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...

OBJECTIVE To show cancer association is a risk factor other than statin exposure for anti-3-hydroxy-3-methylglutaryl coenzyme A reductase autoantibody-positive (anti-HMGCR Ab+) myopathy. METHODS We analyzed the clinical features and courses of 33 patients (23 female and 10 male) with anti-HMGCR Ab+ myopathy among 621 consecutive patients with idiopathic inflammatory myopathies. RESULTS Amon...

Dermatomyositis, an idiopathic inflammatory myopathy with characteristic skin manifestations, is associated with several types of cancer. Only three cases of gallbladder cancer with dermatomyositis have been reported worldwide, and none has been reported in Korea. We present a case of a 71-year-old male with proven dermatomyositis associated with gallbladder cancer to emphasize the necessity of...

Background JDM is the most common idiopathic inflammatory myopathy in children. The Wedderburn scoring system is used to assess JDM muscle biopsies and categorizes muscle involvement into 4 domains: inflammatory, vascular, muscle fiber and connective tissue fibrosis (maximum scores: 12, 3, 10, and 2, respectively) and a 10-cm VAS measuring overall abnormality. A 10 cm VAS for chronicity (endomy...

Idiopathic inflammatory myopathy (IIM) is the umbrella term including dermatomyositis (DM), polymyositis (PM), overlap myositis (OM), sporadic inclusion body (IBM) and necrotising autoimmune (NAM), also known as immune-mediated myopathy. There some debate to whether PM exists a discrete entity, or perhaps an overly generalising encompassing connective tissue disease associated myositis, OM, pre...

Mdx mice develop an inflammatory myopathy characterized at different ages by myonecrosis with scattered inflammatory infiltrates followed by muscular regeneration and later persistent fibrosis. This work aimed to verify the putative anti-inflammatory role of nicotinic acetylcholine receptor (nAChR) in the mdx muscular lesion. Mitigation of myonecrosis and decreased TNFα production were accompan...

how to cite this article: nilipour y. lysosomal myopathies. iran j child neurol autumn 2012; 6:4 (suppl. 1):11. pls see pdf.

Inflammatory myopathies such as dermatomyositis and polymyositis are well-recognized paraneoplastic syndromes. Little is known, however, about necrotizing myopathies in association with cancer. We here describe a case of paraneoplastic necrotizing myopathy with a mild inflammatory infiltrate in a patient with adenocarcinoma. After the rapid development of a severe, disabling muscle weakness, th...

OBJECTIVES Immune-mediated necrotizing myopathy (IMNM) is characterized by the predominant presence of necrotic muscle fibres in muscle biopsy and variable response to immunosuppressive treatment. The aims of this study were to analyse the temporal trend of IMNM incidence in our centre over the past 10 years and to explore the role of statins as possible causative agents. METHODS A retrospect...