Ce mémoire propose une analyse des justifications du paternalisme étatique dans les cas d’acrasie. Nous explorerons d’abord quelques théories et conceptions de l’acrasie, démontrant la nécessité de développer une conception de l’agent acratique qui soit en quelque sorte subdivisible. Mous exposerons par la suite en quoi cette conception de l’individu remet en question certains présupposés fondamentaux du libéralisme. Notre second chapitre sera consacré à une redéfinition de certains principes libéraux en fonction de notre conception de l’individualité. Cette redéfinition nous permettra d’expliquer comment l’intervention étatique de type paternaliste peut être parfois justifiée d’un point de vue libéral. Le cœur de notre argumentation mettra l’accent sur l’importance pour l’État d’assurer l’autonomie de ses citoyens en concevant leur raison comme faculté d’intégration personnelle. Notre troisième chapitre tentera d’explorer divers exemples de cas concrets où les principes développés plus tôt pourront s’appliquer. / The purpose of this master thesis is to analyze paternalistic justifications for State intervention in cases of acrasia. We first start by exploring some theories and conceptions of acrasia showing the necessity to develop a conception of the acratic agent as somehow subdivisible. We then show that this conception of the individual challenges some of the most central presuppositions for political liberalism. Our second chapter means to redefine some liberal principles according to our conception of individuality. This redefinition will enable us to explain how paternalistic State intervention can sometime be justified from a liberal point of view. Our argumentation will focus on the importance for the State to ensure the autonomy of its citizens by securing the role of their reason as a faculty of personal integration. Our third chapter means to explore diverse practical cases in which the principles developed earlier can apply.

Este tesis fue publicado como artículo científico en la Revista Endocrinología y Nutrición (DOI: 10.1016/j.endonu.2015.12.008) / Objetivo: Evaluar la asociación entre la fuerza muscular (FM) y rendimiento físico (RF) en adultos mayores con y sin diabetes de un hospital público de Lima, Perú. Pacientes y método: Estudio transversal en pacientes ≥ 60 años con y sin diabetes. Se midió FM con dinamómetro manual y RF con prueba “Timed get-up-and-go”. Se determinó el estado nutricional con el índice de masa corporal, el porcentaje de grasa corporal con bioimpedanciómetro manual y se evaluó el consumo proteico mediante recordatorio de 24 horas. Además, se registró edad, género, antecedente de hospitalización y suplementación. Se evaluó la asociación con razones de prevalencias ajustadas (RPa). Resultados: Se evaluaron 139 pacientes con diabetes (26,6% con bajo RF y 13,7% con FM disminuida) y 382 sin diabetes (36,6% con bajo RF y 23,0% con FM disminuida). No se halló asociación de DM2 con FM (RPa: 0,99; IC95%: 0,67-1,57) ni RF (RPa: 1,13; IC95%: 0,84-1,52). Tampoco se asoció el consumo proteico ni de suplementos (p> 0,05), pero sí el antecedente de hospitalización, la edad, el género, el estado nutricional y el porcentaje de grasa corporal (p< 0,05). Conclusiones: No se encontró asociación entre DM2, FM y RF. Sin embargo, el bajo RF se asoció con ser mujer y presentar sobrepeso/obesidad; y la FM disminuida con tener alto porcentaje de grasa corporal y bajo peso. Además, ambos se relacionaron con el aumento de edad y tener antecedente de hospitalización. / Objective: To compare the association between physical performance (PP) and muscle strength (MS) in older people with and without diabetes in a public hospital of Lima, Peru. Patients and methods: Cross sectional analysis of 60 years and older patients with and without diabetes. MS was measured with a handheld dynamometer and PP with the “Timed get-up-and-go” Test. The nutritional status was determined using the body mass index, the body fat percentage using a handheld fat loss monitor and the protein intake using the 24 hours Recall. Furthermore, age, gender, history of hospitalization and supplementation were registered. The association was evaluated with adjusted prevalence ratios. Results: 139 patients with diabetes (26.6% had low PP and 13.7% diminished MS) and 382 counterparts without diabetes (36.6% had low PP and 23.0% diminished MS) were evaluated. No association was found between MS and T2D (aPR: 0.99; IC95%: 0.67- 1.57), nor with PP (aPR: 1.13; IC95%: 0.84-1.52). Protein and supplement consumption were not related either (p> 0.05); however, age and history of hospitalization, gender, nutritional status and body fat percentage were linked (p< 0.05). Conclusions: No association was found between T2D, MS and PP. Nevertheless, low PP was associated with female sex and overweight/obesity; and diminished MS was linked to high body fat percentage and underweight. Moreover, MS and PP were related to age and history of hospitalization.

Pacientes com Insuficiência Cardíaca (IC) e fraqueza da musculatura inspiratória (FMI) apresentam limitação na realização de atividades da vida diária devido à dispnéia e cansaço. Objetivo. Avaliar em pacientes com miocardiopatia hipertensiva e FMI o efeito do treinamento da musculatura inspiratória (TMI) sobre a força e resistência dos músculos respiratórios, bem como na atividade simpática, hemodinâmica e a qualidade de vida. Métodos. Vinte e sete pacientes foram alocados em seqüência em dois grupos: Grupo Controle (não realizavam o treinamento) e Grupo TMI. Os pacientes incluídos no Grupo TMI participaram de um programa de exercícios respiratórios com o Threshold Inspiratório durante 12 semanas, sete sessões por semana com duração de 30 minutos por sessão, com carga de 30% da pressão inspiratória máxima (Pimáx) de repouso, ajustada mensalmente. Antes e após 12 semanas ambos os grupos foram avaliados quanto a Pimáx de repouso, variáveis hemodinâmicas em repouso: pressão arterial sistólica (PAS) e diastólica (PAD), freqüência cardíaca (FC), resistência vascular periférica (RVP) e débito cardíaco (DC); capacidade funcional cardiorrespiratória: consumo de oxigênio (VO2), quociente respiratório de produção de gás carbônico (VCO2), limiar anaeróbio (LA), ponto de compensação respiratório (PCR) e duração da atividade física, atividade simpática nervosa periférica (ANSP- microneurografia) e central (variabilidade da FC- componentes LF e HF), fluxo de sangue para o antebraço (FSA) e qualidade de vida (Questionário de Minessota). Resultados. Após 12 semanas não foram encontradas alterações significativas em qualquer parâmetro avaliado nos pacientes incluídos no Grupo Controle. Nos pacientes alocados no Grupo TMI foi possível observar alterações significativas nos seguintes parâmetros: aumento da Pimáx (basal 59,2 cmH2O ± 4,9 vs pós 87,5cmH2O ± 6,5); aumento do VO2 pico (14,4 ml/kg/min ± 1,0 vs 18,9 ml/kg/min ± 1,16); diminuição do VE/VCO2 pico (35,8 ml/kg/min ± 0,4 vs 32,5 ml/kg/min ± 0,8); diminuição do componente LF em valor absoluto (607,2 mms2 ± 153,9 vs 263,5 mms2 ± 53,6), e do componente LF em valor normalizado (6,2 mms2 ± 1,7 vs 5,0 mms2 ± 1,1) e aumento do componente HF em valor absoluto (48,41 mms2 ± 4,4 vs 56,7 mms2 ± 4,4); diminuição da ANSP (37,1 ± 3 disparos /min. vs 29,5 ± 2,3 disparos por minuto) e diminuição na pontuação nos domínio físico (20,2 pontos ± 3,5 vs 7,6 pontos ± 2,2) e geral (23,6 pontos ± 3,8 vs 9,2 pontos ± 2,4) do Questionário de Minessota. Conclusão. O TMI correlacionou-se com o aumento de força e resistência dos músculos respiratórios, melhora da capacidade cardiorrespiratória, diminuição da atividade simpática cardíaca e periférica, acarretando uma melhora na qualidade de vida de pacientes com IC hipertensiva. O TMI pode ser considerado um método seguro, prático e eficaz e uma alternativa no tratamento de pacientes com IC de etiologia hipertensiva / Patients with heart failure and inspiratory muscle weakness (IMW) experience limitations in performing their routine activities due to dyspnea and fatigue. Methods and Results: Twenty-seven patients were sequentially allocated to one of two groups: a control group in which inspiratory muscle training (IMT) was not provided and the IMT group. Patients included in the IMT group participated in a program of respiratory exercises with inspiratory threshold loading consisting of seven 30-minute sessions a week for a period of 12 weeks, with a monthly increase of 30% in maximal inspiratory pressure (Pimax) at rest. Prior to and following the 12-week evaluation period, both groups were assessed for Pimax measured at rest; oxygen consumption (VO2), ratio of ventilation to carbon dioxide production (VE/VCO2), peripheral and cardiac nervous sympathetic activity, and quality of life. Results: In the patients allocated to the IMT group, significant alterations were recorded: an increase in Pimax (59,2 ± 4,9 cmH2O at baseline compared to 87,5 ± 6,5 cmH2O following therapy),; an increase in peak oxygen consumption (14,4 ± 1,03 versus 18,9 ± 1,16 ml/kg/min); a reduction in peak VE/VCO2 (35,8 ± 0,4 versus 32,5 ± 0,8 ml/kg/min), a reduction in the low-frequency (LF) component (6,18 ± 1,7 versus 5,04 ± 1,1 mms2) and an increase in the normalized value of the high frequency (HF) component (48,4 ± 4,4 versus 56,7 ± 4,4 mms2), a reduction in peripheral sympathetic activity (37,1 ± 3 versus 29,5 ± 2,3 bursts/minute) and a reduction in the physical domain (20,2 ± 3,5 versus 7,6 ± 2,2 points) and general scores (23,6 ± 3,8 versus 9,2 ± 2,4 points) of the Minnesota instrument. Conclusion: IMT is associated with an increase in respiratory muscle strength and endurance, an improvement in cardiorespiratory capacity and a reduction in central and peripheral sympathetic activity, resulting in an improvement in the quality of life of patients with hypertensive heart disease

Les myopathies inflammatoires (MI) représentent un groupe hétérogène de maladies caractérisépar une faiblesse musculaire chronique et symétrique associée à une augmentation du taux sérique decréatine phosphokinase (CPK). Les MI sont actuellement subdivisées en 5 entitées : les dermatomyosites,les myopathies nécrosantes auto-immunes, la myosite à inclusion, la polymyosite et les myosites dechevauchement. A ce jour, le diagnostic des MI repose sur l’association de signes cliniques, decaractéristiques anatomopathologiques sur la biopsie musculaire et la présence d’auto-anticorps (aAc). Eneffet, la découverte d’aAc spécifiques et/ou associés aux myosites (MSA/MAA) a considérablementamélioré le diagnostic et le pronostic de la maladie. Cependant, un nombre non négligeable de patientsatteints de MI sont séronégatifs pour les MSA/MAA connus. Par ailleurs, la biopsie musculaire nécessaireau diagnostic est parfois guidée par imagerie par résonance magnétique (IRM), bien qu’il n’ait pas étéprouvé que les données d’imagerie soient corrélées aux signes histologiques. Enfin, le traitement des MIrepose sur l’utilisation d’immunosuppresseurs systémiques, une approche non spécifique de laphysiopathologie de la maladie. Les modèles animaux de MI les plus utilisés sont induits et nonspontanés : ils reposent principalement sur l’immunisation d’animaux contre des protéines telles que lamyosine, la protéine C ou l’histidyl-tRNA synthétase.Les souris NOD (non obese diabetic) sont le modèle classique d’étude du diabète de type 1.Lorsque ces souris sont invalidées pour la voie de costimulation lymphocytaire ICOS/ICOSL, les souris nedéveloppent plus de diabète mais présentent alors une atteinte musculaire. Dans ce travail, nous avonsétudié le phénotype et caractérisé l’atteinte musculaire des souris NOD Icos-/- et NOD Icosl-/-. Nous avonsainsi établi le 1er modèle murin spontané de MI, dont la physiopathologie est médiée par leslymphocytes T CD4+ et la sécrétion d’IFN-γ. Par ailleurs, ces souris présentent un déficit en lymphocytes Trégulateurs. Nous avons également identifié 4 auto-antigènes (aAg) candidats cibles d’aAc chez ces souris.La recherche des aAc correspondants aux aAg orthologues dans le sérum des patients atteints de MI apermis d’identifier, pour l’un d’entre eux, une minorité d’individus séropositifs grâce au développementd’un nouveau test ALBIA (addressable laser bead immunoassay). Il pourrait donc s’agir d’un nouveaubiomarqueur. Dans la perspective de nouvelles évaluations thérapeutiques, nous avons établi desdonnées préliminaires montrant que l’interleukine 2 à faibles doses permet de retarder l’apparition de lamaladie. Enfin, nous avons mis à profit ce modèle et démontré la corrélation entre les données généréespar IRM et par analyse histologique de l’inflammation, confortant le rôle de cette technique d’imagerie àla fois pour le diagnostic et le suivi des MI. / Inflammatory Myopathies (IM) are a heterogeneous group of diseases characterized bychronic and symmetrical muscle weakness associated to increased creatine phosphokinase (CPK)levels, according to entity concerned. Currently, IM are divided into 5 main entities:dematomyositis, immune-mediated necrotizing myopathies, inclusion body myositis, polymyositisand overlap myositis. Nowadays, IM diagnosis is based on clinical signs associated to pathologicfeatures on muscle biopsy and presence of auto-antibodies (aAb). Indeed, the discovery of myositisspecific and/or associated auto-antibodies (MSA/MAA) had considerably improve disease diagnosisand prognosis. However, substantial proportion of IM patients do not display any knownMSA/MAA. Furthermore, diagnosis requires muscle biopsy. This biopsy is sometimes guided bymagnetic resonance imaging (MRI), even though correlation between MRI findings and pathologicalfeatures is not established. Lastly, therapeutics used in IM treatment are systemicimmunosuppressive agents, i.e. not specific to IM pathophysiology. Animal models of IM are mainlybased on active immunization against different proteins as myosin, C protein orhistidyl-tRNA synthetase, while spontaneous models are required to identify pathophysiologicalmechanisms that new therapeutics should target.NOD (non obese diabetic) mice are the main model of type 1 diabetes. When invalidatedfor ICOS/ICOSL costimulation pathway, these mice do not develop diabetes but present musculardisorders. In this work, we study Icos-/- and Icosl-/- NOD mice phenotype and characterize theremuscle lesion. Thus, we have established this model as the first paradigm of IM. Pathophysiologicalstudy in these mice demonstrated that disease is CD4+ T cell dependent and associated to IFN-γproduction. Furthermore, we shown a quantitative defect in regulatory T cells. We have alsoidentified 4 candidate autoantigens (aAg) in Icos-/- and Icosl-/- NOD mice. Searching forcorresponding aAb against ortholog proteins in patients with IM, we identified for one of them, alow percentage of seropositive individuals using a new ALBIA (addressable laser beadimmunoassay). It could be identified as a new biomarker. In order to evaluate new therapies, weestablished preliminary data showing that low dose interleukin 2 therapy allow to delay diseaseonset. Lastly, we took advantage of this new model to demonstrate the correlation betweenMRI findings and histological inflammation features, confirming the valuable role of MRI for thediagnosis and monitoring of IM.

Introduction: In elderly seniors (>80 years), respiratory function may be compromised when, in addition to the presence of comorbidity and loss of mobility, there is also reduced respiratory muscle (RM) strength. The literature has shown that RM training could be an effective method to improve RM function and prevent clinical deterioration, particularly in population with RM weakness.The main purpose of this paper was to assess the effectiveness of RM training on the respiratory muscle strength and endurance of institutionalized elderly women with functional impairment. Method: Fifty-four residents (mean=85 years, SD=6.7) were randomly assigned to either a control (n=27) or training (n=27) group. A supervised training program was developed with Threshold®IMT, five times per week for 6-weeks. The main variables of the intervention were: maximum inspiratory pressure (PImax), maximum expiratory pressure (PEmax) and maximal voluntary ventilation (MVV), all of which were measured at weeks 0, 4, 7 and 10. Results: Statistical analysis revealed no significant differences in PImax (F3,114=1.04, p=0.368, R2=0.027), PEmax (F3,114=1.86, p=0.14, R2=0.047) and MVV (F3,114=1.74, p=0.162, R2=0.044) between the two groups after the intervention. However, the workload significantly improved with the training sessions (F5,100=72.031, p<0.001, R2=0.791). Conclusion: In a 6-week interval-based training program, the threshold loading device does not significantly improve parameters related to RM strength and endurance of the study population.

Introduction: In elderly seniors (>80 years), respiratory function may be compromised when, in addition to the presence of comorbidity and loss of mobility, there is also reduced respiratory muscle (RM) strength. The literature has shown that RM training could be an effective method to improve RM function and prevent clinical deterioration, particularly in population with RM weakness.The main purpose of this paper was to assess the effectiveness of RM training on the respiratory muscle strength and endurance of institutionalized elderly women with functional impairment. Method: Fifty-four residents (mean=85 years, SD=6.7) were randomly assigned to either a control (n=27) or training (n=27) group. A supervised training program was developed with Threshold®IMT, five times per week for 6-weeks. The main variables of the intervention were: maximum inspiratory pressure (PImax), maximum expiratory pressure (PEmax) and maximal voluntary ventilation (MVV), all of which were measured at weeks 0, 4, 7 and 10. Results: Statistical analysis revealed no significant differences in PImax (F3,114=1.04, p=0.368, R2=0.027), PEmax (F3,114=1.86, p=0.14, R2=0.047) and MVV (F3,114=1.74, p=0.162, R2=0.044) between the two groups after the intervention. However, the workload significantly improved with the training sessions (F5,100=72.031, p<0.001, R2=0.791). Conclusion: In a 6-week interval-based training program, the threshold loading device does not significantly improve parameters related to RM strength and endurance of the study population.

This thesis provides a presentation and analysis of Simone Weil’s articulation of the weakness of love vis-à-vis our encounters with others. It does so both as a way to better examine Christian theological claims about the importance of weakness and as a way of accessing the tensional depths of Simone Weil’s work from an often neglected angle. To achieve this engagement, I read Weil alongside Jean Vanier, whose life and work share with Weil a profound emphasis on the centrality of weakness for meeting others truthfully and lovingly. In Chapter One I draw on both Weil and Vanier to present their shared critique of relationships that are reduced to a pursuit of power and influence over others. I call such relationships “territorial” because they posit human beings as competitors for two-dimensional territory and therefore envision human relationships in essentially competitive terms. Chapter Two is a detailed presentation of Weil’s constructive work on the weakness of love that emphasizes her account of the impersonal, non-egoistic, and unattached relationships we ought to pursue with others. Weil claims that we experience the fullness of reality through an uncompromising embrace of all things, which we can only accomplish through the removal of ourselves in the face of others. We get concrete development of these themes in Weil’s presentation of neighbour love and friendship, which she understands as a total openness to another’s position, circumstance, and being. In the third chapter, I use the theme of communion in Vanier’s work to call into question the way Weil demands our total surrender to the other. I suggest that an absent self cannot be truly weak and vulnerable before others. The central problem, I suggest, is a conception in which any sort of positive presence is necessarily an obstacle and any imposition of oneself necessarily competitive. / Thesis / Master of Arts (MA) / In this thesis, I present and examine Simone Weil’s understanding of how we might avoid doing violence to other people and instead treat other people with love and compassion. The first chapter reads Weil together with Jean Vanier to examine the various ways that power and the attempt to dominate others dictates our relationships with others. In the second chapter I systematically present Weil’s understanding of how we might, through a certain kind of personal death, transport ourselves wholly into the perspectives of other people and thereby practice true compassion towards them. In the third and concluding chapter I use Vanier’s compelling presentation of human flourishing as consisting of heart-to-heart interdependent relationships to critique the refusal of being dependent on others that is both implicit and explicit in Weil’s account of compassion.

Background Critical illness myopathy (CIM) and polyneuropathy (CIP) are a common complication of critical illness. Both cause intensive-care-unit-acquired (ICU-acquired) muscle weakness (ICUAW) which increases morbidity and delays rehabilitation and recovery of activities of daily living such as walking ability. Focused physical rehabilitation of people with ICUAW is, therefore, of great importance at both an individual and a societal level. A recent systematic Cochrane review found no randomised controlled trials (RCT), and thus no supporting evidence, for physical rehabilitation interventions for people with defined CIP and CIM to improve activities of daily living. Therefore, the aim of our study is to compare the effects of an additional physiotherapy programme with systematically augmented levels of mobilisation with additional in-bed cycling (as the parallel group) on walking and other activities of daily living.

Étant pourtant un phénomène extrêmement répandu, la procrastination a très peu fait l’objet d’études philosophiques. Inversement, la faiblesse de la volonté et les différents phénomènes qu’on lui rattache sont encore aujourd’hui étudiés en profondeur comme des cas paradigmatiques de défaillance de la rationalité pratique. Or, la procrastination présente un cas de défaillance de la rationalité pratique particulier dans notre rapport avec le temps, rapport qui est très souvent laissé de côté dans l’étude de la faiblesse de la volonté. Après avoir présenté dans le premier chapitre les phénomènes majeurs associés à la faiblesse de la volonté et les problématiques qu’ils soulèvent, dans le deuxième chapitre, nous examinerons leurs liens avec la procrastination. Nous verrons comment la procrastination, avec son emphase sur le caractère temporel de notre agentivité, permet de mieux saisir et d'expliquer les problématiques soulevées par la faiblesse de la volonté en les insérant dans un cadre explicatif plus large. / Even if procrastination is extremely widespread, it is still rarely the subject of philosophical study. Conversely, weakness of the will and the various phenomena which are related to it are still extensively studied as paradigmatic cases of failure of our practical rationality. However, procrastination seems to be a failure of our practical rationality related to our relationship with time, a relationship that is often overlooked in the study of weakness of will. After presenting, in the first chapter, the major phenomena associated with weakness of will and the issues they raise, we will examine their relationship with procrastination in the second chapter. We'll see how procrastination, with its emphasis on the temporal nature of our agency helps us to better understand and explain the issues raised by weakness of will by inserting them in a broader explanatory framework.

Introdução: As disferlinopatias são doenças genéticas causadas por alterações no gene da disferlina (DYSF), também denominadas distrofia muscular de cinturas (DMC) do tipo 2B, sendo a segunda em frequência em diversos países. A determinação de biomarcadores de função muscular desta doença se faz necessária. Objetivo: Estudo de caracterização da força e da função muscular nas disferlinopatias para estabelecer biomarcadores de habilidades motoras. Método: Amostra de 40 pacientes com dados de prontuário de força muscular (Medical Research Council - MRC), índice MRC, tempo de deambulação de 10 metros e, escalas de Vignos, Egen Klassifikation, Avaliação Funcional para distrofia muscular de Duchenne (FES-DMD) e North Star Ambulatory Assessment adaptada (a-NSAA). Resultados: Prevalência da disferlinopatia de 25,5% no Centro de Pesquisa sobre o Genoma Humano e Células Tronco, idade média de 36,5 anos, 52,5% do sexo masculino e 75% deambuladores. Músculos mais acometidos: abdominal, glúteo, íliopsoas, isquiotibial, quadríceps femoral, tibial anterior e deltoide médio. Correlação forte entre MRC e tempo de deambulação de 10 metros (média r=0,77) e, muito forte da MRC distal dos MMII com a-NSAA (r=0,90). Interação da MRC dos membros superiores (MMSS) e membros inferiores (MMII) entre os segmentos proximal e distal (p < 0,001), sendo mais evidente em MMSS do que em MMII. Taxa variável de progressão da doença, com 60% dos pacientes moderadamente ou gravemente afetados com menos de 12 anos de doença. Conclusão: Os achados caracterizam o padrão de fraqueza muscular dos brasileiros com disferlinopatia como proximal e distal dos MMII, com comprometimento associado da região proximal dos MMSS, além de elucidar as habilidades motoras em relação ao processo de locomoção. A forte correlação encontrada entre a força muscular, o tempo de deambulação de 10 metros e a escala a-NSAA, associada ao acompanhamento da evolução do desempenho de alguns grupos músculos podem fornecer um biomarcador adequado para o estudo da doençaCharacterization of muscle strength and function in Brazilian subjects with dysferlinopathy / Introduction: Dysferlinopathies are genetic diseases, caused to changes in the disferlina gene (DYSF), also named limb-girdle dystrophy type 2B, that is the second one in frequency in several countries. The small number of biomarkers of functional performance researches brings the need for studies in this area. Objective: This study characterizes muscle strength and function in subjects with dysferlinopathy to establish biomarkers of motor skills. Method: Data were available from 40 patients and included muscle strength assessment using the Medical Research Council (MRC) power scale, MRC index, timed motor performances for walking and data from the Vignos, Egen Klassifikation, Functional Assessment for Duchenne muscular dystrophy (FES-DMD) and the adapted North Star Ambulatory Assessment (a-NSAA) scales. Results: The prevalence of dysferlinopathy was 25.5% in the Centre for the Study of the Human Genome and Stem Cells (CEGH-CEL), the mean cohort age was 36.5 years, 52.5% were males and 75% were walkers. The weaker muscle found were the abdominal, gluteus, iliopsoas, hamstrings, quadriceps femoris, tibialis anterior and medial deltoid. Strong correlations were observed between the MRC power score and walking time (r = 0.77) and very strong between the MRC distal lower limb power score and a-NSAA (r = 0.90). Interactions of MRC scores were observed between the upper and lower limbs and the proximal and distal regions (p < 0.001) but were more evident in the upper limbs. The disease progression rates were variable with 60% of patients moderately or severely affected after more than 12 years since diagnosis. Conclusion: These findings suggest the pattern of muscular weakness in Brazilians with dysferlinopathy is predominantly in the lower limbs (proximal and distal) with associated involvement of the proximal upper limbs and elucidates the motor abilities in relation to locomotion. Due to the strong correlation with muscle strength, the walking time and the a-NSAA scale, in association with monitoring the evolution of the performance of some specific muscles can provide a suitable biomarker for the study of the disease