1/18. Inclusion body myositis masquerading as polymyositis: a case study.A case of inclusion body myositis masquerading as unresponsive polymyositis is presented. A 56-year-old woman diagnosed with "biopsy-proven" polymyositis in 1991 was referred to our clinic in 1997 with progressive, painless weakness that was unresponsive to steroid therapy. Further evaluation, including electromyography and review of the original muscle biopsy specimen, found a diagnosis of inclusion body myositis, leading to a change in the patient's prognosis and management. Inclusion body myositis is frequently mistaken for polymyositis, despite the fact that it is now the most common inflammatory myopathy affecting people older than 50 years. The purpose of this report is to increase awareness of this disease, to enhance early diagnosis, and to ensure appropriate management. We discuss the clinical findings, pathogenesis, and physiatric management, as well as compare this disease with other idiopathic inflammatory myopathies.- - - - - - - - - - ranking = 1keywords = myopathies (Clic here for more details about this article) |
2/18. Respiratory failure due to muscle weakness in inflammatory myopathies: maintenance therapy with home mechanical ventilation.polymyositis and dermatomyositis are idiopathic inflammatory myopathies. Respiratory complications are a common feature, but ventilatory insufficiency is rare in these patients. We describe here three patients diagnosed with inflammatory myopathy (polymyositis) with respiratory failure due to muscle weakness who did not respond to immunosuppressive therapy. Mechanical ventilation at home with nasal or tracheal intermittent positive pressure resulted in improved chronic hypoventilation. This treatment improves the quality of life of patients with inflammatory myopathies and can be lifesaving in some cases.- - - - - - - - - - ranking = 6keywords = myopathies (Clic here for more details about this article) |
3/18. Multiple deletions of mtDNA remove the light strand origin of replication.Idiopathic inflammatory myopathies are progressive, debilitating muscle diseases. The pathogenesis of these disorders is multifactorial and appears to include mutations of the mitochondrial genome, which are usually indicated by morphological changes of mitochondria. The vast majority of all mitochondrial dna deletions found are located between the origins of replication in the "major region" between nt5760-nt190. Using long distance PCR and sequencing techniques, we detected deletions which were unusually large (ca. 10500-12800 bp) and show uncommon 5'-breakpoints between nt800 and nt3326. Unlike most other deletions, their breakpoints are far upstream of the "major region." The atypical location of these deletions suggests a different pathomechanism. The impact of the mitochondrial dna deletions in the pathogenetic cascade remains uncertain.- - - - - - - - - - ranking = 1keywords = myopathies (Clic here for more details about this article) |
4/18. MRI guided muscle biopsy confirmed polymyositis diagnosis in a patient with interstitial lung disease.Idiopathic inflammatory myopathies, such as polymyositis (PM), may present with general symptoms such as fever and fatigue and only minimal muscle weakness, making it difficult to make a definite diagnosis and provide adequate treatment. Here a case is described in which interstitial lung disease was the first and most prominent manifestation of PM. Later, when muscle weakness became apparent and inflammatory muscle disease was suspected the first muscle biopsy was non-diagnostic. However, magnetic resonance imaging (MRI) scans of the clinically weak thigh muscles showed high signal on T(2) weighted images, suggesting muscle inflammation more proximal to the first biopsy site. A second biopsy at this site disclosed typical histopathological findings for myositis. After treatment with prednisolone in combination with cyclophosphamide both pulmonary and muscle function improved. CONCLUSION: MRI scans of muscles may be helpful in selection of a site for muscle biopsy in patients with suspected inflammatory myopathy when a first muscle biopsy turns out to be negative. Additionally, patients with interstitial lung disease of unknown cause should be tested for muscular function to exclude an associated inflammatory muscle disorder.- - - - - - - - - - ranking = 1keywords = myopathies (Clic here for more details about this article) |
5/18. A deceptive case of amyloid myopathy: clinical and magnetic resonance imaging features.Amyloid myopathy is a well-described, increasingly recognized clinical entity. Similar to inflammatory myopathies, amyloid myopathy presents with proximal muscle weakness and can be associated with elevated levels of muscle enzymes. We report the case of a 58-year-old woman who, at presentation to her physician with proximal muscle weakness and congestive heart failure, was antinuclear antibody positive and had muscle biopsy findings "consistent with inflammatory myopathy." She was referred to Johns Hopkins University Medical Center with the diagnosis of polymyositis. Further investigation revealed a monoclonal gammopathy, a unique patterning of subcutaneous fat reticulation and hypodense bone marrow changes on magnetic resonance imaging (MRI), and an endocardial biopsy sample that was positive for light chain amyloid deposition. paraffin sections of the muscle biopsy sample from the time of her original presentation were obtained, and congo red staining showed diffuse amyloid deposition throughout the sample, but no inflammation. This case not only illustrates that proximal muscle weakness due to primary amyloid myopathy (as found in light chain amyloidosis and transthyretin amyloidosis) can mimic that of polymyositis, but also shows that unique findings on MRI can alert the clinician to the diagnosis of amyloidosis prior to muscle biopsy.- - - - - - - - - - ranking = 1keywords = myopathies (Clic here for more details about this article) |
6/18. Misunderstandings, misperceptions, and mistakes in the management of the inflammatory myopathies.Many misconceptions persist concerning fundamental issues related to the idiopathic inflammatory myopathies. Such misconceptions can lead to frank mistakes in the diagnosis and management of these disorders. In some cases, these misperceptions have resulted from overreliance on out-of-date information and "classic" articles that are no longer classic! In other instances, misperceptions persist because of the many voids in our understanding of these diseases. This review uses case presentations to highlight important caveats in diagnosing and managing the common idiopathic inflammatory myopathies.- - - - - - - - - - ranking = 6keywords = myopathies (Clic here for more details about this article) |
7/18. Mitochondrial disease mimicking polymyositis: a case report.The authors report on a 34-year-old woman who had developed severe weakness and reduction in grip strength in both upper and lower limbs. Laboratory blood tests revealed increased levels of muscle enzyme. The presence of progressive bilateral ptosis and external ophthalmoplegia raised the suspicion of a mitochondrial disease, subsequently confirmed by deltoid biopsy and genetic analysis of mitochondrial dna that showed a deletion indicative of kearns-sayre syndrome. In this report we emphasise the need for a differential diagnosis between myositis and other myopathies, particularly the mitochondrial ones.- - - - - - - - - - ranking = 1keywords = myopathies (Clic here for more details about this article) |
8/18. A case of limb-girdle muscular dystrophy with serum anti-nuclear antibody which led to a mistaken diagnosis of polymyositis.A 45-year-old woman had first been diagnosed with polymyositis because of the presence of focal necrosis, regeneration and inflammatory infiltration in the muscle fibers, and elevated creatinine phosphokinase levels. However, a pathological re-evaluation and family history led to the definite diagnosis of limb-girdle muscular dystrophy (MD). This case suggests that MD should be taken into consideration in the differential diagnosis of the inflammatory myopathies and genetic surveys including dystrophin molecule may be necessary if the condition manifests during or after adolescence, or when the family history is uninformative. In this case, the serum anti-nuclear antibody was positive, and it may represent the first time that ANA positivity has been found in limb-girdle MD.- - - - - - - - - - ranking = 1keywords = myopathies (Clic here for more details about this article) |
9/18. Successful combination therapy of cyclosporine and methotrexate for refractory polymyositis with anti-Jo-1 antibody: a case report.Although corticosteroids have been the initial agent for the treatment of inflammatory myopathies (IM), immunosuppressive agents such as azathioprine, methotrexate, cyclophosphamide, or cyclosporine are commonly required to control the disease except mild cases. On the other hand, the efficacy of combination therapy of cyclosporine and methotrexate in severe rheumatoid arthritis has been proven without serious side effects. However, in treatment-resistant myositis, the experience of such a therapy is very limited, and has not been described in refractory polymyositis with anti-Jo-1 antibody. Here, we report a young female patient with recalcitrant polymyositis and anti-Jo-1 antibody who was successfully treated with the combination therapy of cyclosporine and methotrexate. At first, the myositis did not respond to several agents, such as corticosteroid, monthly pulse cyclophosphamide, azathioprine, or cyclosporine. methotrexate was initially avoided as treatment regimen because of its potential pulmonary toxicity in the case with preexisting lung disease.- - - - - - - - - - ranking = 1keywords = myopathies (Clic here for more details about this article) |
10/18. Inclusion body myositis (IBM). Morphological study.Among the chronic idiopathic inflammatory myopathies inclusion body myositis (IBM) has emerged as a clinicopathologic variant. Slowly progressive weakness of the distal and the proximal muscle groups, the presence of rimmed vacuoles with basophilic granules as well as 15-18-nm filamentous inclusions in affected muscle confirm the clinical and histopathological distinction between inclusion body myositis and chronic polymyositis.- - - - - - - - - - ranking = 1keywords = myopathies (Clic here for more details about this article) |
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