Cases reported "Myositis"

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1/61. S-1 radiculopathy as a possible predisposing factor in focal myositis with unilateral hypertrophy of the calf muscles.

    Associated with chronic S-1 radiculopathy, a 44-year-old man developed unilateral hypertrophy of the calf muscles. electromyography revealed neurogenic alterations in the corresponding limb compatible with S-1 radiculopathy. In addition, MR-tomographic and bioptic findings were consistent with a focal inflammatory myopathy of the enlarged right gastrocnemius muscle. Predisposing factors for the localisation of a focal myositis are unknown. This case report highlights the diagnostic difficulties in distinguishing focal myositis and denervation hypertrophy following S-1 radiculopathy or secondary inflammation related to denervation. We consider the possibility that in our case the inflammatory process might have been triggered by electromyographically proven chronic denervation related to radiculopathy.
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2/61. pyomyositis of the iliacus muscle in a child.

    pyomyositis is rarely seen in temperate climates. Typically, it presents with the formation of an abscess requiring surgical drainage and it has been reported as a differential diagnosis for septic arthritis of the hip. We describe the occurrence of pyomyositis of the iliacus muscle in a ten-year-old girl which was diagnosed by MRI and blood culture. Formation of an abscess did not occur despite marked focal inflammation and swelling of the muscle. Conservative treatment with antibiotics alone led to complete clinical and radiological resolution of the infection. We could find no previous description of pyomyositis in a child in the British orthopaedic literature. Orthopaedic surgeons, particularly those with a paediatric interest, should be aware of this condition and its presentation, diagnosis and treatment.
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3/61. Focal myositis.

    Focal myositis is a rare, benign focal inflammation of muscle. The lesion often presents as a mass that may be mistaken for a soft tissue sarcoma. This report describes the MRI and histopathological features of a case and illustrates how the diagnosis may be suspected on the basis of the MR findings.
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4/61. Congenital inflammatory myopathy: a demonstrative case and proposed diagnostic classification.

    There have been few reports of congenital inflammatory myopathy in the literature, and most of these have been associated with congenital muscular dystrophy. We review the literature and present a case with electromyographic and muscle biopsy evidence of congenital inflammatory myopathy with onset in the perinatal period and no evidence of a congenital muscular dystrophy. There was evidence of subjective improvement following corticosteroid administration (approximately 1 mg/kg per day) with a concomitant normalization of the serum creatine kinase activity. Of particular interest in the case was the history of maternal infection, suggesting a possible postinfectious molecular mimicry as the etiology of the muscle inflammation. This case suggests that a rare form of congenital inflammatory myopathy does exist that is not associated with a congenital muscular dystrophy. A preliminary classification scheme is proposed to separate true congenital cases from those acquired after birth and those cases associated with congenital dystrophy.
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5/61. Monocytic fasciitis: a newly recognized clinical feature of tumor necrosis factor receptor dysfunction.

    Tumor necrosis factor receptor-associated periodic syndrome (TRAPS) is a dominantly inherited autoinflammatory syndrome that results from mutations in TNFRSF1A, the gene that encodes the 55-kd tumor necrosis factor receptor. Clinically, patients present with recurrent episodes of fever in conjunction with localized inflammation at various sites. myalgia is one of the most characteristic features of this syndrome and is frequently associated with an overlying erythematous, macular rash that, together with the myalgia, displays centrifugal migration. This has previously been believed to occur as a result of myositis. We describe herein the case of a 60-year-old man with TRAPS, in whom magnetic resonance imaging of the left thigh demonstrated edematous changes in the muscle compartments and surrounding soft tissues. A full-thickness wedge biopsy was performed, and hematoxylin and eosin staining and immunohistochemistry analysis of the specimen demonstrated normal myofibrils but a severely destructive monocytic fasciitis. These results suggest that the myalgia experienced by individuals with TRAPS is due to a monocytic fasciitis and not to myositis.
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keywords = inflammation
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6/61. Myopathy with antibodies to the signal recognition particle: clinical and pathological features.

    OBJECTIVES: To study myopathies with serum antibodies to the signal recognition particle (SRP), an unusual, myositis specific antibody associated syndrome that has not been well characterised pathologically. methods: Clinical, laboratory, and myopathological features were evaluated in seven consecutive patients with a myopathy and serum anti-SRP antibodies, identified over three years. The anti-SRP myopathy was compared with myopathology in other types of inflammatory and immune myopathies. RESULTS: The patients with anti-SRP antibodies developed weakness at ages ranging from 32 to 70 years. Onset was seasonal (August to January). Weakness became severe and disability developed rapidly over a period of months. Muscle pain and fatigue were present in some patients. No patient had a dermatomyositis-like rash. serum creatine kinase was very high (3000 to 25 000 IU/l). Muscle biopsies showed an active myopathy, including muscle fibre necrosis and regeneration. There was prominent endomysial fibrosis, but little or no inflammation. Endomysial capillaries were enlarged, reduced in number, and associated with deposits of the terminal components of complement (C5b-9, membrane attack complex). Strength improved in several patients after corticosteroid treatment. CONCLUSIONS: Myopathies associated with anti-SRP antibodies may produce severe and rapidly progressive weakness and disability. Muscle biopsies show active myopathy with pathological changes in endomysial capillaries but little inflammation. Corticosteroid treatment early in the course of the illness is often followed by improvement in strength. In patients with rapidly progressive myopathies and a high serum creatine kinase but little inflammation on muscle biopsy, measurement of anti-SRP antibodies and pathological examination of muscle, including evaluation of endomysial capillaries, may provide useful information on diagnosis and treatment.
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ranking = 3
keywords = inflammation
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7/61. Interstitial lung disease in polymyositis and dermatomyositis: analysis of six cases and review of the literature.

    Interstitial pneumonitis may be the presenting manifestation of polymyositis-dermatomyositis, or may occur later in the evolution of the disease. The clinical picture is characterized by non-productive cough, dyspnea and hypoxemia. The chest radiograph demonstrates interstitial infiltrates with predilection for the lung bases, often with an alveolar pattern in addition. The histopathologic features are those of organizing and interstitial pneumonitis and pleuritis, with variable fibrosis. In the present series, the patients with mixed alveolar and interstitial infiltrates on chest radiograph and organizing pneumonia and bronchiolitis obliterans in addition to interstitial pneumonitis. In one patient evolution from pulmonary inflammation to interstitial fibrosis was demonstrated. The etiology of primary lung disease in PM-DM is not known, but cell-mediated autoimmunity to an unidentified component of lung tissue is suggested. Including the present series, 50 percent of patients have responded favorably to corticosteroids with decreased dyspnea, clearing of the chest radiograph and improved pulmonary function tests.
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keywords = inflammation
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8/61. Pathophysiology and treatment of streptococcal toxic shock syndrome.

    We have recently encountered three cases of streptococcal toxic shock syndrome, each of which had a different cause. All the patients had inflammation of soft tissue in the lower extremities, and developed shock and multiple organ failure immediately after the clinical visit. The inflammation of soft tissue was necrotising fasciitis in one case, myositis in one case, and phlegmon in one. In the first case the debridement was incomplete, which resulted in an extensive ulceration. Wary of repeating this experience, we made an early diagnosis and did a thorough debridement in the second case. The patient was ultimately discharged without complications. It is rare that a patient with extensive myositis survives without amputation of the extremity. The third patient responded well to early treatment with antibiotics.
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ranking = 2
keywords = inflammation
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9/61. Chemotherapy-induced radiation recall myositis.

    Myofasciitis syndrome encompasses a group of disorders characterized by chronic inflammation and/or fibrosis of the subcutaneous septa and muscular fascia. We report on a patient in whom myositis was diagnosed in the areas previously irradiated for papillary thyroid carcinoma and anal canal carcinoma respectively 21 and 3 years after radiotherapy. We are not able to explain why myopathy developed at the same time in two different sites at a different interval from the two radiotherapic schemes. We can suppose that the patient developed a subclinical regional myopathy after the first radiotherapic scheme. radiation induced heritable mutations within surviving cells that were unable to tolerate the second damage by systemic chemotherapy. It is unclear how radiosensitization correlates with an ability to reactivate latent effects in normal tissue. physicians using chemotherapic radiosensitizers should be aware of their potential to induce a delayed form of radiosensitization. We report this case to encourage physicians to be alert to the knowledge of the clinical, histologic and morphologic characteristics of radiation myositis in order to distinguish it from an infectious or immune fasciitis or myositis.
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keywords = inflammation
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10/61. GNE mutations causing distal myopathy with rimmed vacuoles with inflammation.

    The authors describe a family in which two individuals have clinical distal myopathy with rimmed vacuoles (DMRV). While the clinical and most of the pathologic features in these patients were compatible with a diagnosis of DMRV, the presence of inflammatory changes in the connective tissue between muscle fibers was not. Gene analysis revealed a compound heterozygous mutation in these individuals, characterized by V572L and I472T.
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ranking = 4
keywords = inflammation
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