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Query: UMLS:C0027121 (myositis)
 4,538 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 38-year-old male developed a new muscle weakness in his left thigh 35 years after having acute paralytic poliomyelitis with residual right distal leg weakness and atrophy. EMG studies showed widespread denervation in proximal and distal muscles regardless the clinical involvement. Muscle biopsy from an affected muscle showed the findings of inclusion-body myositis consisting of perivascular and interstitial mononuclear infiltration, sarcoplasmic granular inclusions with membranous whorls and typical filamentous inclusions in several myonuclei. This raises the possibility of inclusion body myositis in other cases of progressive post-poliomyelitis muscular atrophy, especially those with perivascular infiltration of mononuclear cells in the muscle biopsy.
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PMID:Inclusion body myositis in post-poliomyelitis muscular atrophy. 284 1

The inflammatory myopathies have diverse clinical and pathological features and multiple etiologies. Some are confined to a single muscle or group of muscles (e.g., orbital myositis and localized nodular myositis) while others are diffuse. Infective forms may be due to viral, bacterial, fungal, protozoal, or parasitic organisms. Viruses may cause acute self-limited forms of myositis and have been isolated from muscle in some cases of acute rhabdomyolysis and inclusion body myositis. They have also been implicated in some cases of congenital myopathy and in polymyositis and dermatomyositis, but there is no evidence of viral invasion of muscle in these conditions. In polymyositis and dermatomyositis there are derangements in humoral and cellular immune function, and recent evidence suggests an underlying disturbance of immunoregulation. The roles of genetic factors, drugs, and Toxoplasma infection have been under scrutiny. There is increasing recognition of immunological and pathological differences in polymyositis and juvenile and adult dermatomyositis, and in cases with associated connective tissue diseases, suggesting different underlying pathogenetic mechanisms. Inclusion body myositis, eosinophilic myositis, and granulomatous myositis can be separated from the other idiopathic inflammatory myopathies because of distinctive clinical and pathological features and this may also reflect different mechanisms of muscle injury. Recent developments in the treatment of the idiopathic inflammatory myopathies include the use of plasmapheresis and total-body irradiation in cases that are resistant to corticosteroids and immunosuppressive drugs.
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PMID:Inflammatory myopathies: Part 1. 298 25

Myositis in man may be divided into infectious and non-infectious forms. The myopathologist more often deals with the latter forms which comprise dermatomyositis/polymyositis, inclusion body myositis, mixed connective tissue disease/collagenoses, and granulomatous myopathies. Modern morphological techniques as enzyme-histochemistry, electron microscopy, immunohistology, and morphometry are of different value in various forms of myositis, but are often indispensable techniques in up-to-date diagnostic work up of a myositis.
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PMID:Recent advances in the morphology of myositis. 299 26

The clinical investigation for inflammatory myopathies, which include polymyositis (PM), dermatomyositis (DM) and others, was outlined. The serum creatine kinase (CK) activity increases in the majority of cases of inflammatory myopathies. However, the cases of myositis associated with connective tissue diseases tend to show normal or moderately elevated CK activity. Among the isoenzymes of CK, the MB fraction can increase in the course of treatment as it can originate from regenerating muscle fibres. The macro CK type 1 was reported to appear in association with myositides. Varieties of autoantibodies in the serum such as Jo-1 and Ku have been studied. The Jo-1 antibody is frequently detected in the cases of PM associated with interstitial pulmonary fibrosis. Examination of the heart and lungs is necessary, and so is a search for malignant neoplasms in the cases of DM. Muscle biopsy is mandatory for diagnosing PM, DM and other inflammatory myopathies. Among the latter, inclusion body myositis and granulomatous myopathy need to be identified before treatment as they generally respond poorly. Histological changes of inflammatory myopathies are often distributed unevenly. The magnetic resonance image and ultrasonography are helpful in estimating the distribution of the lesion and therefore in deciding the site of biopsy. Ultrastructural observation of the muscle showed invasion of activated lymphocytes under the basement membrane of the muscle fibres causing degeneration of the myofibrils. The subset analyses of infiltrating cells revealed considerable alterations after the steroid pulse therapy.
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PMID:[Clinical investigation for polymyositis and related disorders]. 747 50

New information regarding myositis specific autoantibodies, histopathologic analysis of muscle biopsy specimens, and immunogenetic features of the different serologic subsets of disease has greatly increased our understanding of the pathogenesis of the inflammatory myopathies. The clinical descriptions of inclusion body myositis and 'amyopathic dermatomyositis' (Euwer and Sontheimer, 1993) are examples of our expanded descriptive capabilities in the evaluation of patients with myopathy. Finally, newer techniques such as cytokine analysis and magnetic resonance imaging may help in the ongoing assessment of disease activity in patients with myositis. The combination of these recently described clinical and laboratory parameters are enough to force a reconsideration of the previously described classification and diagnostic criteria in the inflammatory myopathies.
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PMID:Inflammatory myopathies. 749 35

A 62-year-old woman, who has had slowly progressive muscle weakness and wasting of the four limbs for past 10 years, was evaluated by muscle biopsies; i.e. the deltoid, a moderately atrophic muscle, and the quadriceps femoris, a muscle that was only mildly involved. The specimen from the deltoid disclosed the basic muscle structures having been collapsed, containing many fibers undergoing degeneration. There were rimmed vacuoles and infiltration of macrophages and round cells into the muscle fibers and perimysial space. Inclusion bodies containing fibrillary structures were identified in sarcolemmal nuclei by electron microscopy. When the quadriceps femoris muscle was examined, however, a large number of vacuoles were interspersed between myofibrils that contained numerous lipid droplets. There were no pathological changes suggestive of IBM in this specimen. The case described here is unique in that the muscle biopsies from two separate muscle revealed a distinctly different pathology. Although we were unable to determine the cause of lipid-storage in this case, it is our impression that inclusion body myositis is not a uniform disorder but the characteristic pathology to this particular myositis might be an ultimate feature of several diverse degenerative and/or inflammatory muscle disorders.
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PMID:[A case of inclusion body myositis (IBM) associated with lipid-storage vacuoles]. 761 66

Idiopathic inflammatory myopathies, polymyositis, dermatomyositis, and inclusion body myositis, are increasingly recognized to cause long-term disability in certain subsets of patients. Because these diseases are infrequent, only retrospective analysis of most treatments are available. In this article, identification of subsets of patients with different prognoses and discussion of confounding factors for increasing weakness are emphasized. The advantages and disadvantages of different therapies for myositis and for extraskeletal muscle features are also discussed.
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PMID:The treatment of myositis. How to approach resistant disease. 773 67

The apolipoprotein E genotype was determined for 11 patients with sporadic inclusion-body myositis. Seven cases had the genotype epsilon 3/epsilon 3, the other four cases, epsilon 3/epsilon 4. The frequency of the epsilon 4 allele in this group of patients (0.182) was not significantly increased compared with elderly controls (0.147; n = 58), in contrast to Alzheimer's disease in which there was a significant increase (0.328; n = 67). The epsilon 2 allele was not found in any of the 11 sporadic inclusion-body myositis patients and its frequency was decreased in Alzheimer's disease. Despite certain pathological similarities that exist between inclusion body myositis and Alzheimer's disease, their association with particular apolipoprotein E genotypes is distinct.
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PMID:Apolipoprotein E type epsilon 4 allele frequency is not increased in patients with sporadic inclusion-body myositis. 774 81

Although our understanding of the inflammatory myopathies is still evolving, it is becoming increasingly clear that these syndromes are composed of many separate and distinct disorders with widely divergent clinical signs, symptoms, laboratory abnormalities, and prognoses. Their classification remains controversial, but three approaches of dividing the myositis syndromes appear useful in helping to group disorders with similar features together. The three approaches divide these syndromes on the basis of clinical and histopathologic findings, by serology, and by exposures to known environmental agents. Studies of the prognosis of these disorders are limited by the rarity and heterogeneity of the myositis syndromes. Taken together, however, they suggest that a variety of demographic, clinical, and serologic features are associated with a poor outcome. These include older age at myositis onset, severe myositis, delay to diagnosis and therapy, significant cardiac, pulmonary or gastrointestinal involvement, and the presence of cancer, inclusion body myositis, or antisynthetase or anti-SRP auto-antibodies. It is hoped that our understanding of the classification and prognosis of the inflammatory myopathies will become more complete as we perceive more fully the interrelationships between the genetic and environmental risk factors necessary for the induction of myositis and develop more rational ways of dividing and treating these increasingly recognized syndromes.
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PMID:Classification and prognosis of inflammatory muscle disease. 785 23

In muscle biopsies of 8 sporadic inclusion-body myositis (S-IBM) and 4 hereditary inclusion-body myopathy (H-IBM) patients, vacuolated muscle fibers contained within their vacuoles strongly immunoreactive inclusions with 2 polyclonal and 1 monoclonal antibodies against prion protein (PrP). By light-microscopy, PrP deposits co-localized with beta-amyloid protein (A beta) and ubiquitin (Ub). By immuno-electronmicroscopy, both PrP and A beta were present on amorphous material and on 6-10 nm amyloid-like fibrils; and PrP and Ub co-localized on cytoplasmic twisted tubulofilaments (TTFs) and on amorphous material. Our study provides the first demonstration of abnormally accumulated PrP in pathological tissue other than brain, and it suggests that PrP may play a role in the pathogenesis of IBM.
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PMID:Prion protein is abnormally accumulated in inclusion-body myositis. 828 Aug 54


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