1/44. Heart transplant for dilated cardiomyopathy associated with polymyositis.Cardiac involvement is one of the most significant factors in the poor clinical outcome of polymyositis. The case of a 39 year old African American woman with polymyositis, cardiomyopathy, and severe heart failure who had orthotopic heart transplantation is described. review of the literature reveals that cardiac manifestations of polymyositis are frequent and include conduction system abnormalities, myocarditis, cardiomyopathy, coronary artery atherosclerosis, valvar disease, and pericardial abnormalities.- - - - - - - - - - ranking = 1keywords = myopathy (Clic here for more details about this article) |
2/44. polymyositis with respiratory muscle weakness requiring mechanical ventilation in a patient with metastatic thymoma treated with octreotide.Although most patients with thymoma present with a mediastinal mass amenable to surgical resection, some patients develop metastatic disease requiring systemic therapy. The majority of thymomas express somatostatin receptors as demonstrated by octreotide scanning, an observation which has prompted the clinical use of octreotide in patients with this disease. Many patients with thymoma exhibit autoimmune paraneoplastic syndromes, most frequently myesthenia gravis. We report here the case of a patient with metastatic thymoma who developed a profound autoimmune polymyositis and lupus-like syndrome that flared following treatment with octreotide and was associated with a clinical response to this agent. No evidence for myesthenia gravis was discovered. The severity of the myopathy necessitated mechanical ventilation for 12 weeks. The natural history of thymoma, treatment options including recent combination chemotherapy regimens, and potential mechanisms for flaring of autoimmune paraneoplastic syndromes triggered by therapy of thymoma are discussed.- - - - - - - - - - ranking = 0.16666666666667keywords = myopathy (Clic here for more details about this article) |
3/44. Quadriceps sparing myopathy.magnetic resonance imaging (MRI) has been proven to be a useful tool in the evaluation of myopathy. Myopathic changes secondary to processes such as inflammatory disease, neuropathy, and neuromuscular disorders often involve several muscle groups. We describe a unique case of lower extremity myopathy with sparing of the quadriceps muscle group on MRI evaluation.- - - - - - - - - - ranking = 1keywords = myopathy (Clic here for more details about this article) |
4/44. Amyloid myopathy masquerading as polymyositis.OBJECTIVE: It is not well appreciated that the clinical presentation of amyloid myopathy can mimic that of polymyositis. By retrospective clinicopathologic analysis we determined distinctive features of amyloid myopathy that differentiate the 2 diseases. methods: Two patients with clinical and histologic evidence of an inflammatory myopathy had fatal outcomes despite appropriate treatment for polymyositis. Their clinical course and original pathologic specimens were reviewed. In addition, original tissue samples were obtained and analyzed using congo red staining and immunoperoxidase. RESULTS: The initial diagnosis of polymyositis was supported in both cases by muscle biopsies showing inflammatory infiltrates and elevations of creatine phosphokinase and by classic electromyography. Retrospective evaluation of the initial muscle biopsies disclosed subtle but incontrovertible evidence of vascular amyloid. Further analysis of the original specimens confirmed the presence of immunoglobin light chain (AL) amyloid. CONCLUSION: Amyloid myopathy can mimic polymyositis. Both can have similar clinical symptoms, as well as inflammatory infiltrates on muscle biopsy. Failure to recognize amyloid myopathy deprives patients of potentially life prolonging treatment. congo red staining and immunohistochemical analysis of tissue could prevent misdiagnosis.- - - - - - - - - - ranking = 1.5keywords = myopathy (Clic here for more details about this article) |
5/44. Primary biliary cirrhosis and myopathy: an uncommon association.Primary biliary cirrhosis (PBC) is a cholestatic liver disease, which is characterized by a chronic inflammatory destruction of intrahepatic bile ducts. It is a rare disorder whose precise etiology is still to be elucidated. Even though the liver is the principal target of PBC, other organ systems also might be affected. Muscular involvement has rarely been described in this disease, and in the majority of cases, muscular weakness has been interpreted as polymyositis. We report the case of a 48-year-old woman suffering from classic PBC, in association with a myopathy whose histological features are distinct from the cases reported before. We also performed a medline research for PBC and concomitant muscular diseases.- - - - - - - - - - ranking = 0.83333333333333keywords = myopathy (Clic here for more details about this article) |
6/44. 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 = 0.16666666666667keywords = myopathy (Clic here for more details about this article) |
7/44. 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 = 0.16666666666667keywords = myopathy (Clic here for more details about this article) |
8/44. 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 = 0.16666666666667keywords = myopathy (Clic here for more details about this article) |
9/44. Inflammatory myopathy on HTLV-I infection: case report.We describe a 41 years old woman who 17 years ago presented hypotonia and proximal muscular weakness in the upper and lower limbs. On neurological examination, the biceps, triceps and Achilles reflexes were absent; the brachioradialis reflexes were decreased and the patellar reflexes were normal. There was bilateral Babinski sign. The remainder of the neurological examination was unremarkable. In the investigation a myopathic pattern was found in the electromyography. The nerve-conduction study was normal; a ELISA method for htlv-i antibodies was positive in the blood and in the cerebral spinal fluid. The muscle biopsy showed inflammatory myopathy, compatible with polymyositis. This paper focuses the polymyositis in the beginning of an HTLV-I infection case.- - - - - - - - - - ranking = 0.83333333333333keywords = myopathy (Clic here for more details about this article) |
10/44. 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 = 1.3333333333333keywords = myopathy (Clic here for more details about this article) |
| | Next -> |