OBJECTIVE Little information exists regarding the economic burden related to inflammatory myopathies. Our objective was to estimate health services costs in a large, unselected, population-based sample of patients with inflammatory myopathies. METHODS We identified subjects with polymyositis and dermatomyositis from administrative healthcare databases (covering all beneficiaries, about 7.5 mi...

Idiopathic inflammatory myopathies are a group of autoimmune diseases characterized by proximal muscle weakness and inflammation of skeletal muscle. Specific forms of idiopathic inflammatory myopathy include polymyositis, dermatomyositis, and inclusion body myositis. Inflammatory myopathies have a bimodal distribution, occurring in patients between 10 and 15 years and 45 and 60 years of age, ex...

BACKGROUND Mutations in ryanodine receptor 1 gene (RYR1) are frequent causes of myopathies. They classically present with central core disease; however, clinical variability and histopathologic overlap are being increasingly recognized. PATIENTS Patient 1 is a 15-year-old girl with mild proximal, four-limb weakness from age 5, presenting with a progressive scoliosis starting at age 10. Patien...

Congenital myopathies and congenital myopathic dystrophies are distinct groups of inherited diseases of muscle, genetically heterogeneous, that manifest in early life or infancy. Congenital myopathic dystrophy is characterized by a dystrophic pattern, whereas no necrotic or degenerative changes are present in congenital myopathies. Much progress has been made in recent years in clarifying the c...