S.P.14 Exploring arm function in persons with LGMD by means of questionnaires

Abstract

Abstract Limb-Girdle Muscular Dystrophies (LGMD) mainly involve the pelvic and/or shoulder girdle muscles. One of the major problems is limitations in performing daily activities. The natural course of the disease has been described before, and research is done to investigate functional ability of walking. However, little is known about functional abilities of the arms, which is even more important in performing many daily activities. Clear understanding is essential to improve rehabilitation therapies and for development of new arm supports. The aim of this study is to investigate arm function of people with LGMD on activity level, by using questionnaires. A web-based questionnaire to assess arm function was developed. We used questions from the Brooke scale (grading scale, 1–6), the Capabilities of Upper Extremity questionnaire (CUE) (basic arm functions, 30 items, max score 210, higher score means better function) and Abilhand questionnaire (22 ADL items, max score 44, higher score means lower function). Also questions about disease duration, age, gender were asked. The Dutch patient organization (VSN) distributed the questionnaire. Fifty-one people (age 6–72 y) participated in the period August 2011 till February 2012. Preliminary results show that in this group, 33% has Brooke scale 1 and 51% Brooke 2–4. The CUE total median score was 159 (range 60–210), corresponding with 75.7% of the maximum score. The total Abilhand median score was 39 (range 8–44) corresponding with a 88.6% of the maximum score. Especially activities above shoulder level are problematic. Patterns of limitations in arm function are further analyzed as are correlations with disease course, age, and gender. The project McArm is a national Dutch ‘Pieken in de Delta’ project. It is partially funded by: Agentschap.nl, an agency of the Dutch Ministry of Economic Affairs, Agriculture and Innovation, the Province of Noord Brabant and the Province of Limburg.