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1.
Dev Med Child Neurol ; 62(3): 379-385, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-31602643

RESUMO

AIM: To evaluate whether spasticity measures are related to pain in adults with cerebral palsy (CP). METHOD: This cross-sectional study recruited individuals aged 16 to 89 years with a diagnosis of CP. Participants completed the Penn Spasm Frequency Scale (PSFS), Brief Pain Inventory (BPI), and PROMIS Pain Interference measures. The Modified Ashworth Scale (MAS) and Tardieu spasticity angles of six joints were rated and summed to composite MAS and Tardieu scores for each participant. Associations between spasticity and pain measures were evaluated. RESULTS: Forty-seven participants (27 females, 20 males) with a mean age of 35 years 7 months (range 18-77y) spanning all Gross Motor Function Classification System (GMFCS) levels were included. Twenty-six participants reported their average pain level on BPI as greater than 0 over the past week (median pain level 4.0). Median PSFS was 1.0 (range 0.0-1.0) and this correlated with average BPI and Pain Interference T scores (median 40.7; ρ=0.33 and ρ=0.31 respectively [both p=0.01]). When adjusted for pain medication use and age, MAS correlated with BPI (ρ=0.30; p=0.04). Other pain and spasticity measures, or GMFCS level, were not significantly related with pain interference or BPI rating. Age was weakly associated with BPI (slope=0.10; p<0.01). INTERPRETATION: PROMIS Pain Interference was lower than population-based norms. Patient-rated spasm frequency demonstrated better association with pain levels and interference than physician-rated MAS and Tardieu. WHAT THIS PAPER ADDS: Pain was not associated with Gross Motor Function Classification System level. Pain increased with age, as anticipated. Self-reported spasm scores were associated with increased pain in contrast to clinical examination scales. Adjusted, summed spasticity on the Modified Ashworth Scale was associated with pain scores on the Brief Pain Inventory. Although pain is experienced by adults with cerebral palsy, pain did not interfere with activities.


Assuntos
Paralisia Cerebral/complicações , Espasticidade Muscular/complicações , Dor/complicações , Adolescente , Adulto , Fatores Etários , Idoso , Paralisia Cerebral/fisiopatologia , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Espasticidade Muscular/fisiopatologia , Dor/fisiopatologia , Medição da Dor , Adulto Jovem
2.
World Neurosurg ; 134: 390-395, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31733394

RESUMO

BACKGROUND: Complex spinal deformities are a common issue in pediatric patients with an underlying neurologic diagnosis or syndrome. Management of neuromuscular scoliosis is an awesome responsibility, because these patients present with the most challenging pathologies of the deformed spine. Along with surgical correction of the underlying deformity, an intrathecal baclofen (ITB) pump is considered effective in managing the associated spasticity. CASE DESCRIPTION: We present the case of an 11-year-old female who sustained an episode of severe ischemic encephalopathy accompanied by hydrocephalus and severe spastic quadriplegia. An ITB pump was inserted to manage spasticity. Two years later, a very severe decompensated spinal curvature developed. In addition, malfunction of the pump was noted, and the decision was made to perform revision along with open hemilaminectomy at the L3-4 level. The inability of cerebrospinal fluid (CSF) to access the pump was verified intraoperatively, with the absence of CSF glow through the intrathecal space demonstrating blockage of CSF flow. CONCLUSIONS: The association of cerebral palsy and relevant disorders with the relentless progression of scoliosis is analyzed, along with the possible offending mechanisms. The efficacy of an ITB pump in controlling intractable spasticity associated with neuromuscular scoliosis is reviewed, as well as its potential to accentuate the clinical progression of neuromuscular scoliosis. Although this is an extremely infrequent situation, we must always bear in mind the possibility that malfunction of an ITB pump could be related to obstruction of CSF flow, owing to the extreme severity of the curves established during the course of, most likely untreated, neuromuscular scoliosis.


Assuntos
Baclofeno/administração & dosagem , Hipóxia Encefálica/complicações , Relaxantes Musculares Centrais/administração & dosagem , Espasticidade Muscular/complicações , Escoliose/etiologia , Criança , Falha de Equipamento , Feminino , Humanos , Bombas de Infusão Implantáveis/efeitos adversos , Injeções Espinhais , Espasticidade Muscular/tratamento farmacológico
3.
J Pediatr Orthop B ; 29(1): 22-28, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31770291

RESUMO

The objective of this study is to present the clinical and radiographic data collected from patients who were treated with a varus derotational osteotomy using Rush rod fixation and compare this to published norms of outcomes using blade plate fixation. A retrospective chart and radiograph review was conducted after identifying 44 patients with 61 hips who underwent varus derotational osteotomy with Rush rod fixation at our institution between 2006 and 2016. We identified 44 patients with 61 hips who underwent the procedure. Information from follow-up clinic visits was gathered and any complications were noted. The patients' radiographs were analyzed to measure neck-shaft angle, center-edge angle, and acetabular index. At the time of surgery, 44 patients (61 hips) also had soft tissue releases performed, 44 (61 hips) had an open reduction of the hip, and 39 (55 hips) had Dega acetabular osteotomies performed as well. The average pre-operative neck-shaft angle was measured at 163.0° (range 128-180) with average post-operative neck-shaft angles measuring 111.3° (range 85-167). The acetabular index improved from an average of 33.3° (range 16-60) to 16.4 (range 4-35). Post-operative Center-Edge Angle measured 29.7° (range 5-45). There were no infections or cases of avascular necrosis of the femoral head. We present an alternative fixation method for performing varus derotational osteotomy of the proximal femur in children with cerebral palsy using the Rush rod. In our retrospective analysis of 61 hips undergoing this procedure, we present comparable radiographic outcomes with decreased complication rates. Level of evidence: Retrospective comparative study to previously published results, Level III.


Assuntos
Paralisia Cerebral/complicações , Coxa Vara/cirurgia , Fêmur/cirurgia , Articulação do Quadril/fisiopatologia , Espasticidade Muscular/complicações , Osteotomia/instrumentação , Amplitude de Movimento Articular/fisiologia , Adolescente , Paralisia Cerebral/cirurgia , Criança , Pré-Escolar , Coxa Vara/diagnóstico , Coxa Vara/etiologia , Desenho de Equipamento , Feminino , Fêmur/diagnóstico por imagem , Seguimentos , Articulação do Quadril/diagnóstico por imagem , Humanos , Masculino , Espasticidade Muscular/fisiopatologia , Espasticidade Muscular/cirurgia , Radiografia , Estudos Retrospectivos
4.
Neurocirugía (Soc. Luso-Esp. Neurocir.) ; 30(6): 288-293, nov.-dic. 2019. graf, tab
Artigo em Espanhol | IBECS | ID: ibc-186958

RESUMO

Introducción: La espasticidad representa un problema médico cuya incidencia está aumentando debido a enfermedades como parálisis cerebral, ictus, esclerosis múltiple, traumatismos o encefalopatías, afectando tanto a adultos como a niños. Los tratamientos incluyen rehabilitación, farmacoterapia y cirugía, entre las cuales destacamos las bombas de baclofeno intratecal. Material y métodos: Seleccionamos a los pacientes portadores de bomba de baclofeno intratecal implantada en el Hospital Clínico de Santiago de Compostela entre 2005-2018 y analizamos retrospectivamente los resultados mediante escalas de valoración de espasticidad, como la de Ashworth, así como las complicaciones observadas. Resultados: Se implantaron bombas de baclofeno a 17 pacientes, obteniendo una mejoría de 2 puntos en la escala de Ashworth en el 88,2% y de 1 punto en la escala de Penn en el 94%. Se observaron complicaciones en 3 pacientes. Conclusiones: El tratamiento con baclofeno intratecal es una técnica sencilla con resultados muy positivos para mejorar la calidad de vida de pacientes con espasticidad


Introduction: Spasticity represents a medical problem whose incidence is increasing during the last years due to pathologies such as cerebral palsy, stroke, multiple sclerosis, trauma or encephalopathy, affecting both adults and children. The treatments include rehabilitation, pharmacotherapy and surgery, among which we highlight intrathecal baclofen infusion devices. Material and methods: Intrathecal baclofen devices implanted patients in Clinical Hospital of Santiago de Compostela from 2005 to 2018 were selected for retrospective analysis using assessment of spasticity scales, such as Ashworth scale. Complications are described. Results: Surgery was performed in 17 patients for baclofen pump implant, achieving an improvement of 2 points on the Ashworth Scale in 88,2% of the patients and of 1 point on the Penn Scale in 94%. Complications were seen in 3 patients. Conclusions: Intrathecal baclofen is a simple technique with good results for improving the quality of life of patients with spasticity


Assuntos
Humanos , Masculino , Feminino , Adulto , Baclofeno/administração & dosagem , Espasticidade Muscular/tratamento farmacológico , Estudos Retrospectivos , Espasticidade Muscular/complicações , Esclerose Múltipla/etiologia , Neurofisiologia
5.
PLoS One ; 14(7): e0219670, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31361750

RESUMO

BACKGROUND: Nabiximols (THC/CBD Oromucosal Spray, Sativex) is used as an add-on therapy to treat moderate to severe spasticity of Multiple Sclerosis (MS). OBJECTIVES: To examine the impact of physiotherapy (PT) programs on effectiveness and persistence of nabiximols treatment in people with MS-related spasticity. METHODS: This is an observational multicenter study with a follow-up period of 12 weeks, conducted in routine care settings in Italy. Patients with moderate to severe MS-related spasticity who started nabiximols were included. Spasticity was evaluated by the patient-rated 0-10 numerical rating scale (NRS). Clinical data were collected at baseline (T0), 4 weeks (T1) and 12 weeks (T2) months after enrollment. RESULTS: A total of 297 MS patients were selected, 290 completed the 3 months follow-up period. Mean NRS scores were 7.6 ± 1.1 at T0, 5.8 ± 1.4 at T1 and 5.5 ± 1.5 at T2. At T1, 77% of patients reached ≥20% improvement (initial response, IR); 22% reached ≥30% improvement (clinically relevant response, CRR). At T1, patients undergoing PT had a higher probability to reach CRR (Odds Ratio = 2.6 95% CI 1.3-5.6, p = 0.01). Nabiximols was discontinued in 30/290 (10.3%) patients at T1 (early discontinuers) and in 71/290 (24.5%) patients at T2 (late discontinuers). The probability of being late discontinuers was reduced in patients undergoing PT (Hazard Ratio = 0.41; 95% CI 0.23-0.69, p = 0.001). CONCLUSIONS: Our real-life study confirms nabiximols' effectiveness in MS-related spasticity and suggests that the association of a PT program may improve overall response and persistence to nabiximols treatment.


Assuntos
Canabidiol/uso terapêutico , Dronabinol/uso terapêutico , Esclerose Múltipla/terapia , Espasticidade Muscular/terapia , Sprays Orais , Modalidades de Fisioterapia , Terapia Combinada , Combinação de Medicamentos , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Esclerose Múltipla/complicações , Esclerose Múltipla/tratamento farmacológico , Espasticidade Muscular/complicações , Espasticidade Muscular/tratamento farmacológico , Suspensão de Tratamento
6.
Clin Rehabil ; 33(11): 1819-1830, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31266351

RESUMO

OBJECTIVE: To establish the prevalence of unmet need for spasticity management in care home residents in two counties of the United Kingdom. DESIGN: Cross-sectional observational study with a six-month follow-up arm for participants with identified unmet needs. SETTING: 22 care homes in Derbyshire and Nottinghamshire. SUBJECTS: 60 care home residents with upper motor neuron syndrome-related spasticity. INTERVENTIONS: No intervention. When unmet needs around spasticity management were identified, the participant's general practitioner was advised of these in writing. MAIN MEASURES: Resistance to Passive Movement Scale to assess spasticity; recording of (a) the presence of factors which may aggravate spasticity, (b) potential complications of spasticity, (c) spasticity-related needs and (d) current interventions to manage spasticity. Two assessors judged the presence or absence of needs for spasticity management and whether these needs were met by current care. RESULTS: Out of 60 participants, 14 had no spasticity-related needs; 46 had spasticity-related needs; 11 had needs which were being met by current care and 35 participants had spasticity-related needs at baseline which were not being met by their current care. These were most frequently related to the risk of contracture development or problems with skin hygiene or integrity in the upper limb. In total, 6 participants had one or more pressure sores and 35 participants had one or more established joint contractures. A total of 31 participants were available for follow-up. Informing general practitioners of unmet needs resulted in no change to spasticity management in 23/31 cases. CONCLUSION: Care home residents in this study had high levels of unmet need for spasticity management.


Assuntos
Necessidades e Demandas de Serviços de Saúde , Doença dos Neurônios Motores/complicações , Espasticidade Muscular/complicações , Idoso , Idoso de 80 Anos ou mais , Contratura/etiologia , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Lesão por Pressão/etiologia , Prevalência , Instituições Residenciais , Higiene da Pele , Reino Unido
7.
J Pediatr Orthop ; 39(Issue 6, Supplement 1 Suppl 1): S33-S37, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31169645

RESUMO

BACKGROUND: Hip displacement in children with cerebral palsy is common and related to a child's gross motor function. Progressive lateral hip displacement can result in severe pain, impaired function and quality of life. The purpose of this paper is to review the literature to identify the natural history of untreated spastic hip displacement in children with cerebral palsy. METHODS: A search of the literature was carried out using PubMed to identify papers describing the natural history of spastic hip displacement in children with cerebral palsy. Population-based studies, large retrospective cohort series, and randomized trials were included when available; expert opinion and case series were excluded. RESULTS: A total of 79 articles were reviewed. Articles were then subdivided into 4 main categories: epidemiology, pathophysiology, outcome, and prevention. The prevalence of hip displacement as defined as a migration percentage >30% was found to be 33% in pooled population-based studies. The risk of hip displacement was found to be linked to a child's gross motor function. Hip surveillance programs have been shown to be sustainable and capable of preventing hip dislocation. The majority of children with hip dislocation do develop pain, decreased function, and impaired health-related quality of life (HRQOL). CONCLUSIONS: Children with cerebral palsy are at risk of progressive lateral hip displacement proportional to their gross motor function. Untreated progressive lateral hip displacement has been shown to negatively impact a child's HRQOL and hip surveillance can decrease the incidence of hip dislocation.


Assuntos
Paralisia Cerebral/complicações , Paralisia Cerebral/fisiopatologia , Luxação do Quadril/etiologia , Dor Musculoesquelética/etiologia , Adolescente , Criança , Pré-Escolar , Luxação do Quadril/epidemiologia , Luxação do Quadril/fisiopatologia , Luxação do Quadril/prevenção & controle , Humanos , Incidência , Espasticidade Muscular/complicações , Prevalência , Qualidade de Vida
8.
Gene ; 704: 68-73, 2019 Jul 01.
Artigo em Inglês | MEDLINE | ID: mdl-30986448

RESUMO

AIM: The purpose of the present study is to evaluate and understand the association of global and MTHFR gene specific methylation in preeclampsia and recurrent miscarriages in light of MTHFR C677T polymorphism. METHODS: The subjects comprised of recurrent miscarriage cases, their gestation matched controls, preeclampsia cases and matched controls. A set of women at full term were also recruited. Fasting blood sample (~5 ml) was drawn from all the participants followed by DNA extraction, global DNA methylation and MTHFR gene specific methylation. MTHFR C677T polymorphism was analysed by PCR followed by RFLP. RESULTS HIGHER: Global DNA methylation at maternal front (p = 0.04) and hypomethylation of MTHFR gene at fetal front (p = 0.001) might be a characteristic of preeclampsia. Recurrent miscarriage cases were having significantly (p = 0.002) hyper MTHFR gene specific methylation as compared to controls. Women carrying CT genotype were found to be having significantly (p = 0.001) higher global DNA methylation in PE cases and MTHFR gene specific methylation (p = 0.005) in RM cases. Intergenerational analysis revealed similar patterns of global DNA methylation and MTHFR gene specific methylation among both PE and RM cases at maternal and fetal fronts. CONCLUSION: The study highlights the importance of global DNA methylation in Preeclampsia and MTHFR gene specific methylation in recurrent miscarriages. MTHFR C677T gene polymorphism in association with global and gene specific methylation seem to play a pivotal role in PE and RM respectively.


Assuntos
Aborto Habitual/genética , Metilação de DNA , Metilenotetra-Hidrofolato Redutase (NADPH2)/genética , Pré-Eclâmpsia/genética , Adulto , Estudos de Casos e Controles , Feminino , Estudos de Associação Genética , Predisposição Genética para Doença , Homocistinúria/complicações , Homocistinúria/genética , Humanos , Índia , Metilenotetra-Hidrofolato Redutase (NADPH2)/deficiência , Espasticidade Muscular/complicações , Espasticidade Muscular/genética , Polimorfismo de Fragmento de Restrição , Polimorfismo de Nucleotídeo Único , Pré-Eclâmpsia/diagnóstico , Gravidez , Segundo Trimestre da Gravidez/genética , Terceiro Trimestre da Gravidez/genética , Transtornos Psicóticos/complicações , Transtornos Psicóticos/genética
9.
BMJ Case Rep ; 12(4)2019 Apr 23.
Artigo em Inglês | MEDLINE | ID: mdl-31015239

RESUMO

A 55-year-old ambulatory woman with hemiplegia and varus foot deformity had several problems in her daily life, including load pain and stance instability in the affected foot, easy fatigue of the non-paralysed leg, low back pain, neck stiffness and rapid shoe-rubber wear on the deformed side. We began repeated focal blockades using botulinum toxin to the tibialis posterior muscle to control varus spasticity. Distant influences presenting in the whole body were relieved soon after the first blockade, and shoe wear also stopped. Although, neither the deformed appearance nor foot contact pattern on walking changed in the initial period after beginning the blockade, the foot contact pattern revealed gradual improvement over several years. Generally, surgical correction is indicated for the treatment of deformed feet. The present case suggests that, in case of varus-deformed foot with some spastic elements, trial of focal blockade for varus spasticity may be worthwhile.


Assuntos
Toxinas Botulínicas/uso terapêutico , Pé/patologia , Espasticidade Muscular/tratamento farmacológico , Inibidores da Liberação da Acetilcolina/uso terapêutico , Toxinas Botulínicas/administração & dosagem , Diagnóstico Diferencial , Feminino , Deformidades Adquiridas do Pé/diagnóstico , Deformidades Adquiridas do Pé/etiologia , Hemiplegia/complicações , Humanos , Pessoa de Meia-Idade , Espasticidade Muscular/complicações , Resultado do Tratamento
10.
Int Wound J ; 16(3): 847-851, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30895715

RESUMO

The aim of this study was to identify and characterise the association between the prevalence of pressure ulcers, spasticity levels, and advanced dementia in disable elderly patients. Data were obtained from the patient medical files. Patients were hospitalised in the geriatric skilled nursing department. A total of 40 frail elderly patients, bedbound and suffering from advanced chronic diseases, advanced dementia, and high-grade pressure ulcers, were examined. Pressure ulcer grades and spasticity in advanced dementia versus non-dementia patients were evaluated. Logistic regression indicated that only advanced dementia and spasticity were significantly associated with the development of pressure ulcers versus those without dementia or without spasticity. Patients with advanced dementia displayed a significantly higher prevalence of severe spasticity. In multivariate logistic regression analyses, spasticity was significantly associated with pressure ulcers. The strong association of spasticity with the onset of pressure ulcers in advanced dementia should encourage clinicians to implement preventative measures to delay the onset of pressure ulcers.


Assuntos
Demência/complicações , Idoso Fragilizado/estatística & dados numéricos , Espasticidade Muscular/complicações , Lesão por Pressão/etiologia , Idoso , Idoso de 80 Anos ou mais , Estudos Transversais , Feminino , Humanos , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Razão de Chances , Prevalência , Fatores de Risco
13.
J Neurol ; 266(5): 1260-1266, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30840144

RESUMO

We aimed to provide proof-of-principle evidence that intensive home-based speech treatment can improve dysarthria in complex multisystemic degenerative ataxias, exemplified by autosomal recessive spastic ataxia Charlevoix-Saguenay (ARSACS). Feasibility and piloting efficacy of speech training specifically tailored to cerebellar dysarthria was examined through a 4-week program in seven patients with rater-blinded assessment of intelligibility (primary outcome) and naturalness and acoustic measures of speech (secondary outcomes) performed 4 weeks before, immediately prior to, and directly after training (intraindividual control design). Speech intelligibility and naturalness improved post treatment. This provides piloting evidence that ataxia-tailored speech treatment might be effective in degenerative cerebellar disease.


Assuntos
Disartria/etiologia , Disartria/reabilitação , Espasticidade Muscular/complicações , Fonoterapia/métodos , Ataxias Espinocerebelares/congênito , Retroalimentação Sensorial/fisiologia , Feminino , Humanos , Masculino , Avaliação de Resultados em Cuidados de Saúde , Projetos Piloto , Ataxias Espinocerebelares/complicações , Estatísticas não Paramétricas
14.
Arch Phys Med Rehabil ; 100(9): 1703-1725, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-30796921

RESUMO

OBJECTIVE: To provide a comprehensive overview of reported effects and scientific robustness of botulinum toxin (BoNT) treatment regarding the main clinical goals related to poststroke upper limb spasticity, using the International Classification of Functioning, Disability and Health. DATA SOURCES: Embase, PubMed, Wiley/Cochrane Library, and Ebsco/CINAHL were searched from inception up to May 16, 2018. STUDY SELECTION: We included randomized controlled trials comparing upper limb BoNT injections with a control intervention in patients with a history of stroke. A total of 1212 unique records were screened by 2 independent reviewers. Forty trials were identified, including 2718 patients with history of stroke. DATA EXTRACTION: Outcome data were pooled according to assessment timing (ie, 4-8wk and 12wk after injection), and categorized into 6 main clinical goals (ie, spasticity-related pain, involuntary movements, passive joint motion, care ability, arm and hand use, and standing and walking performance). Sensitivity analyses were performed for the influence of study and intervention characteristics, involvement of pharmaceutical industry, and publication bias. DATA SYNTHESIS: Robust evidence is shown for the effectiveness of BoNT in reducing resistance to passive movement, as measured with the (Modified) Ashworth Score, and improving self-care ability for the affected hand and arm after intervention (P<.005) and at follow-up (P<.005). In addition, robust evidence is shown for the absence of effect on arm-hand capacity at follow-up. BoNT was found to significantly reduce involuntary movements, spasticity-related pain, and caregiver burden, and improve passive range of motion, while no evidence was found for arm and hand use after intervention. CONCLUSIONS: In view of the robustness of current evidence, no further trials are needed to investigate BoNT for its favorable effects on resistance to passive movement of the spastic wrist and fingers, and on self-care. No trials are needed to further confirm the lack of effects of BoNT on arm-hand capacity, whereas additional trials are needed to establish the suggested favorable effects of BoNT on other body functions, which may result in clinically meaningful outcomes at activity and participation levels.


Assuntos
Toxinas Botulínicas/uso terapêutico , Espasticidade Muscular/tratamento farmacológico , Espasticidade Muscular/etiologia , Neurotoxinas/uso terapêutico , Acidente Vascular Cerebral/complicações , Braço , Cuidadores , Mãos , Humanos , Movimento , Espasticidade Muscular/complicações , Dor Musculoesquelética/tratamento farmacológico , Dor Musculoesquelética/etiologia , Amplitude de Movimento Articular , Autocuidado
15.
NeuroRehabilitation ; 44(1): 157-159, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30714982

RESUMO

BACKGROUND: Spasticity is a frequent symptom after traumatic brain injury. OBJECTIVE: To assess the effects of different interventions used for muscle spasticity after TBI. METHODS: To summarize a rehabilitation perspective "Cochrane Review" conducted by Synnot et al.RESULTS:Nine studies were involved for the Cochrane review. Poor report on the results of the studies that tested the effectiveness of interventions in spasticity are responsible for the low quality of the evidence. Most of the studies reported results in terms of decreasing spasticity and limiting effects in terms of how the decrease spasticity and no reports on the beneficial effects in terms of improving activities and participation. CONCLUSIONS: High quality adequately powered trials in patients with TBI should be encouraged.


Assuntos
Lesões Encefálicas Traumáticas/reabilitação , Espasticidade Muscular/reabilitação , Reabilitação Neurológica/métodos , Lesões Encefálicas Traumáticas/complicações , Lesões Encefálicas Traumáticas/diagnóstico , Ensaios Clínicos como Assunto/métodos , Humanos , Espasticidade Muscular/complicações , Espasticidade Muscular/diagnóstico
16.
Mol Neurobiol ; 56(6): 4175-4191, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30288696

RESUMO

Folate is an important B vitamin required for methylation reactions, nucleotide and neurotransmitter synthesis, and maintenance of homocysteine at nontoxic levels. Its metabolism is tightly linked to that of choline, a precursor to acetylcholine and membrane phospholipids. Low folate intake and genetic variants in folate metabolism, such as the methylenetetrahydrofolate reductase (MTHFR) 677 C>T polymorphism, have been suggested to impact brain function and increase the risk for cognitive decline and late-onset Alzheimer's disease. Our study aimed to assess the impact of genetic and nutritional disturbances in folate metabolism, and their potential interaction, on features of cognitive decline and brain biochemistry in a mouse model. Wild-type and Mthfr+/- mice, a model for the MTHFR 677 C>T polymorphism, were fed control or folate-deficient diets from weaning until 8 and 10 months of age. We observed short-term memory impairment measured by the novel object paradigm, altered transcriptional levels of synaptic markers and epigenetic enzymes, as well as impaired choline metabolism due to the Mthfr+/- genotype in cortex or hippocampus. We also detected changes in mRNA levels of Presenillin-1, neurotrophic factors, one-carbon metabolic and epigenetic enzymes, as well as reduced levels of S-adenosylmethionine and acetylcholine, due to the folate-deficient diet. These findings shed further insights into the mechanisms by which genetic and dietary folate metabolic disturbances increase the risk for cognitive decline and suggest that these mechanisms are distinct.


Assuntos
Envelhecimento/patologia , Encéfalo/patologia , Dieta , Ácido Fólico/metabolismo , Homocistinúria/complicações , Metilenotetra-Hidrofolato Redutase (NADPH2)/deficiência , Espasticidade Muscular/complicações , Peptídeos beta-Amiloides/metabolismo , Animais , Ansiedade/complicações , Ansiedade/fisiopatologia , Encéfalo/fisiopatologia , Sobrevivência Celular , Córtex Cerebral/patologia , Córtex Cerebral/fisiopatologia , Colina/metabolismo , Ilhas de CpG/genética , Metilação de DNA/genética , Epigênese Genética , Ácido Glutâmico/metabolismo , Homocistinúria/fisiopatologia , Fígado/metabolismo , Masculino , Transtornos da Memória/complicações , Transtornos da Memória/fisiopatologia , Memória de Curto Prazo , Metilação , Camundongos Endogâmicos BALB C , Espasticidade Muscular/fisiopatologia , Fatores de Crescimento Neural/metabolismo , Neurônios/patologia , Fosfolipídeos/metabolismo , Transtornos Psicóticos/complicações , Transtornos Psicóticos/fisiopatologia , RNA Mensageiro/genética , RNA Mensageiro/metabolismo , S-Adenosilmetionina/metabolismo , Transmissão Sináptica
17.
Brain Dev ; 41(3): 276-279, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30482549

RESUMO

BACKGROUND: The human GATA zinc finger domain containing 2B (GATAD2B) encodes a subunit of the MeCP1-Mi-2/nucleosome remodeling and deacetylase complex, which is involved in chromatin modification and transcription. Recently, patients with severe intellectual disabilities and characteristic features associated with GATAD2B mutations have been identified. CASE REPORT: The patient was a 4-year-old male with dysmorphic features, including frontal bossing, hypertelorism, epicanthal folds, down-slanting palpebral fissures, a flat nasal bridge, a high arched palate, and micrognathia. He spoke no meaningful words and exhibited severe intellectual disability. Hypermetropic astigmatism and mild spasticity of the lower extremities were noted. Whole-exome sequencing revealed a de novo missense mutation in GATAD2B (NM_020699:exon4:c.502C>T; p.(Glu168∗)). CONCLUSION: We report a novel GATAD2B mutation in a boy exhibiting bilateral leg spasticity and white matter abnormalities on brain magnetic resonance imaging.


Assuntos
Deficiências do Desenvolvimento/genética , Fatores de Transcrição GATA/genética , Deficiência Intelectual/genética , Mutação/genética , Encéfalo/diagnóstico por imagem , Pré-Escolar , Deficiências do Desenvolvimento/complicações , Deficiências do Desenvolvimento/diagnóstico por imagem , Humanos , Deficiência Intelectual/complicações , Deficiência Intelectual/diagnóstico por imagem , Imagem por Ressonância Magnética , Masculino , Espasticidade Muscular/complicações , Espasticidade Muscular/genética
18.
Mult Scler Relat Disord ; 27: 383-390, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30502644

RESUMO

BACKGROUND: Studies suggest cannabis may improve symptoms like pain and muscle spasticity in patients with multiple sclerosis (PwMS). Despite cannabis' new-found legality and availability, few studies have explored the profile of PwMS cannabis users and characteristics of their use, particularly in a state where cannabis is legal both for recreational and medicinal use. The purpose of the current study was to evaluate cannabis use among PwMS at a large academic multiple sclerosis (MS) clinic, specifically: (1) prevalence, (2) products used (e.g., cannabidiol vs Δ9-tetrahydocannabinol), (3) symptom treatment, and (4) patient characteristics. METHODS: PwMS completed questions assessing personal opinions about cannabis use, characteristics of cannabis use, MS history, and sociodemographic details, as well as the self-reported disability-Patient Determined Disease Steps (PDDS), overall quality of life-the Patient Reported Outcome Measure Information System (PROMIS-10), and cognition-the Neuro-QoL ACGC v1.0 measures. RESULTS: Thirty-eight percent (n = 96) of PwMS were current Cannabis users (CUs). Although there were no sociodemographic or clinical differences (p ≤ 0.05) between CUs and Non-Cannabis users (NUs), CUs had significantly higher median disability compared to NUs (PDDS = 2 vs. 1; p = 0.02). Among CUs, 57% categorized their use as strictly medicinal. CUs reported using cannabis most often for pain and insomnia/poor sleep and experienced greater than 60% benefit/relief from cannabis use. Over 90% of respondents desire more research on cannabis for MS, and 74% indicated they would consider using cannabis for their MS. CONCLUSION: As cannabis legalization has impacted the variety of cannabis products available, there appears to be growing numbers of PwMS using cannabis, with this study's CUs reporting use of highly efficacious products with minimal side-effects.


Assuntos
Fumar Maconha/psicologia , Maconha Medicinal/uso terapêutico , Esclerose Múltipla/tratamento farmacológico , Adulto , Feminino , Humanos , Masculino , Fumar Maconha/efeitos adversos , Maconha Medicinal/efeitos adversos , Pessoa de Meia-Idade , Esclerose Múltipla/complicações , Espasticidade Muscular/complicações , Dor/complicações , Qualidade de Vida , Fatores de Risco
19.
Dev Med Child Neurol ; 61(1): 69-74, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-30051908

RESUMO

AIM: People with cerebral palsy (CP) are often unable to express pain owing to cognitive or speech impairments. Reports that rely on observation can be inaccurate, because behaviours such as grimacing, common in people with spastic CP, resemble pain expressions. We examined preliminary validity and reliability of the revised Face, Legs, Activity, Cry, and Consolability (r-FLACC) scale in people with spastic CP. METHOD: Forty-eight young people and adults (35 females, 13 males; mean [SD] age 29y 2mo [13y]) were video-recorded during a standard examination, rating their pain (0-10) afterwards. Two raters completed the r-FLACC using the video recordings. Interrater reliability was assessed with an unconditional cross-classified random-effects model and item response theory approach; Pearson correlations measured agreement between raters and participants. RESULTS: Mean (SD) participant (n=48) pain scores were 2.48 (2.5) and mean (SD) r-FLACC scores were 1.46 (1.68). There was moderate agreement between raters (intraclass coefficient 0.41 and 0.57 respectively) but low agreement between participants and raters (r=0.26). There were no significant effects for raters (lay observers, nurses, physicians, and inexperienced raters). INTERPRETATION: Results provide mixed support for the interrater reliability of the r-FLACC in people with spastic CP. WHAT THIS PAPER ADDS: The revised Face, Legs, Activity, Cry, and Consolability (r-FLACC) scale can be reliably used by experts and lay raters for people with spastic cerebral palsy (CP). Support is mixed for interrater reliability of the r-FLACC scale used with people with spastic CP.


Assuntos
Paralisia Cerebral , Medição da Dor , Dor/diagnóstico , Adolescente , Adulto , Paralisia Cerebral/complicações , Paralisia Cerebral/diagnóstico , Competência Clínica , Expressão Facial , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Atividade Motora , Espasticidade Muscular/complicações , Espasticidade Muscular/diagnóstico , Variações Dependentes do Observador , Dor/complicações , Exame Físico , Postura , Reprodutibilidade dos Testes , Autorrelato , Gravação em Vídeo , Adulto Jovem
20.
Orthop Traumatol Surg Res ; 105(1S): S133-S141, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30056240

RESUMO

The hip is the joint most exposed to orthopaedic complications in cerebral palsy (CP), which is the main cause of spasticity in paediatric patients. The initial immaturity of the hip allows the forces applied by the spastic and retracted muscles to displace the femoral head, eventually causing it to dislocate. The risk of hip dislocation increases with the severity and extent of CP, exceeding 70% in the most severe cases. Hip dislocation causes pain in up to 30% of cases, carries a risk of orthopaedic and cutaneous complications and hinders patient installation and nursing care. These adverse outcomes warrant routine screening, which has been proven effective in lessening the frequency and severity of hip displacement. Preventive techniques including physical therapy, orthoses and treatments to alleviate spasticity are strongly recommended in every case. The beneficial effects of treating spasticity, if needed via neurosurgical procedures, have been convincingly established. Orthopaedic surgery is required when prevention fails. Soft-tissue release is designed to correct the asymmetry in the forces applied by the muscles. Femoral osteotomy creates the possibility for spontaneous correction of secondary acetabular dysplasia. Progress has been made in standardising the use of multilevel surgery involving the soft tissues, femur and pelvis, which is often effective in correcting the morphological abnormalities and stabilising the joint. When hip pain or alterations are severe, hip resection or total hip arthroplasty are highly effective in alleviating the pain and improving patient comfort. The spastic hip is a complex condition in which currently available screening protocols and treatment strategies have been proven effective in benefitting patient outcomes.


Assuntos
Paralisia Cerebral/complicações , Luxação do Quadril/etiologia , Luxação do Quadril/terapia , Espasticidade Muscular/complicações , Adolescente , Artrodese , Criança , Tratamento Conservador , Fêmur/cirurgia , Luxação do Quadril/diagnóstico , Articulação do Quadril/diagnóstico por imagem , Articulação do Quadril/cirurgia , Humanos , Procedimentos Neurocirúrgicos , Osteotomia , Dor/etiologia , Exame Físico , Qualidade de Vida , Radiografia
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