Gait Abnormalities in Late-Onset Pompe Disease (LOPD) — Gower’s Sign

Pompe disease is a degenerative and potentially life-threatening genetic muscle disorder. Late-onset Pompe disease (LOPD) is characterized by progressive weakness in the trunk and proximal muscles as well as the group of muscles surrounding the pelvis. This may affect a person’s mobility, making it difficult to walk, climb stairs, stand up from a chair, and reach overhead. Weakness in the diaphragm and other respiratory muscles also cause progressive respiratory disease. One of the visible symptoms of LOPD is difficulty rising from a seated position, also referred to as Gower’s sign, which is characterized by core muscle weakness and the reliance on upper body strength to push oneself up. Using motion capture footage of actual patients diagnosed with LOPD, this video shows what Gower’s sign can look like in patients with differing severity and duration of disease. If you notice any of these gait characteristics in your patients, consider a GAA enzyme assay or genetic testing to test for Pompe disease. See more gait abnormalities in LOPD: - Waddling gait: - Climbing stairs: - Reaching overhead: MAT-US-2008160 May 2020