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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.
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.
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).
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.
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To investigate the immediate effect of a single session of whole body vibration (WBV) on lower extremity spasticity in children with cerebral palsy (CP).
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.
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.
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.
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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.
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).
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.
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.
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To investigate the effect of deep brain stimulation (DBS) on reducing dystonia and disability in adults with cerebral palsy (CP) and to compare the therapeutic outcomes between primary dystonia patients and CP patients over two years after bilateral pallidal DBS.
Five patients with primary dystonia and seven CP patients with dystonia were recruited. All subjects received DBS surgery in both globus pallidus. Burke-Fahn-Marsden dystonia rating scale consisting of dystonia movement score and disability score and subjective satisfaction scale were assessed after 1 month and every 6 months over two years following DBS treatment.
On the dystonia movement scale, both groups of primary dystonia patients and CP patients showed a significant decrease over time following DBS. On the disability scale, patients with primary dystonia showed a significant decrease over time, whereas the disability score of CP patients did not change over the two years. Comparing the dystonia movement and disability scores of CP patients at each assessment, patients with primary dystonia showed a significant reduction after 6 months. Comparing the satisfaction scores of CP patients after DBS, patients with primary dystonia showed significantly higher subjective satisfaction.
Whereas dystonia can be significantly reduced in patients with primary dystonia, CP patients showed a modest improvement on the dystonia movement scale, but not on the disability scale. Therefore, DBS may be considered with caution as a treatment modality of CP patients with dystonia.
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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.
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Isodicentric chromosome 15 [idic(15)] is a rare chromosomal abnormality that occurs due to inverted duplication of chromosome 15q. It is associated with many clinical findings such as early central hypotonia, developmental delay, cognitive dysfunction, autism spectrum disorders, and seizure. Delayed development is a common problem referred to pediatric rehabilitation clinics. A 9-month-old boy with delayed development was referred to our clinic for assessment and treatment. On chromosomal analysis, he was diagnosed as idic(15) syndrome with 47,XY,+idic(15)(q12) on karyotyping. Herein we describe his clinical manifestations and provide a brief review of the related literature.
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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.
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To determine reliability and clinical use of two methods of migration index (MI) in CP patients with or without hip dysplasia.
The materials included radiographs of 200 hips of children with cerebral palsy. Conventional anteroposterior radiographs of the pelvis were taken with the child in the supine position with standardized methods. Two rehabilitation doctors measured the migration index using two methods. In the classic method, the lateral margin of the acetabular roof was used as a landmark and in the modified method the lateral margin of the sourcil was used as a landmark. Each rater measured the migration index at three separate times with a time interval of at least one week. Intraclass correlation (ICC) was used to test the inter- and intra-rater reliability.
MI shows excellent intra-rater reliability in both the classic and modified methods, but the inter-rater reliability was higher in the classic method than in the modified method. When categorized according to the sourcil classification, inter-rater reliability was higher in the normal sourcil type and lower in the dysplastic sourcil types.
Generally, the classic method showed higher reliability than the modified method, even though the reliability of the MI measurement was relatively high with both methods.
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Dystonia is a symptom defined by involuntary and irregular contractions of the muscles, which cause movement disorders and postural problems. Deep brain stimulation (DBS) in globus pallidus interna (GPi) is a good option for controlling dystonia. DBS has already been shown to have significant effects on primary dystonia as well as Parkinson's disease. Dystonia is very difficult to manage, as seen in cerebral palsy (CP) mixed with spasticity. As CP patients grow, their musculoskeletal problems may require orthopedic surgery. However, the outcome of orthopedic surgery is not usually suitable due to dystonia. Therefore, we attempted to control dystonia through DBS initially and perform orthopedic surgery to correct musculoskeletal deformities after treatment of dystonia. Herein, we report a case that showed remarkable improvement in terms of the dystonia rating scale and gait pattern after combined therapy of DBS and orthopedic surgery.
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To investigate the reliability of inspection-based classification of sagittal gait patterns in children with bilateral spastic cerebral palsy (CP).
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.
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).
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.
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