Tetraplegic Cerebral Palsy Research Articles

Power Mobility, Supported Standing and Stepping Device Use in the First Two Years of Life: A Case Report of Twins Functioning at GMFCS V

Mobility experience has a positive impact on activity, participation, socialisation, language and cognition, but children with cerebral palsy (CP), Gross Motor Function Classification System (GMFCS) level V require assistive devices or assistance in all environments. Supported standing devices afford upright, weight-bearing positions to promote muscle, bone, joint and overall health. Supported stepping devices afford stepping and upright independent mobility, positively impacting self-esteem and participation, while power mobility is the only possibility for effective, independent community mobility. These devices and opportunities should be introduced at the age when children who are typically developing are pulling to stand, moving and exploring their environment. A detailed case description including lived experience and device use data is presented for female twins with dystonic tetraplegic CP born at 25 weeks gestational age and functioning at GMFCS level V. The feasibility of using power mobility, standing and stepping devices in home and community settings within the first two years is illustrated. The twins transitioned from spending 24 h in lying positions or being held in arms to spending more than 2 h daily in upright positions and having opportunities to move independently. Positioning and mobility devices can help to address all the F-words for child development: functioning, family, fitness, fun, friends and future.

Whole Genome Expression Profiling of Semitendinosus Tendons from Children with Diplegic and Tetraplegic Cerebral Palsy.

Cerebral palsy (CP) is the most common movement disorder in children, with a prevalence ranging from 1.5 to 4 per 1000 live births. CP is caused by a non-progressive lesion of the developing brain, leading to progressive alterations of the musculoskeletal system, including spasticity, often leading to the development of fixed contractures, necessitating tendon lengthening surgery. Total RNA-sequencing analysis was performed on semitendinosus tendons from diplegic and tetraplegic CP patients subjected to tendon lengthening surgery compared to control patients undergoing anterior cruciate ligament reconstructive surgery. Tetraplegic CP patients showed increased expression of genes implicated in collagen synthesis and extracellular matrix (ECM) turnover, while only minor changes were observed in diplegic CP patients. In addition, tendons from tetraplegic CP patients showed an enrichment for upregulated genes involved in vesicle-mediated transport and downregulated genes involved in cytokine and apoptotic signaling. Overall, our results indicate increased ECM turnover with increased net synthesis of collagen in tetraplegic CP patients without activation of inflammatory and apoptotic pathways, similar to observations in athletes where ECM remodeling results in increased tendon stiffness and tensile strength. Nevertheless, the resulting increased tendon stiffness is an important issue in clinical practice, where surgery is often required to restore joint mobility.

Clinical Characteristics and Possible Risk Factors of Epilepsy in Children with Cerebral Palsy: A Tertiary-Center Experience

Objective: The aim of this study was to evaluate the clinical characteristics of children with cerebral palsy (CP) and to investigate possible risk factors and prognosis of epilepsy in children with cerebral palsy (CP) with a special emphasis on drug-resistant epilepsy (DRE). Materials and Methods: A total of 145 pediatric patients who were followed up with a diagnosis of CP between 2019 and 2022 were evaluated. Demographic features, prenatal/perinatal history, etiology and type of CP, degree of impairment in motor and cognitive functions, seizure type, neuroimaging, and electroencephalography (EEG) findings were obtained retrospectively from hospital records. The patients were divided into two groups: CP patients with epilepsy and patients without epilepsy. Study variables were compared between these two groups and also between DRE and controlled epilepsy groups. Results: There were 91 (63%) boys and 54 (37%) girls with a mean age of 11.1 ± 4.2 years (3-18 years). Epilepsy was present in 107 (73.7%) cases and 40.1% of them had refractory epilepsy. Epilepsy was most common in the tetraplegic form of CP (p=0.028). Term gestation, birth weight of ≥2500 g, and history of neonatal seizures were significantly higher in patients with epilepsy (p=0.03, 0.01, and 0.03, respectively). Children with DRE were more likely to have tetraplegic CP (50%) and severe intellectual disability (56%). Conclusion: Determination of potential risk factors is important in predicting the development of epilepsy in patients with CP, as it may provide closer follow-up of patients at high risk. Particular attention should be paid to the early identification and treatment of comorbid epilepsy in children with CP.

EFFECTS OF TASK-ORIENTED TRAINING ON WALKING IN CHILDREN WITH CEREBRAL PALSY

ABSTRACT. 
 BACKGROUND: Cerebral Palsy (CP) is a common neurological deficit in children which hinders the child’s functional performance. Task-oriented training is considered to focus on functional aspects of the patient to improve movements and overall performance of functional tasks.
 OBJECTIVE: To determine the effects of task-oriented training on walking and balance in children with spastic diplegic Cerebral Palsy (CP).
 METHODS: A randomized control trial (ClinicalTrials.gov ID: NCT04561349) was conducted on 44 CP children. Both male and female children were randomized into an experimental group (Task-oriented training) and control group (mat activities and range of motion exercises). Children with spasticity ? 2, age 4-14 years, who could walk and follow commands were included. However, children with cognitive impairment, lower limb surgery in the last 6 months, tetraplegic CP were excluded. Children were assessed before and after an intervention. The outcome measures were Gross Motor Function Classification Scale (GMFC), Timed Up and Go (TUG), Functional Walking Test (FWT), and Modified Ashworth scale.
 RESULTS: Out of 38, 53.3% males and 44.7% females with the mean age of 9.3 ± 2.9 years. The experimental group had significant improvement in walking as the p-value for both TUG and FWT was significant after the 6th week (p< 0.05) but no significant change in MMT and MAS was observed (P>0.05).
 CONCLUSION: It is concluded that both techniques are effective to improve walking and balance in CP children. However, task-oriented training has a significant improvement in walking and balance in spastic CP children.
 KEYWORDS: Cerebral Palsy, Cognitive Impairment, Spasticity, Walking.

Analysis of Selected Risk Factors Depending on the Type of Cerebral Palsy.

Background: Cerebral palsy (CP) is not a defined, separate disease classification, but a set of etiologically diverse symptoms that change with the child’s age. According to the up-to-date definition, CP is a group of permanent but not unchanging disorders of movement and/or posture and motor function, which are due to a nonprogressive interference, lesion, or abnormality of the developing/immature brain. CP is one of the most frequent causes of motor disability in children. The aim of the present study was to analyze whether selected risk factors may vary depending on particular types of CP. Methods: 181 children with CP (aged 4–17 years), hospitalized at the Department of Pediatrics and Developmental Age Neurology in Katowice in the years 2008–2016 were retrospectively analyzed in the present study. The assumed risk factors of CP were divided into two groups: 1—pre-conception and prenatal (mother’s age, family history of epilepsy, burdened obstetric history, mother’s systemic diseases, pregnancy order, multiple pregnancy, duration of pregnancy, bleedings from the genital tract during gestation, arterial hypertension during pregnancy, infections during pregnancy, preterm contractions, maintained pregnancy, premature rupture of membranes, abruptio placentae, and others), 2—perinatal and postnatal (mode of delivery, birth weight, Apgar score at the first and fifth minute, neonatal convulsions, respiratory failure, infections in neonatal and infant period, and intraventricular bleeding). The division into particular CP types was based on Ingram’s classification. Results: The following risk factors were the most frequent in the total group: respiratory failure, infections, intraventricular bleeding, and prematurity. Among the analyzed preconception and prenatal factors, the duration of pregnancy and preterm contractions during pregnancy significantly differentiated the subgroups of patients depending on the type of CP. The prevalence of almost all analyzed perinatal, neonatal, and infant-related risk factors (i.e., birth weight, Apgar score at the first and fifth minute, neonatal convulsions, respiratory failure, infections in neonatal and infant period, and intraventricular bleeding) significantly differed between CP types, apart from the mode of delivery. However, in multivariate regression, only intraventricular bleeding was an independent predictor for tetraplegic CP type when compared to joined extrapyramidal and ataxic types (OR = 2.801, p = 0.028). Conclusions: As CP is a syndrome of multifactorial etiology, the identification of CP risk factors entails the need for careful observation and comprehensive care of children in the risk group. The presence of certain risk factors may be a prognostic indicator for particular types of CP. The knowledge about the association between the risk factor(s) and the CP type could be a very useful tool for pediatricians looking after the child at risk of developmental disorders.

Evaluation of Child Development Centre Based Intervention for Children with Multiple Disability

Background: Children with multiple disabilities usually have more than one significant disability that may include speech, physical mobility, learning, mental retardation, visual, hearing, and possibly others.
 Objective: The purpose of the present study was to evaluate the types of multiple disabilities in children with cerebral palsy and epilepsy and their fate after the intervention.
 Methodology: This retrospective observational study was conducted at Dhaka Shishu hospital from January 2011 to Decemberb2011.Children with multiple disabilities were referred to the child development center of Dhaka Shishu Hospital and were assessed for impairments and appropriate intervention done in special clinics.
 Result: Out of 540 children 66% were male. 43% of children were age group from 12-23 months. 58% had tetraplegic cerebral palsy (CP) with epilepsy and the second-highest group was diplegic CP with epilepsy 75(14.0%). Among 72 selected children who came in regular follow up, all children had motor disabilities. Cognitive delay, speech delay, vision and hearing impairment were found in 50%, 79%, 58% and 54% respectively. After intervention more improvement was found in motor function (92%).
 Conclusion: Intervention in specialized clinics improved the quality of life of children with multiple disability.
 Journal of National Institute of Neurosciences Bangladesh, January 2021, Vol. 7, No. 1, pp. 38-41

Assessment of the effect of incobotulinumtoxinA on muscle tone in children with spastic diplegic or tetraplegic cerebral palsy

Assessment of the effect of incobotulinumtoxinA on muscle tone in children with spastic diplegic or tetraplegic cerebral palsy

Jakość życia dzieci i młodzieży z postacią czterokończynową mózgowego porażenia dziecięcego

ENWEndNote BIBJabRef, Mendeley RISPapers, Reference Manager, RefWorks, Zotero AMA Michalska A, Markowska M, Śliwiński Z, Pogorzelska J. Quality of life in children and young people with tetraplegic cerebral palsy. Medical Studies/Studia Medyczne. 2018;34(2):112-119. doi:10.5114/ms.2018.76871. APA Michalska, A., Markowska, M., Śliwiński, Z., & Pogorzelska, J. (2018). Quality of life in children and young people with tetraplegic cerebral palsy. Medical Studies/Studia Medyczne, 34(2), 112-119. https://doi.org/10.5114/ms.2018.76871 Chicago Michalska, Agata, Małgorzata Markowska, Zbigniew Śliwiński, and Justyna A. Pogorzelska. 2018. "Quality of life in children and young people with tetraplegic cerebral palsy". Medical Studies/Studia Medyczne 34 (2): 112-119. doi:10.5114/ms.2018.76871. Harvard Michalska, A., Markowska, M., Śliwiński, Z., and Pogorzelska, J. (2018). Quality of life in children and young people with tetraplegic cerebral palsy. Medical Studies/Studia Medyczne, 34(2), pp.112-119. https://doi.org/10.5114/ms.2018.76871 MLA Michalska, Agata et al. "Quality of life in children and young people with tetraplegic cerebral palsy." Medical Studies/Studia Medyczne, vol. 34, no. 2, 2018, pp. 112-119. doi:10.5114/ms.2018.76871. Vancouver Michalska A, Markowska M, Śliwiński Z, Pogorzelska J. Quality of life in children and young people with tetraplegic cerebral palsy. Medical Studies/Studia Medyczne. 2018;34(2):112-119. doi:10.5114/ms.2018.76871.

Autosomal recessive spastic tetraplegia caused by AP4M1 and AP4B1 gene mutation: Expansion of the facial and neuroimaging features

Adaptor protein complex-4 (AP4) is a component of intracellular transportation of proteins, which is thought to have a unique role in neurons. Recently, mutations affecting all four subunits of AP4 (AP4M1, AP4E1, AP4S1, and AP4B1) have been found to cause similar autosomal recessive phenotype consisting of tetraplegic cerebral palsy and intellectual disability. The aim of this study was analyzing AP4 genes in three new families with this phenotype, and discussing their clinical findings with an emphasis on neuroimaging and facial features. Using homozygosity mapping followed by whole-exome sequencing, we identified two novel homozygous mutations in AP4M1 and a homozygous deletion in AP4B1 in three pairs of siblings. Spastic tetraplegia, microcephaly, severe intellectual disability, limited speech, and stereotypic laughter were common findings in our patients. All patients also had similar facial features consisting of coarse and hypotonic face, bitemporal narrowing, bulbous nose with broad nasal ridge, and short philtrum which were not described in patients with AP4M1 and AP4B1 mutations previously. The patients presented here and previously with AP4M1, AP4B1, and AP4E1 mutations shared brain abnormalities including asymmetrical ventriculomegaly, thin splenium of the corpus callosum, and reduced white matter volume. The patients also had hippocampal globoid formation and thin hippocampus. In conclusion, disorders due to mutations in AP4 complex have similar neurological, facial, and cranial imaging findings. Thus, these four genes encoding AP4 subunits should be screened in patients with autosomal recessive spastic tetraplegic cerebral palsy, severe intellectual disability, and stereotypic laughter, especially with the described facial and cranial MRI features.

Ocular disorders in children with spastic subtype of cerebral palsy.

To document common ocular abnormalities in children with spastic subtype of cerebral palsy (CP) and to find out whether any correlation exists between their occurance and etiologic factors. Totally 194 patients with the diagnosis of spastic type CP were enrolled in this retrospective study. Detailed ophthalmic examinations were performed. Demographic data and neuroradiological findings were documented. Kruskal-Wallis, Mann Whitney U, Pearson Chi-square tests and Student's t tests were used in the statistical analysis. The mean age was 64.7±44.2 months on the first ophthalmic examination. Prevalences of diplegia (47.4%) and tetraplegia (36.1%) were found to be higher than the frequency of hemiplegia (16.5%) in our study population. Etiologic factor was asphyxia in 60.8% of the patients. Abnormal ocular findings were present in 78.9% of the patients. Statistically significant poor vision was detected in tetraplegia group among all the spastic ubtypes of CP (P=0.000). Anisometropia and significant refractive error were found in 14.4% and 70.1% of the patients, respectively. Thirty-six children (18.6%) had nystagmus and 107 children (55.2%) had strabismus. Lower gestational age and birth weight were statistically higher in patients with esotropia than exotropia (P=0.009 and P=0.024, respectively). Abnormal morphology of the optic disc was present in 152 eyes (39.2%). Severe periventricular leukomalacia (PVL) was found in 48 patients and statistically significant poor vision was detected in the presence of PVL (P=0.000). Spastic diplegic or tetraplegic CP patients with positive neuroradiological symptoms, younger gestational age and lower birth weight ought to have detailed ophthalmic examinations as early as possible to provide best visual rehabilitation.

Improved Gait After Repetitive Locomotor Training in Children with Cerebral Palsy

The aim of this study was to evaluate the effectiveness of repetitive locomotor training with an electromechanical gait trainer in children with cerebral palsy. In this randomized controlled trial, 18 ambulatory children with diplegic or tetraplegic cerebral palsy were randomly assigned to an experimental group or a control group. The experimental group received 30 mins of repetitive locomotor training with an applied technology (Gait Trainer GT I) plus 10 mins of passive joint mobilization and stretching exercises. The control group received 40 mins of conventional physiotherapy. Each subject underwent a total of 10 treatment sessions over a 2-wk period. Performance on the 10-m walk test, 6-min walk test, WeeFIM scale, and gait analysis was evaluated by a blinded rater before and after treatment and at 1-mo follow-up. The experimental group showed significant posttreatment improvement on the 10-m walk test, 6-min walk test, hip kinematics, gait speed, and step length, all of which were maintained at the 1-mo follow-up assessment. No significant changes in performance parameters were observed in the control group. Repetitive locomotor training with an electromechanical gait trainer may improve gait velocity, endurance, spatiotemporal, and kinematic gait parameters in patients with cerebral palsy.

Natural History of Scoliosis in Nonambulatory Spastic Tetraplegic Cerebral Palsy

To analyze the development and progression of scoliosis in children and adolescents with nonambulatory spastic tetraplegic cerebral palsy. Retrospective longitudinal review. Pediatric nursing home. A total of 110 children and adolescents <18 years of age, with scoliosis. N/A. Cobb angle, age, weight, height, history of hip dislocation, tracheostomy. The Cobb angle increased with age, weight, and height in a nonlinear fashion. A square root transformation of the Cobb angle was chosen to model the nonlinear relationship between the Cobb angle and predictors. Age, height, and weight were significant univariate predictors of the square root of the Cobb angle (slopes = 0.377, 0.067, and 0.06, respectively). In the multivariate mixed model, age remained a significant predictor of the Cobb angle (slope = 0.456), but height and weight did not. If the Cobb angle was > 40° by age 12 years, scoliosis was more likely to progress than if the Cobb angle was ≤ 40°. The effect of age was stronger for those with history of tracheostomy (age slope = 0.631 vs 0.281) than those without. The relationship of age and Cobb angle did not differ significantly between hip dislocated and non-hip-dislocated groups. Age was found to be the most significant predictor of Cobb angle, and the effect of age was greater in the tracheostomy group than in the nontracheostomy group. After adjustment for age, the weight and height were not significant predictors of Cobb angle. Cobb angles of > 40° by the age of 12 years were associated with greater increases in Cobb angle with age.

Mutation in the AP4M1 Gene Provides a Model for Neuroaxonal Injury in Cerebral Palsy

Cerebral palsy due to perinatal injury to cerebral white matter is usually not caused by genetic mutations, but by ischemia and/or inflammation. Here, we describe an autosomal-recessive type of tetraplegic cerebral palsy with mental retardation, reduction of cerebral white matter, and atrophy of the cerebellum in an inbred sibship. The phenotype was recorded and evolution followed for over 20 years. Brain lesions were studied by diffusion tensor MR tractography. Homozygosity mapping with SNPs was performed for identification of the chromosomal locus for the disease. In the 14 Mb candidate region on chromosome 7q22, RNA expression profiling was used for selecting among the 203 genes in the area. In postmortem brain tissue available from one patient, histology and immunohistochemistry were performed. Disease course and imaging were mostly reminiscent of hypoxic-ischemic tetraplegic cerebral palsy, with neuroaxonal degeneration and white matter loss. In all five patients, a donor splice site pathogenic mutation in intron 14 of the AP4M1 gene (c.1137+1G-->T), was identified. AP4M1, encoding for the mu subunit of the adaptor protein complex-4, is involved in intracellular trafficking of glutamate receptors. Aberrant GluRdelta2 glutamate receptor localization and dendritic spine morphology were observed in the postmortem brain specimen. This disease entity, which we refer to as congenital spastic tetraplegia (CST), is therefore a genetic model for congenital cerebral palsy with evidence for neuroaxonal damage and glutamate receptor abnormality, mimicking perinatally acquired hypoxic-ischemic white matter injury.

Long‐term effects of botulinum toxin A in children with cerebral palsy

The long-term effects of botulinum toxin A (BoNT-A) treatment in children with cerebral palsy (CP) are still elusive. We studied a prospective clinical cohort of 94 children with different subtypes (50% spastic diplegic CP, 22% hemiplegic CP, 25% tetraplegic CP, 3% dyskinetic CP), sex (55% male, 45% female), severity according to Gross Motor Function Classification System (29% Level I, 15% Level II, 16% Level III, 17% Level IV, 23% Level V), and age (median 5y 4mo, range 11mo-17y 8mo). The longest follow-up time was 3 years 7 months (median 1y 6mo) and included a maximum of eight injections per muscle (median two injections to a specific muscle). Outcome measurements were muscle tone (Modified Ashworth Scale) and joint range of motion (ROM). Assessments were made at a minimum before and 3 months after each injection. Ninety-five per cent confidence intervals for differences from baseline were used to identify significant changes. BoNT-A injections induced reduction of long-term spasticity in all muscle-groups examined: the gastrocnemius, hamstring, and adductor muscles. The reduction in tone was most distinct in the gastrocnemius muscle, and each repeated injection produced an immediate reduction in muscle tone. However, improvement in ROM was brief and measured only after the first injections, whereupon the ROM declined. Thus, the results suggest that BoNT-A can be effective in reducing muscle tone over a longer period, but not in preventing development of contractures in spastic muscles. The dissociation between the effects on muscle tone and ROM indicates that development of contractures is not coupled to increased muscle tone only, but might be caused by other mechanisms.

Risk factors and clinical profiles in Turkish children with cerebral palsy: analysis of 625 cases

The aim of the study was to investigate risk factors, clinical profiles and gross motor function levels of Turkish children with cerebral palsy (CP). A total of 625 consecutive children with CP, who were rehabilitated in the pediatrics rehabilitation clinic between 2000 and 2004 years, were included. Factors causing CP were investigated by interviewing the families and by scanning medical files. Risk factors were recorded as consanguineous marriage, maternal disorder, preterm birth, birth asphyxia, low birth weight, multiple pregnancy, neonatal convulsion, kernicterus, postnatal central nervous system infection and brain injury. Swedish classification was followed in CP typing. Of 625 children with CP, 370 (59.2%) were males and 255 (40.8%) were females, with ages ranging between 2 and 13 years (the mean age was 5.11+/-2.19 years). It was determined that 47.8% of the cases were spastic diplegic CP, 27.7% were spastic tetraplegic CP, 12.8% spastic hemiplegic CP and 11.7% were other types (ataxic, dyskinetic and mixed CP types). The most frequently encountered risk factors were low birth weight (45.1%), preterm birth (40.5%), birth asphyxia (34.6%) and consanguineous marriage (23.8%). Low birth weight, preterm birth, birth asphyxia and consanguineous marriage were top-ranked risk factors that were determined in Turkish children with CP. Compared with other countries, consanguineous marriage is still an important problem in Turkey.

Higher amount of MyHC IIX in a wrist flexor in tetraplegic compared to hemiplegic cerebral palsy

Spastic cerebral palsy can be divided into diagnostic groups by the relative severity of the arm impairment. This study investigates if hemiplegic, tetraplegic or diplegic cerebral palsy (CP) results in different patterns of myosin heavy chain (MyHC) expression in the flexor carpi ulnaris muscle from 17 young patients with CP. Using enzyme-immunohistochemistry and gel electrophoresis techniques we found a higher percentage of fibers expressing fast MyHC IIx (52%) in tetraplegic CP compared to hemiplegic patients (32%), ( p < 0.05). Tetraplegic CP also resulted in a lower amount of fibers expressing slow MyHC I (18%) compared to hemiplegic CP (40%), ( p < 0.005). The proportion of muscle fibers containing fetal MyHC was higher in tetraplegic CP compared to other groups, ( p < 0.005). Taken together theses results indicate that tetraplegic CP is associated with a shift from slow to fast myosins and that regenerative events are more prominent in tetraplegic CP compared with milder brain damage.

Outcome after intrapartum hypoxic ischaemia at term

We consider the range of childhood disabilities that have been attributed to perinatal hypoxic ischaemia at term and review the strength of evidence for each. The strongest evidence is for a causal link between acute profound hypoxic ischaemia and dyskinetic tetraplegic cerebral palsy (CP). Hemiplegic CP is not usually due to a perinatal hypoxic ischaemic insult at term; an important cause is focal cerebral infarction or 'stroke'. Characteristically, diplegic CP is seen in ex-preterm children with periventricular leukomalacia. Ataxic CP is unlikely to be due to perinatal asphyxia. Recent careful follow-up studies have shown that childhood survivors of perinatal hypoxic ischaemia are at risk for cognitive deficits even in the absence of functional motor disorders. There is no evidence that, in isolation, either attention deficit hyperactivity disorder or autism is caused by hypoxic ischaemia. As effective neuroprotective therapies are introduced, notably cooling, it is possible that the prevalence of CP may be reduced.

Spastic Cerebral Palsy: Clinical Magnetic Resonance Imaging Correlation of 129 Children

A prospective study was undertaken of 129 children with spastic cerebral palsy to clarify the relationship between magnetic resonance imaging (MRI) findings and clinical features of cerebral palsy. Low birth weight, asphyxia, prematurity, seizures, mental development, Gross Motor Function Classification System, and MRI findings were analyzed. Significant abnormalities relevant to the cerebral palsy were evident on imaging in 123 (95.3%). A similar percentage of MRI abnormalities were detected in the groups, 45 (100%) in patients with tetraplegic cerebral palsy, 37 (92.5%) in children with diplegic cerebral palsy, and 42 (95.4%) with hemiplegic cerebral palsy. Periventricular leukomalacia was detected more frequently in the children with spastic diplegia than in the patients with tetraplegia or hemiplegia. Cerebral atrophy was found more often in the tetraplegic group compared to the diplegic patients. Porencephalic cysts were detected more frequently in children with spastic hemiplegia. Congenital brain anomalies were found in a higher proportion in tetraplegic children. Significant correlations between the MRI findings and Gross Motor Function Classification System in the diplegic and tetraplegic patients were found. No correlations between the MRI results and risk factors for cerebral palsy in the tetraplegic patients were noted. Early detection of brain abnormalities in children with cerebral palsy may help in the prognosis and in the initiation of appropriate therapy

Spastic wrist flexors are more severely affected than wrist extensors in children with cerebral palsy

Morphological properties of skeletal muscle were compared between wrist flexors and extensors within the same children (n=8, six females, two males; age range 4 to 9y, median age 7y)with wrist muscle imbalance secondary to spastic cerebral palsy (CP). Five patients had hemiplegic CP, two diplegic CP, and one patient had tetraplegic CP. Muscle biopsies were taken during either tendon transfer or tendon lengthening procedures. Analyses included distribution of muscle fibre types, fibre sizes, and expression of developmental myosins. Extensor fibre area was significantly greater than flexor fibre area for type 2A fibres and type 2B fibres but not for type 1 fibres. Coefficient of variation (CV) of fibre size for all three fibre types was greater for flexors compared with extensors. The greatest CV was observed for the type 2A fibres in flexors (39.5 [3.6%]). A wide variation was observed for expression of developmental myosin with the magnitude of the expression being greater, but not statistically significant, in flexors compared with extensors (5.4/mm2 vs 0.53/mm2). These data demonstrate that significant secondary myopathy of wrist flexor muscles results from CP.

Spastic wrist flexors are more severely affected than wrist extensors in children with cerebral palsy

Morphological properties of skeletal muscle were compared between wrist flexors and extensors within the same children (n = 8, six females, two males; age range 4 to 9y, median age 7 y) with wrist muscle imbalance secondary to spastic cerebral palsy (CP). Five patients had hemiplegic CP, two diplegic CP, and one patient had tetraplegic CP. Muscle biopsies were taken during either tendon transfer or tendon lengthening procedures. Analyses included distribution of muscle fibre types, fibre sizes, and expression of developmental myosins. Extensor fibre area was significantly greater than flexor fibre area for type 2A fibres and type 2B fibres but not for type 1 fibres. Coefficient of variation (CV) of fibre size for all three fibre types was greater for flexors compared with extensors. The greatest CV was observed for the type 2A fibres in flexors (39.5 [3.6%]). A wide variation was observed for expression of developmental myosin with the magnitude of the expression being greater, but not statistically significant, in flexors compared with extensors (5.4/mm2 vs 0.53/mm2). These data demonstrate that significant secondary myopathy of wrist flexor muscles results from CP.