Starling et al. (2004) reported a Brazilian family in which 12 members had a mild adult-onset form of autosomal dominant limb-girdle muscular dystrophy. Age at onset ranged from 30 to 47 years with proximal lower limb weakness in ... Starling et al. (2004) reported a Brazilian family in which 12 members had a mild adult-onset form of autosomal dominant limb-girdle muscular dystrophy. Age at onset ranged from 30 to 47 years with proximal lower limb weakness in most patients, muscle cramps in 1 patient, and upper limb weakness in 1 patient. Nine of 10 patients eventually had upper limb weakness. With the exception of the youngest patient, all patients developed progressive and permanent restriction of finger and toe flexion and reduced range of movement in the interphalangeal joints. Normal strength was retained in the intrinsic hand muscles. Serum creatine kinase was increased in all but 2 patients, and muscle biopsy of 1 patient showed a myopathic pattern with rimmed vacuoles. All 5 patients and 3 unaffected family members older than 45 years had type II diabetes mellitus (125853).