Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C1762617 (weakness)
 37,932 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Twenty five patients with idiopathic myositis attended this department for long term follow up from 1980 to 1989. Twelve patients had primary polymyositis (four men, eight women) and six had primary dermatomyositis (three men, three women); five women had an overlap syndrome. Two patients had a malignant condition associated with the myositis. The mean age at diagnosis was 40 years. All of the patients had proximal muscle weakness, 18/25 had a raised creatine kinase value (mean 2325 IU/l), 19/20 had an abnormal electromyogram, and 19/24 had positive muscle biopsy samples. Of the disease specific antibodies, anti-Jo-1 was detected in only 1/21 patients tested (three patients with fibrosing alveolitis were negative for this antibody), but the 56 kDa antibody was detected in 12/17 patients. The HLA data analysed in the white patients (17/25) showed that 6/8 of those tested were HLA-DR3 positive. All patients were treated with prednisolone and azathioprine was used for 14/25 patients. Only three deaths occurred during the eight year follow up, but there was a substantial morbidity, which may reflect the referral pattern. Muscle strength tests and creatine kinase levels were useful in recording the response to treatment in some patients. These data emphasise that careful long term follow up of patients with myositis is mandatory and that although the present treatment strategy has substantially reduced the death rate, morbidity associated with the disease remains a major problem.
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PMID:Idiopathic myositis: a rheumatological view. 154 36

Polymyositis is a rare disease entity belonging to the class of enigmatic disorders known as the collagen vascular diseases. It is characterized by proximal muscle weakness. More than 50% of the patients with this disease have development of head and neck manifestations, most commonly a heliotrope rash or dysphagia. Weakness and atrophy of neck muscles, and lolling of the neck have also been described. We report a case of polymyositis presenting as a neck mass, a heretofore undescribed manifestation of this disease in the head and neck. A review of the literature and an interdisciplinary approach to the diagnosis and management of this unusual disease will be described.
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PMID:Polymyositis presenting as a neck mass. 155 55

Polymyositis-dermatomyositis (PM-DM) is an inflammatory disease of muscle and skin mediated by autoimmune and cellular events. Most typically, muscle weakness is the usual presentation. This review emphasizes that often the systemic components of this disease may mask the usual presentation and actually may be the presenting and only manifestations; more often than not they are the causes of increased morbidity and mortality. In particular, the cardiopulmonary manifestations may dominate the disease course. Cardiac complications include congestive heart failure resulting from a primary cardiomyopathy, disrhythmias and atrioventricular conduction disturbances, sick sinus syndrome, and cor pulmonale either secondary to interstitial lung disease (ILD) or primary pulmonary artery hypertension. Recurrent aspiration pneumonia results from pharyngeal muscle involvement by the myositic process. Several histologic patterns of ILD can emerge with varying outcomes and responses to immunosuppresive therapy. Involvement of the muscles of respiration can lead to hypercapnic respiratory failure, diaphragmatic dysfunction, hypostatic pneumonia, and restrictive lung disease.
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PMID:Pulmonary and cardiac manifestations of polymyositis-dermatomyositis. 157 25

A 55-year-old woman was admitted to our hospital, complaining of general malaise, muscular weakness, anorexia and weight loss. She had a history of ingesting of a certain germanium (Ge) compound over the preceding 19 months, with a total dose of 47 g as Ge element. She was found to have renal failure (blood urea nitrogen, 44 mg/dl; serum creatinine, 2.6 mg/dl) without abnormal findings in urinalysis, and muscular and nervous damage. Initially, polymyositis was diagnosed and prednisolone administered. However, no improvement was seen, and neuromuscular symptoms and signs steadily worsened, ending in death. Microscopic study of the kidney showed that lipofuscin granules increased in the cells of the thick ascending limb of Henle's loop to the distal convoluted tubule accompanying mild tubular atrophy and that some of the tubules of these segments had vacuolar degeneration or desquamation. No apparent glomerular and vascular changes were observed. High Ge content was found in serum, urine and various tissues, e.g., spleen, liver, kidney, adrenal gland and myocardium, while in controls Ge could not be detected in sera, urine or tissues. We also review case reports about Ge toxicity, and discuss the pathogenesis of renal failure induced by Ge compounds.
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PMID:Nephrotoxicity of germanium compounds: report of a case and review of the literature. 158 20

The case of a 41 year old woman with amyloid myopathy is reported. Clinical involvement consisted of limb girdle muscle weakness, mild scapular muscle atrophy and dysphagia. In contrast with the published cases, abnormal firmness, pseudohypertrophy of the musculature and macroglossia were absent. Muscle biopsy showed endo- and perimysial amyloid deposits but also inflammatory infiltrates. Inflammatory cells typing was studied by immunocytochemical methods and revealed a predominant T-helper cell infiltration. Free kappa light chains were present in serum and urine. Serum immunoglobulin levels were reduced. Bone marrow examination revealed mild plasmocytosis without abnormal cells. Immunofluorescence and immunoperoxidase techniques for identification of the type of amyloid fibrils showed positivity with antisera to kappa light chains. A 4-year follow-up revealed a progressive worsening of muscle weakness despite immunosuppressive treatment. No malignant plasmocytosis occurred. The unusual inflammatory muscle infiltration observed in this case may suggest an associated polymyositis.
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PMID:[Non hypertrophic amyloid myopathy with muscular inflammation in plasma cell dyscrasia]. 160 34

We describe an outbreak of trichinosis in 3 members of a rural family. In the 3 patients eating raw pork was the source of infection. They presented with myalgias and severe proximal muscle weakness mimicking polymyositis. The diagnosis was made by demonstration of larvae of Trichinella spiralis in the muscle biopsy and also by the presence of anti-Trichinella antibodies detected by double immunodiffusion in their sera. We call attention to the unusual clinical presentation of trichinosis in our patients that was manifested by severe muscle weakness that may be confused with polymyositis.
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PMID:Trichinosis with severe myopathic involvement mimicking polymyositis. Report of a family outbreak. 162 35

The IIM are a heterogeneous group of systemic rheumatic diseases which share the common features of chronic muscle weakness and mononuclear cell infiltrates in muscle. A number of classification schemes have been proposed for them, but none takes into consideration the marked immunologic, clinical, and genetic heterogeneity of the various clinical groups. We compared the usefulness of myositis-specific autoantibodies (anti-aminoacyl-tRNA synthetases, anti-SRP, anti-Mi-2 and anti-MAS) to the standard clinical categories (polymyositis, dermatomyositis, overlap myositis, cancer-associated myositis, and inclusion body myositis) in predicting clinical signs and symptoms, HLA types, and prognosis in 212 adult IIM patients. Although patients with inclusion body myositis (n = 26) differed in having significantly more asymmetric and distal weakness, falling, and atrophy than other patients, there were few other significant differences among the other clinical groups. In contrast, autoantibody status defined distinct sets of patients and each patient had only 1 myositis-specific autoantibody. Patients with anti-amino-acyl-tRNA synthetase autoantibodies (n = 47), compared to those without these antibodies, had significantly more frequent arthritis, fever, interstitial lung disease, and "mechanic's hands"; HLA-DRw52; higher mean prednisone dose at survey, higher proportion of patients receiving cytotoxic drugs, and higher death rates. Those with anti-signal recognition particle antibodies (n = 7) had increased palpitations; myalgias; DR5, DRw52; severe, refractory disease; and higher death rates. Patients with anti-Mi-2 antibodies (n = 10) had increased "V-sign" and "shawl-sign" rashes, and cuticular overgrowth; DR7 and DRw53; and a good response to therapy. The 2 patients with anti-MAS antibodies were the only ones with alcoholic rhabdomyolysis preceding myositis; both had insulin-dependent diabetes mellitus, and both had HLA-B60, -C3, -DR4, and -DRw53. These findings suggest that myositis-specific autoantibody status is a more useful guide than clinical group in assessing patients with myositis, and that specific associations of immunogenetics, immune responses, and clinical manifestations occur in IIM. Thus the myositis-specific autoantibodies aid in interpreting the diverse symptoms and signs of myositis patients and in predicting their clinical course and prognosis. We propose, therefore, that an adjunct classification of the IIM, based on the myositis-specific autoantibody status, be incorporated into future studies of their epidemiology, etiology, and therapy.
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PMID:A new approach to the classification of idiopathic inflammatory myopathy: myositis-specific autoantibodies define useful homogeneous patient groups. 165 47

Peripheral nerve involvement is rare in polymyositis and dermatomyositis. We describe a patient in whom the clinical appearance was suggestive of a markedly asymmetrical presentation of polymyositis. Nerve conduction studies, however, were consistent with multiple neuropathies and suggest that neurological involvement may have been the basis of the asymmetrical weakness. Both the muscle weakness and the neurological abnormalities resolved with corticosteroid therapy. This case illustrates a previously undescribed neurological complication of polymyositis.
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PMID:Asymmetrical weakness in polymyositis associated with neuropathic involvement. 166 48

Polymyositis and dermatomyositis are inflammatory myopathies characterized by proximal muscle weakness and myopathic electromyographic and histological findings. While the causes of myositis are not known, the close association of these disorders with a spectrum of autoantibodies suggests an etiologic and/or pathogenetic role for autoimmune processes. Of particular interest in this regard are antibodies directed against histidyl as well as other tRNA synthetases which are almost uniquely associated with myositis and may define a distinct subset of patients. Recently we isolated the histidyl tRNA synthetase gene which encodes the autoantigen representing the most frequent target of the myositis autoimmune response. The isolation and expression of this gene has allowed us to investigate both the autoreactive epitopes on histidyl-tRNA synthetase and the extent to which these correlate with functional epitopes on the molecule. As described here, the results of these studies as well as other recent data pertaining to the immunopathogenesis of myositis, provide a framework for delineating the mechanisms which render synthetases and other translation-related proteins autoantigenic in myositis, and allow one to examine the significance of such autoimmune responses in the etiology and pathogenesis of inflammatory myopathy.
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PMID:Anti-Jo-1 autoantibodies and the immunopathogenesis of autoimmune myositis. 172 33

Animal models have proven very useful in furthering insight into a number of muscle diseases. Studies of ethanol-fed rats are being used to understand the pathogenetic mechanisms underlying acute and chronic myopathy induced by ethanol. Several animal species, including mice, dogs, and cats, develop X-linked muscular dystrophies, which have genetic defects identical to those of Duchenne muscular dystrophy. As in the human disease, these animals lack dystrophin. They are being used to investigate the mechanisms by which lack of dystrophin results in weakness and to examine myoblast transfer as a treatment modality. A model of eosinophilia-myalgia syndrome has recently been induced in Lewis rats by the feeding of L-tryptophan samples that were implicated in the clinical syndrome in humans, making possible studies of the pathogenesis of this interesting new entity. A dermatomyositis-like syndrome occurs spontaneously in dogs, and polymyositis-like illnesses can be induced in mice by immunization with muscle or following infection with selected viruses, especially enteroviruses. Study of the latter is helping us understand mechanisms in the etiology and pathogenesis of inflammatory myositis and virus-induced autoimmunity.
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PMID:Animal models of myopathy. 177 47


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