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Specialty: Neurology
Condition: Polymyositis

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Total 96 results found since Jan 2013.

Lipid storage myopathy due to late-onset multiple Acyl-CoA dehydrogenase deficiency with novel mutations in ETFDH: A case report
ConclusionOur observations extend the spectrum of ETFDH variants in Chinese the population and reinforce the role of NGS in diagnosis of MADD. Early diagnosis and appropriate treatment of LSM lead to great clinical efficacy and avoid some lethal complications.
Source: Frontiers in Neurology - December 15, 2022 Category: Neurology Source Type: research

Antibody Therapies in Autoimmune Inflammatory Myopathies: Promising Treatment Options
AbstractInflammatory myopathies, including polymyositis (PM), dermatomyositis (DM), inclusion body myositis (IBM), necrotizing myopathy (NM), antisynthetase syndrome (ASS) and overlap myositis (OM), in short myositis, are rare diseases. All forms of myositis have progressive muscle weakness in common, with each subtype characterized by different autoantibody profiles, histological findings and extramuscular manifestations. Due to better understanding of the pathogenesis of the muscle inflammation in myositis, new molecular pathways for targeted therapy have been discovered. Current therapies aim at different components of ...
Source: Neurotherapeutics - April 8, 2022 Category: Neurology Source Type: research

What Is in the Myopathy Literature?
This edition concentrates on inflammatory myopathies with reports of reclassification of polymyositis, cancer associations, evaluation of subclinical cardiac involvement, myositis-specific and -associated antibodies, and immune checkpoint inhibitor myositis. A number of reports address sporadic late-onset nemaline myopathy and point out its diagnostic difficulty and the importance of identifying an associated monoclonal gammopathy that is likely of clinical significance and may warrant aggressive immunotherapy. Finally, treatment of nondystrophic channelopathies is addressed.
Source: Journal of Clinical Neuromuscular Disease - November 29, 2021 Category: Neurology Tags: Review Article Source Type: research

Autoimmune & inflammatory nmd
Anti-transcription intermediary factor 1 γ (TIF1γ) antibodies are one of the myositis-specific antibodies frequently detected in dermatomyositis (DM). To elucidate the characteristics of anti-TIF1γ antibody-positive myositis, we extracted cases with anti-TIF1γ antibodies from idiopathic inflammatory myopathy (IIM) patients who underwen t muscle biopsy from 2008 to 2019, and analyzed the clinicopathological findings. We found 87 cases of IIM (40 DM, 3 polymyositis, 25 immune-mediated necrotizing myopathy, 14 antisynthetase syndrome, and 5 nonspecific myositis), among which anti-TIF1γ antibodies were positive in 13 case...
Source: Neuromuscular Disorders - September 19, 2021 Category: Neurology Authors: A. Yamanaka, N. Eura, M. Yamaoka, M. Ozaki, T. Shiota, H. Nanaura, K. Sugie Source Type: research

New insights into cellular or muscle function
Analysis of human muscle diseases highlights the role of mitochondrial dysfunction in the skeletal muscle. Our previous work revealed that diverse upstream events correlated with altered mitochondrial proteome in human muscle biopsies. However, several proteins showed relatively unchanged expression suggesting that posttranslational modifications, mainly protein phosphorylation could influence their activity and regulate mitochondrial processes. We conducted mitochondrial phosphoprotein profiling, by proteomics approach, of healthy human skeletal muscle (n=10) and three muscle diseases (n=10 each): Dysferlinopathy, polymyo...
Source: Neuromuscular Disorders - September 19, 2021 Category: Neurology Authors: B. Sunitha, M. Kumar, N. Gowthami, S. Unni, N. Gayathri, T. Keshava Prasad, A. Nalini, K. Polavarapu, S. Vengalil, K. Veeramani Preethish, B. Padmanabhan, M. Srinivas Bharath Source Type: research

Diagnostic Accuracy of Anti-CN1A on the Diagnosis of Inclusion Body Myositis. A Hierarchical Bivariate and Bayesian Meta-analysis
In this study, we investigated the diagnostic accuracy of anti-CN1A antibodies for sporadic IBM in comparison with other inflammatory myopathies, autoimmune disorders, motor neurone disease, using a hierarchical bivariate approach, and a Bayesian model taking into account the variable prevalence. The results of the present analysis show that anti-CN1A antibodies have moderate sensitivity, and despite having high specificity, they are not useful biomarkers for the diagnosis of IBM, polymyositis or dermatomyositis, other autoimmune conditions, or neuromuscular disorders. Neither the hierarchical bivariate nor the Bayesian an...
Source: Journal of Clinical Neuromuscular Disease - August 28, 2021 Category: Neurology Tags: Review Article Source Type: research

A Case of Inflammatory Myopathy in Graft vs Host Disease - A Potential Role for Ibrutinib
Idiopathic inflammatory myopathies are a group of systemic autoimmune conditions with dominant manifestations on skeletal muscle and include the subgroups dermatomyositis, polymyositis, inclusion body myositis and necrotising myopathy. These subgroups share the clinical features of skeletal muscle weakness, raised serum levels of creatine kinase (CK), myopathic changes on electromyography and magnetic resonance imaging, but are distinguished by autoantibody profiles, and importantly by muscle histopathology which reflects the distinct pathogenetic mechanisms [1].
Source: Neuromuscular Disorders - July 4, 2021 Category: Neurology Authors: Michael Wilkinson, David Yeung, Vidya Limaye Tags: Case report Source Type: research

Inflammatory myopathies: update on diagnosis, pathogenesis and therapies, and COVID-19-related implications.
Authors: Dalakas MC Abstract The inflammatory myopathies constitute a heterogeneous group of acquired myopathies that have in common the presence of endomysial inflammation. Based on steadily evolved clinical, histological and immunopathological features and some autoantibody associations, these disorders can now be classified in five characteristic subsets: Dermatomyositis (DM) Polymyositis (PM), Necrotizing Autoimmune Myositis (NAM), Anti-synthetase syndrome-overlap myositis (Anti-SS-OM), and Inclusion-Body-Myositis (IBM). Each inflammatory myopathy subset has distinct immunopathogenesis, prognosis and response t...
Source: Acta Myologica - January 20, 2021 Category: Neurology Tags: Acta Myol Source Type: research

Autoimmune myopathies
Scleroderma/systemic sclerosis (Scl/SSc) is a heterogeneous disease which can involve internal organ and cause death. When myopathy occurred in Scl/SSc, it is associated with poor prognosis and with more frequent cardiopulmonary involvement. Because the myositis disease concept is changing and the diagnosis of polymyositis may not exist, it may affect Scl/SSc with ``myositis overlap''. In the current study, we try to elucidate the features of myopathy in Scl/SSc patients mainly from a histopathological point of view.
Source: Neuromuscular Disorders - September 28, 2020 Category: Neurology Authors: Y. Chen, M. Inoue, M. Ogasawara, Y. Saito, L. Indrawati, J. Tanboon, T. Kumutpongpanich, M. Okubo, W. Yohioka, S. Hayashi, S. Noguchi, I. Nishino Source Type: research

Autoimmune myopathies
Inflammatory myopathies consist of heterogenous group of immune-mediated muscle diseases including dermatomyositis, polymyositis and so on. Patients with inflammatory myopathies occasionally have malignancies such as breast, lung, colorectal cancer and nasopharyngeal carcinoma. There have been a few reports of patients with inflammatory myopathy and lymphoma. Herein, we report an unusual case of a 67-year-old male with inflammatory myopathy and diffuse large B cell lymphoma who initially presented with slowly progressive quadriparesis, axial weakness and abnormality in the electromyography and muscle magnetic resonance image (MRI).
Source: Neuromuscular Disorders - September 28, 2020 Category: Neurology Authors: I. Yoo, J. Shin Source Type: research

Where are we moving in the classification of idiopathic inflammatory myopathies?
Purpose of review Discoveries of myositis-specific antibodies, transcriptomic signatures, and clinicoseropathological correlation support classification of idiopathic inflammatory myopathies (IIM) into four major subgroups: dermatomyositis, immune-mediated necrotizing myopathy (IMNM), antisynthetase syndrome (ASS), and inclusion body myositis (IBM) whereas leaving polymyositis as a historical nonspecific diagnosis of exclusion. This review summarizes and comments on recent knowledge regarding the major subgroup of IIM. Recent findings Type 1 interferon (IFN1) pathway activation is the most prominent in dermatomyositis...
Source: Current Opinion in Neurology - September 4, 2020 Category: Neurology Tags: MUSCULAR DISEASE: Edited by John Vissing Source Type: research

What is new in idiopathic inflammatory myopathies: Mechanisms and therapies
Satish V Khadilkar, Megha C DhamneAnnals of Indian Academy of Neurology 2020 23(4):458-467 Idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of disorders that cause muscle weakness and also have extramuscular manifestations involving various organ systems; namely the lung, skin, heart, and joints. Previously classified broadly as dermatomyositis (DM) and polymyositis now the spectrum of the disease has evolved into more clinical subtypes. There are now five clinicoserological subtypes recognized worldwide DM, antisynthetase syndrome (AS), overlap myositis (OM), immune mediated necrotizing myopathy (IMNM)...
Source: Annals of Indian Academy of Neurology - August 20, 2020 Category: Neurology Authors: Satish V Khadilkar Megha C Dhamne Source Type: research

Juvenile Dermatomyositis and the Inflammatory Myopathies
Semin Neurol DOI: 10.1055/s-0040-1705120The inflammatory myopathies comprise disorders of immune-mediated muscle injury. The histopathology and clinical features help distinguish them. Juvenile dermatomyositis (JDM) is the most common form of myositis in children and adolescents. Children with JDM present with proximal muscle weakness and characteristic rashes. The presentation is similar in children and adults, but JDM is a primary disorder and the adult form often is concerning for a paraneoplastic syndrome. Proximal muscle weakness occurs with dermatomyositis, polymyositis, and immune-mediated necrotizing myopathy, but ...
Source: Seminars in Neurology - April 5, 2020 Category: Neurology Authors: Swafford, Collin Roach, E Steve Tags: Review Article Source Type: research

Clinical and histological features of immune-mediated necrotising myopathy: a multi-centre South Australian cohort study
The idiopathic inflammatory myopathies (IIMs) are a group of systemic autoimmune diseases characterised primarily by muscle inflammation but also potentially accompanied by a range of extra-muscular manifestations. Dermatomyositis (DM), polymyositis (PM) and inclusion body myositis (IBM) constitute well-characterised subtypes of IIM, with the entity of non-specific idiopathic inflammatory myopathy (NSIIM) being more recently described [1]. Whilst these IIM subtypes are distinguished on clinical, serological and histological grounds, they are unified by the presence of a typically prominent intramuscular lymphocytic infiltrate.
Source: Neuromuscular Disorders - February 17, 2020 Category: Neurology Authors: Jessica Day, Sophia Otto, Kathy Cash, Vidya Limaye Source Type: research