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1.
Scand J Med Sci Sports ; 31(11): 2144-2155, 2021 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-34409660

RESUMO

This study aimed to determine deficits in knee extensor muscle function through the torque-time and torque-velocity relationships and whether these deficits are associated with reduced functional performance in postmenopausal women with knee osteoarthritis (KOA). A clinical sample of postmenopausal women with established KOA (n = 18, ≥55 years) was compared to an age-matched healthy control sample (CON) (n = 26). The deficits in different parameters of the knee extensor torque-time (maximal isometric torque and rate of torque development) and torque-velocity relationship (maximum muscle power, maximal velocity and torque at 0-500°·s-1 ) were assessed through a protocol consisting of isometric, isotonic and isokinetic tests. Functional performance was evaluated with sit-to-stand and stair-climbing tasks using a sensor-based technology (ie, time- and power-based outcomes). Postmenopausal women with KOA showed reduced maximal isometric torque (Hedge's g effect size (g) = 1.05, p = 0.001) and rate of torque development (g = 0.77-1.17, all p ≤ 0.02), combined with impaired torque production at slow to moderate velocities (g = 0.92-1.70, p ≤ 0.004), but not at high or maximal velocities (g = 0.16, p > 0.05). KOA were slower (g = 0.81-0.92, p ≤ 0.011) and less powerful (g = 1.11-1.29, p ≤ 0.001) during functional tasks. Additionally, knee extensor deficits were moderately associated with power deficits in stair climbing (r = 0.492-0.659). To conclude, knee extensor muscle weakness was presented in postmenopausal women with KOA, not only as limited maximal and rapid torque development during isometric contractions, but also dynamically at low to moderate velocities. These deficits were related to impaired functional performance. The assessment of knee extensor muscle weakness through the torque-time and torque-velocity relationships might enable individual targets for tailored exercise interventions in KOA.


Assuntos
Debilidade Muscular/fisiopatologia , Osteoartrite do Joelho/fisiopatologia , Pós-Menopausa , Idoso , Estudos Transversais , Teste de Esforço , Feminino , Humanos , Pessoa de Meia-Idade , Torque
2.
Sci Rep ; 11(1): 16213, 2021 08 10.
Artigo em Inglês | MEDLINE | ID: mdl-34376701

RESUMO

Dropped head syndrome (DHS) exhibits cervical deformity due to weakness of the cervical extensor group, and sarcopenia is characterized by progressive and systemic reduction in skeletal muscle mass. These clinical finding are associated with reduced activity of daily living, reduced quality of life, and increased risk of mortality. We collected and reviewed prospective registry data for 16 patients with idiopathic DHS continuously collected without dropping out and 32 healthy individuals who matched their gender and age. The prevalence of sarcopenia and body composition data were compared. There were no differences in the prevalence of sarcopenia, appendicular muscle mass, and leg muscle mass between DHS patients and the healthy elderly. Trunk muscle mass in DHS patients was significantly lower than that in healthy individuals. A significant correlation was found between appendicular muscle mass and trunk muscle mass in healthy subjects but not in DHS patients. Sarcopenia was not associated with the onset of idiopathic DHS. The prevalence of sarcopenia was not high in patients with idiopathic DHS due to the preservation of their appendicular skeletal muscle mass. Patients with DHS were characterized by a significant loss of trunk muscle mass that may be related to the disease but not aging.


Assuntos
Vértebras Cervicais/fisiopatologia , Cifose/fisiopatologia , Debilidade Muscular/fisiopatologia , Doenças Musculares/fisiopatologia , Sarcopenia/epidemiologia , Idoso , Idoso de 80 Anos ou mais , Estudos de Casos e Controles , Estudos Transversais , Feminino , Voluntários Saudáveis , Humanos , Pessoa de Meia-Idade , Sarcopenia/patologia
3.
Sci Rep ; 11(1): 16317, 2021 08 11.
Artigo em Inglês | MEDLINE | ID: mdl-34381086

RESUMO

Diaphragm weakness affects up to 60% of ventilated patients leading to muscle atrophy, reduction of muscle fiber force via muscle fiber injuries and prolonged weaning from mechanical ventilation. Electromagnetic stimulation of the phrenic nerve can induce contractions of the diaphragm and potentially prevent and treat loss of muscular function. Recommended safety distance of electromagnetic coils is 1 m. The aim of this study was to investigate the magnetic flux density in a typical intensive care unit (ICU) setting. Simulation of magnetic flux density generated by a butterfly coil was performed in a Berlin ICU training center with testing of potential disturbance and heating of medical equipment. Approximate safety distances to surrounding medical ICU equipment were additionally measured in an ICU training center in Bern. Magnetic flux density declined exponentially with advancing distance from the stimulation coil. Above a coil distance of 300 mm with stimulation of 100% power the signal could not be distinguished from the surrounding magnetic background noise. Electromagnetic stimulation of the phrenic nerve for diaphragm contraction in an intensive care unit setting seems to be safe and feasible from a technical point of view with a distance above 300 mm to ICU equipment from the stimulation coil.


Assuntos
Nervo Frênico/fisiopatologia , Gestão da Segurança/métodos , Berlim , Diafragma/fisiopatologia , Fenômenos Eletromagnéticos , Humanos , Unidades de Terapia Intensiva , Contração Muscular/fisiologia , Debilidade Muscular/fisiopatologia , Atrofia Muscular/fisiopatologia , Respiração Artificial/métodos
4.
J Am Heart Assoc ; 10(13): e021030, 2021 07 06.
Artigo em Inglês | MEDLINE | ID: mdl-34212761

RESUMO

Background The activation of AT2 (angiotensin II type 2 receptor ) and Mas receptor by angiotensin II and angiotensin-(1-7), respectively, is the primary process that counteracts activation of the canonical renin-angiotensin system (RAS). Although inhibition of canonical RAS could delay the progression of physiological aging, we recently reported that deletion of Mas had no impact on the aging process in mice. Here, we used male mice with a deletion of only AT2 or a double deletion of AT2 and Mas to clarify whether these receptors contribute to the aging process in a complementary manner, primarily by focusing on aging-related muscle weakness. Methods and Results Serial changes in grip strength of these mice up to 24 months of age showed that AT2/Mas knockout mice, but not AT2 knockout mice, had significantly weaker grip strength than wild-type mice from the age of 18 months. AT2/Mas knockout mice exhibited larger sizes, but smaller numbers and increased frequency of central nucleation (a marker of aged muscle) of single skeletal muscle fibers than AT2 knockout mice. Canonical RAS-associated genes, inflammation-associated genes, and senescence-associated genes were highly expressed in skeletal muscles of AT2/Mas knockout mice. Muscle angiotensin II content increased in AT2/Mas knockout mice. Conclusions Double deletion of AT2 and Mas in mice exaggerated aging-associated muscle weakness, accompanied by signatures of activated RAS, inflammation, and aging in skeletal muscles. Because aging-associated phenotypes were absent in single deletions of the receptors, AT2 and Mas could complement each other in preventing local activation of RAS during aging.


Assuntos
Força Muscular , Debilidade Muscular/fisiopatologia , Músculo Esquelético/fisiopatologia , Proteínas Proto-Oncogênicas/deficiência , Receptor Tipo 2 de Angiotensina/deficiência , Receptores Acoplados a Proteínas G/deficiência , Fatores Etários , Animais , Fibrose , Regulação da Expressão Gênica , Predisposição Genética para Doença , Força da Mão , Mediadores da Inflamação/metabolismo , Masculino , Camundongos Endogâmicos C57BL , Camundongos Knockout , Força Muscular/genética , Debilidade Muscular/genética , Debilidade Muscular/metabolismo , Debilidade Muscular/patologia , Músculo Esquelético/metabolismo , Músculo Esquelético/patologia , Fenótipo , Proteínas Proto-Oncogênicas/genética , Receptor Tipo 2 de Angiotensina/genética , Receptores Acoplados a Proteínas G/genética , Sistema Renina-Angiotensina/genética
5.
Crit Care ; 25(1): 252, 2021 07 17.
Artigo em Inglês | MEDLINE | ID: mdl-34274000

RESUMO

BACKGROUND: Muscle weakness is a complication of critical illness which hampers recovery. In critically ill mice, supplementation with the ketone body 3-hydroxybutyrate has been shown to improve muscle force and to normalize illness-induced hypocholesterolemia. We hypothesized that altered cholesterol homeostasis is involved in development of critical illness-induced muscle weakness and that this pathway can be affected by 3-hydroxybutyrate. METHODS: In both human critically ill patients and septic mice, the association between circulating cholesterol concentrations and muscle weakness was assessed. In septic mice, the impact of 3-hydroxybutyrate supplementation on cholesterol homeostasis was evaluated with use of tracer technology and through analysis of markers of cholesterol metabolism and downstream pathways. RESULTS: Serum cholesterol concentrations were lower in weak than in non-weak critically ill patients, and in multivariable analysis adjusting for baseline risk factors, serum cholesterol was inversely correlated with weakness. In septic mice, plasma cholesterol correlated positively with muscle force. In septic mice, exogenous 3-hydroxybutyrate increased plasma cholesterol and altered cholesterol homeostasis, by normalization of plasma mevalonate and elevation of muscular, but not hepatic, expression of cholesterol synthesis genes. In septic mice, tracer technology revealed that 3-hydroxybutyrate was preferentially taken up by muscle and metabolized into cholesterol precursor mevalonate, rather than TCA metabolites. The 3-hydroxybutyrate protection against weakness was not related to ubiquinone or downstream myofiber mitochondrial function, whereas cholesterol content in myofibers was increased. CONCLUSIONS: These findings point to a role for low cholesterol in critical illness-induced muscle weakness and to a protective mechanism-of-action for 3-hydroxybutyrate supplementation.


Assuntos
Colesterol/análise , Homeostase/efeitos dos fármacos , Ácido 3-Hidroxibutírico , Idoso , Idoso de 80 Anos ou mais , Animais , Colesterol/metabolismo , Estado Terminal/terapia , Modelos Animais de Doenças , Feminino , Humanos , Metabolismo dos Lipídeos/efeitos dos fármacos , Masculino , Camundongos , Camundongos Endogâmicos C57BL/metabolismo , Camundongos Endogâmicos C57BL/fisiologia , Pessoa de Meia-Idade , Análise Multivariada , Debilidade Muscular/fisiopatologia
6.
Neurology ; 97(7 Suppl 1): S99-S110, 2021 08 17.
Artigo em Inglês | MEDLINE | ID: mdl-34230196

RESUMO

OBJECTIVE: To determine a suitable outcome measure for assessing muscle strength in neurofibromatosis (NF) type 1 and NF2 clinical trials, we evaluated the intraobserver reliability of handheld dynamometry (HHD) and developed consensus recommendations for its use in NF clinical trials. METHODS: Patients ≥5 years of age with weakness in at least 1 muscle group by manual muscle testing (MMT) were eligible. Maximal isometric muscle strength of a weak muscle group and the biceps of the dominant arm was measured by HHD. An average of 3 repetitions per session was used as an observation, and 3 sessions with rest period between each were performed on the same day by a single observer. Intrasession and intersession intraclass correlation coefficients (ICCs) and coefficients of variation (CVs) were calculated to assess reliability and measurement error. RESULTS: Twenty patients with NF1 and 13 with NF2 were enrolled; median age was 12 years (interquartile range [IQR] 9-17 years) and 29 years (IQR 22-38 years), respectively. By MMT, weak muscle strength ranged from 2-/5 to 4+/5. Biceps strength was 5/5 in all patients. Intersession ICCs for the weak muscles were 0.98 and 0.99 in the NF1 and NF2 cohorts, respectively, and for biceps were 0.97 and 0.97, respectively. The median CVs for average session strength were 5.4% (IQR 2.6%-7.3%) and 2.9% (IQR 2.0%-6.2%) for weak muscles and biceps, respectively. CONCLUSION: HHD performed by a trained examiner with a well-defined protocol is a reliable technique to measure muscle strength in NF1 and NF2. Recommendations for strength testing in NF1 and NF2 trials are provided.


Assuntos
Contração Isométrica/fisiologia , Força Muscular/fisiologia , Debilidade Muscular/fisiopatologia , Músculo Esquelético/fisiologia , Adolescente , Adulto , Criança , Humanos , Masculino , Pessoa de Meia-Idade , Dinamômetro de Força Muscular , Debilidade Muscular/diagnóstico , Neurofibromatoses/fisiopatologia
8.
PLoS One ; 16(6): e0253531, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34143856

RESUMO

The maximal power generating capacity of a muscle declines with age and has a negative impact on the performance of daily life activities. As muscle power is the product of force and velocity, we recruited 20 young (10 men, 10 women: 20-31 years) and 20 older (10 men, 10 women: 65-86 years) people to investigate which of these components contributes to the lower power and performance in old age. After determination of the maximal isometric knee extension torque (MVC), they performed a countermovement jump (CMJ) in 1) the normal situation (normal), 2) with an extra load of 15% body weight (loaded) and 3) 15% lower body weight (unloaded with a pulley system), and a timed up-and-go test (TUG) in the normal or loaded condition. The TUG and CMJ performance was lower in old than young participants (p<0.001). Below a critical CMJ peak power of ~23.7 W·kg-1 TUG showed a progressive decrease. The CMJ take-off velocity (Voff) in the normal condition was lower in old than young participants (p<0.001). However, the Voff vs. body weight/MVC relationship of the normal, loaded and unloaded data combined was similar in the old and young participants and fitted the Hill equation (R2 = 0.396). This indicates that 1) only when peak power drops below a critical threshold TUG becomes impaired and 2) there was no evidence for intrinsic slowing of the muscle contractile properties in older people, but rather the older people were working on a slower part of the force-velocity relationship due to weaker muscles.


Assuntos
Limitação da Mobilidade , Contração Muscular/fisiologia , Força Muscular/fisiologia , Debilidade Muscular/fisiopatologia , Músculo Esquelético/fisiopatologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Adulto Jovem
9.
Int J Mol Sci ; 22(9)2021 May 07.
Artigo em Inglês | MEDLINE | ID: mdl-34066978

RESUMO

Bone and muscle are highly synergistic tissues that communicate extensively via mechanotransduction and biochemical signaling. Osteogenesis imperfecta (OI) is a heritable connective tissue disorder of severe bone fragility and recently recognized skeletal muscle weakness. The presence of impaired bone and muscle in OI leads to a continuous cycle of altered muscle-bone crosstalk with weak muscles further compromising bone and vice versa. Currently, there is no cure for OI and understanding the pathogenesis of the skeletal muscle weakness in relation to the bone pathogenesis of OI in light of the critical role of muscle-bone crosstalk is essential to developing and identifying novel therapeutic targets and strategies for OI. This review will highlight how impaired skeletal muscle function contributes to the pathophysiology of OI and how this phenomenon further perpetuates bone fragility.


Assuntos
Osso e Ossos/patologia , Debilidade Muscular/patologia , Músculo Esquelético/patologia , Osteogênese Imperfeita/patologia , Animais , Fenômenos Biomecânicos , Osso e Ossos/fisiopatologia , Metabolismo Energético , Humanos , Debilidade Muscular/fisiopatologia , Músculo Esquelético/fisiopatologia , Osteogênese Imperfeita/metabolismo , Osteogênese Imperfeita/fisiopatologia
10.
Thorax ; 76(7): 664-671, 2021 07.
Artigo em Inglês | MEDLINE | ID: mdl-33931570

RESUMO

PURPOSE: Functional electrical stimulation-assisted cycle ergometry (FESCE) enables in-bed leg exercise independently of patients' volition. We hypothesised that early use of FESCE-based progressive mobility programme improves physical function in survivors of critical care after 6 months. METHODS: We enrolled mechanically ventilated adults estimated to need >7 days of intensive care unit (ICU) stay into an assessor-blinded single centre randomised controlled trial to receive either FESCE-based protocolised or standard rehabilitation that continued up to day 28 or ICU discharge. RESULTS: We randomised in 1:1 ratio 150 patients (age 61±15 years, Acute Physiology and Chronic Health Evaluation II 21±7) at a median of 21 (IQR 19-43) hours after admission to ICU. Mean rehabilitation duration of rehabilitation delivered to intervention versus control group was 82 (IQR 66-97) versus 53 (IQR 50-57) min per treatment day, p<0.001. At 6 months 42 (56%) and 46 (61%) patients in interventional and control groups, respectively, were alive and available to follow-up (81.5% of prespecified sample size). Their Physical Component Summary of SF-36 (primary outcome) was not different at 6 months (50 (IQR 21-69) vs 49 (IQR 26-77); p=0.26). At ICU discharge, there were no differences in the ICU length of stay, functional performance, rectus femoris cross-sectional diameter or muscle power despite the daily nitrogen balance was being 0.6 (95% CI 0.2 to 1.0; p=0.004) gN/m2 less negative in the intervention group. CONCLUSION: Early delivery of FESCE-based protocolised rehabilitation to ICU patients does not improve physical functioning at 6 months in survivors. TRIAL REGISTRATION NUMBER: NCT02864745.


Assuntos
Estado Terminal/reabilitação , Ergometria/métodos , Terapia por Exercício/métodos , Unidades de Terapia Intensiva , Força Muscular/fisiologia , Debilidade Muscular/reabilitação , Qualidade de Vida , Respiração Artificial/métodos , Estimulação Elétrica , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Debilidade Muscular/fisiopatologia , Estudos Prospectivos , Fatores de Tempo
11.
Muscle Nerve ; 64(2): 219-224, 2021 08.
Artigo em Inglês | MEDLINE | ID: mdl-34037996

RESUMO

INTRODUCTION/AIMS: We studied a patient with a congenital myasthenic syndrome (CMS) caused by a dominant mutation in the synaptotagmin 2 gene (SYT2) and compared the clinical features of this patient with those of a previously described patient with a recessive mutation in the same gene. METHODS: We performed electrodiagnostic (EDX) studies, genetic studies, muscle biopsy, microelectrode recordings and electron microscopy (EM). RESULTS: Both patients presented with muscle weakness and bulbar deficits, which were worse in the recessive form. EDX studies showed presynaptic failure, which was more prominent in the recessive form. Microelectrode studies in the dominant form showed a marked reduction of the quantal content, which increased linearly with higher frequencies of nerve stimulation. The MEPP frequencies were normal at rest but increased markedly with higher frequencies of nerve stimulation. The EM demonstrated overdeveloped postsynaptic folding, and abundant endosomes, multivesicular bodies and degenerative lamellar bodies inside small nerve terminals. DISCUSSION: The recessive form of CMS caused by a SYT2 mutation showed far more severe clinical manifestations than the dominant form. The pathogenesis of the dominant form likely involves a dominant-negative effect due to disruption of the dual function of synaptotagmin as a Ca2+ -sensor and modulator of synaptic vesicle exocytosis.


Assuntos
Mutação/genética , Síndromes Miastênicas Congênitas/genética , Junção Neuromuscular/genética , Sinaptotagmina II/genética , Adulto , Pré-Escolar , Feminino , Humanos , Síndrome Miastênica de Lambert-Eaton/genética , Síndrome Miastênica de Lambert-Eaton/fisiopatologia , Masculino , Debilidade Muscular/genética , Debilidade Muscular/fisiopatologia , Síndromes Miastênicas Congênitas/diagnóstico , Junção Neuromuscular/fisiopatologia
12.
Crit Care ; 25(1): 157, 2021 04 22.
Artigo em Inglês | MEDLINE | ID: mdl-33888128

RESUMO

Around one third of intensive care unit (ICU) patients will develop severe neuromuscular alterations, known as intensive care unit-acquired weakness (ICUAW), during their stay. The diagnosis of ICUAW is difficult and often delayed as a result of sedation or delirium. Indeed, the clinical evaluation of both Medical Research Council score and maximal voluntary force (e.g., using handgrip and/or handheld dynamometers), two independent predictors of mortality, can be performed only in awake and cooperative patients. Transcutaneous electrical/magnetic stimulation applied over motor nerves combined with the development of dedicated ergometer have recently been introduced in ICU patients in order to propose an early and non-invasive measurement of evoked force. The aim of this narrative review is to summarize the different tools allowing bedside force evaluation in ICU patients and the related experimental protocols. We suggest that non-invasive electrical and/or magnetic evoked force measurements could be a relevant strategy to characterize muscle weakness in the early phase of ICU and diagnose ICUAW.


Assuntos
Potenciais Evocados , Estudos Transversais , Ergometria/instrumentação , Força da Mão/fisiologia , Humanos , Unidades de Terapia Intensiva/organização & administração , Unidades de Terapia Intensiva/tendências , Debilidade Muscular/etiologia , Debilidade Muscular/fisiopatologia , Narração
13.
Phys Ther ; 101(7)2021 07 01.
Artigo em Inglês | MEDLINE | ID: mdl-33764475

RESUMO

OBJECTIVE: Impairments associated with cerebral palsy (CP) can affect gait quality and limit activity and participation. The purpose of this study was to quantify (1) which of 6 factors (pain, weakness, endurance, mental ability, safety concerns, balance) were perceived to limit walking ability the most in individuals with CP and (2) whether age or Gross Motor Function Classification System (GMFCS) level is related to that perception. METHODS: This cross-sectional study queried data from a gait laboratory database. Perceived walking limitation was quantified using a 5-point Likert scale ("never" to "all of the time"). Included were 1566 children and adults (mean age = 10.9 y [SD = 6.8]; range = 3.0-72.1 y) with CP (GMFCS level I: 502; II: 564; III: 433; IV: 67). RESULTS: Patients or caregivers perceived balance to limit walking ability to the greatest extent, followed by endurance, weakness, safety, mental ability, and pain. Balance was perceived to always limit walking ability in 8%, 22%, 30%, and 34% of individuals in GMFCS levels I through IV, respectively. Endurance was perceived to always limit walking ability in 5%, 13%, 13%, and 27% of individuals in GMFCS levels I through IV, respectively. There were minor differences in the perceived extent of limitation caused by the factors by GMFCS level. Only weak associations between age and pain, mental ability, safety, and balance were observed (Spearman rho = -0.13 to 0.24). CONCLUSION: Patients or caregivers perceived decreased balance and endurance to most strongly limit walking ability. Efforts should be made to clinically track how both perceived and objective measures of these limiting factors change with age and intervention. IMPACT: Following a patient- and family-centered care model, therapy that places greater emphasis on balance and cardiovascular endurance may have the greatest effect on walking ability for individuals with CP. Future research should quantify which therapeutic, surgical, and pharmacologic interventions minimize these impairments and optimize activity and participation. LAY SUMMARY: Balance and endurance are perceived to be the greatest factors limiting walking in people with CP. If you have CP, your physical therapist might emphasize balance and cardiovascular endurance to improve your walking ability.


Assuntos
Paralisia Cerebral/fisiopatologia , Transtornos Neurológicos da Marcha/fisiopatologia , Adolescente , Adulto , Criança , Pré-Escolar , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Debilidade Muscular/fisiopatologia , Medição da Dor , Resistência Física/fisiologia , Equilíbrio Postural/fisiologia , Adulto Jovem
14.
Thorax ; 76(6): 561-567, 2021 06.
Artigo em Inglês | MEDLINE | ID: mdl-33712505

RESUMO

PURPOSE: To assess the association between respiratory muscle weakness (RMW) at intensive care unit (ICU) discharge and 5-year mortality and morbidity, independent from confounders including peripheral muscle strength. METHODS: Secondary analysis of the prospective 5-year follow-up of the EPaNIC cohort (ClinicalTrials.gov: NCT00512122), limited to 366 patients screened for respiratory and peripheral muscle strength in the ICU with maximal inspiratory pressure (MIP) after removal of the artificial airway, and the Medical Research Council sum score. RMW was defined as an absolute value of MIP <30 cmH2O. Associations between RMW at (or closest to) ICU discharge and all-cause 5-year mortality, and key measures of 5-year physical function, comprising respiratory muscle strength (MIP), hand-grip strength (HGF), 6 min walk distance (6MWD) and physical function of the SF-36 quality-of-life questionnaire (PF-SF-36), were assessed with Cox proportional hazards and linear regression models, adjusted for confounders including peripheral muscle strength. RESULTS: RMW was present in 136/366 (37.2%) patients at ICU discharge. RMW was not independently associated with 5-year mortality (HR with 95% CI 1.273 (0.751 to 1.943), p=0.352). Among 156five-year survivors, those with, as compared with those without RMW demonstrated worse physical function (MIP (absolute value, cmH2O): 62(42-77) vs 94(78-109), p<0.001; HGF (%pred): 67(44-87) vs 96(68-110), p<0.001; 6MWD (%pred): 87(74-102) vs 99 (80-111), p=0.009; PF-SF-36 (score): 55 (30-80) vs 80 (55-95), p<0.001). Associations between RMW and morbidity endpoints remained significant after adjustment for confounders (effect size with 95% CI: MIP: -23.858 (-32.097 to -15.027), p=0.001; HGF: -18.591 (-30.941 to -5.744), p=0.001; 6MWD (transformed): -1587.007 (-3073.763 to -179.253), p=0.034; PF-SF-36 (transformed): 1.176 (0.144-2.270), p=0.036). CONCLUSIONS: RMW at ICU discharge is independently associated with 5-year morbidity but not 5-year mortality.


Assuntos
Cuidados Críticos/métodos , Unidades de Terapia Intensiva/estatística & dados numéricos , Força Muscular/fisiologia , Debilidade Muscular/fisiopatologia , Alta do Paciente/tendências , Insuficiência Respiratória/terapia , Músculos Respiratórios/fisiopatologia , Idoso , Progressão da Doença , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Debilidade Muscular/etiologia , Prognóstico , Estudos Prospectivos , Qualidade de Vida , Insuficiência Respiratória/complicações , Insuficiência Respiratória/fisiopatologia , Fatores de Risco , Inquéritos e Questionários , Fatores de Tempo
15.
Muscle Nerve ; 63(6): 897-901, 2021 06.
Artigo em Inglês | MEDLINE | ID: mdl-33759219

RESUMO

INTRODUCTION: Periodic paralysis (PP) is thought to be limited to episodes of muscle weakness, but there are reports of fibromyalgia-like pain in PP. We aimed to evaluate pain and comorbid sleep, fatigue, and mood disorders in PP patients. METHODS: We administered a cross-sectional survey to PP patients at the 2019 Periodic Paralysis Conference. The survey consisted of the Brief Pain Inventory, Widespread Pain Index, Pittsburgh Sleep Quality Index, Modified Fatigue Impact Scale, and ten-question Center for Epidemiologic Studies Depression Scale (CESD-10). Descriptive statistics for PP patients were calculated and compared with earlier studies. RESULTS: Forty-four individuals with PP took the survey. Of these patients, 52.3% reported a moderate to severe interference of pain on their lives, and 45.5% met the study criteria for fibromyalgia. Patients with SCN4A mutations had higher rates of fibromyalgia than the next most prevalent gene mutation, CACNA1S. In patients with pain, there were increased rates of comorbid fatigue, depression, and poor sleep quality. DISCUSSION: Pain, akin to fibromyalgia, is a significant symptom of PP and can affect quality of life. Pain in PP was more prevalent than in the general population, at a rate comparable with other chronic neuromuscular disease groups. PP patients could benefit from a multidisciplinary approach to assess their pain, sleep, fatigue, and mood.


Assuntos
Fibromialgia/complicações , Debilidade Muscular/complicações , Dor/etiologia , Paralisias Periódicas Familiares/complicações , Adulto , Estudos Transversais , Feminino , Fibromialgia/genética , Fibromialgia/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Debilidade Muscular/genética , Debilidade Muscular/fisiopatologia , Canal de Sódio Disparado por Voltagem NAV1.4/genética , Dor/genética , Dor/fisiopatologia , Paralisias Periódicas Familiares/genética , Paralisias Periódicas Familiares/fisiopatologia , Qualidade de Vida
16.
BMC Anesthesiol ; 21(1): 64, 2021 03 02.
Artigo em Inglês | MEDLINE | ID: mdl-33653264

RESUMO

BACKGROUND: Few studies have evaluated muscle strength in COVID-19 ICU survivors. We aimed to report the incidence of limb and respiratory muscle weakness in COVID-19 ICU survivors. METHOD: We performed a cross sectional study in two ICU tertiary Hospital Settings. COVID-19 ICU survivors were screened and respiratory and limb muscle strength were measured at the time of extubation. An ICU mobility scale was performed at ICU discharge and walking capacity was self-evaluated by patients 30 days after weaning from mechanical ventilation. RESULTS: Twenty-three patients were included. Sixteen (69%) had limb muscle weakness and 6 (26%) had overlap limb and respiratory muscle weakness. Amount of physiotherapy was not associated with muscle strength. 44% of patients with limb weakness were unable to walk 100 m 30 days after weaning. CONCLUSION: The large majority of COVID-19 ICU survivors developed ICU acquired limb muscle weakness. 44% of patients with limb weakness still had severely limited function one-month post weaning.


Assuntos
COVID-19/complicações , Debilidade Muscular/etiologia , Idoso , Braço/fisiopatologia , COVID-19/fisiopatologia , Estudos Transversais , Feminino , Seguimentos , França , Humanos , Perna (Membro)/fisiopatologia , Pulmão/fisiopatologia , Masculino , Pessoa de Meia-Idade , Debilidade Muscular/fisiopatologia , Estudos Retrospectivos , SARS-CoV-2
17.
NeuroRehabilitation ; 48(2): 243-245, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33664161

RESUMO

BACKGROUND: Progressive muscle weakness is a feature of neuromuscular diseases (NMDs), a heterogeneous group of conditions with variable onset, presentation and prognosis that affect both children and adults. Respiratory muscle weakness compromises respiratory function and may lead to respiratory failure. OBJECTIVE: To assess the effects of respiratory muscle training (RMT) in adults and children with NMD. METHODS: A Cochrane Review by Silva et al. was summarized with comments. RESULTS: Eleven studies involving 250 randomized participants with NMD were included. While the studies showed that RMT may lead to improvements in lung function and respiratory muscle strength in people with ALS and DMD, this was not a consistent finding. The evidence from all the included trials was of low or very low certainty. CONCLUSIONS: There may be some improvement in lung capacity and respiratory muscle strength following RMT in some NMD. There appears to be no clinically meaningful effect of RMT on physical functioning and quality of life in ALS. The low certainty of the evidence means that the results need to be interpreted with caution.


Assuntos
Exercícios Respiratórios/métodos , Força Muscular/fisiologia , Doenças Neuromusculares/terapia , Músculos Respiratórios/fisiologia , Adulto , Criança , Feminino , Humanos , Masculino , Debilidade Muscular/fisiopatologia , Debilidade Muscular/terapia , Doenças Neuromusculares/fisiopatologia , Qualidade de Vida , Testes de Função Respiratória/métodos
18.
J Peripher Nerv Syst ; 26(1): 83-89, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33555098

RESUMO

Guillain-Barré syndrome (GBS) is the most common cause of acute flaccid paralysis in children. The objective of this study was to investigate the preceding infections, clinical, serological and electrophysiological characteristics and outcome of childhood GBS in Bangladesh. We included 174 patients with GBS aged <18 years from a prospective cohort in Bangladesh between 2010 and 2018. We performed multivariate logistic regression to determine the risk factors for poor outcome. Among 174 children with GBS, 74% (n = 129) were male. Around half of the patients (49%, n = 86) had severe muscle weakness, 65% (n = 113) were bedbound (GBS disability score 4) and 17% (n = 29) patients required mechanical ventilation at admission. Campylobacter jejuni serology and anti-GM1 IgG antibody were positive in 66% and 21% of the patients respectively. One hundred and forty-three (82%) patients did not receive standard treatment and half of them recovered fully or with minor deficits at 6-month. Twenty patients (11%) died throughout the study period. At 3-month of onset of weakness, complete recovery or recovery with minor deficit was significantly higher in demyelinating GBS patients compared to axonal GBS patients (86% vs 51%, P = .001). Cranial nerve palsy (OR = 4.00, 95%CI = 1.55-10.30, P = .004) and severe muscle weakness (OR = 0.16, 95%CI = 0.06-0.45, P = .001) were the important risk factors of poor outcome in children with GBS. Further large-scale studies are required for better understanding of factors associated with mortality and morbidity in childhood GBS.


Assuntos
Anticorpos Antibacterianos/sangue , Autoanticorpos/sangue , Doenças dos Nervos Cranianos , Síndrome de Guillain-Barré , Debilidade Muscular , Adolescente , Bangladesh , Campylobacter jejuni/imunologia , Criança , Pré-Escolar , Doenças dos Nervos Cranianos/diagnóstico , Doenças dos Nervos Cranianos/etiologia , Doenças dos Nervos Cranianos/fisiopatologia , Feminino , Gangliosídeos/imunologia , Síndrome de Guillain-Barré/sangue , Síndrome de Guillain-Barré/complicações , Síndrome de Guillain-Barré/diagnóstico , Síndrome de Guillain-Barré/fisiopatologia , Humanos , Lactente , Masculino , Debilidade Muscular/diagnóstico , Debilidade Muscular/etiologia , Debilidade Muscular/fisiopatologia , Prognóstico , Estudos Prospectivos , Fatores de Risco , Análise de Sobrevida
19.
Neuromuscul Disord ; 31(4): 265-280, 2021 04.
Artigo em Inglês | MEDLINE | ID: mdl-33610434

RESUMO

This study aims to determine clinically relevant phenotypic differences between the two most common phenotypic classifications in dysferlinopathy, limb girdle muscular dystrophy R2 (LGMDR2) and Miyoshi myopathy (MMD1). LGMDR2 and MMD1 are reported to involve different muscles, with LGMDR2 showing predominant limb girdle weakness and MMD1 showing predominant distal lower limb weakness. We used heatmaps, regression analysis and principle component analysis of functional and Magnetic Resonance Imaging data to perform a cross-sectional review of the pattern of muscle involvement in 168 patients from the Jain Foundation's international Clinical Outcomes Study for Dysferlinopathy. We demonstrated that there is no clinically relevant difference in proximal vs distal involvement between diagnosis. There is a continuum of distal involvement at any given degree of proximal involvement and patients do not fall into discrete distally or proximally affected groups. There appeared to be geographical preference for a particular diagnosis, with MMD1 being more common in Japan and LGMDR2 in Europe and the USA. We conclude that the dysferlinopathies do not form two distinct phenotypic groups and therefore should not be split into separate cohorts of LGMDR2 and MM for the purposes of clinical management, enrolment in clinical trials or access to subsequent treatments.


Assuntos
Miopatias Distais/diagnóstico , Atrofia Muscular/diagnóstico , Distrofia Muscular do Cíngulo dos Membros/diagnóstico , Adolescente , Adulto , Criança , Pré-Escolar , Progressão da Doença , Feminino , Humanos , Lactente , Recém-Nascido , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Debilidade Muscular/fisiopatologia , Fenótipo , Adulto Jovem
20.
Aging (Albany NY) ; 13(5): 7247-7258, 2021 02 26.
Artigo em Inglês | MEDLINE | ID: mdl-33640880

RESUMO

INTRODUCTION: Low muscle function determined unfavorable clinical outcome than low muscle mass; nevertheless, comparison of detrimental parameters among dynapenia, presarcopenia and sarcopenia was sparse. We hypothesized that adiposity is implicated in low muscle function related adverse events. METHODS: We recruited community elders to measure handgrip strength and walking speed. Using bioelectronics impedance analyzer to examine body compositions. The faller is indicated of having a fall event in the past one year. Associations of different obesity parameters, metabolic syndrome (MetS) and fall among the groups were analyzed. RESULTS: Among 765 participants, the dynapenia group had higher metabolic profiles, body fat percentage (BFP), waist circumference, and fat to muscle ratio (FMR) than the other groups, whereas the presarcopenia subjects had the lowest obesity parameters. The fallers tended to have poorer muscle function than non-fallers (p<0.001). The dynapenia individuals had the highest risk for MetS (odds ratio [OR]= 5.79; 95% confidence interval [CI]= 2.45-13.73), and the highest fall risk (OR= 3.11; 95% CI=1.41-6.87). Among obesity parameters, FMR had better diagnostic performance to estimate low muscle function, followed by BFP. CONCLUSION: Dynapenia individual had higher risk of obese-related adverse events. Increased adiposity irrespective of muscle mass is relevant to reduced muscle function among elders.


Assuntos
Adiposidade , Debilidade Muscular/patologia , Músculo Esquelético/patologia , Sarcopenia/patologia , Acidentes por Quedas , Idoso , Estudos Transversais , Feminino , Força da Mão , Humanos , Masculino , Síndrome Metabólica/patologia , Síndrome Metabólica/fisiopatologia , Debilidade Muscular/fisiopatologia , Músculo Esquelético/fisiopatologia , Obesidade/patologia , Obesidade/fisiopatologia , Fatores de Risco , Sarcopenia/fisiopatologia
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