Inclusion Body Myositis

Dimachkie, Mazen M.; Barohn, Richard J.

doi:10.1016/j.ncl.2014.04.001

Cited by 101 publications

(110 citation statements)

References 69 publications

(102 reference statements)

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“…Dysphagia occurs at some stage of the disease in 51-65% of cases , Cox et al, 2009, Dimachkie and Barohn 2013. In one series dysphagia was the presenting symptom in 15% of cases and predated other manifestations by up to 10 years in some cases (Dimachkie and Barohn 2013).…”

Section: Swallowingmentioning

confidence: 92%

“…In one series dysphagia was the presenting symptom in 15% of cases and predated other manifestations by up to 10 years in some cases (Dimachkie and Barohn 2013).…”

Section: Swallowingmentioning

confidence: 93%

“…Detailed analysis of several large patient cohorts has provided a clearer appreciation of the typical patterns of muscle involvement and degree of variability in the clinical phenotype, as well as atypical presentations and other disease manifestations (Needham et al, 2008, Dimachkie andBarohn 2013).…”

Section: Clinical Phenotypementioning

confidence: 99%

“…A systematic study of hand function in a cohort of 45 IBM patients showed that whilst hand-grip and pinch strength were markedly impaired, compensatory strategies were commonly employed, and fine motor abilities were relatively well preserved (Eriksson and Lindberg 2012). In one series, 24% of cases were considered to have atypical phenotypes, such as a limb-girdle pattern of weakness, scapular winging, footdrop, or prominent forearm weakness with sparing of the quadriceps (Dimachkie and Barohn 2013). Mild to moderate weakness of the facial muscles is common and in occasional cases it may precede other manifestations , Dimachkie and Barohn 2013, Ghosh et al, 2014, Mastaglia and Needham 2015.…”

Section: Patterns Of Muscle Involvementmentioning

confidence: 99%

“…In one series, 24% of cases were considered to have atypical phenotypes, such as a limb-girdle pattern of weakness, scapular winging, footdrop, or prominent forearm weakness with sparing of the quadriceps (Dimachkie and Barohn 2013). Mild to moderate weakness of the facial muscles is common and in occasional cases it may precede other manifestations , Dimachkie and Barohn 2013, Ghosh et al, 2014, Mastaglia and Needham 2015. Weakness of the paraspinal muscles may develop as the disease progresses, resulting in dropped head or camptocormia, and is the presenting feature in some cases (Goodman et al, 2012, Ma et al, 2013.…”

Section: Patterns Of Muscle Involvementmentioning

confidence: 99%

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Sporadic inclusion body myositis: A review of recent clinical advances and current approaches to diagnosis and treatment

Needham

Mastaglia

2016

Clinical Neurophysiology

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Sporadic inclusion body myositis is the most frequent acquired myopathy of middle and later life and is distinguished from other inflammatory myopathies by its selective pattern of muscle involvement and slowly progressive course, and by the combination of inflammatory and degenerative muscle pathology and multi-protein deposits in muscle tissue. This review summarises the findings of recent studies that provide a more complete picture of the clinical phenotype and natural history of the disease and its global prevalence and genetic predisposition. Current diagnostic criteria, including the role of electrophysiological and muscle imaging studies and the recently identified anti-5'-nucleotidase (anti-cN1A) antibody in diagnosis are also discussed as well as current trends in the treatment of the disease.

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Section: Swallowingmentioning

confidence: 92%

“…In one series dysphagia was the presenting symptom in 15% of cases and predated other manifestations by up to 10 years in some cases (Dimachkie and Barohn 2013).…”

Section: Swallowingmentioning

confidence: 93%

Section: Clinical Phenotypementioning

confidence: 99%

Section: Patterns Of Muscle Involvementmentioning

confidence: 99%

Section: Patterns Of Muscle Involvementmentioning

confidence: 99%

See 3 more Smart Citations

Sporadic inclusion body myositis: A review of recent clinical advances and current approaches to diagnosis and treatment

Needham

Mastaglia

2016

Clinical Neurophysiology

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NMR‐based serum and muscle metabolomics for diagnosis and activity assessment in idiopathic inflammatory myopathies

Guleria

Kumar

et al. 2021

Analytical Science Advances

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Objectives Differentiating smoldering disease activity from weakness due to fatty replacement of atrophied muscle can often be a challenge in the idiopathic inflammatory myositis (IIM). We aimed to identify the metabolic disturbances associated with IIM and if these changes can aid in the assessment of disease activity. Methods Metabolic profiles of sera (N = 99) and muscle (N = 21) from patients with IIM (ACR‐EULAR criteria) were compared with healthy control (HC) samples (N = 75 for serum and N = 12 for muscle tissues) employing 800 MHz NMR (Nuclear Magnetic Resonance) spectroscopy. Metabolic disparity between IIM and HC was established based on Partial Least Squares Discriminant Analysis (PLS‐DA) and the discriminatory metabolites were identified based on variable importance in projection (VIP) statistics (P‐value < .05, corrected for false discovery rate (FDR)). Results Serum metabolomics profiles were distinctive in IIM as compared to HC, with a visible shift to anaerobic metabolism (increased lactate, low glucose), oxidative defect (high Phenylalanine/tyrosine), decreased muscle mass (low serum creatinine), increased muscle catabolism (increased branched‐chain amino acids), and dyslipidemia (higher lipids, higher very low‐density lipoprotein [VLDL]/low‐density lipoprotein [LDL] ratio, lower polyunsaturated fatty acid [PUFA]). The sera of active IIM patients were characterized by anaerobic metabolism (low glucose), loss of muscle mass (low creatinine, amino acids), and oxidative defect (high Phenylalanine/tyrosine). Three metabolites (isopropanol, succinate, and glycine) were distinctive in muscle tissue metabolomics. NMR‐based serum metabolic disparity was lacking between different clinical subsets of IIM. Conclusion Serum and muscle tissue metabolomics have the potential to distinguish (a) IIM from HC and (b) active IIM from inactive IIM irrespective of disease subtype.

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Functional regeneration of tissue engineered skeletal muscle in vitro is dependent on the inclusion of basement membrane proteins

et al. 2019

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Skeletal muscle has a high regenerative capacity, injuries trigger a regenerative program which restores tissue function to a level indistinguishable to the pre‐injury state. However, in some cases where significant trauma occurs, such as injuries seen in military populations, the regenerative process is overwhelmed and cannot restore full function. Limited clinical interventions exist which can be used to promote regeneration and prevent the formation of non‐regenerative defects following severe skeletal muscle trauma. Robust and reproducible techniques for modelling complex tissue responses are essential to promote the discovery of effective clinical interventions. Tissue engineering has been highlighted as an alternative method, allowing the generation of three‐dimensional in vivo like tissues without laboratory animals. Reducing the requirement for animal models promotes rapid screening of potential clinical interventions, as these models are more easily manipulated, genetically and pharmacologically, and reduce the associated cost and complexity, whilst increasing access to models for laboratories without animal facilities. In this study, an in vitro chemical injury using barium chloride is validated using the C2C12 myoblast cell line, and is shown to selectively remove multinucleated myotubes, whilst retaining a regenerative mononuclear cell population. Monolayer cultures showed limited regenerative capacity, with basement membrane supplementation or extended regenerative time incapable of improving the regenerative response. Conversely tissue engineered skeletal muscles, supplemented with basement membrane proteins, showed full functional regeneration, and a broader in vivo like inflammatory response. This work outlines a freely available and open access methodology to produce a cell line‐based tissue engineered model of skeletal muscle regeneration.

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Inclusion Body Myositis

Cited by 101 publications

References 69 publications

Sporadic inclusion body myositis: A review of recent clinical advances and current approaches to diagnosis and treatment

Sporadic inclusion body myositis: A review of recent clinical advances and current approaches to diagnosis and treatment

NMR‐based serum and muscle metabolomics for diagnosis and activity assessment in idiopathic inflammatory myopathies

Functional regeneration of tissue engineered skeletal muscle in vitro is dependent on the inclusion of basement membrane proteins

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