| Main clinical features
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characterized by progressive proximal leg weakness, with/without proximal arm weakness, absent ankle deep-tendon reflexes and elevated creatine-kinase values
onset of muscle weakness in the pelvic girdle muscles between the fourth and sixth decade, later involvement of the shoulder girdle, and marked walking difficulties in the eighth decade (PMID: 21376592))
at the early stages of the disease fatty degeneration in T1-weighed MRI sequences were observed in the soleus, adductor magnus, semimembranosus and biceps femoris muscles followed by medial gastrocnemius, adductor longus and later by vasti muscles of the quadriceps (PMID: 23865856)) |