The 6 Minute Walk Test and Performance of Upper Limb in Ambulant Duchenne Muscular Dystrophy Boys

Conclusions In conclusion, our results confirm that upper limb involvement can already be found in DMD boys even in the ambulant phase. The scores on the PUL largely reflect the overall impairment observed on the 6MWT but the correlation was not linear. The use of the PUL appears to be less relevant in the very strong patients with 6MWT above 400 meters, that are often excluded from clinical trials. In patients with lower 6MWT early signs of involvement are not only limited to shoulder involvement but also, in some cases, to the loss of the ability to fully perform some activities in the middle and distal domain. The severity of upper limb involvement was variable and could not always be predicted by the overall weakness and fatigability measured by the 6MWT or by the use of steroids. The concomitant use of the PUL appears to be even more relevant in the patients with 6MWT results below 350 meters, and especially in those below 230 meters who are more likely to lose ambulation within two years6 . In these boys the PUL will provide the possibility to monitor possible functional changes also during and after loss of ambulation. Longitudinal studies are in process in both ambulant and non ambulant DMD boys and adults in order to better understand the changes of the PUL scores over time and the possible effect of different variables, such as age and steroids on the progression of upper limb involvement. Competing Interests The authors have declared that no competing interests e...
Source: PLOS Currents Muscular Dystrophy - Category: Neurology Authors: Source Type: research