OBJECT: Neurological conditions including cerebral palsy, brain injury, and stroke often result in severe spasticity, which can lead to significant deformity and interfere with function. Treatments for spasticity include oral medications, intramuscular botulinum toxin type A injections, orthopedic surgeries, intrathecal baclofen pump implantation, and selective dorsal rhizotomy (SDR). Selective dorsal rhizotomy, which has been well studied in children with spastic diplegia, results in significant reduction in spasticity and improved function in children. To the authors’ knowledge, there are no published outcome data for SDR in patients with spastic hemiparesis. The object of this study was to examine the effects of SDR on spastic hemiparesis.

METHODS: A 2-year study was undertaken including all children with spastic hemiparesis who underwent SDR at the authors’ institution. The degree of spasticity, as measured by the Modified Ashworth Scale or quality of gait rated using the visual gait assessment scale, the gait parameters, and velocity were compared in patients before and after undergoing SDR.

RESULTS: Thirteen children (mean age 6 years 7 months) with spastic hemiparesis underwent SDR performed by the same surgeon during a 2-year period. All of the patients had a decrease in tone in the affected lower extremity after the procedure. The mean reduction in tone in 4 muscle groups (hip adductors, knee flexors, knee extensors, and ankle plantar flexors) according to the modified Ashworth scale score was 2.6 ± 1.26 (p < 0.0001). The quality of gait was assessed in 7 patients by using the visual gait assessment scale. This score improved in 6 patients and remained the same in 1. Stride length and gait velocity were measured in 4 children. Velocity increased in 3 patients and decreased in a 3-year-old child. Parents and clinicians reported an improvement in quality of gait after the procedure. Stride length increased bilaterally in 3 patients and increased on one side and decreased on the other in the other patient.

CONCLUSIONS: Selective dorsal rhizotomy showed efficacy in the treatment of spastic hemiparesis in children. All of the patients had decreased tone after SDR as measured by the modified Ashworth scale. The majority of patients had qualitative and quantitative improvements in gait.

OBJECTIVE: To establish the test-retest reliability of linear and nonlinear measures, including intra- and intersession reliability, when used to analyze the center of pressure (COP) time series during the development of infant sitting postural control in infants with or at risk for cerebral palsy (CP).

DESIGN: Longitudinal study.

SETTING: University hospital laboratory.

PARTICIPANTS: Infants with or at risk for CP (N=18; mean age ± SD at entry into the study, 13.7±3.6mo).

INTERVENTIONS: Not applicable.

MAIN OUTCOME MEASURES: Infant sitting COP data were recorded for 3 trials at each session (2 sessions for each month within 1 week) for 4 consecutive months. The linear COP parameters of the root mean square, the range of sway for both the anterior-posterior and the medial-lateral directions, and the sway path were calculated. In addition, the nonlinear parameters of approximate entropy, Lyapunov exponent (LyE), and the correlation dimension for both directions were also calculated. Intra- and intersession reliability was computed by the intraclass correlation coefficient (ICC).

RESULTS: Regarding nonlinear measures, LyE showed high intra- and intersession ICC values in comparison with all other parameters evaluated. Intrasession and intersession reliability increased overall in the last 2 months of data collection and as sitting posture improved.

CONCLUSIONS: Our results suggested that the methodology presented is a reliable way of examining the development of sitting postural control in infants with or at risk for CP, and the reliability results generally parallel values found in sitting postural behavior in typical infants. Therefore, this methodology may be helpful in examining efficacy of therapy protocols directed at advancing sitting postural control in infants with motor developmental delays.

Copyright © 2010 American Congress of Rehabilitation Medicine. Published by Elsevier Inc. All rights reserved.

Hereditary spastic paraplegia (HSP) is a neurodegenerative disease characterized by progressive spasticity in the lower extremities. Mutations in the atlastin GTPase 1 (ATL1) gene cause approximately 10% of autosomal dominantly inherited HSP. For many subjects with an ATL1 mutation, spastic gait begins in early childhood and does not significantly worsen, even over many years; such cases resemble spastic diplegic cerebral palsy. Herein we report a heterozygous R239C mutation in the ATL1 gene in a Korean family. The family members exhibited early onset pure spastic paraplegia and had been previously diagnosed with the diplegic form of cerebral palsy. We suggest that spastic paraplegia type 3 (SPG3A) be included in the differential diagnosis of early onset spastic paraplegia. To the best of our knowledge, this is the first report of a genetically confirmed family affected with SPG3A in Korea.

OBJECTIVES: To assess the association of Apgar score 5 minutes after birth with cerebral palsy in both normal weight and low birthweight children, and also the association with the cerebral palsy subdiagnoses of quadriplegia, diplegia, and hemiplegia.

DESIGN: Population based cohort study.

SETTING: The Medical Birth Registry of Norway was used to identify all babies born between 1986 and 1995. These data were linked to the Norwegian Registry of Cerebral Palsy in Children born 1986-95, which was established on the basis of discharge diagnoses at all paediatric departments in Norway.

POPULATION: All singletons without malformations born in Norway during 1986-95 and who survived the first year of life (n=543?064).

MAIN OUTCOME MEASURE: Cerebral palsy diagnosed before the age of 5 years.

RESULTS: 988 children (1.8 in 1000) were diagnosed with cerebral palsy before the age of 5 years. In total, 11% (39/369) of the children with Apgar score of less than 3 at birth were diagnosed with cerebral palsy, compared with only 0.1% (162/179?515) of the children with Apgar score of 10 (odds ratio (OR) 53, 95% CI 35 to 80 after adjustment for birth weight). In children with a birth weight of 2500 g or more, those with an Apgar score of less than 4 were much more likely to have cerebral palsy than those who had an Apgar score of more than 8 (OR 125, 95% confidence interval 91 to 170). The corresponding OR in children weighing less than 1500 g was 5 (95% CI 2 to 9). Among children with Apgar score of less than 4, 10-17% in all birthweight groups developed cerebral palsy. Low Apgar score was strongly associated with each of the three subgroups of spastic cerebral palsy, although the association was strongest for quadriplegia (adjusted OR 137 for Apgar score <4 v Apgar score >8, 95% CI 77 to 244).

CONCLUSIONS: Low Apgar score was strongly associated with cerebral palsy. This association was high in children with normal birth weight and modest in children with low birth weight. The strength of the association differed between subgroups of spastic cerebral palsy. Given that Apgar score is a measure of vitality shortly after birth, our findings suggest that the causes of cerebral palsy are closely linked to factors that reduce infant vitality.

Background/Aims: This study examined the spectral characteristics of American English vowels in dysarthria associated with cerebral palsy (CP), and investigated the relationship between a speaker’s overall speech intelligibility and vowel contrast. Methods: The data were collected from 12 American English native speakers (9 speakers with a diagnosis of CP and 3 controls). Primary measures were F(1) and F(2) frequencies of 3 corner vowels /i, a, u/ and 3 noncorner vowels /I, 3, */. Six acoustic variables were derived from the formant measures, and were regressed against intelligibility: corner vowel space, noncorner vowel space, mean distance between vowels, F(1) and F(2) variability, and overlap degree among vowels. Results: First, the effect of vowel was significant for both F(1) and F(2) measures for all speakers, but post hoc analysis revealed a reduced distinction at lower intelligibility. Second, regression functions relating intelligibility and acoustic variables were significant for overlap degree among vowels, F(1) variability, corner vowel space and mean distance between vowels. Overlap degree among vowels accounted for the greatest amount of variance in intelligibility scores. Conclusion: A speaker’s overall intelligibility in dysarthric speech is better represented by the overlap degree among vowels than by the vowel space.

Copyright © 2010 S. Karger AG, Basel.

Advances in medical and surgical care over the past 20 years have resulted in children who formerly would have died at birth or infancy now surviving well into adulthood, many with permanent physical disabilities, including those caused by cerebral palsy. Inadequate medical and surgical diagnoses and intervention are prevalent in the adult cerebral palsy population. Decreased physical activity and participation in physical therapy and fitness programs, along with loss of strength, contractures, and pain are common factors in the loss of functional weight bearing, self-care, and daily performance over time. Increased awareness of these problems is needed by adult health care providers who provide care to these individuals and also by pediatric providers who may be able to intervene and prevent some of the long-term problems. Early identification and intervention in the child and younger adult remain the ideal in the pursuit of optimal musculoskeletal function and lifestyle throughout the adult years.

Copyright © 2010 Elsevier Inc. All rights reserved.

Clinical decision making for the management of foot deformities in children with cerebral palsy is based on the collection and integration of data from 5 sources: the clinical history, physical examination, plain radiographs, observational gait analysis, and quantitative gait analysis (which includes kinematic/kinetic analyses, dynamic electromyography, and dynamic pedobarography). The 3 most common foot segmental malalignments in children with CP are equinus, equinoplanovalgus, and equinocavovarus. The 2 most common associated deformities are ankle valgus and hallux valgus. Foot and ankle deformities caused by dynamic overactivity and imbalance of muscles are best treated with pharmacologic or neurosurgical interventions designed to manage muscle tone and spasticity, or muscle tendon unit transfers. Deformities caused by fixed or myostatic soft tissue imbalance without fixed skeletal malalignment are best treated with muscle tendon unit lengthening surgery. Deformities characterized by structural skeletal malalignment associated with fixed or myostatic soft tissue imbalance are best treated with a combination of soft tissue and skeletal surgeries.

Copyright © 2010 Elsevier Inc. All rights reserved.

This article discusses the sagittal gait patterns in children with spastic diplegia, with an emphasis on the knee, as well as the concept of the “dose” of surgery that is required to correct different gait pathologies. The authors list the various interventions in the order of their increasing dose. The concept of dose is useful in the consideration of the management of knee dysfunction.

Copyright © 2010 Elsevier Inc. All rights reserved.

Hip abnormalities affect most children with cerebral palsy. Dedicated surveillance programs have been shown to be effective means of identifying hips at risk and preventing pathologic dislocation. Patients who are ambulatory and correlate with Gross Motor Function Classification Score I and II experience deformities that affect mobility and gait, but rarely dislocations. Marginal and nonambulatory patients have an increasing risk of dislocation. Once subluxation has been identified, early surgical intervention is indicated. Long-term postoperative follow-up is needed to monitor for recurrence. Individuals who recur or who do not respond to initial soft tissue releases benefit from bony surgery. Comprehensive reconstruction of the hip has become the predominant treatment approach when acetabular and proximal femoral dysplasia is present. The painful arthritic dislocated hip has numerous treatment options. Hip arthroplasty procedures show promising results and may supplant other salvage options in the future.

Copyright © 2010. Published by Elsevier Inc.