory volume at one second (FEV1), FEV1/FVC ratio, peak expiratory flow (PEF), and MPT were assessed as outcome measures before and after 4 weeks of training. RESULTS: There were significant improvements in FVC, FEV1, PEF, and MPT in the experimental group, but not in the control group. In addition, the improvements in FVC, FEV1, and MPT were significantly greater in the experimental group than in the control group. CONCLUSION: The results of this randomized controlled study support the use of ISE for enhancing pulmonary function and breath control for speech production in children with CP.

Botulinum Toxin Type A Injection for Spastic Equinovarus Foot in Children with Spastic Cerebral Palsy: Effects on Gait and Foot Pressure Distribution

PURPOSE: To investigate the effect of intramuscular Botulinum toxin type A (BoNT-A) injection on gait and dynamic foot pressure distribution in children with spastic cerebral palsy (CP) with dynamic equinovarus foot. MATERIALS AND METHODS: Twenty-five legs of 25 children with CP were investigated in this study. BoNT-A was injected into the gastrocnemius (GCM) and tibialis posterior (TP) muscles under the guidance of ultrasonography. The effects of the toxin were clinically assessed using the modified Ashworth scale (MAS) and modified Tardieu scale (MTS), and a computerized gait analysis and dynamic foot pressure measurements using the F-scan system were also performed before injection and at 1 and 4 months after injection. RESULTS: Spasticity of the ankle plantar-flexor in both the MAS and MTS was significantly reduced at both 1 and 4 months after injection. On dynamic foot pressure measurements, the center of pressure index and coronal index, which represent the asymmetrical weight-bearing of the medial and lateral columns of the foot, significantly improved at both 1 and 4 months after injection. The dynamic foot pressure index, total contact area, contact length and hind foot contact width all increased at 1 month after injection, suggesting better heel contact. Ankle kinematic data were significantly improved at both 1 and 4 months after injection, and ankle power generation was significantly increased at 4 months after injection compared to baseline data. CONCLUSION: Using a computerized gait analysis and foot scan, this study revealed significant benefits of BoNT-A injection into the GCM and TP muscles for dynamic equinovarus foot in children with spastic CP.

Neuropsychological Outcomes of Preterm Birth in Children With No Major Neurodevelopmental Impairments in Early Life

OBJECTIVES: To investigate cognition, social adaptive functioning, behavior, and emotional development in the preschool period and to determine the effects of the age of onset of walking on those developmental areas in children who were born preterm without major neurodevelopmental impairments (NDI) early in life. METHODS: Fifty-eight children who were born preterm without major NDI early in life participated in this study. The Korean versions of the Wechsler Preschool and Primary Scale of Intelligence or the Bayley Scales of Infant Development, the social maturity scale, the Korean version of the Child Behavior Checklist (CBCL), Conners' abbreviated parent/teacher rating scale, the Childhood Autism Rating Scale, and a speech developmental test were administered. The participants were divided into two groups: early walkers (group A) and late walkers (group B). RESULTS: The full-scale intelligence quotient (IQ) and performance IQ were significantly lower in group B than in group A, while the verbal IQ did not differ significantly between the groups. The children in group B had greater risks of cognitive deficits than did the children in group A, especially in performance skills. The social quotient (SQ) was significantly lower in group B than in group A (p<0.05). The rates of mild or significant deficits based on SQ and the CBCL did not differ significantly between the groups. Four children in group A and one child in group B had attention/hyperactivity problems. One child in group A had autistic behavior. Only one child in group B showed a significant speech developmental delay. CONCLUSIONS: Problems in cognition, social adaptive functioning, and emotional and behavioral development can occur in children without major NDI early in life. Late walkers had significantly lower scores in cognition and social adaptive functioning than did early walkers.

Reliability and Validity of the Upper Limb Physician's Rating Scale in Children with Cerebral Palsy

PURPOSE: The Upper Limb Physician's Rating Scale (ULPRS) is a tool that assesses movement quality of the upper limbs. It is used as an outcome measure after botulinum toxin type A injection in children with cerebral palsy (CP). This study aimed to investigate the reliability and validity of the ULPRS in children with spastic CP. MATERIALS AND METHODS: Thirty children with spastic CP (M:F=17:13) aged 5 to 13 years old were recruited. The ULPRS was scored based on recorded videotapes by four physicians on two separate occasions. The Melbourne Assessment of Unilateral Upper Limb Function (MUUL) was scored by an occupational therapist. Intraclass correlation coefficients (ICCs), 95% confidence intervals and weighted kappa statistics were calculated for the scores of ULPRS to obtain interrater and intrarater reliability. The relationship between ULPRS and MUUL was assessed using Pearson correlation coefficients. RESULTS: The ICCs for the total ULPRS scores were 0.94 between raters and 0.99 to 1.00 within raters. The weighted kappa statistics for subitem scores for the ULPRS ranged from 0.67 to 1.00 within raters and from 0.46 to 0.86 between raters. The relationship between ULPRS and MUUL was strong (Pearson correlation coefficient=0.751; p<0.05). CONCLUSION: The results demonstrated the high reliability of the total ULPRS score within and between raters. A significant concurrent validity between ULPRS and MUUL also supports the clinical utility of the ULPRS as an outcome measure of spastic upper limb in children with CP.

Estimation of Gastrocnemius Muscle Volume Using Ultrasonography in Children with Spastic Cerebral Palsy

PURPOSE: This study aimed to investigate useful parameters for estimating gastrocnemius (GCM) muscle volume (MV) using ultrasonography (US) and anthropometry in children with spastic cerebral palsy (CP). MATERIALS AND METHODS: Eighteen legs from nine children with spastic CP aged 2 to 6 years were investigated in this study. Tibial length (TL) of each leg was measured and muscle thickness (MT) and anatomical cross-sectional area (aCSA) of GCM muscles were assessed using US. The volume of the GCM was measured by magnetic resonance imaging (MRI) scans. The relationship of TL, MT, and aCSA with MV measured by MRI was investigated. Simple and multiple regression analyses were performed to establish muscle volume prediction equations. RESULTS: Resting MT, aCSA, and TL were highly related to MV of both medial and lateral head of GCM determined by MRI. The MV prediction equation based on simple regression analysis resulted in r2 values ranging from 0.591 to 0.832 (p<0.05). The r2 values were higher using aCSA as independent variable than using MT. The MV prediction equation based on multiple regression analysis resulted in r2 values ranging from 0.779 to 0.903 (p<0.05). However, the relatively high standard error of the estimate values ranged from 18.0-33.6% on simple regression and 15.5-25.6% on multiple regression. The contribution of aCSA was higher than that of MT for predicting MV of GCM. CONCLUSION: Our study demonstrated the suitability of US assessment of aCSA and MT combined with TL for estimating MV of GCM in children with spastic CP and showed that aCSA is more useful parameter than MT.

Architectural Changes of the Gastrocnemius Muscle after Botulinum Toxin Type A Injection in Children with Cerebral Palsy

PURPOSE: This study used ultrasonography (US) to investigate the architectural changes in gastrocnemius muscles (GCM) after botulinum toxin injection (BoNT-A) in children with cerebral palsy (CP). MATERIALS AND METHODS: Thirteen children with CP who received a BoNT-A injection into their GCM to treat equinus were recruited (9 males and 4 females). Architectural changes in both the medial and lateral heads of the GCM from a total of 20 legs were assessed using B-mode, real-time US. Muscle thickness (MT), fascicle length (FL), and fascicle angle (FA) were measured over the middle of the muscle belly in both a resting and neutral ankle position. Measures at 1 and 3 months after the injection were compared with baseline data taken before the injection. RESULTS: The mean age of the subjects was 5.8 (+/-1.6) years. Spasticity was significantly reduced when measured by both the modified Tardieu scale and the modified Ashworth scale at 1 and 3 months after injection (p<0.05). The MT and FA of both the medial and lateral heads of the GCM were significantly reduced for both neutral and resting ankle positions at 1 and 3 months after the injection. The FL of both the medial and lateral heads of the GCM were significantly increased in a resting position (p<0.05), but not in a neutral position. CONCLUSION: Our results demonstrated muscle architectural changes induced by BoNT-A injection. The functional significances of these changes were discussed.

Effects of Hippotherapy on Gross Motor Function and Functional Performance of Children with Cerebral Palsy

PURPOSE: The purpose of our study was to investigate the effects of hippotherapy on gross motor function and functional performance in children with spastic cerebral palsy (CP). MATERIALS AND METHODS: We recruited 34 children (M:F=15:19, age: 3-12 years) with spastic CP who underwent hippotherapy for 45 minutes twice a week for 8 weeks. Twenty-one children with spastic CP were recruited for control group. The distribution of gross motor function classification system level and mean age were not significantly different between the two groups. Outcome measures, including the Gross Motor Function Measure (GMFM)-66, GMFM-88 and the Pediatric Evaluation of Disability Inventory: Functional Skills Scale (PEDI-FSS), were assessed before therapy and after the 8-weeks intervention as outcome measures. RESULTS: There were no significant differences between intervention and control groups in mean baseline total scores of GMFM-66, GMFM-88 or PEDI-FSS. After the 8-weeks intervention, mean GMFM-66 and GMFM-88 scores were significantly improved in both groups. However, the hippotherapy group had significantly greater improvement in dimension E and GMFM-66 total score than the control group. The total PEDI-FSS score and the sub-scores of its 3 domains were significantly improved in the hippotherapy group, but not in the control group. CONCLUSION: The results of our study demonstrate the beneficial effects of hippotherapy on gross motor function and functional performance in children with CP compared to control group. The significant improvement in PEDI-FSS scores suggests that hippotherapy may be useful to maximize the functional performance of children with CP.

The Effect of Obturator Nerve Block on Hip Lateralization in Low Functioning Children with Spastic Cerebral Palsy

PURPOSE: Hip adductor spasticity has a great impact on developing hip displacement in children with cerebral palsy (CP). Obturator nerve (ON) block is less invasive intervention rather than soft tissue surgery for reduction of hip adductor spasticity. The aim of this study is to investigate the effect of ON block on hip lateralization in low functioning children with spastic CP. MATERIALS AND METHODS: The study was performed by retrospective investigation of the clinical and radiographic follow-up data of low functioning children [gross motor function classification system (GMFCS) level III to V] with spastic cerebral palsy whose hip was subluxated. Migration percentage (MP) was measured on hip radiographs and its annual change was calculated. In intervention group, ON block was done with 50% ethyl alcohol under the guidance of electrical stimulation. RESULTS: The data of 49 legs of 25 children for intervention group and the data of 41 legs of 23 children for nonintervention group were collected. In intervention group, the MP were significantly reduced at 1st follow-up and the MPs at 2nd and last follow-up did not show significant differences from initial MP. Whereas in nonintervention group, the MPs at 1st, 2nd and last follow-up were all significantly increased compared to initial MPs. CONCLUSION: ON block with ethyl alcohol is useful as an early effective procedure against progressive hip displacement in these children with spastic CP.

Clinical Features and Associated Abnormalities in Children and Adolescents With Corpus Callosal Anomalies

Callosal anomalies are frequently associated with other central nervous system (CNS) and/or somatic anomalies. We retrospectively analyzed the clinical features of corpus callosal agenesis/hypoplasia accompanying other CNS and/or somatic anomalies. We reviewed the imaging and clinical information of patients who underwent brain magnetic resonance imaging in our hospital, between 2005 and 2012. Callosal anomalies were isolated in 13 patients, accompanied by other CNS anomalies in 10 patients, associated with only non-CNS somatic anomalies in four patients, and with both CNS and non-CNS abnormalities in four patients. Out of 31 patients, four developed normally, without impairments in motor or cognitive functions. Five of nine patients with cerebral palsy were accompanied by other CNS and/or somatic anomalies, and showed worse Gross Motor Function Classification System scores, compared with the other four patients with isolated callosal anomaly. In addition, patients with other CNS anomalies also had a higher seizure risk.

Relation among the Gross Motor Function, Manual Performance and Upper Limb Functional Measures in Children with Spastic Cerebral Palsy

PURPOSE: The objective of this study was to describe hand function in relation with gross motor function and subtype of spastic cerebral palsy and to investigate the relationships among gross motor function, bimanual performance, unimanual capacity and upper limb functional measures in children with spastic cerebral palsy (CP). MATERIALS AND METHODS: We collected upper extremity data of 140 children with spastic CP. The Gross Motor Functional Classification System (GMFCS) was used to assess gross motor function, Manual Ability Classification System (MACS) for bimanual performance, and Modified House Functional Classification (MHC) for the best capacity of each hand. Upper limb functions were evaluated by using the Upper Limb Physician's Rating Scale and Upper Extremity Rating Scale. RESULTS: There was a good correlation between GMFCS and MACS in children with bilateral CP, but the correlation was not strong in children with unilateral CP. No significant difference between GMFCS and MACS was found in children with bilateral CP, but children with unilateral CP scored higher on GMFCS than on MACS. A strong correlation was observed between MACS and MHC in children with bilateral CP, but not in children with unilateral CP. The upper limb functional measures in each hand were highly related with MACS and MHC in bilateral CP, but not in unilateral CP. CONCLUSION: Gross motor function, bimanual performance and the best capacity of each hand are closely related with each other in children with bilateral CP, but not in children with unilateral CP.

Novel Influenza A (H1N1)-Associated Acute Necrotizing Encephalopathy: A Case Report

Several cases of acute necrotizing encephalopathy (ANE) with influenza A (H1N1) have been reported to date. The prognosis of ANE associated with H1N1 is variable; some cases resulted in severe neurologic complication, whereas other cases were fatal. Reports mostly focused on the diagnosis of ANE with H1N1 infection, rather than functional recovery. We report a case of ANE with H1N1 infection in a 4-year-old Korean girl who rapidly developed fever, seizure, and altered mentality, as well as had neurologic sequelae of ataxia, intentional tremor, strabismus, and dysarthria. Brain magnetic resonance imaging showed lesions in the bilateral thalami, pons, and left basal ganglia. To our knowledge, this is the first report of ANE caused by H1N1 infection and its long-term functional recovery in Korea.

Reliability of Visual Classification of Sagittal Gait Patterns in Patients with Bilateral Spastic Cerebral Palsy

OBJECTIVE: To investigate the reliability of inspection-based classification of sagittal gait patterns in children with bilateral spastic cerebral palsy (CP). METHOD: Video clip recordings of gait patterns and sagittal kinematic data obtained by a computerized motion analysis system from 91 patients with bilateral spastic CP were reviewed. The abnormal gait patterns were classified into 4 groups using the method described by Rodda et al. Visual observation-based classification (visual classification) was compared with classification by 3D analysis-based methods (3D classification). The reliabilities of visual classifications made by an experienced physician and a trainee physician were analyzed. RESULTS: The consistency of inspection-based gait classification using kinematic data analysis was demonstrated by an experienced physician (Kappa coefficient (k)=0.67, p<0.001). However, the consistency was low for the trainee physician (k=0.37, p<0.001). Group III (apparent equinus) was commonly confused with group IV (crouch gait) by the trainee physician, resulting in lower agreement for those two evaluation groups than for other patterns. Video observation showed low reliability in comparisons made between the experienced and the trainee physician (k=0.37, p<0.001). CONCLUSION: There was substantial agreement of gait classification between video observation and kinematic data analysis by the experienced physician, but not by the trainee physician. Low reliability was also demonstrated for inspection-based gait classification.