Cross-sectional retrospective study of muscle function in patients with glycogen storage disease type III

Decostre V, Laforêt P, Nadaj-Pakleza A, De Antonio M, Leveugle S, Ollivier G, Canal A, Kachetel K, Petit F, Eymard B, Behin A, Wahbi K, Labrune P, Hogrel JY 
Neuromuscul Disord. 2016. 26(9):584-92.
 This study aimed to identify promising muscle function measures for future studies on natural disease progression and therapeutic trials in patients with glycogen storage disease type III.
The age-effect on the manual muscle testing (MMT), the hand-held dynamometry (HHD), the motor function measure (MFM), and the Purdue pegboard test was evaluated. The MFM score and handgrip strength were age-sensitive in patients older than 29 and 37 years old.

PubMed link

Keywords: Debranching enzyme deficiency; Glycogen storage disease type III; Metabolic myopathy; Outcome measures

Articles dans les revues scientifiques