Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0011168 (dysphagia)
 15,644 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

From January 1, 1981 to December 31, 1992, we experienced nine patients with childhood onset of dermatomyositis and polymyositis. The mean age of disease onset was 12 years (range 7 to 16 years). Seven of them fulfilled the criteria of dermatomyositis, the remaining two were polymyositis. Girls were more predominant than boys in 6:3 ratio. The clinical features included extremities muscle weakness, skin rash, periorbital swelling and dysphagia. Increased muscle enzymes including creatine phosphokinase (CPK) or lactic dehydrogenase (LDH) were all positive in nine patients. All of our nine patients were treated with prednisolone after the diagnosis was established. The duration of treatment ranged from 3 to 65 months (mean: 25.3 months). Two of the nine patients also received immunosuppressive agents, hydroxychloroquine and azathioprine respectively. At present six patients survive without treatment. Two patients continue with corticosteroid and immunosuppressive therapy. One patient died from primary peritonitis, six months after being diagnosed with JDMS. In conclusion our study shows there is a female dominance; monocyclic clinical course is more common; and the prognosis is good in general, in the cases of juvenile dermatomyositis and polymyositis.
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PMID:Dermatomyositis and polymyositis in childhood. 794 27

A 36-year-old woman gradually developed dysphagia and muscle weakness of the lower extremities. Diagnosis of polymyositis was given from elevation of serum creatine kinase and pathological findings of a muscle biopsy. Despite oral prednisolone and intravenous pulse methylprednisolone therapy, her muscle weakness persisted, and then pulse intravenous cyclophosphamide (IVCY) therapy was initiated and repeated five times in total, which resulted in significant improvement in muscle strength. Thereafter, weekly administration of methotrexate at low dosage further normalized the serum creatine kinase level. We may conclude that IVCY and low-dose weekly methotrexate together could be an alternative in refractory polymyositis.
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PMID:Successful treatment of refractory polymyositis with pulse intravenous cyclophosphamide and low-dose weekly oral methotrexate therapy. 814 84

Myositis due to the etiologic agent of Lyme disease, Borrelia burgdorferi, has been reported nine times in the English-language literature; there has been but a single report of exacerbation of dermatomyositis due to B. burgdorferi in a patient with known dermatomyositis. Multiple infectious agents, but not B. burgdorferi, have been hypothesized to trigger dermatomyositis. We report the first case of dermatomyositis that appears to have been triggered by B. burgdorferi. This case involved an individual from Westchester County, NY, who presented with skin lesions suggestive of erythema migrans and who was seropositive for Lyme disease. He soon developed a clinical syndrome suggestive of dermatomyositis: periorbital edema, dysphagia, proximal muscle weakness, and a markedly elevated level of creatine phosphokinase. We also review the clinical presentation and response to treatment of patients with Lyme myositis.
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PMID:Dermatomyositis associated with Lyme disease: case report and review of Lyme myositis. 816 22

We report a family of mitochondrial myopathy which appeared to be interited as an autosomal dominant trait. The proband is a 58-year-old Japanese male, who presented with bilateral ptosis, chronic progressive ophthalmopletia, dysphagia, and atrophy of proximal muscles in the upper extremities. There was no cataract or retinal degeneration. Serum creatine kinase (CK) and lactic acid levels were normal. Cardiac evaluations were normal. Muscle biopsy revealed 7% of ragged red fibers. Cytochrome c oxidase activity in the muscle was decreased to 50% of the control value. PCR analysis of muscle mitochondrial DNA revealed 3 large-scale deletions in the non-D-loop regions, ranging in size from 4.2 kb to 5.2 kb. His father, three siblings, and the two children had symptoms similar to the proband. We have reviewed forty-five individuals from six families, including our family, who had mitochondrial myopathy with autosomal dominant inheritance. Frequent manifestations include chronic progressive ophtalmoplegia (91.2%), ptosis (95.6%), hearing loss (72.7%), dysphagia (60.0%), limb weakness (74.1%), and respiratory muscle weakness (75.0%). Interestingly, there is no individual with retinal degeneration or cardiac involvement. Serum CK and lactic acid levels may be elevated. CT of the head is normal. Muscle biopsy shows ragged red fibers and the frequency of cytochrome c oxidase-negative fibers ranges from 0 to 38%. Multiple large-scale deletions of mitochondrial DNA, ranging in size from 4.2 to 8.3 kb, are found in the muscle, all of which are located in the non-D-loop region of the mitochondrial DNA. The multiplicity of deletions may be one to the characteristic features of this form of mitochondrial myopathy.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Mitochondrial myopathy with autosomal dominant inheritance--report of a family and review of the literature]. 831 87

This is the first report on idiopathic inflammatory myopathies (IIM) in French Canadians. We reviewed retrospectively 30 French Canadian adults (20 women and 10 men) with IIM seen consecutively over 12 years. The median age at diagnosis was 45 years. The IIM were 8 (27%) primary polymyositis (PM), 9 (30%) primary dermatomyositis (DM), 5 (17%) IIM with neoplasia (lymphoma, breast, esophageal, colonic, and skin cancer) and 8 (27%) IIM with a connective tissue disease (4 with systemic sclerosis, 2 with mixed connective tissue disease, and 2 with rheumatoid arthritis). The most common presenting symptom was proximal muscle weakness (n = 10,33%). Of the remaining 20 patients, 6 (20%) had the onset of their weakness within 1 month of the presenting symptom. Only 3 (10%) patients did not have proximal muscle weakness. Twenty-six (87%) patients had weakness in the pelvic girdle, 25 (83%) in the shoulder girdle, and 7 (23%) in the neck muscles. Other common symptoms included dyspnea on exertion and dysphagia, each present in 13 (43%) patients. Gottron's papules and the heliotrope rash were the most common skin lesions documented in 11 (37%) and 10 (33%) patients, respectively. The serum creatine kinase (CK) level was between 171 and 1,000 U/L in 13 (43%) patients and between 1,001 and 6,000 U/L in 13 (43%) patients. Antinuclear antibodies (ANA) on HEp-2 cells were positive in 16 (53%) patients, of which 2 (13%) expressed autoantibodies to nuclear pore complexes. Autoantibody specificities were anti-La (n = 4, 13%), anti-U1RNP (n = 3, 10%), and anti-Ro (n = 2, 7%). None of the patients expressed anti-Jo-1, anti-topoisomerase I, or anticentromere antibodies. Twenty-eight (93%) patients received corticosteroid therapy, and 8 (27%) patients responded to prednisone alone. Thirteen (43%) patients were treated with methotrexate, and 9 (69%) responded. The mean follow-up was 62 months: 23 (77%) had their disease controlled, 3 (10%) patients were lost to follow-up, and 4 (13%) died (no death occurred because of IIM or its treatment). Therapy was discontinued because of remission in 5 (17%) patients. Cumulative survival rates at 2, 5, and 10 years were 89%, 89%, and 85%, respectively. The presence of autoantibodies to nuclear pore complexes and anti-La autoantibodies, the rare occurrence of anti-Jo-1 autoantibodies, the response to conventional therapies, and a high survival rate may distinguish IIM in French Canadians from that of other reported series.
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PMID:Distinctive features of idiopathic inflammatory myopathies in French Canadians. 887 Jan 12

A 52-year-old woman was admitted to the hospital with a rash, periorbital edema, dysphagia, and muscle weakness. The rash had appeared on the back of her neck nine months earlier, and because she had recently returned from a wildlife preserve on Assateague Island, Maryland, she assumed that it was due to tick bites. Over the next two months, the rash spread to her forehead, back, chest, and upper extremities, and scaly lesions appeared over metacar-pophalangeal joints. Initial laboratory tests indicated that her creatine kinase level was elevated. She was given prednisone and the level decreased. The rash also improved, but in the next two months the muscle weakness worsened. She became feverish and increasingly fatigued, depressed, and irritable. These later symptoms were attributed to the medication, but when she was weaned from it, her rash, weakness, and dysphagia increased. Over the ensuing months, she was given intramuscular injections of methotrexate (up to 25 mg/wk), followed by oral doses of hydroxychloroquine and azathioprine, but the symptoms persisted.
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PMID:A woman with a post-vacation rash. 910 5

We evaluated the clinical and myopathological features of all patients with granulomas in muscle biopsy specimens identified over a 5-year period (1992-1996) at the Washington University Medical Center. Ten patients were found to have granulomas in their muscle biopsy specimens. Of these, eight patients had myopathic changes. Seven had dysphagia as a major functional difficulty during the course of their disease. None had elevated levels of serum creatine kinase (CK). Four of the patients with myopathy had systemic sarcoidosis and relatively severe proximal weakness with functional disability. Treatment with corticosteroids was followed by marked improvement in strength and functional disability. The four other patients with myopathy had no systemic signs of sarcoidosis. Weakness was especially prominent distally in three of these patients. The two patients in this group treated with corticosteroids did not improve. The final two patients, who had granulomas in muscle but no myopathic changes, had clinical syndromes of mononeuritis multiplex and eosinophilic fasciitis (Shulman syndrome). We conclude that granulomatous myopathy, in the presence or absence of systemic sarcoidosis, is commonly associated with dysphagia (87%) and a normal serum CK. Clinical features in patients with sarcoidosis included severe proximal weakness with functional disability that often responded to corticosteroid treatment. Granulomatous myopathy without systemic sarcoidosis was associated with milder, but more predominantly distal weakness.
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PMID:Clinical correlates of granulomas in muscle. 974 15

The neuroleptic malignant syndrome (NMS) consists in an idiosyncratic reaction to neuroleptic drugs, probably related to a blockage of dopamine receptors in basal ganglia. Research criteria for diagnosing NMS from DSM-IV require severe rigidity and fever accompanied by 2 of 10 minor features including diaphoresis, dysphagia, tremor, incontinence, altered mentation, mutism, tachycardia, elevated or labile blood pressure, leukocytosis and elevation of creatine phosphokinase. From a clinical point of view, the NMS may range a large spectrum of presentations. Haloperidol is the most frequent drug associated with this syndrome. We report the case of a 30 year-old man who developed NMS at two different occasions, the first related to haloperidol and chlorpromazine and the second related to olanzapine, to our knowledge without previous mention in the indexed literature.
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PMID:[Neuroleptic malignant syndrome: case report with recurrence associated with the use of olanzapine]. 1002 91

A patient with polymyositis manifesting severe myocardial damage and conduction block is described. A 57-year-old man presented dysarthria, dysphagia, proximal-dominant muscle weakness and wasting of the extremities. Muscle biopsy revealed degeneration and regeneration of muscle fibers and infiltration of mononuclear cells. After admission, muscle weakness rapidly progressed and mechanical ventilation was needed for respiratory failure. Simultaneously, cardiac symptom developed and resulted in bradycardia and trifascicular conduction block, which required a pacemaker. Echocardiogram revealed diffuse hypokinesia, ventricular enlargement and thickened wall. Marked elevations of serum CK-MB, cardiac myosin light chain I and cardiac troponin T were observed. High dose administration of methylprednisolone resulted in improvement of muscular and cardiac symptoms, and prevented complete heart block. Immediate and high dose of steroid therapy was considered to be effective for severe myocarditis in polymyositis.
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PMID:[A patient of polymyositis with severe myocardial damage and conduction block]. 1039 Oct 81

Inclusion body myositis has been recognized as a major form of idiopathic inflammatory myopathy. An old male patient with insidious onset and slowly progressive muscular weakness and artrophy has been reported in this article. The duration of symptom before biopsy was 23 years. The first symptom was dysphagia, and muscular weakness developed seven years later. Muscular atrophy was predominant symmetrically and proximally, particularly the quadriceps femoris muscles. Cervical and abdominal muscles were also affected. Myalgia was absent. Electromyogrophy showed myopathic alterations. Erythrocyte sedimentation rate, creatine kinase, immunoglobulins G increased slightly or moderately. Rheumatoid factor was positive, and he had been diagnosed as having rheumatoid arthritis for 23 years. Inclusion body myositis was ultimately diagnosed based on the muscle biopsy which showed mononuclear cell invasion of nonnecrotic muscle fibers, the characteristic rimmed vacuoles in cryostat sections and cytoplasmic inclusion bodies consisted of plenty of tubulofilaments by electron microscope.
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PMID:[Inclusion body myositis: clinical and myopathological features]. 1043 72


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