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1.
Adv Exp Med Biol ; 1190: 333-343, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31760654

RESUMO

Chronic inflammatory demyelinating polyneuropathy (CIDP) is immune-mediated neuropathy defined by clinical progression for more than 2 months, and electrodiagnostic evidence of peripheral nerve demyelination. However, there are several clinical phenotypes, classified into "typical CIDP," and "atypical CIDP" such as "multifocal acquired demyelinating sensory and motor neuropathy (MADSAM)." Typical CIDP is a most common form, characterized by symmetric proximal and distal muscle weakness and motor-dominant manifestation. In typical CIDP, demyelination predominantly affects the distal nerve terminals and nerve roots, where the blood-nerve barrier is anatomically deficient. These features suggest antibody-mediated demyelination in typical CIDP. By contrast, MADSAM is characterized by multifocal demyelination in the nerve trunks, and such distribution of lesions results in multiple mononeuropathy or asymmetric polyneuropathy. In MADSAM, cellular immunity is likely to be involved in the breakdown of the blood-nerve barrier at the site of conduction block. Clinical features are probably determined by the distribution of demyelinative lesions and reflect the different immunopathogenesis of each CIDP subtype that would require different treatment strategy.


Assuntos
Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/fisiopatologia , Humanos , Imunidade Celular , Debilidade Muscular/fisiopatologia , Condução Nervosa , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/imunologia
3.
Muscle Nerve ; 60(6): 679-686, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31566774

RESUMO

INTRODUCTION: The purpose of this study was to comprehensively evaluate respiratory muscle function in adults with facioscapulohumeral muscular dystrophy (FSHD). METHODS: Fourteen patients with FSHD (9 men, 53 ± 16 years of age) and 14 matched controls underwent spirometry, diaphragm ultrasound, and measurement of twitch gastric and transdiaphragmatic pressures (twPgas and twPdi; n = 10) after magnetic stimulation of the lower thoracic nerve roots and the phrenic nerves. The latter was combined with recording of diaphragm compound muscle action potentials (CMAPs; n = 14). RESULTS: The following parameters were significantly lower in patients vs controls: forced vital capacity (FVC); maximum inspiratory and expiratory pressure; peak cough flow; diaphragm excursion amplitude; and thickening ratio on ultrasound, twPdi (11 ± 5 vs 20 ± 6 cmH2 O) and twPgas (7 ± 3 vs 25 ± 20 cmH2 O). Diaphragm CMAP showed no group differences. FVC correlated inversely with the clinical severity scale score (r = -0.63, P = .02). DISCUSSION: In FSHD, respiratory muscle weakness involves both the diaphragm and the expiratory abdominal muscles.


Assuntos
Diafragma/fisiopatologia , Debilidade Muscular/fisiopatologia , Distrofia Muscular Facioescapuloumeral/fisiopatologia , Músculos Respiratórios/fisiopatologia , Potenciais de Ação/fisiologia , Adulto , Idoso , Estudos de Casos e Controles , Estudos Transversais , Diafragma/diagnóstico por imagem , Feminino , Humanos , Masculino , Pressões Respiratórias Máximas , Pessoa de Meia-Idade , Debilidade Muscular/etiologia , Distrofia Muscular Facioescapuloumeral/complicações , Condução Nervosa , Nervo Frênico , Raízes Nervosas Espinhais , Espirometria , Vértebras Torácicas , Ultrassonografia , Capacidade Vital
4.
Muscle Nerve ; 60(6): 724-731, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31531865

RESUMO

INTRODUCTION: There is currently little evidence regarding oculopharyngeal muscular dystrophy (OPMD) disease burden reported by patients. In this study we aim to elicit direct patient input regarding OPMD disease burden. METHODS: We conducted semistructured interviews with 25 participants with genetically confirmed OPMD and a wide range of disease duration (15 ± 8 years). Using the Framework Technique, themes and categories were then extracted. RESULTS: Analyses revealed 7 themes (physical impact, mental impact, social impact, perception of progression, treatment perceptions, coping strategies, and access to disease information), encompassing 27 categories of OPMD disease burden. The most frequent categories were related to dysphagia, coping strategies for dysphagia, and impaired mobility. DISCUSSION: This study demonstrates the importance of considering, when providing clinical care, the broad range of coping strategies patients use to deal with OPMD symptoms, especially dysphagia, to properly assess limitations and monitor real disease progression.


Assuntos
Distrofia Muscular Oculofaríngea/fisiopatologia , Distrofia Muscular Oculofaríngea/psicologia , Acesso à Informação , Adaptação Psicológica , Adolescente , Adulto , Idoso , Atitude Frente a Saúde , Transtornos Dismórficos Corporais , Criança , Efeitos Psicossociais da Doença , Transtornos de Deglutição/etiologia , Transtornos de Deglutição/fisiopatologia , Progressão da Doença , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Limitação da Mobilidade , Debilidade Muscular/etiologia , Debilidade Muscular/fisiopatologia , Distrofia Muscular Oculofaríngea/complicações , Distrofia Muscular Oculofaríngea/terapia , Dor/etiologia , Dor/fisiopatologia , Medidas de Resultados Relatados pelo Paciente , Pesquisa Qualitativa , Participação Social , Distúrbios da Voz/etiologia , Distúrbios da Voz/fisiopatologia , Desempenho Profissional , Adulto Jovem
5.
Muscle Nerve ; 60(6): 658-661, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31531870

RESUMO

Repetitive nerve stimulation (RNS) of the median nerve is rarely studied in myasthenia gravis (MG). We performed a retrospective analysis of RNS studies performed on 448 patients at our center between 2010 and 2016. Among 95 patients with MG, an abnormal decrement of the compound muscle action potential amplitude was seen in 40.7% of median, 38.4% of spinal accessory, and 57.1% of facial nerve RNS studies. Median nerve RNS was abnormal in 10 patients with normal spinal accessory and 5 patients with normal facial nerve RNS. MG patients with abnormal median nerve RNS were more likely to be seropositive and have limb weakness. No differences were observed in the incidence of abnormal median nerve RNS in patients with and without a median neuropathy at the wrist. Median nerve RNS is easy to perform, reproducible, and should be considered in the electrodiagnostic evaluation of MG.


Assuntos
Nervo Mediano/fisiopatologia , Debilidade Muscular/fisiopatologia , Miastenia Gravis/diagnóstico , Adulto , Idoso , Estimulação Elétrica , Eletrodiagnóstico , Eletromiografia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Miastenia Gravis/fisiopatologia , Sensibilidade e Especificidade
6.
J Foot Ankle Res ; 12: 41, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31406508

RESUMO

Background: To reduce gait problems in individuals with non-spastic calf muscle weakness, spring-like ankle-foot orthoses (AFOs) are often applied, but they are not individually optimized to treatment outcome. The aim of this proof-of-concept study was to evaluate the effects of modifying the stiffness for two spring-like AFO types with shoes-only as reference on gait outcomes in three individuals with calf muscle weakness due to polio. Methods: We assessed 3D gait biomechanics, walking speed and walking energy cost for shoes-only and five stiffness conditions of a dorsal-leaf-spring AFO and a spring-hinged AFO. Outcomes were compared between stiffness conditions in the two AFOs and three subjects. Results: Maximum ankle dorsiflexion angle decreased with increasing stiffness in both AFOs (up to 6-8°) and all subjects. Maximum knee extension angle changed little between stiffness conditions, however different responses between the AFOs and subjects were observed compared to shoes-only. Walking speed remained unchanged across conditions. For walking energy cost, we found fairly large differences across stiffness conditions with both AFOs and between subjects (range 3-15%). Conclusions: Modifying AFO stiffness in individuals with non-spastic calf muscle weakness resulted in substantial differences in ankle biomechanics and walking energy cost with no effect on speed. Our results provide proof-of-concept that individually optimizing AFO stiffness can clinically beneficially improve gait performance.


Assuntos
Desenho de Equipamento , Órtoses do Pé , Transtornos Neurológicos da Marcha/terapia , Tono Muscular , Debilidade Muscular/terapia , Adulto , Tornozelo/fisiopatologia , Fenômenos Biomecânicos , Feminino , Pé/fisiopatologia , Marcha/fisiologia , Transtornos Neurológicos da Marcha/etiologia , Transtornos Neurológicos da Marcha/fisiopatologia , Humanos , Joelho , Masculino , Debilidade Muscular/complicações , Debilidade Muscular/fisiopatologia , Estudo de Prova de Conceito , Amplitude de Movimento Articular , Sapatos , Resultado do Tratamento , Caminhada/fisiologia
7.
BMJ Case Rep ; 12(7)2019 Jul 27.
Artigo em Inglês | MEDLINE | ID: mdl-31352397

RESUMO

Muscular symptoms in hypothyroidism are common, including myalgia, fatigue and cramps; however, a significantly raised creatine kinase and muscle weakness are rare. Differential diagnosis of patients presenting with muscle weakness and a raised creatine kinase is wide, and hypothyroidism is rarely considered. We report this case of a 30-year-old female presenting with proximal muscle weakness as her primary symptom, hypothyroid symptoms of 3-month duration and a significantly raised creatine kinase. After ruling out other causes of a raised creatine kinase, thyroxine replacement was commenced, which led to complete resolution of her proximal weakness, myalgia and normalisation of creatine kinase level. This case illustrates severe proximal myopathy can be secondary to hypothyroidism, symptoms can resolve with thyroxine replacement and emphasises the importance of measuring thyroid function in patients with proximal weakness/myalgia and a significantly raised creatine kinase.


Assuntos
Doença de Hashimoto/complicações , Debilidade Muscular/etiologia , Tiroxina/uso terapêutico , Adulto , Creatina Quinase/sangue , Feminino , Doença de Hashimoto/sangue , Doença de Hashimoto/fisiopatologia , Terapia de Reposição Hormonal , Humanos , Debilidade Muscular/sangue , Debilidade Muscular/tratamento farmacológico , Debilidade Muscular/fisiopatologia , Tiroxina/sangue , Resultado do Tratamento
8.
Muscle Nerve ; 60(4): 392-399, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-31298737

RESUMO

INTRODUCTION: The prevalence and impact of symptoms affecting individuals with pediatric forms of myotonic dystrophy type-1 (DM1) are not well understood. METHODS: Patients from the United States, Canada, and Sweden completed a survey that investigated 20 themes associated with pediatric-onset DM1. Participants reported the prevalence and importance of each theme affecting their lives. Surveys from participants were matched with surveys from their caregivers for additional analysis. RESULTS: The most prevalent symptomatic themes included problems with hands or fingers (79%) and gastrointestinal issues (75%). Problems with urinary/bowel control and gastrointestinal issues were reported to have the greatest impact on patients' lives. Responses from participants and their caregivers had varying levels of agreement among symptomatic themes. DISCUSSION: Many symptoms have meaningful impact on disease burden. The highest levels of agreement between caregivers and individuals with pediatric forms of myotonic dystrophy were found for physical activity themes.


Assuntos
Distrofia Miotônica/fisiopatologia , Distrofia Miotônica/psicologia , Atividades Cotidianas , Adolescente , Adulto , Cuidadores , Criança , Pré-Escolar , Comunicação , Efeitos Psicossociais da Doença , Transtornos de Deglutição/etiologia , Transtornos de Deglutição/fisiopatologia , Feminino , Dedos/fisiopatologia , Gastroenteropatias/etiologia , Gastroenteropatias/fisiopatologia , Mãos/fisiopatologia , Humanos , Masculino , Limitação da Mobilidade , Debilidade Muscular/etiologia , Debilidade Muscular/fisiopatologia , Miotonia/etiologia , Miotonia/fisiopatologia , Distrofia Miotônica/complicações , Medidas de Resultados Relatados pelo Paciente , Adulto Jovem
9.
Gait Posture ; 73: 168-172, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-31344605

RESUMO

BACKGROUND: Muscle weakness is one of the most prevalent symptoms in children with cerebral palsy (CP). Although recent studies show that functional power training can improve strength and functional capacity in young children with CP, effects on specific gait parameters have not previously been reported. RESEARCH QUESTION: What are the effects of functional power training on gait in children with CP? Specifically, we investigated effects of training on gait kinematics and spatiotemporal parameters, and whether these were dependent on walking speed. METHODS: Ten children with CP (age 5-10 years, GMFCS I-II) participated in a functional power training program. At the start and end of the program, children underwent 3D gait analysis on a treadmill at a gradual range of walking speeds (70-175% of their comfortable walking speed). Multilevel (linear mixed model) analysis was used to evaluate effects pre-post training at different walking velocities. RESULTS: Although children's self-chosen comfortable walking speed improved (0.71 ±â€¯0.25 to 0.85 ±â€¯0.25 m/s, p < .05), effects on gait kinematics at similar speed were limited and only exceeded statistical and clinically meaningful thresholds when children walked at higher walking speed. At fast speeds, improvements up to 5° were found in knee and hip extension during stance (p < .01). SIGNIFICANCE: This study demonstrates that gait kinematics can improve after functional power training, but the magnitude of effects is dependent on walking speed. In this light, improvements are underestimated when evaluating gait at pre-training comfortable walking speed only.


Assuntos
Debilidade Muscular/reabilitação , Fenômenos Biomecânicos/fisiologia , Paralisia Cerebral/fisiopatologia , Paralisia Cerebral/reabilitação , Criança , Pré-Escolar , Feminino , Análise da Marcha , Transtornos Neurológicos da Marcha/fisiopatologia , Transtornos Neurológicos da Marcha/reabilitação , Humanos , Masculino , Debilidade Muscular/fisiopatologia , Modalidades de Fisioterapia , Velocidade de Caminhada/fisiologia
10.
Medicine (Baltimore) ; 98(28): e16389, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31305444

RESUMO

INTRODUCTION: Various phenotypes have been identified for MYH7 gene mutation-related myopathy. Here, we describe a patient with severe muscular weakness and skeletal deformity with de novo heterozygous MYH7 gene mutation. PATIENT CONCERNS: A 33-year-old woman presented with early onset of muscular weakness, with delayed motor development during infancy. At age 8 years, she was unable to walk, with signs of skeletal deformity, including the progression of kyphoscoliosis. At age 31 years, she developed dyspnea. DIAGNOSIS: She diagnosed with esophageal hiatal hernia with abdominal CT. In electromyography, short duration, small amplitude motor unit action potential (MUAP), and early recruitment patterns were observed in the involved proximal muscles, suggesting myopathy. Muscle histopathology showed fiber-type disproportion. INTERVENTIONS: Next-generation sequencing study revealed a heterozygous in-frame deletion variation in the exon 14 of the MYH7 gene (c.1498_1500del/p.Glu500del), which is a novel variation confirmed by conventional Sanger sequencing. Compared with the parental test, this variant was concluded as de novo. OUTCOMES: She received laparoscopic hiatal hernia repair and Nissen fundoplication for esophageal hiatal hernia. After surgery, her postural dyspnea improved. As there is no fundamental treatment for MYH7-related myopathies, she continued conservative treatment for her symptoms. CONCLUSION: Here, we presented a rare case of de novo mutation of the myosin head domain in the MYH7 gene. This report broadens both the phenotypic and genotypic spectra of MYH7-related myopathies.


Assuntos
Miosinas Cardíacas/genética , Cifose/genética , Debilidade Muscular/genética , Mutação , Cadeias Pesadas de Miosina/genética , Escoliose/genética , Adulto , Idade de Início , Dispneia/etiologia , Dispneia/genética , Dispneia/cirurgia , Feminino , Humanos , Cifose/fisiopatologia , Debilidade Muscular/patologia , Debilidade Muscular/fisiopatologia , Fenótipo , Escoliose/fisiopatologia
11.
Muscle Nerve ; 60(3): 292-298, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-31269226

RESUMO

INTRODUCTION: There are no validated, practical, and quantitative measures of disease severity in Lambert-Eaton myasthenia (LEM). METHODS: Data from the Effectiveness of 3,4-Diaminopyridine in Lambert-Eaton Myasthenic Syndrome (DAPPER) trial were analyzed to assess triple timed up-and-go (3TUG) reproducibility and relationships between 3TUG times and other measures of LEM severity. RESULTS: The coverage probability technique showed ≥0.90 probability for an acceptable 3TUG difference of ≤0.2, indicating that it is reproducible in LEM patients. The correlation between 3TUG times and lower extremity function scores was significant in subjects who continued and in those who were withdrawn from 3,4-diaminopyridine free base. Worsening patient-reported Weakness Self-Assessment Scale and Investigator Assessment of Treatment Effect scores corresponded with prolongation of 3TUG times. DISCUSSION: The 3TUG is reproducible, demonstrates construct validity for assessment of lower extremity function in LEM patients, and correlates with changes in patient and physician assessments. These findings, along with prior reliability studies, indicate 3TUG is a valid measure of disease severity in LEM.


Assuntos
Síndrome Miastênica de Lambert-Eaton/fisiopatologia , Extremidade Inferior/fisiopatologia , Debilidade Muscular/fisiopatologia , Humanos , Programas de Rastreamento/métodos , Debilidade Muscular/tratamento farmacológico , Bloqueadores dos Canais de Potássio/uso terapêutico , Reprodutibilidade dos Testes , Índice de Gravidade de Doença
12.
Intern Med ; 58(13): 1851-1858, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31257275

RESUMO

Objective Amyotrophic lateral sclerosis (ALS) is an adult-onset neurodegenerative disease characterized by the progressive loss of the upper and lower motor neurons that progresses to paralysis of almost all skeletal muscles of the extremities, bulbar, and respiratory system. Although most ALS cases are sporadic, about 10% are dominantly inherited. We herein report an atypical phenotype of familial ALS (fALS). To elucidate the phenotype-genotype correlation of this atypical phenotype of fALS, clinical and genetic investigations were performed. Methods and Patients Five sibling patients (three men, two women) from a Japanese family and one healthy sibling (a woman) were clinically interviewed and examined. Genetic analyses, including genome-wide linkage analyses and whole-exome sequencing, were performed using genomic DNA extracted from the peripheral blood samples of these siblings. Results The clinical features of fALS are characterized by slow progression (mean duration of the disease±standard deviation [SD]: 19.6±3.9 years) and lower extremities-predominant late-onset muscular weakness (mean onset of muscular weakness±SD: 52.8±2.6 years). Genetic analyses revealed novel heterozygous missense mutations of c.2668C>T, p.R890C in the PLEC gene and c.421G>C, p.V141L in the ST3GAL6 gene in all affected siblings. Conclusion A new atypical fALS family with a benign clinical course is herein reported. We identified two candidate gene mutations of PLEC and ST3GAL6 linked to this phenotype.


Assuntos
Esclerose Amiotrófica Lateral/genética , Esclerose Amiotrófica Lateral/mortalidade , Predisposição Genética para Doença , Neurônios Motores/fisiologia , Debilidade Muscular/fisiopatologia , Doenças Neurodegenerativas/genética , Doenças Neurodegenerativas/fisiopatologia , Idoso , Idoso de 80 Anos ou mais , Esclerose Amiotrófica Lateral/fisiopatologia , Grupo com Ancestrais do Continente Asiático , Evolução Fatal , Feminino , Genótipo , Humanos , Extremidade Inferior/fisiopatologia , Masculino , Pessoa de Meia-Idade , Músculo Esquelético/fisiopatologia , Mutação , Doenças Neurodegenerativas/mortalidade , Fenótipo
13.
Crit Care ; 23(1): 236, 2019 Jul 01.
Artigo em Inglês | MEDLINE | ID: mdl-31262340

RESUMO

BACKGROUND: ICU-acquired weakness is a debilitating consequence of prolonged critical illness that is associated with poor outcome. Recently, premorbid obesity has been shown to protect against such illness-induced muscle wasting and weakness. Here, we hypothesized that this protection was due to increased lipid and ketone availability. METHODS: In a centrally catheterized, fluid-resuscitated, antibiotic-treated mouse model of prolonged sepsis, we compared markers of lipolysis and fatty acid oxidation in lean and obese septic mice (n = 117). Next, we compared markers of muscle wasting and weakness in septic obese wild-type and adipose tissue-specific ATGL knockout (AAKO) mice (n = 73), in lean septic mice receiving either intravenous infusion of lipids or standard parenteral nutrition (PN) (n = 70), and in lean septic mice receiving standard PN supplemented with either the ketone body 3-hydroxybutyrate or isocaloric glucose (n = 49). RESULTS: Obese septic mice had more pronounced lipolysis (p ≤ 0.05), peripheral fatty acid oxidation (p ≤ 0.05), and ketogenesis (p ≤ 0.05) than lean mice. Blocking lipolysis in obese septic mice caused severely reduced muscle mass (32% loss vs. 15% in wild-type, p < 0.001) and specific maximal muscle force (59% loss vs. 0% in wild-type; p < 0.001). In contrast, intravenous infusion of lipids in lean septic mice maintained specific maximal muscle force up to healthy control levels (p = 0.6), whereas this was reduced with 28% in septic mice receiving standard PN (p = 0.006). Muscle mass was evenly reduced with 29% in both lean septic groups (p < 0.001). Lipid administration enhanced fatty acid oxidation (p ≤ 0.05) and ketogenesis (p < 0.001), but caused unfavorable liver steatosis (p = 0.01) and a deranged lipid profile (p ≤ 0.01). Supplementation of standard PN with 3-hydroxybutyrate also attenuated specific maximal muscle force up to healthy control levels (p = 0.1), but loss of muscle mass could not be prevented (25% loss in both septic groups; p < 0.001). Importantly, this intervention improved muscle regeneration markers (p ≤ 0.05) without the unfavorable side effects seen with lipid infusion. CONCLUSIONS: Obesity-induced muscle protection during sepsis is partly mediated by elevated mobilization and metabolism of endogenous fatty acids. Furthermore, increased availability of ketone bodies, either through ketogenesis or through parenteral infusion, appears to protect against sepsis-induced muscle weakness also in the lean.


Assuntos
Tecido Adiposo/fisiopatologia , Lipólise/fisiologia , Debilidade Muscular/etiologia , Sepse/complicações , Animais , Modelos Animais de Doenças , Ácidos Graxos/metabolismo , Ácidos Graxos/farmacocinética , Cetonas/metabolismo , Metabolismo dos Lipídeos/fisiologia , Masculino , Camundongos , Debilidade Muscular/metabolismo , Debilidade Muscular/fisiopatologia , Obesidade/fisiopatologia , Fatores de Proteção , Sepse/metabolismo , Sepse/fisiopatologia
14.
BMC Musculoskelet Disord ; 20(1): 273, 2019 Jun 03.
Artigo em Inglês | MEDLINE | ID: mdl-31159812

RESUMO

BACKGROUND: Previous studies have indicated that trunk muscle strength decreases with chronic low back pain, and is associated with poor balance, poor functional performance, and falls in older adults. Strengthening exercises for chronic low back pain are considered the most effective intervention to improve functional outcomes. We developed an innovative exercise device for abdominal trunk muscles that also measures muscle strength. The correlation between muscle weakness, as measured by our device, the presence of chronic low back pain, and decreased physical ability associated with a risk of falling were evaluated in older women. METHODS: Thirty-eight elderly women, who could walk without support during daily activities and attended our outpatient clinic for treatment of chronic low back pain, knee or hip arthritis, or osteoporosis, were included in this study. Anthropometric measurements were performed. Grip power and one-leg standing time with eyes open were measured, and abdominal trunk muscle strength was measured using our device. History of falling in the previous 12 months was noted. Subjects with chronic low back pain (visual analog scale score ≥ 20 mm) for over 3 months were assigned to the low back pain group (n = 21). The remaining subjects formed the non-low back pain group (n = 17). RESULTS: Abdominal muscle strength of subjects in the low back pain group, and with history of falling, was significantly lower compared with that of subjects in the non-low back pain group, and in subjects without a history of falling, respectively. There was a moderate positive correlation between abdominal trunk muscle strength and one-leg standing time with eyes open. CONCLUSION: We measured abdominal muscle strength in older women with chronic low back pain using our device, and it was significantly lower than that of those without chronic low back pain. Muscle weakness was associated with a history and risk of falling.


Assuntos
Músculos Abdominais/fisiopatologia , Acidentes por Quedas/estatística & dados numéricos , Dor Lombar/complicações , Debilidade Muscular/diagnóstico , Acidentes por Quedas/prevenção & controle , Idoso , Idoso de 80 Anos ou mais , Estudos de Coortes , Terapia por Exercício/instrumentação , Terapia por Exercício/métodos , Feminino , Humanos , Dor Lombar/fisiopatologia , Dor Lombar/terapia , Força Muscular/fisiologia , Debilidade Muscular/etiologia , Debilidade Muscular/fisiopatologia
15.
BMC Neurol ; 19(1): 118, 2019 Jun 08.
Artigo em Inglês | MEDLINE | ID: mdl-31176377

RESUMO

BACKGROUND: In patients suffering from Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) disease severity is assessed by Medical Research Counsil (MRC) Scale or Inflammatory Neuropathy Cause and Treatment (INCAT) disability score. However, none of these methods is appropriate to objectively assess muscle weakness or to detect very small subclinical changes. More objective and quantitative measures are needed in order to evaluate treatment efficiency or to detect subclinical affection of upper limps for early diagnosis. The goal of our study was to objectively quantify muscular weakness in CIDP patients with the non-invasive Quantitative Motor (Q-Motor) test of Grip Force Assessment (QGFA) as well as the Involuntary Movement Assessment (QIMA) and to search for differences between typical and atypical CIDP variants. In addition, we hypothesized that Q-Motor findings correlate with disease severity scales such as MRC or INCAT score. METHODS: In this cross-sectional exploratory proof-of-concept study subjects with confirmed diagnosis of typical or atypical CIDP were examined and compared to healthy controls (HC). For Q-Motor tests all subjects had to lift a device (250 g and 500 g) equipped with an electromagnetic sensor that measured grip force (GF) and three-dimensional changes in position and orientation. The measures "grip force variability" (GFV), "position index" (PI) and "orientation index" (OI) were provided to assess involuntary movements due to muscular weakness. RESULTS: 33 patients with CIDP and 28 HC were included. All measures were significantly elevated in CIDP patients for both devices in the right and left hand compared to healthy controls. Subgroup analysis revealed no differences between typical and atypical CIDP variants. INCAT score only weakly correlated with OI and PI. However, there was a stronger correlation between MRC and QIMA parameters in both hands. CONCLUSION: Q-Motor assessments were capable to objectively assess muscular weakness in CIDP. In particular, QIMA measures detected subclinical generalized muscle weakness even in patients with milder disability. Sensitivity and rater-independence of Q-Motor assessments support a further exploration of QIMA measures as potential endpoints for future clinical trials in CIDP.


Assuntos
Força da Mão/fisiologia , Debilidade Muscular , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica , Adulto , Idoso , Idoso de 80 Anos ou mais , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Debilidade Muscular/diagnóstico , Debilidade Muscular/etiologia , Debilidade Muscular/fisiopatologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/complicações , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/fisiopatologia , Estudo de Prova de Conceito
17.
Phys Ther ; 99(9): 1167-1176, 2019 09 01.
Artigo em Inglês | MEDLINE | ID: mdl-31197369

RESUMO

BACKGROUND: Neuromuscular electrical stimulation (NMES) is a viable intervention for improving impaired muscle function in individuals with rheumatoid arthritis (RA). However, there is limited evidence about the dose-response relationship between NMES and muscle function in these individuals. OBJECTIVE: The objectives of this study were to investigate the dose-response relationship between NMES and muscle function in individuals with RA and to establish the minimal NMES training intensity for promoting improvements. DESIGN: This study was a secondary analysis of data obtained before and after an NMES intervention in a randomized study. METHODS: The study took place at a research clinic. Only adults diagnosed with RA were included. The intervention consisted of 36 NMES treatment sessions for the quadriceps muscles over 16 weeks. Muscle function was measured before and after the intervention; quadriceps cross-sectional area and muscle quality were assessed using computed tomography, and strength was measured with an isokinetic dynamometer. NMES training intensity was calculated as a percentage by dividing NMES-elicited quadriceps muscle torque by the maximum voluntary isometric contraction. Improvements in muscle function were calculated using paired-sample t tests. The dose-response relationship was determined using curve estimation regression statistics. The minimum NMES training intensity was defined as that sufficient to significantly improve all muscle function measures. RESULTS: Twenty-four people (48 legs) participated (75% women; mean [SD] age = 58 [8] years; mean body mass index = 32 [7] kg/m2). Quadriceps cross-sectional area, muscle quality, and strength improved after the intervention. Associations between NMES training intensity and muscle quality (r2 = 0.20) and strength (r2 = 0.23) were statistically significant, but that between NMES training intensity and muscle cross-sectional area was not (r2 = 0.02). The minimum NMES training intensity necessary to improve all measures of muscle function ranged from 11% to 20% of the maximum voluntary isometric contraction. LIMITATIONS: The relatively small sample size was a limitation. CONCLUSIONS: The minimum NMES training intensity for significant gains in muscle function was ∼15%. Higher NMES intensities may promote better muscle quality and strength in individuals with RA.


Assuntos
Artrite Reumatoide/terapia , Terapia por Estimulação Elétrica/métodos , Contração Isométrica/fisiologia , Músculo Quadríceps/fisiopatologia , Artrite Reumatoide/fisiopatologia , Índice de Massa Corporal , Terapia por Estimulação Elétrica/estatística & dados numéricos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Força Muscular/fisiologia , Debilidade Muscular/fisiopatologia , Debilidade Muscular/terapia , Músculo Esquelético/fisiopatologia , Atrofia Muscular/fisiopatologia , Atrofia Muscular/terapia , Músculo Quadríceps/diagnóstico por imagem , Análise de Regressão , Tamanho da Amostra , Torque
18.
BMC Neurol ; 19(1): 135, 2019 Jun 19.
Artigo em Inglês | MEDLINE | ID: mdl-31216995

RESUMO

BACKGROUND: Myotonic Dystrophy 1 (DM1) causes progressive myopathy of extremity muscles. DM1 may also affect muscles of the trunk. The aim of this study was to investigate fat infiltration and muscle size in trunk muscles in DM1 patients, and in an age and gender matched control group. Further, explore how fat infiltration and degree of atrophy in these muscles are associated with motor and respiratory function in DM1 patients. METHOD: We measured fat infiltration and trunk muscle size by MRI in 20 patients with genetically confirmed classic form of DM1, and compared these cases with 20 healthy, age and gender matched controls. In the DM1 group, we investigated correlations between MRI findings and clinical measures of muscle strength, mobility and respiration. We used sum scores for fat infiltration and muscle size in trunk flexors and trunk extensors in the analysis of group differences and correlations. RESULTS: Significant differences between cases and controls were present for fat infiltration in trunk flexors (p = 0.001) and trunk extensors (p = < 0.001), and for muscle size in trunk flexors (p = 0.002) and trunk extensors (p = 0.030). Fat infiltration in trunk flexors were significant correlated to back extension strength (rho = - 0.523 p = 0.018), while muscle size in trunk flexors was significantly correlated to trunk flexion strength (rho = 0.506 p = 0.023). Fat infiltration in trunk flexors was significantly correlated with lower general mobility (rho = - 0.628, p = 0.003), reduced balance (rho = 0.630, p < 0.003) and forced vital capacity (rho - 0.487 p = 0.040). CONCLUSIONS: Trunk muscles in DM1 patients had significant higher levels of fat infiltration and reduced muscle size compared to age and gender matched controls. In DM1 patients, fat infiltration was associated with reduced muscle strength, mobility, balance and lung function, while muscle size was associated with reduced muscle strength and lung function. These findings are of importance for clinical management of the disease and could be useful additional outcome measures in future intervention studies.


Assuntos
Atrofia/patologia , Músculo Esquelético/patologia , Distrofia Miotônica/patologia , Distrofia Miotônica/fisiopatologia , Respiração , Tronco/patologia , Adulto , Estudos de Casos e Controles , Feminino , Humanos , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Força Muscular/fisiologia , Debilidade Muscular/fisiopatologia , Músculo Esquelético/fisiopatologia , Adulto Jovem
19.
Phys Ther Sport ; 38: 36-43, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31042614

RESUMO

OBJECTIVES: To investigate the effect of sex on measures of quadriceps strength, rate of torque development, and knee function during the first year following ACLR. DESIGN: Cross-sectional; SETTING: University community; PARTICIPANTS: Sixty individuals (29 men) with unilateral ACLR in the last 12 months. MAIN OUTCOME MEASURES: Participants completed bilateral knee extension maximal voluntary isometric contraction (MVIC, Nm/kg) strength assessments. Rates of torque development (Nm*kg-1*s-1) were assessed from contraction initiation to 100 ms (RTD100) and from 100 ms to 200 ms after contraction initiation (RTD200). The effects of sex MVIC strength, RTD, and limb symmetry were assessed using separate ANCOVAs. RESULTS: Women displayed weaker involved limb (Men = 2.72 ±â€¯0.72 Nm*kg-1, Women = 2.01 ±â€¯0.50 Nm*kg-1, p < 0.001) and contralateral limb (Men = 3.15 ±â€¯0.52 Nm*kg-1, Women = 2.66 ±â€¯0.58 Nm*kg-1, p < 0.001) MVIC, and slower involved limb RTD100 (Men = 8.36 ±â€¯3.16 Nm*kg-1*s-1, Women = 6.50 ±â€¯2.41 Nm*kg-1*s-1, p = 0.01) and RTD200 (Men = 9.49 ±â€¯3.45 Nm*kg-1*s-1, Women = 9.49 ±â€¯3.45 Nm*kg-1*s-1, p < 0.001) when compared to men. CONCLUSIONS: Within the first year after ACLR, women displayed bilateral quadriceps weakness and slower involved limb quadriceps RTD when compared to men. Specific focus on facilitating quadriceps hypertrophy and improving neural drive to the quadriceps is indicated when treating female patients attempting to make a return to sport after ACLR.


Assuntos
Reconstrução do Ligamento Cruzado Anterior/reabilitação , Terapia por Exercício/métodos , Contração Isométrica/fisiologia , Força Muscular/fisiologia , Debilidade Muscular/reabilitação , Músculo Quadríceps/fisiopatologia , Adolescente , Adulto , Estudos Transversais , Feminino , Seguimentos , Humanos , Articulação do Joelho/fisiopatologia , Masculino , Debilidade Muscular/etiologia , Debilidade Muscular/fisiopatologia , Fatores Sexuais , Fatores de Tempo , Adulto Jovem
20.
BMJ Case Rep ; 12(5)2019 May 08.
Artigo em Inglês | MEDLINE | ID: mdl-31068355

RESUMO

Necrotising autoimmune myopathy (NAM) is an immune-mediated myopathy that may be associated with statin use, malignancy or an autoimmune connective tissue disease, but it can also be idiopathic. Anti-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) myopathy is an extremely rare side effect of statin use, occurring in approximately 2-3 out of every 100 000 patients who use statins. Patients typically present with subacute proximal muscle weakness and creatine kinase levels >10 times the upper limit of normal. The diagnosis is suggested by muscle biopsy showing necrotic fibres with minimal inflammation along with positive anti-HMGCR antibodies. Treatment nearly always requires multiple immunosuppressive agents, the earlier use of which is associated with improved outcomes. Reports of statin-induced NAM leading to heart failure are limited. We present the case of a 69-year-old woman with statin-induced NAM who presented with acute systolic heart failure. Early initiation of high-dose corticosteroids and IVIG resulted in significant improvement in her symptoms.


Assuntos
Anti-Inflamatórios/administração & dosagem , Anticolesterolemiantes/efeitos adversos , Atorvastatina/efeitos adversos , Insuficiência Cardíaca Sistólica/induzido quimicamente , Hidroximetilglutaril-CoA Redutases/efeitos dos fármacos , Metilprednisolona/administração & dosagem , Debilidade Muscular/induzido quimicamente , Doenças Musculares/induzido quimicamente , Idoso , Creatina Quinase , Feminino , Insuficiência Cardíaca Sistólica/fisiopatologia , Humanos , Debilidade Muscular/fisiopatologia , Doenças Musculares/fisiopatologia , Resultado do Tratamento
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