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Total 442 results found since Jan 2013.

Magnetic Resonance Imaging Findings of the Lower Limb Muscles in Anti-Mitochondrial M2 Antibody-Positive Myositis
Group of disorders characterized by myositis symptoms, including progressive muscle weakness and muscle inflammation in a setting of autoimmune abnormalities are collectively known as autoimmune myositis (AIM) or idiopathic inflammatory myopathy [1,2], including dermatomyositis (DM), anti-synthetase syndrome (ASS), immune-mediated necrotizing myopathy (IMNM), inclusion body myositis (IBM) [3,4] and other less well-characterized conditions.
Source: Neuromuscular Disorders - July 25, 2023 Category: Neurology Authors: Hirotaka Nomiya, Tadanori Hamano, Naoko Takaku, Hirohito Sasaki, Kojiro Usui, Sayaka Sanada, Tomohisa Yamaguchi, Yuki Kitazaki, Yoshinori Endo, Tomoko Kamisawa, Soichi Enomoto, Norimichi Shirahuzi, Akiko Matsunaga, Asako Ueno, Masamichi Ikawa, Osamu Yamam Tags: Research paper Source Type: research

Diagnosis, pathogenesis and treatment of myositis: recent advances.
Abstract Dermatomyositis (DM), polymyositis (PM), necrotizing myopathy (NM) and inclusion body myositis (IBM) are four distinct subtypes of idiopathic inflammatory myopathies -in short myositis. Recent studies have shed some light on the unique pathogenesis of each entity. Some of the clinical features are distinct, but muscle biopsy is indispensable for making a reliable diagnosis. The use of magnetic resonance imaging of skeletal muscles and detection of myositis-specific autoantibodies have become useful additions to our diagnostic repertoire. Only few controlled trials are available to substantiate current tre...
Source: Clinical and Developmental Immunology - August 27, 2013 Category: Allergy & Immunology Authors: Carstens PO, Schmidt J Tags: Clin Exp Immunol Source Type: research

Diagnosis, pathogenesis and treatment of myositis: recent advances
Abstract Dermatomyositis (DM), polymyositis (PM), necrotizing myopathy (NM) and inclusion body myositis (IBM) are four distinct subtypes of idiopathic inflammatory myopathies –in short myositis. Recent studies have shed some light on the unique pathogenesis of each entity. Some of the clinical features are distinct, but muscle biopsy is indispensable for making a reliable diagnosis. The use of magnetic resonance imaging of skeletal muscles and detection of myositis‐specific autoantibodies have become useful additions to our diagnostic repertoire. Only few controlled trials are available to substantiate current treatmen...
Source: Clinical and Experimental Immunology - August 27, 2013 Category: Allergy & Immunology Authors: Per‐Ole Carstens, Jens Schmidt Tags: Review Source Type: research

Neurologic involvement in scleroderma: A systematic review
Conclusions: Previously considered a rare event, nervous system involvement in scleroderma has been increasingly recognized. Seizures and headache are the most reported features in LS en coup de sabre, while peripheral and autonomic nervous systems involvement predominate in SSc. Moreover, recently, reports have frequently documented white matter lesions in asymptomatic SSc patients, suggesting smaller branches and perforating arteries involvement.
Source: Seminars in Arthritis and Rheumatism - July 3, 2013 Category: Rheumatology Authors: Tiago Nardi Amaral, Fernando Augusto Peres, Aline Tamires Lapa, João Francisco Marques-Neto, Simone Appenzeller Tags: Connective Tissue Diseases Source Type: research

The clinical features, diagnosis and classification of dermatomyositis.
Abstract Dermatomyositis (DM) is an idiopathic inflammatory myopathy (IIM) characterized by an inflammatory infiltrate primarily affecting the skeletal muscle and skin. Most common and peculiar cutaneous lesions include Gottron's papules, Gottron's sign and heliotrope rash. Different DM subsets have been identified until now encompassing classic DM, amyopathic DM, hypomyopathic DM, post-myopathic DM, and DM sine dermatitis. Patients with DM have a higher incidence rate of malignancy than the normal population. In these patients cancer occurs in about 30% of cases with higher occurrence in men and in elderly people...
Source: Journal of Autoimmunity - January 24, 2014 Category: Allergy & Immunology Authors: Iaccarino L, Ghirardello A, Bettio S, Zen M, Gatto M, Punzi L, Doria A Tags: J Autoimmun Source Type: research

Electrophysiological Findings in Acute Solanum Torvum Toxicity (P5.103)
CONCLUSIONS:Acute intoxication with poisonous Solanum torvum berries appears to cause a toxic myopathy. The chemical directly responsible remains unknown, however, is likely of the class of solanaceous steroidal glycoalkaloids.Study Supported by: NoneDisclosure: Dr. Glover has nothing to disclose. Dr. Patterson has nothing to disclose. Dr. Connors has nothing to disclose. Dr. Smith has nothing to disclose. Dr. Milstein has nothing to disclose. Dr. Swerdlow has nothing to disclose.
Source: Neurology - April 9, 2014 Category: Neurology Authors: Glover, R., Patterson, D., Connors, N., Smith, S., Milstein, M., Swerdlow, M. Tags: Neurophysiology/EMG Source Type: research

Cardiac and Muscular Involvement in Idiopathic Inflammatory Myopathies: Noninvasive Diagnostic Assessment and the Role of Cardiovascular and Skeletal Magnetic Resonance Imaging.
Abstract Idiopathic inflammatory myopathies (IIMs) are rare autoimmune diseases and include dermatomyositis, polymyositis, necrotizing myopathy and inclusion body myositis; they are characterized by inflammation of skeletal muscle and other internal organs and may potentially lead to irreversible damage and death. Only a small percentage of IIM has clinically overt cardiac disease; however, heart involvement is one of the leading causes of death and therefore, early detection remains a challenge. Biochemical markers and non-invasive methods such as the electrocardiogram and echocardiography have a role in diagnosi...
Source: Inflammation and Allergy Drug Targets - May 26, 2014 Category: Allergy & Immunology Authors: Mavrogeni S, Sfikakis PP, Dimitroulas T, Kolovou G, Kitas GD Tags: Inflamm Allergy Drug Targets Source Type: research

Intravoxel incoherent motion magnetic resonance imaging findings in the acute phase of MELAS: a case report
ConclusionThese findings imply a breakdown of autoregulation with hyperperfusion and vasogenic edema during the acute phase of MELAS, as described in previous reports. IVIM imaging is a valuable, noninvasive tool that simultaneously quantifies perfusion and diffusion parameters. We report a patient in the acute phase of MELAS who appears to show characteristic radiological findings using IVIM imaging. This type of imaging is a valuable technique that allows noninvasive and simultaneous measurement of both regional perfusion and diffusion.
Source: Brain and Behavior - September 11, 2014 Category: Neurology Authors: Ryuji Uehara, Koji Yamashita, Akio Hiwatashi, Osamu Togao, Kazufumi Kikuchi, Jun Yokoyama, Dai Matsuse, Takashi Yoshiura, Hiroshi Honda Tags: Methods Source Type: research

The value of respiratory muscle testing in a child with congenital muscular dystrophy
We report the case of a 12‐year‐old boy with congenital muscular dystrophy (CMD) in whom invasive and non‐volitional respiratory muscle tests showed an elective diaphragmatic dysfunction with the preservation of expiratory muscle strength. This finding, coupled with a clinical phenotype associating diffuse muscle atrophy with finger hyperlaxity and proximal contractures, strengthened the suspicion of Ullrich CMD. Skin‐cultured fibroblasts showed intracellular retention of collagen 6 (COL6), muscle magnetic resonance imaging was typical of COL6 myopathy, and molecular studies identified a COL6 gene mutation (COL6A2 ...
Source: Respirology Case Reports - June 30, 2014 Category: Respiratory Medicine Authors: Sonia Khirani, Ivana Dabaj, Alessandro Amaddeo, Adriana Ramirez, Susana Quijano‐Roy, Brigitte Fauroux Tags: Case Report Source Type: research

Non-Neuromuscular Manifestations of Spinal and Bulbar Muscular Atrophy (P7.054)
CONCLUSIONS: These non-neuromuscular observations provide new insight into spinal and bulbar muscular atrophy and offer additional avenues for investigating the disease mechanism. Study Supported by:Disclosure: Dr. Bakar has nothing to disclose. Dr. Grunseich has nothing to disclose. Dr. Kats has nothing to disclose. Dr. Kokkinis has nothing to disclose. Dr. Schindler has nothing to disclose. Dr. Rinaldi has nothing to disclose. Dr. Fischbeck has nothing to disclose.
Source: Neurology - April 8, 2015 Category: Neurology Authors: Bakar, D., Grunseich, C., Kats, I., Kokkinis, A., Schindler, A., Rinaldi, C., Fischbeck, K. Tags: Neuromuscular Disease: Clinical Science Source Type: research

Exclude pregnancy, vigorous exercise and myopathy before diagnosing noncompaction in healthy subjects
With interest we read the article by Andre et al. about the reference values for left and right trabeculation and non-compacted myocardium [1]. They investigated 117 healthy volunteers by cardiac magnetic resonance imaging (CMRI) and measured the cardiac structures. They found that a considerable amount of healthy volunteers fulfils the current diagnostic criteria for left ventricular hypertrabeculation/noncompaction (LVHT). We have the following questions and concerns:
Source: International Journal of Cardiology - April 16, 2015 Category: Cardiology Authors: Claudia Stöllberger, Josef Finsterer Tags: Letters to the Editor Source Type: research

Polyneuritis cranialis with generalized hyperreflexia as a presenting manifestation of thyrotoxicosis
Gaurav M Kasundra, Amita Narendra Bhargava, Bharat Bhushan, Khichar Shubhakaran, Isha SoodAnnals of Indian Academy of Neurology 2015 18(2):240-242A 22-year-old male student with no past medical illness, presented with acute onset dysarthria, binocular diplopia, and dysphagia over 10 hours. On examination, he had tachycardia, hypertension, generalized hyper-reflexia, and bilateral pupil sparing oculomotor, troclear, abducens, trigeminal, facial, glossopharyngeal, and vagus nerve palsy. Rest examination was unremarkable. Facial nerve conduction study (NCS) showed decreased amplitude bilaterally and neurogenic pattern on elec...
Source: Annals of Indian Academy of Neurology - May 8, 2015 Category: Neurology Authors: Gaurav M KasundraAmita Narendra BhargavaBharat BhushanKhichar ShubhakaranIsha Sood Source Type: research

Myocarditis in Patients With Antisynthetase Syndrome: Prevalence, Presentation, and Outcomes
Abstract: Antisynthetase syndrome (aSS) corresponds to an overlapping inflammatory myopathy identified by various myositis-specific autoantibodies (directed against tRNA-synthetases). Myocardial involvement in this condition is poorly described. From a registry of 352 aSS patients, 12 cases of myocarditis were retrospectively identified on the basis of an unexplained increase in troponin T/I levels associated with either suggestive cardiac magnetic resonance imaging (MRI) findings, nonsignificant coronary artery abnormalities or positive endomyocardial biopsy. The prevalence of myocarditis in aSS is 3.4% and was not linked...
Source: Medicine - July 1, 2015 Category: Internal Medicine Tags: Research Article: Observational Study Source Type: research

Myositides : What is the current situation?
This article gives a review of the classification, diagnostic procedures and treatment of idiopathic inflammatory myopathies from a neurological point of view. The myositis syndromes can be subdivided into four groups, polymyositis (PM), dermatomyositis (DM), inclusion body myositis (IBM) and necrotizing myopathy (NM), which substantially differ clinically and pathophysiologically. Myositis may also occur in association with cancer or autoimmune systemic diseases (overlap syndrome). Diagnosis of inflammatory myopathies is based on clinical symptoms, determination of creatine phosphokinase and acute phase parameters in bloo...
Source: Zeitschrift fur Rheumatologie - July 18, 2015 Category: Rheumatology Authors: Rösler KM, Scheidegger O Tags: Z Rheumatol Source Type: research

The Emerging Role of Cardiovascular Magnetic Resonance Imaging in the Evaluation of Metabolic Cardiomyopathies
Horm Metab ResDOI: 10.1055/s-0035-1555913The aim of this review is to discuss the role of Cardiovascular Magnetic Resonance (CMR) in the diagnosis, risk stratification, and follow-up of metabolic cardiomyopathies. The classification of myocardial diseases, proposed by WHO/ISFC task force, distinguished specific cardiomyopathies, caused by metabolic disorders, into 4 types: 1) endocrine disorders, 2) storage or infiltration disorders (amyloidosis, hemochromatosis and familial storage disorders), 3) nutritional disorders (Kwashiorkor, beri-beri, obesity, and alcohol), and 4) diabetic heart. Thyroid disease, pheochromocytoma,...
Source: Hormone and Metabolic Research - July 21, 2015 Category: Endocrinology Authors: Mavrogeni, S.Markousis-Mavrogenis, G.Markussis, V.Kolovou, G. Tags: Review Source Type: research