La loi du 11 février 2005 a permis de changer le regard que nous portons sur le handicap. Parallèlement à l'évolution des modèles et définitions, les associations de personnes en situation de handicap (PSH) continuent à se battre pour une société plus accessible. C'est dans ce contexte que nos travaux de recherche revendiquent l'intérêt d'une conception de l'innovation qui prend en compte les besoins de ces utilisateurs considérés comme une population de niche de la part des industriels. En effet, si l'on considère que les PSH représentent environ 50 millions d'européens, ce marché est-il encore considéré comme un marché de niche ? L'analyse du marché des technologies pour l'autonomie montre qu'il existe un réel champ d'action pour une démarche d'innovation centrée sur les besoins des PSH afin de pallier les manques existants. Pouvoir définir les caractéristiques et besoins de ces utilisateurs cibles, nécessite alors de comprendre et de caractériser ce qui crée une situation de handicap. Enfin, certains besoins spécifiques des PSH ont déjà donné lieu à des inventions et des progrès technologiques dont la majorité de la population dispose aujourd'hui (e.g. la télécommande, les robinets mitigeurs, la reconnaissance vocale, etc.). Nous essaierons donc de démontrer au travers d'expérimentations menées en partenariat avec l'Association Française contre les Myopathies que la connaissance des besoins de ces utilisateurs cibles peut donc être considérée comme une source potentielle d'innovation pour tous.

Assessment in patients with NMD consists principally of measures of motor impairment since they are well known by clinicians and their measures do not require much equipment. The conventional treatments in patients with NMD are above-all focused on the diminution of motor impairments by maintaining or improving joint mobility, muscle strength and endurance. Nevertheless, a reduction of motor impairments does not directly lead to a higher ability in performing daily activities. Therefore, activity limitations should be measured specifically. A new scale of activity limitations was first developed in children and adults with NMD. The use of the Rasch model provided a scale to assess the fundamental psychometric qualities. Secondly, relationships between motor impairments and activity limitations were investigated to verify the assumption that reduced motor impairments do not necessarily lead to higher activity levels. Finally, to complete the investigation of psychometric qualities, a longitudinal study of the developed questionnaire was carried out to evaluate its sensitivity to change. Chapter 1 presents the development of ACTIVLIM, a Rasch-built measure of activity limitations and its validation in children and adults with NMD. ACTIVLIM is a self-reported questionnaire that assesses the difficulties adult patients and parents of affected children perceive when they or their children perform daily activities. This questionnaire originally included 126 daily activities and was submitted to 369 patients. The Rasch model selected 22 daily activities to define a linear and unidimensional measure of activity limitations in patients with NMD. The validity and the reproducibility of the results were also studied. A second section of Chapter 1 demonstrates why the measure of activity limitations in children with NMD as assessed using the ACTIVLIM questionnaire is based upon the perception of their parents. A third section of Chapter 1 compares the difficulties self-perceived by the patients with the difficulties observed by external examiners. The agreement between both measures is very good, indicating that the use of ACTIVLIM as a self-reporting questionnaire is a valid method to assess activity limitations in patients with NMD. Chapter 2 investigates the relationships between motor impairments and activity limitations as measured with the ACTIVLIM questionnaire. As the anatomical basis and pathophysiology are different from one NMD to another, the relationships between impairments and activity limitations were investigated in six main diagnostic groups and in the whole sample without diagnostic distinction. Gait speed and muscle weakness in proximal and flexor muscle groups were significantly but moderately correlated to the activity limitations, indicating that the latter cannot simply be inferred from motor impairments but should be independently measured and treated. Chapter 3 investigates the sensitivity to change of the ACTIVLIM questionnaire. As NMD are progressive disorders, it is important that the ACTIVLIM questionnaire be able to assess the change over time in the activity level of patients with NMD in order to characterize the disease course and to quantify the effects of new treatments on activity limitations in these patients. Finally, the last section discusses the results of the different chapters and presents perspectives for future research.

Depuis son apparition à la fin de la seconde guerre mondiale, la pratique sportive des personnes atteintes de déficiences motrices a connu de nombreuses et rapides évolutions. Conçue initialement dans une logique rééducative, elle intègre de plus en plus le principe de performance en se rapprochant du mouvement sportif « ordinaire ». Toutefois, ce processus de sportivisation est variable selon l'espace social et culturel dans lequel il se réalise. Initié dans les années 1970, le mouvement handisportif guadeloupéen rencontre des difficultés récurrentes à s'organiser et à se fédérer. Au cours de ce travail, nous avons réalisé une enquête de terrain auprès des associations handisportives locales. Des entretiens avec les acteurs principaux de ce mouvement ont été menés, des archives recueillies et des observations directes et participantes effectuées afin de retracer l'histoire collective et de repérer les logiques individuelles mobilisées dans la structuration du mouvement handisport en Guadeloupe. Pour cela nous avons utilisé des outils de la socio-histoire et de l'analyse organisationnelle et traité les discours à partir d'une analyse des relations par opposition (ARO). Les principaux résultats mettent en évidence que les dynamiques associatives structurant la pratique sportive des personnes handicapées motrices en Guadeloupe sont imprégnées par une logique sportive doublée d'enjeux sociaux, culturels et identitaires. Ainsi, la thèse principale défendue au terme de cette recherche est que l'organisation du mouvement handisport en Guadeloupe est marquée par des stratégies fortement contextualisées où la conjugaison des identités – sportive, antillaise, de personne handicapée - produit des ajustements singuliers. / Since its apparition at the end of the second world word, the practice of sport for people suffering with motor deficiency has known numerous and quick evolutions. Initially conceived in the science of rehabilitation therapy, it now integrates more and more the principle of performance by getting closer to “ordinary” sport movements. However, this process of sportivisation is variable depending on the social and cultural context in which it is taking place. Initiated at the start of the 1970's, Guadeloupe's Movement for Sport for People with motor impairments has been meeting recurring difficulties in how to organise and federate itself. During this work, we have conducted a survey directly with the local disabled sport associations. Meetings with the principal actors of this movement had been taking place, archives had been gathered and both direct and participative observations had been carried out in order to retrace the collective history and to identify the individual theories utilised in the formation of Guadeloupe's Movement for Sport for Disabled People. For this we have used the tools of the socio-history and of the organizational analysis and handled the speeches with an analyse of the relationship by opposition (ARO). The main results bring to light that the associative dynamics which structure the sport practices of Guadeloupe's disabled people are impregnated by a sport logic combined with social, cultural and identity stakes. Therefore the principal thesis defended in the process of the research is that the organisation of Guadeloupe's Movement for Sport for Disabled People is impregnated by the highly contextual strategies where the identities -sport, Caribbean, disabled- produces some very particular adjustments.