نتایج جستجو برای: inclusion body myositis

تعداد نتایج: 845562  

Journal: :Current opinion in neurology 2014
Pedro M Machado Mazen M Dimachkie Richard J Barohn

PURPOSE OF THE REVIEW To describe new insights and developments in the pathogenesis, diagnosis and treatment of sporadic inclusion body myositis (IBM). RECENT FINDINGS Various hypothesis about the pathogenesis of IBM continue to be investigated, including autoimmune factors, mitochondrial dysfunction, protein dyshomeostasis, altered nucleic acid metabolism, myonuclear degeneration and the rol...

2013
Ken-ya Murata Ken Kouda Fumihiro Tajima Tomoyoshi Kondo

Here, we describe balloon catheter dilation at the upper esophageal sphincter (UES) in three sporadic inclusion body myositis (s-IBM) patients with dysphagia. Initially, we performed IVIg therapy, and, three months later, switched to balloon dilation therapy. A 12-Fr balloon catheter was inserted from the mouth under fluoroscopy and the balloon inflated at the UES. The catheter was pulled back ...

Journal: :Neurology 2006
Robert C Griggs

There is no established treatment that improves, arrests, or slows the progression of inclusion-body myositis (IBM). Many anti-inflammatory, immunosuppressant, or immunomodulating agents have been administered to patients with IBM but the design of clinical trials was such that it can only be concluded that none produced rapid improvement. The natural history of the disease is for stabilization...

Journal: :Muscle & nerve 2009
Ashok Verma Rup Tandan

TDP-43 is a 414 amino acid protein encoded by the transactive response (TAR) DNA binding protein gene on chromosome 1p36. It was first cloned as a human protein capable of binding to the TAR DNA of HIV-1.11 It was later identified as part of a complex involved in splicing of the cystic fibrosis transmembrane conductance regulator gene.3 TDP-43 is highly conserved, ubiquitously expressed and pre...

Journal: :Autoimmunity reviews 2014
M Catalán A Selva-O'Callaghan J M Grau

Sporadic inclusion body myositis (sIBM) is the most common acquired muscle disease in elderly individuals, particularly men. Its prevalence varies among ethnic groups but is estimated at 35 per one million people over 50. Genetic as well as environmental factors and autoimmune processes might both have a role in its pathogenesis. Unlike other inflammatory myopathies, sIBM causes very slowly pro...

2017
Daiki Takekawa Hirotaka Kinoshita Tomoyuki Kudo Masato Kitayama Tetsuya Kushikata Kazuyoshi Hirota

Inclusion body myositis (IBM) is an inflammatory muscle disease characterized by slowly progressive muscle weakness and wasting, especially affecting proximal leg and distal arm. We report a successful anesthetic management of a 68-year-old male patient with IBM undergoing ventriculoperitoneal shunt. Anesthesia was induced and maintained with total intravenous anesthesia using propofol, remifen...

Journal: :Science translational medicine 2016
Mhoriam Ahmed Pedro M Machado Adrian Miller Charlotte Spicer Laura Herbelin Jianghua He Janelle Noel Yunxia Wang April L McVey Mamatha Pasnoor Philip Gallagher Jeffrey Statland Ching-Hua Lu Bernadett Kalmar Stefen Brady Huma Sethi George Samandouras Matt Parton Janice L Holton Anne Weston Lucy Collinson J Paul Taylor Giampietro Schiavo Michael G Hanna Richard J Barohn Mazen M Dimachkie Linda Greensmith

Sporadic inclusion body myositis (sIBM) is the commonest severe myopathy in patients more than 50 years of age. Previous therapeutic trials have targeted the inflammatory features of sIBM but all have failed. Because protein dyshomeostasis may also play a role in sIBM, we tested the effects of targeting this feature of the disease. Using rat myoblast cultures, we found that up-regulation of the...

Journal: :Neurology 2014
Anthony A Amato Kumaraswamy Sivakumar Namita Goyal William S David Mohammad Salajegheh Jens Praestgaard Estelle Lach-Trifilieff Anne-Ulrike Trendelenburg Didier Laurent David J Glass Ronenn Roubenoff Brian S Tseng Steven A Greenberg

OBJECTIVE To study activin signaling and its blockade in sporadic inclusion body myositis (sIBM) through translational studies and a randomized controlled trial. METHODS We measured transforming growth factor β signaling by SMAD2/3 phosphorylation in muscle biopsies of 50 patients with neuromuscular disease (17 with sIBM). We tested inhibition of activin receptors IIA and IIB (ActRII) in 14 p...

Journal: :British medical journal 1953
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Journal: :Muscle & nerve 2009
Mohammad Salajegheh Jack L Pinkus J Paul Taylor Anthony A Amato Remedios Nazareno Robert H Baloh Steven A Greenberg

The nucleic acid binding protein TDP-43 was recently identified in normal myonuclei and in the sarcoplasm of inclusion body myositis (IBM) muscle. Here we found TDP-43 sarcoplasmic immunoreactivity in 23% of IBM myofibers, while other reported IBM biomarkers were less frequent, with rimmed vacuoles in 2.8%, fluorescent Congo red material in 0.57%, SMI-31 immunoreactivity in 0.83%, and focal R12...

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