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1.
J Physiol ; 602(14): 3489-3504, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-39008710

RESUMO

Cerebral palsy (CP) describes some upper motoneuron disorders due to non-progressive disturbances occurring in the developing brain that cause progressive changes to muscle. While longer sarcomeres increase muscle stiffness in patients with CP compared to typically developing (TD) patients, changes in extracellular matrix (ECM) architecture can increase stiffness. Our goal was to investigate how changes in muscle and ECM architecture impact muscle stiffness, gait and joint function in CP. Gracilis and adductor longus biopsies were collected from children with CP undergoing tendon lengthening surgery for hamstring and hip adduction contractures, respectively. Gracilis biopsies were collected from TD patients undergoing anterior cruciate ligament reconstruction surgery with hamstring autograft. Muscle mechanical testing, two-photon imaging and hydroxyproline assay were performed on biopsies. Corresponding data were compared to radiographic hip displacement in CP adductors (CPA), gait kinematics in CP hamstrings (CPH), and joint range of motion in CPA and CPH. We found at matched sarcomere lengths muscle stiffness and collagen architecture were similar between TD and CP hamstrings. However, CPH stiffness (R2 = 0.1973), collagen content (R2 = 0.5099) and cross-linking (R2 = 0.3233) were correlated to decreased knee range of motion. Additionally, we observed collagen fibres within the muscle ECM increase alignment during muscular stretching. These data demonstrate that while ECM architecture is similar between TD and CP hamstrings, collagen fibres biomechanics are sensitive to muscle strain and may be altered at longer in vivo sarcomere lengths in CP muscle. Future studies could evaluate the impact of ECM architecture on TD and CP muscle stiffness across in vivo operating ranges. KEY POINTS: At matched sarcomere lengths, gracilis muscle mechanics and collagen architecture are similar in TD patients and patients with CP. In both TD and CP muscles, collagen fibres dynamically increase their alignment during muscle stretching. Aspects of muscle mechanics and collagen architecture are predictive of in vivo knee joint motion and radiographic hip displacement in patients with CP. Longer sarcomere lengths in CP muscle in vivo may alter collagen architecture and biomechanics to drive deficits in joint mobility and gait function.


Assuntos
Paralisia Cerebral , Colágeno , Humanos , Paralisia Cerebral/fisiopatologia , Paralisia Cerebral/patologia , Criança , Masculino , Feminino , Colágeno/metabolismo , Fenômenos Biomecânicos , Adolescente , Músculo Grácil , Amplitude de Movimento Articular , Músculo Esquelético/fisiologia , Músculo Esquelético/fisiopatologia , Marcha/fisiologia , Músculos Isquiossurais/fisiologia , Músculos Isquiossurais/fisiopatologia , Matriz Extracelular/fisiologia
2.
Exp Neurol ; 378: 114835, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38789024

RESUMO

Cerebral Palsy (CP) is the main motor disorder in childhood resulting from damage to the developing brain. Treatment perspectives are required to reverse the primary damage caused by the early insult and consequently to recover motor skills. Resveratrol has been shown to act as neuroprotection with benefits to skeletal muscle. This study aimed to investigate the effects of neonatal resveratrol treatment on neurodevelopment, skeletal muscle morphology, and cerebellar damage in CP model. Wistar rat pups were allocated to four experimental groups (n = 15/group) according CP model and treatment: Control+Saline (CS), Control+Resveratrol (CR), CP + Saline (CPS), and CP + Resveratrol (CPR). CP model associated anoxia and sensorimotor restriction. CP group showed delay in the disappearance of the palmar grasp reflex (p < 0.0001) and delay in the appearance of reflexes of negative geotaxis (p = 0.01), and free-fall righting (p < 0.0001), reduced locomotor activity and motor coordination (p < 0.05) than CS group. These motor skills impairments were associated with a reduction in muscle weight (p < 0.001) and area and perimeter of soleus end extensor digitorum longus muscle fibers (p < 0.0001), changes in muscle fibers typing pattern (p < 0.05), and the cerebellum showed signs of neuroinflammation due to elevated density and percentage of activated microglia in the CPS group compared to CS group (p < 0.05). CP animals treated with resveratrol showed anticipation of the appearance of negative geotaxis and free-fall righting reflexes (p < 0.01), increased locomotor activity (p < 0.05), recovery muscle fiber types pattern (p < 0.05), and reversal of the increase in density and the percentage of activated microglia in the cerebellum (p < 0.01). Thus, we conclude that neonatal treatment with resveratrol can contribute to the recovery of the delay neurodevelopment resulting from experimental CP due to its action in restoring the skeletal muscle morphology and reducing neuroinflammation from cerebellum.


Assuntos
Animais Recém-Nascidos , Cerebelo , Paralisia Cerebral , Microglia , Músculo Esquelético , Ratos Wistar , Resveratrol , Resveratrol/farmacologia , Animais , Cerebelo/efeitos dos fármacos , Cerebelo/patologia , Ratos , Microglia/efeitos dos fármacos , Microglia/patologia , Paralisia Cerebral/tratamento farmacológico , Paralisia Cerebral/patologia , Músculo Esquelético/efeitos dos fármacos , Músculo Esquelético/patologia , Modelos Animais de Doenças , Estilbenos/farmacologia , Estilbenos/uso terapêutico , Masculino , Recuperação de Função Fisiológica/efeitos dos fármacos , Feminino
3.
Pediatr Neurol ; 153: 131-136, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38382245

RESUMO

BACKGROUND: The early prediction of cerebral palsy (CP) could enable the follow-up of high-risk infants during the neuroplasticity period. This study aimed to explore the predictive value of fidgety movement assessment (FMA) and brain magnetic resonance imaging (MRI) for the development of CP in clinic rehabilitation setting. METHODS: This retrospective observational study included infants who underwent FMA and brain MRI at age nine to 20 weeks at Children's Hospital, Zhejiang University School of Medicine, between March 2018 and September 2019. The area under the receiver operating characteristic curve (AUC), sensitivity, specificity, and accuracy of FMA and MRI for predicting the development of CP were assessed. RESULTS: A total of 258 infants (169 males, gestational age 37.4 ± 3.0 weeks, birth weight 2987.9 ± 757.1 g) were included. Fifteen children had CP after age two years. The diagnostic value of FMA and brain MRI combination showed 86.7% sensitivity (95% confidence interval [CI]: 58.4% to 97.7%), 98.4% specificity (95% CI: 95.6% to 99.5%), and 97.7% accuracy (95% CI: 95.0% to 99.1%); the combination diagnostic value also showed a significantly higher AUC for predicting CP after age two years than FMA alone (AUC: 0.981 vs 0.893, P = 0.013). CONCLUSIONS: The diagnostic value of FMA and brain MRI combination during infancy showed a high predictive value for CP development in clinical rehabilitation setting.


Assuntos
Paralisia Cerebral , Humanos , Lactente , Masculino , Peso ao Nascer , Encéfalo/diagnóstico por imagem , Encéfalo/patologia , Paralisia Cerebral/diagnóstico por imagem , Paralisia Cerebral/patologia , Imageamento por Ressonância Magnética , Movimento , Feminino
4.
Neuroimage Clin ; 41: 103575, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38354671

RESUMO

Brain damage in children with unilateral cerebral palsy (UCP) affects motor function, with varying severity, making it difficult the performance of daily actions. Recently, qualitative and semi-quantitative methods have been developed for lesion classification, but studies on mild to moderate hand impairment are lacking. The present study aimed to characterize lesion topography and preserved brain areas in UCP children with specific patterns of hand manipulation. A homogeneous sample of 16 UCP children, aged 9 to 14 years, was enrolled in the study. Motor assessment included the characterization of the specific pattern of hand manipulation, by means of unimanual and bimanual measures (Kinematic Hand Classification, KHC; Manual Ability Classification System, MACS; House Functional Classification System, HFCS; Melbourne Unilateral Upper Limb Assessment, MUUL; Assisting Hand Assessment, AHA). The MRI morphological study included multiple methods: (a) qualitative lesion classification, (b) semi-quantitative classification (sq-MRI), (c) voxel-based morphometry comparing UCP and typically developed children (VBM-DARTEL), and (d) quantitative brain tissue segmentation (q-BTS). In addition, functional MRI was used to assess spared functional activations and cluster lateralization in the ipsilesional and contralesional hemispheres of UCP children during the execution of simple movements and grasping actions with the more affected hand. Lesions most frequently involved the periventricular white matter, corpus callosum, posterior limb of the internal capsule, thalamus, basal ganglia and brainstem. VMB-DARTEL analysis allowed to detect mainly white matter lesions. Both sq-MRI classification and q-BTS identified lesions of thalamus, brainstem, and basal ganglia. In particular, UCP patients with synergic hand pattern showed larger involvement of subcortical structures, as compared to those with semi-functional hand. Furthermore, sparing of gray matter in basal ganglia and thalamus was positively correlated with MUUL and AHA scores. Concerning white matter, q-BTS revealed a larger damage of fronto-striatal connections in patients with synergic hand, as compared to those with semi-functional hand. The volume of these connections was correlated to unimanual function (MUUL score). The fMRI results showed that all patients, but one, including those with cortical lesions, had activation in ipsilesional areas, regardless of lesion timing. Children with synergic hand showed more lateralized activation in the ipsilesional hemisphere both during grasping and simple movements, while children with semi-functional hand exhibited more bilateral activation during grasping. The study demonstrates that lesion localization, rather than lesion type based on the timing of their occurrence, is more associated with the functional level of hand manipulation. Overall, the preservation of subcortical structures and white matter can predict a better functional outcome. Future studies integrating different techniques (structural and functional imaging, TMS) could provide further evidence on the relation between brain reorganization and specific pattern of manipulation in UCP children.


Assuntos
Paralisia Cerebral , Hemiplegia , Criança , Humanos , Hemiplegia/diagnóstico por imagem , Hemiplegia/complicações , Encéfalo , Paralisia Cerebral/patologia , Imageamento por Ressonância Magnética/métodos , Movimento , Mãos
5.
Toxins (Basel) ; 16(2)2024 01 30.
Artigo em Inglês | MEDLINE | ID: mdl-38393147

RESUMO

Botulinum toxin-A (BoNT-A) injection is known to exert beneficial effects on muscle tone, joint mobility and gait in children with cerebral palsy (CP). However, recent animal and human studies have raised the concern that BoNT-A might be harmful to muscle integrity. In CP-children, the impact of BoNT-A on muscle structure has been poorly studied, and inconsistent results have been reported. This study was aimed at determining the time course effect of a single BoNT-A administration on medial gastrocnemius (MG) morphology in CP-children. MG microbiopsies from 12 ambulant and BoNT-A-naïve CP-children (age, 3.4 (2.3) years, ranging from 2.5 to 7.8 years; seven boys and five girls; GMFCS I = 5, II = 4 and III = 3) were collected before and 3 and 6 months after BoNT-A treatment to analyze the fiber cross-sectional area (fCSA) and proportion; capillarization; and satellite cell (SC) content. Compared with the baseline, the fCSA decreased at 3 months (-14%, NS) and increased at 6 months (+13%, NS). Fiber size variability was significantly higher at 3 months (type I: +56%, p = 0.032; type IIa: +37%, p = 0.032) and 6 months (type I: +69%, p = 0.04; type IIa: +121%, p = 0.032) compared with the baseline. The higher type I proportion seen at 3 months was still present and more pronounced at 6 months (type I: +17%, p = 0.04; type IIx: -65%, p = 0.032). The capillary fiber density was reduced at 3 months (type I: -43%, NS; type II: -44%, p = 0.0320) but normalized at 6 months. There was a non-significant increase in SC/100 fibers at 3 months (+75%, NS) and 6 months (+40%, NS) compared with the baseline. These preliminary data suggest that BoNT-A induced alterations in the MG of children with CP, which were still present 6 months after BoNT-A injection but with signs of muscle recovery.


Assuntos
Toxinas Botulínicas Tipo A , Paralisia Cerebral , Fármacos Neuromusculares , Masculino , Feminino , Humanos , Pré-Escolar , Projetos Piloto , Fármacos Neuromusculares/uso terapêutico , Paralisia Cerebral/tratamento farmacológico , Paralisia Cerebral/patologia , Espasticidade Muscular/tratamento farmacológico , Injeções Intramusculares , Resultado do Tratamento , Músculo Esquelético , Toxinas Botulínicas Tipo A/uso terapêutico
6.
J Med Primatol ; 53(1): e12691, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38345330

RESUMO

BACKGROUND: Cerebral palsy is a severe motor disability in childhood that poses challenges for children, families, and society. Rhesus macaques are the preferred animals for cerebral palsy model, but surgical excision of motor cortex has low success rate and high cost. In this work, we created cerebral palsy rhesus macaque models by intrathecal injection of bilirubin. METHODS: The puncture point for injection was identified as the intervertebral disc space two, located below the intersection of the iliac crest line and the posterior median line. RESULTS: The models showed abnormal posture and increased muscle tension. Diffuse deposits of bilirubin were found in the basal ganglia from the magnetic resonance imaging. Pathological slides also revealed the presence of brain lesions, such as vacuole formation, contraction of neuronal nuclei, and deep staining of nuclei in the histopathological sections of the hippocampus and basal ganglia. CONCLUSION: The model's symptoms closely resemble those observed in humans with spastic cerebral palsy.


Assuntos
Paralisia Cerebral , Pessoas com Deficiência , Transtornos Motores , Humanos , Animais , Paralisia Cerebral/veterinária , Paralisia Cerebral/patologia , Macaca mulatta , Análise Custo-Benefício
7.
Histopathology ; 84(6): 1024-1037, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38253913

RESUMO

AIMS: Histological chorioamnionitis (HCA) is a condition linked to preterm birth and neonatal infection and its relationship with various pathological stages in extremely preterm neonates, and with their associated short- and long-term consequences, remains a subject of research. This study investigated the connection between different pathological stages of HCA and both short-term complications and long-term outcomes in preterm infants born at or before 32 weeks of gestational age. METHODS: Preterm infants born at ≤ 32 weeks of gestation who underwent placental pathology evaluation and were followed-up at 18-24 months of corrected age were included. Neonates were classified based on their exposure to HCA and were further subdivided into different groups according to maternal inflammatory responses (MIR) and fetal inflammatory responses (FIR) stages. We compared short-term complications during their hospital stay between the HCA-exposed and -unexposed groups and examined the influence of HCA stages on long-term outcomes. RESULTS: The HCA group exhibited distinct characteristics such as higher rates of premature rupture of membranes > 18 h, reduced amniotic fluid, early-onset sepsis, bronchopulmonary dysplasia and intraventricular haemorrhage (IVH) grades III-IV (P < 0.05). The moderate-severe HCA group displayed lower gestational age, lower birth weight and higher incidence of IVH (grades III-IV) and preterm sepsis compared with the mild HCA group (P < 0.05). After adjusting for confounders, the MIR stages 2-3 group showed associations with cognitive impairment and cerebral palsy (P < 0.05), and the FIR stages 2-3 group also showed poor long-term outcomes and cognitive impairment (P < 0.05). CONCLUSIONS: Moderate-severe HCA was associated with increased early-onset sepsis, severe IVH and poor long-term outcomes, including cognitive impairment and cerebral palsy. Vigilant prevention strategies are warranted for severe HCA cases in order to mitigate poorer clinical outcomes.


Assuntos
Paralisia Cerebral , Corioamnionite , Ruptura Prematura de Membranas Fetais , Nascimento Prematuro , Sepse , Lactente , Recém-Nascido , Humanos , Feminino , Gravidez , Recém-Nascido Prematuro , Corioamnionite/epidemiologia , Corioamnionite/etiologia , Corioamnionite/patologia , Placenta/patologia , Ruptura Prematura de Membranas Fetais/patologia , Paralisia Cerebral/complicações , Paralisia Cerebral/patologia , Nascimento Prematuro/etiologia , Nascimento Prematuro/epidemiologia , Nascimento Prematuro/patologia , Fatores de Risco , Idade Gestacional , Sepse/complicações , Sepse/patologia
8.
Neuroradiology ; 66(2): 261-269, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38129651

RESUMO

PURPOSE: Preterm children with cerebral palsy (CP) often have varying hand dysfunction, while the specific brain injury with periventricular leukomalacia (PVL) cannot quite explain its mechanism. We aimed to investigate glymphatic activity using diffusion tensor image analysis along the perivascular space (DTI-ALPS) method and evaluate its association with brain lesion burden and hand dysfunction in children with CP secondary to PVL. METHODS: We retrospectively enrolled 18 children with bilateral spastic CP due to PVL and 29 age- and sex-matched typically developing controls. The Manual Ability Classification System (MACS) was used to assess severity of hand dysfunction in CP. A mediation model was performed to explore the relationship among the DTI-ALPS index, brain lesion burden, and the MACS level in children with CP. RESULTS: There were significant differences in the DTI-ALPS index between children with CP and their typically developing peers. The DTI-ALPS index of the children with CP was lower than that of the controls (1.448 vs. 1.625, P = 0.003). The mediation analysis showed that the DTI-ALPS index fully mediated the relationship between brain lesion burden and the MACS level (c' = 0.061, P = 0.665), explaining 80% of the effect. CONCLUSION: This study provides new insights into the neural basis of hand dysfunction in children with CP, demonstrating an important role of glymphatic impairment in such patients. These results suggest that PVL might affect hand function in children with CP by disrupting glymphatic drainage.


Assuntos
Paralisia Cerebral , Sistema Glinfático , Leucomalácia Periventricular , Criança , Recém-Nascido , Humanos , Paralisia Cerebral/complicações , Paralisia Cerebral/diagnóstico por imagem , Paralisia Cerebral/patologia , Leucomalácia Periventricular/complicações , Leucomalácia Periventricular/diagnóstico por imagem , Leucomalácia Periventricular/patologia , Sistema Glinfático/patologia , Estudos Retrospectivos , Mãos/patologia
9.
Eur J Neurosci ; 59(2): 298-307, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38128061

RESUMO

Children with cerebral palsy (CP) exhibit impaired motor control and significant muscle weakness due to a brain lesion. However, studies that assess the relationship between brain activity and performance on dynamic functional muscle strength assessments in CP are needed. The aim of this study was to determine the effect of a progressive lateral step-up test on prefrontal cortex (PFC) hemodynamic activity in children with CP. Fourteen ambulatory children with spastic CP (Gross Motor Function Classification System level I; 5-11 y) and 14 age- and sex-matched typically developing control children completed a progressive lateral step-up test at incremental step heights (0, 10, 15 and 20 cm) using their non-dominant lower limb. Hemodynamic activity in the PFC was assessed using non-invasive, portable functional neuroimaging (functional near-infrared spectroscopy). Children with CP completed fewer repetitions at each step height and exhibited lower PFC hemodynamic activity across step heights compared to controls. Lower PFC activation in CP was maintained after statistically controlling for the number of repetitions completed at each step height. PFC hemodynamic activity was not associated with LSUT task performance in children with CP, but a positive relationship was observed in controls at the most challenging 20 cm step height. The results suggest there is an altered PFC recruitment pattern in children with CP during a highly dynamic test of functional strength. Further studies are needed to explore the mechanisms underlying the suppressed PFC activation observed in children with CP compared to typically developing children.


Assuntos
Paralisia Cerebral , Criança , Humanos , Paralisia Cerebral/diagnóstico por imagem , Paralisia Cerebral/patologia , Espectroscopia de Luz Próxima ao Infravermelho/métodos , Extremidade Inferior , Córtex Pré-Frontal/fisiologia , Hemodinâmica , Força Muscular/fisiologia
10.
Am J Physiol Cell Physiol ; 326(2): C573-C588, 2024 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-38105751

RESUMO

Inconsistent alterations in skeletal muscle histology have been reported in adolescents with cerebral palsy (CP) and whether alterations are present in young children and differ from older children is not yet known. This study aimed to define histological alterations in the medial gastrocnemius (MG) of ambulant CP (gross-motor classification system, GMFCS I-III) stratified in two age groups (preschool children, PS: 2-5 and school age children, SA: 6-9-yr old) compared with age-matched typically developing (TD) children. We hypothesized that alterations in muscle microscopic properties are already present in PS-CP and are GMFCS level specific. Ultrasound guided percutaneous microbiopsies were collected in 46 CP (24-PS) and 45 TD (13-PS) children. Sections were stained to determine fiber cross-sectional area (fCSA) and proportion, capillary, and satellite cell amount. Average absolute and normalized fCSA were similar in CP and TD, but a greater percentage of smaller fibers was found in CP. Coefficient of variation (CV) was significantly larger in PS-CP-GMFCS I-II and for type I fiber. In SA-CP, all fiber types contributed to the higher CV. Type IIx proportion was higher and type I was lower in PS-CP-GMFCS-III and for all SA-CP. Reduced capillary-to-fiber ratio was present in PS-CP-GMFCS II-III and in all SA-CP. Capillary fiber density was lower in SA-CP. Capillary domain was enhanced in all CP, but capillary spatial distribution was maintained as was satellite cell content. We concluded that MG histological alterations are already present in very young CP but are only partly specific for GMFCS level and age.NEW & NOTEWORTHY Inconsistent histological alterations have been reported in children with cerebral palsy (CP) but whether they are present in very young and ambulant CP children and differ from those reported in old CP children is not known. This study highlighted for the first time that enhanced muscle fiber size variability and loss of capillaries are already present in very young CP children, even in the most ambulant ones, and these alterations seem to extend with age.


Assuntos
Paralisia Cerebral , Humanos , Pré-Escolar , Adolescente , Criança , Paralisia Cerebral/patologia , Músculo Esquelético/patologia
11.
Artigo em Inglês | LILACS, BBO - Odontologia | ID: biblio-1386799

RESUMO

Abstract Objective: To evaluate the barriers to access and oral health care faced by children and adolescents with Cerebral Palsy (CP) according to their motor impairment through the perception of caregivers. Material and Methods: A case series study was carried out at three health institutions in Pernambuco, Brazil. The study sample consisted of 94 caregivers of 5-to-18-year-old patients with CP, according to GMFCS (The Gross Motor Function Classification System). Data were collected using a semi-structured form to evaluate the barriers to access and analyzed statistically by the chi-square and Fisher exact tests, adopting a 5% level of significance. In addition, binary logistic regression was performed to determine the weight of the variables in explaining the outcome variable. Results: There were major difficulties involving transportation (p=0.04) and structural accessibility to dental services (p<0.01) among children and adolescents with severe CP. In addition, the more severe the CP, the greater the difficulty of accessibility (OR=4.09,) and the lower the income (OR=8.80), the greater the motor impairment. Conclusion: Despite the availability of access to dental services, low-income families have more severe CP patients, contributing to the daily difficulties already faced by them in oral health care.


Assuntos
Humanos , Masculino , Feminino , Pré-Escolar , Criança , Adolescente , Percepção Social , Paralisia Cerebral/patologia , Saúde Bucal , Cuidadores , Assistência Odontológica para a Pessoa com Deficiência , Estudos Epidemiológicos , Modelos Logísticos , Inquéritos e Questionários , Estatísticas não Paramétricas , Crianças com Deficiência , Estudos Observacionais como Assunto
12.
Revista Digital de Postgrado ; 9(2): 205, ago. 2020. tab
Artigo em Espanhol | LILACS, LIVECS | ID: biblio-1102879

RESUMO

La Parálisis Cerebral (PC) es un conjunto de alteraciones motrices no progresivas en la población infantojuvenil, ocasionadas por lesión ­a nivel cerebral- de neuronas o fibras de esa vía, de sus aferencias o de las que la modulan; para su diagnóstico deben conocerse otras patologías también frecuentes y que pueden incidir simultánea o causalmente en la motricidad del paciente; la resultante sería disfunción motora tanto voluntaria como involuntaria, refleja o con propósito, de la postura y/o del tono muscular. Objetivo: detectar errores innatos metabólicos (EIM) que causan o se asocian con PC en una serie significativa. Métodos: Estudio descriptivo-interpretativo, se revisaron los expedientes clínicos del Centro de Parálisis Cerebral de Caracas, en cuyos diagnósticos se presentaron ambas alteraciones, entre los años 1988 y 2018. Resultados: De las 2.000 historias clínicas revisadas, el exámen clínico y las pruebas de laboratorio permitieron seleccionar 174 casos de EIM. Conclusiones: Se tipificaron los errores innatos metabólicos en diez formas clínicas distintas, se evidenciaron en pacientes con PC atendidos en un centro público de Caracas, es posible que la casuística sea varias veces mayor en Venezuela dado que ya no se aplica la pesquisa en los centros de atención pública(AU)


Cerebral Palsy (CP) is a set of non-progressive motor alterations in the child and youth population, caused by injury - at the brain level - of neurons or fibers of that pathway, their afferences or those that modulate it; for its diagnosis, other pathologies that are also frequent and that can simultaneously or causally affect the motor skills of the same patient must be known; The result would be both voluntary and involuntary motor dysfunction, reflected or with purpose, of posture and / or muscle tone. Objective: to detect inborn metabolic errors (EIM) that cause or are associated with CP in a significant series. Methods: Descriptive-interpretive study, we reviewed the clinical records of the Cerebral Palsy Center of Caracas, in whose diagnoses both alterations were presented, between the years 1988 and 2018. Results: Of the 2,000 clinical histories reviewed, the clinical examination and tests Laboratory tests allowed the selection of 174 cases of IMD. Conclusions: Inborn metabolic errors were typified in ten different clinical forms, they were evidenced in patients with CP treated in a public center in Caracas, it is possible that the casuistry is several times greater in Venezuela since the investigation is no longer applied in the centers of public attention(AU)


Assuntos
Humanos , Masculino , Feminino , Pré-Escolar , Criança , Adolescente , Paralisia Cerebral/patologia , Erros Inatos do Metabolismo , Neurônios/metabolismo , Pediatria , Doenças do Sistema Nervoso
13.
Artigo em Inglês | LILACS, BBO - Odontologia | ID: biblio-1056826

RESUMO

Abstract Objective: To evaluate factors associated with the caregivers' perception of the oral health of children and adolescents with cerebral palsy. Material and Methods: A cross-sectional study was carried out with 80 children and adolescents with cerebral palsy aged 2-18 years, registered at a reference institution and their respective caregivers. Caregivers provided socioeconomic, systemic and health perception and access to dental services data. A calibrated researcher (Kappa=0.75-0.98) performed oral examinations using DMF-T, dmf-t, dental trauma, gingival bleeding index, community periodontal index, malocclusion index and the dental aesthetic index. The Poisson Regression was used (α=0.05) Results: According to the caregivers' perception, the oral health reports of children and adolescents with cerebral palsy considered good and poor was 73.8% and 26.3%, respectively. The poor oral health perception is associated with the presence of dental caries in anterior teeth (PR 1.34, 95% CI=1.11-1.63) and bleeding during tooth brushing (PR 1.20, 95% CI=1.04-1.40) Conclusion: Children and adolescents with cerebral palsy who presented lesions of caries in the anterior teeth and gingival bleeding during tooth brushing, more frequently, had poor oral health by their caregivers.


Assuntos
Humanos , Masculino , Feminino , Pré-Escolar , Criança , Adolescente , Paralisia Cerebral/patologia , Criança , Saúde Bucal , Cuidadores , Brasil/epidemiologia , Estudos Transversais/métodos , Análise Multivariada , Análise de Regressão , Cárie Dentária/prevenção & controle
14.
Acta fisiátrica ; 25(4)dez. 2018.
Artigo em Inglês, Português | LILACS | ID: biblio-1000034

RESUMO

A aquisição da marcha sob o aspecto neuromotor da reabilitação vem a ser o principal desígnio do terapeuta durante a elaboração do plano de tratamento do paciente com paralisia cerebral, uma vez que a marcha representa não só um ganho da habilidade de locomover-se, mas também um conjunto de reações e padrões de movimento que auxiliam na função motora. Objetivo: Analisar os efeitos de um tratamento intensivo por meio do suporte de peso corporal em crianças com paralisia cerebral. Métodos: Participaram do estudo 10 crianças com paralisia cerebral, GMFCS nível IV ou V, idade entre 4 a 9 anos. Onde foram realizadas sessões diárias com suporte de peso corporal em esteira ergométrica com auxílio de terapeutas para executar o padrão de marcha mais próximo da normalidade, com pontos chaves em joelho e tornozelo com duração de 30 minutos de tratamento e intervalo de 24 horas, por um período de 10 dias. Para fins de avaliação pré e pós-intervenção, utilizando a escala de medição da função motora grossa GMFM, e da flexibilidade pelo Flexiteste. Resultados: Foi possível constatar um aumento relevante na função motora grossa dos sujeitos, onde o domínio Deitar e Rolar obteve maior pontuação, com um aumento de 10,77%. Sentar demonstrou aumento de 3,80%, Engatinhar e Ajoelhar 6,43% e o domínio Em Pé 3,45%. Relativo ao Flexiteste, a média entre os sujeitos relatou aumento expressivo de 4,2 pontos. Já em análise individual, percebe-se que 3 indivíduos obtiveram aumento de score de 6 pontos. Conclusão: Um protocolo intensivo de curta duração é capaz de trazer ganhos de flexibilidade e motores rápidos a crianças que possuem quadro de paralisia cerebral.


The acquisition of gait under the neuromotor aspect of rehabilitation is the main goal of the therapist during the elaboration of the treatment plan of the patient with cerebral palsy, since gait represents not only a gain in the ability to move, but also a set of reactions and movement patterns that aid in motor function. Objective: To analyze the effects of intensive treatment by means of body weight support in children with cerebral palsy. Method: Ten children with cerebral palsy, GMFCS level IV or V, age between 4 and 9 years participated in the study. Where daily sessions were performed with body weight support on a treadmill with the help of therapists to perform the walking pattern closest to normal, with key points in the knee and ankle lasting 30 minutes of treatment and 24 hour intervals for one period of 10 days. For purposes of pre and post-intervention evaluation, using the GMFM gross motor function measurement scale, and Flexitest flexibility. Results: It was possible to observe a significant increase in the gross motor function of the subjects, where the lie down and roll domain obtained a higher score, with an increase of 10.77%. To sit demonstrated a 3.80% increase, Crawling and Kneeling 6.43% and the Standing Foot 3.45%. Relative to Flexitest, the mean between subjects reported a significant increase of 4.2 points. In the individual analysis, it was observed that 3 subjects had a 6-point score increase. Conclusions: An intensive short-duration protocol is capable of bringing flexibility gains and fast motor to children with cerebral palsy.


Assuntos
Humanos , Pré-Escolar , Criança , Paralisia Cerebral/patologia , Modalidades de Fisioterapia/instrumentação , Marcha , Atividade Motora
15.
Int. j. morphol ; 36(3): 886-894, Sept. 2018. tab, graf
Artigo em Inglês | LILACS | ID: biblio-954203

RESUMO

Cerebral Palsy (CP) refers to chronic childhood encephalopathy. The objective of this study was to verify effects of CP model that combines prenatal exposure to LPS, perinatal anoxia and sensorimotor restriction on EDL muscle. Male Wistar rat pups were separated: a) Control - pups of mothers injected with saline during pregnancy and b) Cerebral Palsy - pups of mothers injected with LPS during pregnancy, and submitted to perinatal anoxia and sensorimotor restriction. The CP group presented hypertrophy in the type IIB fibers and increase of nuclei/fiber and capillary/fiber ratios. The intrafusal fibers of CP group presented 26 % atrophy in the crosssectional area and intramuscular collagen volume increase 34 %. CP group showed myofibrillar disruption and Z-line disorganization and the NMJs presented increases of 22 % in area.This animal model of CP produces motor deficits and macro and microscopic alterations and in the ultrastructure of the EDL muscle.


La parálisis cerebral (PC) se refiere a la encefalopatía crónica infantil. El objetivo de este estudio fue verificar los efectos del modelo PC que combina la exposición prenatal a LPS, la anoxia perinatal y la restricción sensitivo-motora en el músculo extensor largo de los dedos (MELD). Se separaron las crías de ratas Wistar machos: a) Control: crías de madres inyectadas con solución salina durante la preñez y b) Parálisis cerebral: crías de madres inyectadas con LPS durante la preñez y sometidas a anoxia perinatal y restricción sensitivo-motora. El grupo PC presentó hipertrofia en las fibras tipo IIB y aumento de la relación núcleo / fibra y capilar / fibra. Las fibras intrafusales del grupo PC presentaron un 26 % de atrofia en el área de la sección transversal y el volumen de colágeno intramuscular aumentó un 34 %. El grupo PC mostró disrupción miofibrilar y desorganización de la línea Z y los NMJ presentaron aumentos de 22 % en el área. Este modelo animal de PC produce déficit motores y alteraciones macro y microscópicas y cambios en la ultraestructura del MELD.


Assuntos
Animais , Ratos , Paralisia Cerebral/patologia , Músculo Esquelético/patologia , Ratos Wistar , Microscopia Eletrônica de Transmissão , Modelos Animais de Doenças
16.
Int. j. odontostomatol. (Print) ; 9(1): 101-106, Apr. 2015. ilus
Artigo em Espanhol | LILACS | ID: lil-747485

RESUMO

Los avances en medicina y en particular en pediatría han permitido sobrevivir a más niños que nacen prematuros. Aunque la mayoría de los sobrevivientes no tienen inconvenientes en su desarrollo, un significativo número de niños demuestran discapacidad en su neurodesarrollo. En los prematuros, las complicaciones y las secuelas neurológicas, aumentan con la disminución de la edad gestacional y el peso al nacer. La parálisis cerebral es la discapacidad neuromuscular más frecuente que afecta a los niños, y constituyen un grupo de alto riesgo de patologías orales. El reporte del presente caso clínico, muestra el tratamiento odontológico de un pre-escolar con parálisis cerebral, realizado en forma ambulatoria, sin premedicación ni anestesia general, sustentado en un conocimiento profundo de la patología basal médica y características individuales. La compresión de las enfermedades orales, la aplicación de técnicas de manejo conductual y la sensibilización del clínico, permitieron controlar y mejorar el deplorable estado de salud oral del pre-escolar, contribuyendo por lo tanto a una mejor calidad de vida. La promoción, la prevención y recuperación de la salud oral en los pacientes con necesidades de cuidados especiales de salud debe ser una parte integral de la odontología, para lo cual el odontopediatra debe estar integrado al equipo multidisciplinario, que busca brindar atenciones adecuadas a las necesidades de este grupo en particular.


Advances in medicine, particularly in pediatric medicine, have enabled more premature babies to survive. While the majority of the survivors present trouble-free development, a significant number of all children present disabilities in their neural development. In premature babies, the neurological complications and consequences increase inversely with the gestational age and weight at birth. Cerebral palsy is the most common neuromuscular disability affecting these children, and as a group they also present a high risk of oral pathologies. The report of the present clinical case describes the dental treatment of a pre-school child with cerebral palsy, in outpatient conditions and without either pre-medication or general anaesthesia, based on a profound knowledge of the basic medical pathology and individual characteristics. The understanding of oral disease, the use of behavioral management techniques and raising awareness in the clinic, enabled the child's deplorable state of oral health to be controlled and improved, thus contributing to a better quality of life. Preventative measures and the promotion and recovery of oral health in patients requiring special health care must be an integral part of dentistry. This requires the dental-pediatrician to form part of a multidisciplinary team specializing in providing proper treatment for the needs of this particular group.


Assuntos
Humanos , Feminino , Pré-Escolar , Paralisia Cerebral/patologia , Assistência Odontológica , Saúde Bucal , Crianças com Deficiência , Placa Dentária , Gengivite , Consentimento Livre e Esclarecido
17.
Arq. neuropsiquiatr ; 72(5): 360-367, 05/2014. tab
Artigo em Inglês | LILACS | ID: lil-709366

RESUMO

Objective: Evaluate side-to-side discrepancies in children with hemiplegic cerebral palsy (HCP), and investigate associations of these discrepancies with patients’ age at initiation of physical therapy, motor and cognitive function, and degree of activities and social participation. Method: We obtained eight side-to-side measurements from 24 HCP children with mean age 49.3±5.2 months. Results: Early initiation of physical therapy was associated with lower discrepancy in hand length (p=0.037). Lower foot length discrepancy was associated with lower requirement for caregiver assistance in activities related to mobility. Increased side-to-side discrepancy was associated with reduced wrist extension and increased spasticity. Discrepancy played a larger role in children with hemineglect and in those with right involvement. Conclusion: Increased discrepancy in HCP children was associated with reduced degree of activity/social participation. These results suggest an association between functional use of the extremities and limb growth. .


Objetivo: Avaliar a discrepância entre o crescimento dos lados do corpo em crianças com paralisia cerebral hemiplégica (PCH), e investigar sua associação com a idade de início do tratamento de fisioterapia, função motora e cognitiva, grau de atividades e participação social. Método: Comparamos oito medidas obtidas de 24 crianças com PCH e com média de idade de 49,3±5,2 meses. Resultados: O início precoce da fisioterapia se relacionou à menor discrepância no comprimento da mão (p=0,037). A menor discrepância no comprimento do pé se relacionou à menor necessidade de ajuda do cuidador em atividades de mobilidade. A maior discrepância esteve relacionada à menor extensão de punho e à maior espasticidade. A discrepância foi mais importante em crianças com heminegligência e com envolvimento à direita. Conclusão: Crianças com PCH com maior discrepância apresentaram menor atividade/participação social. Os resultados sugerem associação entre o uso funcional da mão e o crescimento das extremidades. .


Assuntos
Pré-Escolar , Feminino , Humanos , Masculino , Paralisia Cerebral , Cognição/fisiologia , Crescimento/fisiologia , Hemiplegia , Atividade Motora/fisiologia , Participação Social , Fatores Etários , Antropometria , Estudos Transversais , Paralisia Cerebral/patologia , Paralisia Cerebral/fisiopatologia , Paralisia Cerebral/terapia , Hemiplegia/patologia , Hemiplegia/fisiopatologia , Hemiplegia/terapia , Força Muscular , Modalidades de Fisioterapia , Valores de Referência , Fatores de Tempo , Escalas de Wechsler
18.
Rev. venez. cir. ortop. traumatol ; 43(2): 9-15, dic. 2011. ilus, graf
Artigo em Espanhol | LILACS | ID: lil-654077

RESUMO

La marcha agazapada es una de las alteraciones más frecuentes en los pacientes con parálisis cerebral infantil, y aunque es fácilmente reconocible, tampoco ha sido claramente definida. En ella, la rodilla carece de una extensión dinámica, durante la fase de apoyo, el tobillo lo podemos encontrar en flexión plantar, dorsal o neutro, y las caderas generalmente están flexionadas y frecuentemente en rotación interna y en aducción. El presente es un estudio prospectivo, realizado en el Hospital Ortopédico Infantil, empleando los datos obtenidos del departamento de historias médicas y del laboratorio de análisis de la marcha, en el cual hemos utilizado la nueva clasificación cinemática de Michael Schwartz, Director de Bioingeniería del Laboratorio de Análisis de la Marcha del Gillette Children´s Hospital, Saint Paul, Minnesota, para sujetos que presentaban excesiva flexión de rodilla al contacto inicial. Con lo cual se obtiene 5 subtipos cinemáticas de marcha agazapada, de acuerdo a los datos obtenidos en el plano sagital, y que a la vez nos permiten saber a qué nivel se encuentran las alteraciones principales de los pacientes, proporcionándonos de esta manera una guía que nos ayude en la decisión del tratamiento a emplear y al mismo tiempo nos permite realizar una valoración post-quirúrgica


Crouch gait is one of the most frequent alterations in patients with cerebral palsy, and although it is easily recognizable, has not been clearly defined. In it, the knee lacks a dynamic extension during the stance phase, the ankle can be found in plantar flexion, dorsal or neutral, and hips are usually bent and often in internal rotation and adduction. This is a prospective study conducted at the Children's Orthopedic Hospital, using data obtained from the department of medical and gait laboratory, which we have used the new classification Schwartz Michaels kinematics, Director of Bioengineering, Laboratory of Analysis of the March of Gillette Children's Hospital, Saint Paul, Minnesota, for subjects with excessive knee flexion initial contact. Thus obtained five subtypes kinematic crouched running, according to data obtained in the sagittal plane and at the same time let us know at what level are the main changes of the patients, thereby providing a guide help us in deciding the treatment to be applied at the same time allows us to perform a post-surgical evaluation


Assuntos
Humanos , Fenômenos Biomecânicos/métodos , Marcha , Paralisia Cerebral/fisiopatologia , Paralisia Cerebral/patologia , Traumatismos do Joelho/cirurgia , Traumatologia/métodos
19.
Acta odontol. venez ; 46(1): 52-55, mar. 2008. ilus
Artigo em Espanhol | LILACS | ID: lil-630140

RESUMO

La Parálisis Cerebral Infantil (PCI) constituye un grupo de desórdenes del desarrollo del movimiento y postura causando limitación. Estos son atribuidos a disturbios no progresivos que ocurren durante el desarrollo fetal o cerebro infantil. Los desórdenes motores en PCI están frecuentemente acompañados por alteraciones sensoriales, cognitivas, de comunicación, percepción, de comportamiento y/o por ataques recurrentes. Para muchos pacientes con PCI, las disfunciones orofaciales y las patologías bucodentales constituyen un severo problema de salud. El 10 por ciento de la población mundial presenta algún tipo de discapacidad, tres cuartas partes de la cual no recibe atención odontológica, por lo cual las necesidades de estos pacientes no son solventadas, complicando aún más su calidad de vida. Este estudio estableció como propósito determinar la prevalencia de patologías bucodentales en pacientes pediátricos con diagnóstico médico de PCI, dentro de las cuales se consideró la caries dental, las alteraciones del esmalte, enfermedad periodontal, candidiasis bucal, maloclusiones dentales, bruxismo, patologías asociadas a disfunciones anatómicas y funcionales como la presencia de babeo, y otras alteraciones relacionadas con la enfermedad de base, que pueden asociarse directamente con la afección del sistema estomatognático como cuadros convulsivos o autolesiones. Para obtener la información se tomó una muestra de 30 pacientes con edades comprendidas entre 3 y 12 años, atendidos en la Clínica Odontológica para Pacientes Discapacitados de la Cruz Roja en Valencia, España; utilizándose para la recolección de los datos, la entrevista estructurada a los padres y la hoja de registro de datos clínicos. Los datos agrupados se presentan graficados para determinar de forma porcentual la prevalencia de cada patología, observándose cifras alarmantes para muchos de los ítems estudiados. Se concluye comparando los resultados con los previamente descritos por la literatura


The Cerebral Palsy (CP) compromises a group of developmental disorders, resulting in motor and posture limitations. The cause for these are “non-progressive disturbances” that take place in-uterus, or during infant brain development. The motor disorders associated to CP also result in multiple development sensorial, cognitive, perception, communication, behavior alterations, as well as seizures. For most CP patients, the dental pathologies and oral-facial dysfunctions become a severe health problem. About 10 percent of the world population has some sort of this incapacity, and three quarters of these do not receive “odontological” attention, worsening considerably their quality of life. The objective of this study is to determine the presence of dental pathologies on CP-diagnosed pediatric patients. The study considered several alterations associated with the main disease, which are directly related with malfunctions of the stomatognathic system, seizures and self-injuries. The specific alterations considered included dental caries, enamel alterations, periodontal disease, oral candidiasis, dental maloclusions, bruxism, and drooling, among others. The study base was 30 patients between 3 - 12 years of age, treated in the Red Cross “Destistry Clinic for handicapped patients”, in Valencia, Spain. The methodology consisted of data collection via surveys given to the parents of the children, as well as their clinical chart. Data is presented on graphs and charts, showing percentages of presence of each pathology, resulting in alarming figures for many of the items under study. To close the analysis, results are compared with previous documented results, providing recommendations on actions that could be taken to improve the oral health of these patients


Assuntos
Criança , Boca/patologia , Serviços de Saúde Bucal , Doenças da Boca , Prevalência , Paralisia Cerebral/patologia , Odontologia
20.
Clinics ; 63(5): 601-606, 2008.
Artigo em Inglês | LILACS | ID: lil-495033

RESUMO

OBJECTIVE: To compare skin folds in the dominant and nondominant halves of the body in a group (A) of 20 individuals with cerebral palsy and spastic hemiplegia and a group (B) of 30 normal volunteers. METHOD: Body mass, height and skin folds were measured, and the percentage of body fat was estimated by adipose tissue measurement and densitometry. The mean age in group (A) was 24.6 ± 5.6 years (ranging from 16.1 to 38.1 years). The mean age in group (B) was 25.3 ± 3.8 years (ranging from 19.0 to 34.11 years). RESULTS: Statistically significant differences were observed between the dominant and nondominant halves of the body for biceps, triceps, thoracic, suprailiac, thigh and midcalf skin folds in group A; the biceps, subscapular, midaxillary, suprailiac, abdominal, thigh and midcalf skin folds in group B; and the percentage fat obtained by adipose tissue measurement in both groups. Statistically significant differences were observed for the triceps skin fold when the dominant halves of the body in groups A and B were compared. Statistically significant differences were also observed for the biceps, triceps, thigh and midcalf skin folds as well as the adipose tissue measurements between the dominant and nondominant halves of the body in the two groups. The percentage fat as estimated by densitometry was significantly correlated with the adipose tissue measurement. CONCLUSION: There were statistically significant differences between the skin folds in the dominant and nondominant halves of the body, both in group A and in group B (greater in group A). There was a statistically significant correlation in the percentage fat as estimated by densitometry and as measured by adipose tissue in groups A and B.


Assuntos
Adolescente , Adulto , Feminino , Humanos , Masculino , Adulto Jovem , Antropometria/métodos , Composição Corporal/fisiologia , Paralisia Cerebral/patologia , Lateralidade Funcional , Hemiplegia/patologia , Dobras Cutâneas , Absorciometria de Fóton , Análise de Variância , Tecido Adiposo/patologia , Estudos de Casos e Controles , Estatísticas não Paramétricas , Imagem Corporal Total , Adulto Jovem
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