1/32. Skeletal muscle pathology in 2 siblings infected with toxoplasma gondii.Skeletal muscle can be the site of inflammatory diseases that lead to muscle weakness, pain, and increased myogenic serum enzymes. Most of these inflammatory myopathies are idiopathic. In some cases inflammatory myopathies are due to infectious agents. We describe the pathological aspects of muscle biopsies of 2 Brazilian siblings who acquired toxoplasmosis at the same time and in similar conditions. One developed a tetraplegia that was confirmed to be due to inflammatory myositis due to toxoplasma. The other developed myocarditis, with heart failure, without skeletal muscle weakness. In both cases many toxoplasma organisms were observed in the muscle biopsies, but in case 1 only was there an inflammatory myopathy with myofiber necrosis; the inflammatory cells were predominantly macrophages with some CD4 cells and rare CD20 cells. In case 1, expression of CD54 was observed in many inflammatory cells as well in endothelial cells, but only in endothelial cells in case 2. After treatment with clindamycin and corticosteroids both cases had only partial improvement, case 1 with a residual muscle weakness and case 2 with residual cardiac insufficiency (requiring digoxin). These cases show that the presence of the parasite in myofibers is not enough to induce an inflammatory myositis with muscle cell necrosis. This suggests that immunological disturbances may contribute to the development of inflammatory myositis due to toxoplasma.- - - - - - - - - - ranking = 1keywords = myopathies (Clic here for more details about this article) |
2/32. Granulomatous myositis, primary biliary cirrhosis, pancytopenia, and thymoma.Granulomatous myopathies are rare. Most cases are associated with sarcoidosis. We report a case of granulomatous myopathy associated with primary biliary cirrhosis, pancytopenia, and thymoma. The literature in regard to granulomatous myopathy and its pathogenesis is reviewed. Intermittent pulsed intravenous methylprednisolone may be useful as maintenance therapy for granulomatous myopathy and other neuromuscular syndromes for patients intolerant of oral corticosteroids.- - - - - - - - - - ranking = 0.5keywords = myopathies (Clic here for more details about this article) |
3/32. Inflammatory myopathy associated with mixed connective tissue disease and scleroderma renal crisis.mixed connective tissue disease is a rheumatological syndrome with neuromuscular manifestations that may include inflammatory myopathy and carpal tunnel syndrome. We report a patient who presented with an inflammatory myopathy and bilateral carpal tunnel syndrome prior to the diagnosis of a connective tissue disorder. Early in the course of treatment, a syndrome of acute renal failure developed. knowledge of this syndrome, scleroderma renal crisis, and its possible relation to corticosteroid treatment is important to clinicians involved in the management of patients with inflammatory myopathies.- - - - - - - - - - ranking = 0.5keywords = myopathies (Clic here for more details about this article) |
4/32. 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 = 3keywords = myopathies (Clic here for more details about this article) |
5/32. 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.- - - - - - - - - - ranking = 1.5keywords = myopathies (Clic here for more details about this article) |
6/32. Necrotising myopathy, an unusual presentation of a steroid-responsive myopathy.OBJECTIVE: To evaluate the clinical features, muscle pathology and response to treatment in patients with a necrotising myopathy, without mononuclear cell infiltrates. BACKGROUND: Mononuclear cell infiltrates in the muscle biopsy specimen are the diagnostic hallmark of the immune-mediated idiopathic inflammatory myopathies (IIM). In patients with the typical clinical features of IIM, absence of these infiltrates in the muscle biopsy specimen casts doubt on the diagnosis and leads to uncertainty about therapeutical strategies. methods: A detailed description is given of the clinical, laboratory, and histopathological features of eight patients suspected of having an idiopathic inflammatory myopathy, in whom mononuclear cell infiltrates in their muscle biopsy specimens were lacking. RESULTS: Eight patients (five men, three women, age range 40-69 years) had severe, symmetrical proximal weakness with a subacute onset. There were no skin abnormalities suggesting dermatomyositis. serum creatine kinase activity was more than 10 times elevated. Repeated muscle biopsy specimens, taken from a symptomatic muscle prior to immunosuppressive treatment showed widespread necrosis, regeneration, and atrophy of muscle fibres, but no mononuclear cell infiltrates. Known causes of necrotising myopathy were excluded. Three patients had a malignancy. Adequately dosed and sustained immunosuppressive treatment eventually resulted in normal or near normal muscle strength in seven patients. One patient showed marked improvement. CONCLUSION: Occasionally, patients who clinically present as an idiopathic inflammatory myopathy may lack mononuclear cell infiltrates in their muscle biopsy specimens. This subacute-onset progressive necrotising myopathy should not deter the clinician from timely and appropriate treatment as we consider this myopathy to be steroid-responsive with a possible immune-mediated pathogenesis.- - - - - - - - - - ranking = 0.5keywords = myopathies (Clic here for more details about this article) |
7/32. Management of dysphagia in inclusion body myositis.Inclusion body myositis is an inflammatory myopathy in which dysphagia has been considered a rare finding. However, recent literature finds dysphagia an increasingly common symptom as more cases of inclusion body myositis are identified. Unlike some inflammatory myopathic disorders, inclusion body myositis is resistant to treatment with corticosteroids, and therefore, the otolaryngologist may be consulted regarding surgical options for relief of dysphagia. A patient is described in whom severe progressive dysphagia associated with inclusion body myositis developed. Impaired pharyngeal wall motion and cricopharyngeal achalasia were demonstrated by videofluoroscopic evaluation, and the patient was successfully treated by cricopharyngeal myotomy. The pathophysiologic nature of inclusion body myositis and the mechanisms of cervical dysphagia in the inflammatory myopathies are reviewed.- - - - - - - - - - ranking = 0.5keywords = myopathies (Clic here for more details about this article) |
8/32. 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 = 0.5keywords = myopathies (Clic here for more details about this article) |
9/32. Treatment of idiopathic inflammatory myopathies with cyclophosphamide pulses: clinical experience and a review of the literature.Short-term cyclophosphamide pulse therapy was administered to five patients with idiopathic inflammatory myopathies (IIM) which were refractory to corticosteroids and, in two cases, to other aggressive therapies. Both disease remission and a reduction in corticosteroid dosage were obtained in four patients. Three of them have been in remission over since, taking only minimal doses of steroids during the subsequent two years. In the fourth patient a less intensive, maintenance pulse therapy could not be continued due to unacceptable side effects. On the basis of these results and of data from the literature, herein reviewed, cyclophosphamide pulse therapy may be recommended for adult patients with IIM who do not respond to or who do not tolerate conventional corticosteroid treatment, as well as when a steroid-sparing effect is desired.- - - - - - - - - - ranking = 2.5keywords = myopathies (Clic here for more details about this article) |
10/32. A case of anti-Jo1 myositis with pleural effusions and pericardial tamponade developing after exposure to a fermented Kombucha beverage.The pathogenesis of the idiopathic inflammatory myopathies has been postulated to be an environmental trigger causing the expression of the disease in a genetically predisposed patient. We report a case of anti-Jo1 antibody-positive myositis which was associated with pleural effusions, pericardial effusion with tamponade, and 'mechanic's hands', probably related to the consumption of a fermented Kombucha beverage. Kombucha 'mushroom', a symbiosis of yeast and bacteria, is postulated to be the trigger for our patient's disease owing to the proximity of his symptoms to the consumption of the Kombucha beverage.- - - - - - - - - - ranking = 0.5keywords = myopathies (Clic here for more details about this article) |
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