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)

Forty-one children with dermatomyositis who were treated with corticosteroids and who have been followed over a period of 15 years have been presented. Progressive proximal muscle weakness was seen in all and 60% had muscle pain. The skin rash considered classic for dermatomyositis was seen in 33 children at the time of diagnosis. Elevation of serum muscle enzymes, electromyographic abnormalities, and muscle biopsy evidence of acute myositis were of confirmatory diagnostic value. The course of the disease in this study group has reconfirmed the efficacy of adrenal corticosteroid treatment in conjunction with an individualized physical therapy program and consistent followup. Prognosis for life and minimal functional disability has been good. There have been 3 deaths recorded in this series, only one of which was certain in its relationship to dermatomyositis.
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PMID:Dermatomyositis in the pediatric patient. 26 8

A case of eosinophilic polymyositis is reported. Tender muscle swelling was followed by proximal weakness, creatinine kinase elevation, and electromyographic features typical of polymyositis. Severe myocarditis, pericarditis and heart failure were present. Muscle biopsy specimen showed active myositis with eosinophil infiltrate. Unlike previous cases, blood eosinophils count was normal. The clinical response to corticosteroids was excellent, and a relapse occurring as steroid dose was lowered responded rapidly to an increased dose of prednisolone. Eosinophilic polymyositis may be a component of a general systemic illness with prominent cardiac involvement.
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PMID:Eosinophilc polymyositis. 50 94

Six patients with eosinophilic fasciitis are presented. This syndrome is characterized by indurative swellings of arms and legs, with rapidly progressing difficulties in extending elbows, wrists, and fingers, and often limited motion of shoulders and ankle joints. Pain when contracting muscles, and weakness of proximal muscles and hand grip are common features. The frequent occurrence of localized skin lesions has presented differential diagnostic difficulties to systemic sclerosis and to polymyositis. Visceral involvement and Raynaud's phenomena, however, are absent or mild. Blood eosinophilia, hypergammaglobulinemia, and unspecific signs of inflammation are found. Biopsy of muscle fascia gives characteristic histopathological findings of cell infiltrations (mostly mononuclear cells, frequently eosinophils) and vascular proliferation, in the middle layer of a thickened fascia. Skin changes are prevalent, but not conclusive for the diagnosis, and myositis in some patients might be difficult to distinguish from polymyositis. The importance of the clinical recognition of eosinophilic fasciitis and the inclusion of fascia in diagnostic muscle biopsies, is underlined.
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PMID:Eosinophilic fasciitis. Review and report of six cases. 53 17

Four school children, aged 6 to 9 years, had acute postinfectious myositis. The prodromal illness usually involved the upper respiratory tract, but gastrointestinal symptoms were also seen. Fever and nonspecific malaise were characteristic. After cessation of the illness, myalgia involved the calves and thighs. Arm and neck muscles were less frequently affected. Weakness was less marked than muscle pain. Serum creatine phosphokinase (CPK) was markedly increased in all cases. Myalgia and CPK levels subsided in less than a week, although one child was not back to normal for 4 weeks. Two children had electromyography, and patchy myopathic changes were found. Viral studies were not helpful in any of the cases.
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PMID:Benign acute childhood myositis. 57 38

Myoglobin was detected in the sera of patients with dermatomyositis, polymyositis, scleroderma, and systemic lupus erythematosus (LE) with active myopathy. Overall, myoglobinemia was detected in 74.1% of sera taken from patients with active myositis before therapy, with slightly greater frequency in the groups with dermatomyositis and polymositis. With steroid therapy, this frequency fell to 43.4% and to 9.5% in patients in clinical remission not requiring therapy. Serum enzyme (creatine phosphokinase, lactic dehydrogenase, and SGOT) activity was higher in samples containing myoglobin, but there was considerable overlap between those with and without myoglobinemia. Sequential serum determinations in six patients demonstrated rapid reduction in the levels of serum myoglobin with therapy, usually before enzyme values had returned to normal. In one patient followed up for 30 months, myoglobinemia correlated with clinically observed exacerbations of rash and weakness to a greater degree than did enzyme determinations.
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PMID:Myoglobinemia in inflammatory myopathies. 57 36

In eight women with polymyositis (three patients), systemic lupus erythematosus (SLE) (three patients), rheumatoid arthritis (one patient) and shoulder-hand syndrome (one patient), weakness developed during high dose prednisone therapy. These women were studied using serial functional and manual muscle tests, determination of serum glutamic oxaloacetic transminase (SGOT), creatine phosphokinase (CPK) and serum aldolase levels, and urinary excretion of creatine. Insidious onset of weakness was characteristic. Myalgias were seen in five patients and unusual sudden weakness in two. Weakness was always most severe in the pelvic girdle muscles; there was a lesser involvement of shoulder girdle and distal muscles. Serum muscle enzyme levels were normal in all cases, but urinary creatine excretion was invariably increased and proved to be the most sensitive laboratory indicator for clinical diagnosis and for monitoring patient improvement. Serial urinary creatine excretion and serum enzyme studies were of value in differenting steroid myopathy from a flare of myositis in patients with connective tissue disease. Diagnosis and effective management were achieved by the use of readily available laboratory and clinical procedures without resorting to muscle biopsy.
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PMID:Steroid myopathy in connective tissue disease. 97 43

Ten children, three girls and seven boys, aged 4-10 years, were admitted with benign acute childhood myositis during spring and winter months (March 1988 to March 1990). They presented with an acute onset of symmetrical calf muscle pain and tenderness, weakness and inability to walk a few days after a flu-like illness. All had raised serum creatine kinase and a normal erythrocyte sedimentation rate, and the majority had low peripheral white blood cell counts with relative lymphocytosis. Serum aspartate aminotransferase, alanine aminotransferase and lactate dehydrogenase were found to be raised in four patients. Virological studies performed in six children gave negative results. Full clinical recovery was achieved within 1-7 days. One child developed a second episode. These ten cases of benign acute childhood myositis are the first to be reported from this region.
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PMID:Benign acute childhood myositis in Kuwait. 128 48

Interstitial lung disease (ILD) has been recognized as a manifestation of polymyositis or dermatomyositis (PM/DM). Patients with PM/DM with anti-Jo-1 antibody had a higher incidence of ILD. Thirty-five patients with PM/DM were studied the antibodies. Six (16.1%) were found to have them. ILD occurred in all 6 anti-Jo-1 positive patients, as opposed to 10 (34.5%) of 29 anti-Jo-1 negative patients. Only 2 cases of undifferentiated connective tissue disease among 1320 patients with various kinds of collagen vascular diseases other than PM/DM were positive for anti-Jo-1 antibody. These two patients also had lung involvement. Among six PM/DM patients with anti-Jo-1 antibody, dyspnea preceded proximal muscle weakness in three patients. In one case, the anti-Jo-1 antibody had been detected one month prior to the onset of myositis. Anti-Jo-1 antibody is a useful marker for PM/DM with ILD.
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PMID:Anti-Jo-1 antibody in patients with polymyositis/dermatomyositis. 130 48

Twenty-nine patients with myositis, including 10 with polymyositis (PM), 6 with dermatomyositis (DM), and 13 with myositis associated with a connective tissue disease (CTD), were followed up for a mean observation time of 49 months. The 13 patients with CTD-associated myositis were further separated by the presence or absence of anti-RNP antibodies. The functional disability at diagnosis was pronounced without differences between the groups. The patients with anti-RNP antibodies did not differ from the other patients regarding initial muscle weakness, erythrocyte sedimentation rate, or creatinine phosphokinase values, but the histopathological muscle changes were generally milder. Rapid improvement of muscle strength on moderate doses of corticosteroids was seen in most patients. At the end of study, corticosteroid treatment had been withdrawn from 17 patients because of remission, including 6 of the 7 patients with anti-RNP antibodies. The presence of electromyographic changes compatible with myositis, pronounced muscle weakness before treatment, and a low erythrocyte sedimentation rate seemed to indicate a less favorable outcome unrelated to diagnostic subgroupings. However, the combination was rarely found among the anti-RNP-positive patients.
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PMID:A clinical, serological, and histopathological study of myositis patients with and without anti-RNP antibodies. 143 44

A young woman with slowly progressive muscular weakness and atrophy localized in both thighs is reported. Laboratory, electromyographic and histological findings suggested that the patient suffered from chronic myositis with a background of autoimmune disorder. Quadriceps myositis is a rare condition. The previously reported cases of this disease in the literature are reviewed.
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PMID:Quadriceps myositis. 145 May 3


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