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Experimental allergic myositis: ultrastructural, histochemical, immunological and immunohistochemical studies.

The quadriceps femoris muscles of experimental allergic myositis, in strain 13 guinea pigs immunised with rabbit myosin B fraction, were subjected to histochemical, immunohistochemical and electron microscopic studies. They demonstrated a variety of degenerative changes of muscle fibres, infiltration of lymphocytes and macrophages along with deposition of immunoglobulin G (IgG) and complement factor 3 on the surface of the muscle fibres. One third of the infiltrating cells were macrophages with acid phosphatase activity in the cytoplasm. The serum IgG of the model had an affinity for the surface of normal guinea pig muscle fibres and for thick filaments and other organelles. Its affinity for the heavy and light chains of myosin, actin, troponin T and for other proteins was shown by the immunoblotting method combined with one- and two-dimensional electrophoreses.

Animals↗

[Myositis ossificans following head injury (author's transl)].

This is a report on 14 patients with myositis ossificans with simultaneous head injury. Predilected sites are the hip and elbow joints as well as thigh musculature, where particularly in the youthful and younger adults ossifications occur 5--8 weeks after the accident. Premature operative treatment is useless because of the danger of recurrence. The process of ossification usually comes to a standstill 8--12 weeks following the trauma. The diagnosis is made on the basis of clinical examination (hardening of muscles, immobility of joints) and X-ray pictures. The pathogenesis and modes of therapy are discussed in detail. Furthermore, the incidence of this complication of head injury in various age groups is evaluated statistically.

Adolescent↗

Ultrastructural evidence of myofibroblasts in pseudomalignant myositis ossificans.

Seven cases of pseudomalignant ossifying myositis with a typical clinical symptomatology have been reported. None of the cases had experienced an injury. All the lesions were intramuscular and all of them showed a zonal arrangement. Electron microscopy in three cases allowed the demonstration of cells showing morphological features of myofibroblasts and monocytic cells of the macrophage type. These previously unreported features together with the zonal pattern of the lesions indicate their reparative nature.

Adolescent↗

Inclusion body myositis. A "slow-virus" infection of skeletal musculature?

We report the case of a 56-years old patient with clinical symptoms of an unresolved neuromuscular disease. The light microscopic studies of a muscle biopsy from the m. triceps shows the picture of a diffuse muscular atrophy. By electron microscopy, myelin-like degeneration zones with tubular-filamentous inclusions can be shown in the cytoplasma of the atrophic muscle cells. These filamentous structures correspond morphologically to the nucleocapside of paramyxoviruses. These results lead, even without the proof of inflammatory cells, to the diagnosis of an "inclusion body" myositis also taking into account the clinical and electrophysiological findings.

Biopsy↗

[Fasciitis in granulomatous myositis--an atypical manifestation of sarcoidosis?].

We report about two female patients with a histopathological proven granulomatous myositis associated with a fasciitis. One patient showed noncaseating granulomas in the muscle fascia. She suffered from symmetrical contractures of the digital flexors and ankles. The second patient showed uveitis and polyneuropathy, too. In addition, increased activities of the angiotensin-converting-enzyme and lysozyme in the sera suggested that both cases represent a new form of sarcoidosis.

Adipose Tissue↗

Simultaneous primary infection with HIV and CMV leading to severe pancytopenia, hepatitis, nephritis, perimyocarditis, myositis, and alopecia totalis.

Simultaneous primary infection with HIV and CMV in an 18-year-old woman led to an acute cytotoxic reaction, manifesting as pancytopenia, hepatitis, nephritis, perimyocarditis, and myositis. Within 14 days parameters indicating acute cell damage reverted to normal. Two weeks later transient alopecia totalis developed. Initially, HIV-antigen but no HIV antibodies were present. Within 3 weeks HIV-IgG antibodies appeared while HIV antigen disappeared. Anti-CMV-IgM was clearly and anti-CMV-IgG questionably positive; IgM persisted further, while IgG remained definitely undetectable. We speculate that a particular HIV-induced imbalance of the immune system led to a generalized severe cytotoxic reaction to a simultaneous infection with CMV.

Adolescent↗

Three-dimensional CT diagnosis of myositis ossificans of the sacrospinous ligament.

We present the case of a 4-year-old female with a complex fracture of the left hemipelvis who, on follow-up CT imaging, developed new ossific densities within the peripelvic soft tissues of the contralateral side. Three-dimensional surface reformations of the pelvis demonstrated myositis ossificans along the course of the right sacrospinous ligament, thus elucidating unsuspected ligamentous injury and implying prior instability.

Child, Preschool↗

Chronic intoxication by ethane-1-hydroxy-1,1-diphosphonate (EHDP) in a child with myositis ossificans progressiva.

A child with myositis ossificans progressiva was treated for 8 years with ethane-1-hydroxy-1,1-diphosphonate (EHDP) 30-40 mg/kg per day. Latterly he complained of severe, progressive bone and joint pain which made standing and walking almost impossible. A radiographic skeletal survey showed diffuse ricket-like lesions. Withdrawal of EHDP therapy produced substantial improvement in his general condition as well as in the radiographic appearance of the bones. Multiple exostoses were observed in this case and, particularly those around the knees, presented a peculiar morphology. This supports the theory that exostoses originate from a defect of metaphyseal modelling.

Adolescent↗

Localized nodular myositis and the diagnosis of the localized muscle mass.

Localized nodular myositis (LNM) is a rare variant of polymyositis beginning with inflammatory nodules within muscles. Only seven cases have so far been reported in the literature. We describe a probable further case of LNM in a 67-year-old man with ischemic claudication of the left leg for three years who presented with painful nodules in the left gastrocnemius muscle and signs of systemic disease; a complete follow-up was not possible, because the patient died after only two months and autopsy was not performed. Muscle biopsy showed localized areas of necrotic and inflammatory pleomorphic changes, in keeping with the features of the other known cases. The ultrastructural findings (not previously reported in this disease) were characterized by marked changes of endomysial capillaries, with fibroblastic metamorphosis of the endothelial cells, and by the presence of filamentous inclusions in the myonuclei. The differential diagnosis of LNM from other localized muscle masses, chiefly from muscle infarct, is discussed.

Aged↗

The diagnostic value of serum autoantibodies in endocrine orbitopathy and idiopathic ocular myositis.

Sera of patients with Graves' orbitopathy (GOP) often contain antibodies against retroorbital tissue components. The presence of such autoantibodies has been considered to indicate an autoimmune pathogenesis of the disease. However, their specificity has not been conclusively tested, because studies demonstrating autoantibodies used controls with no lesion in the extraocular eye muscles. Although ocular myositis (OM) is clinically distinct from GOP, damage to the retroorbital muscles is a common histopathological finding in both conditions. Using an immunoblot technique, reactions of sera from patients suffering from either disease were compared. Sera from both groups contained antibodies against a variety of antigenic determinants recognized by both sera. These autoantibodies may have been induced secondary to tissue damage and should be considered to be nonspecific. Because the role of tissue damage was not accounted for in previous studies, evidence concerning antigens supposedly specific for GOP should be reevaluated. The reaction patterns of OM and GOP sera were slightly different. These differences were specific enough to suggest that sera from patients with GOP contain antibodies against eye muscle components that are not present in the sera of patients with idiopathic OM. These findings support the assumption that GOP is an autoimmune disease. However, the major autoimmune targets remain to be identified and their pathogenic relevance is still unclear.

Adult↗

Focal myositis: a pseudotumoral form of polymyositis.

The authors report a case of pseudotumoral focal myositis found in a 55 year-old man, in the anteroexternal area of the right leg. The diagnosis was based on the pathological findings, the absence of any further clinical signs or laboratory findings, and no further development over time. They compare their patient's characteristics with cases found in the literature (24 cases during the past 20 years). Diagnostic certainty confirms the benign character of this pathology, which contrasts with the gravity of the classical form of polymyositis.

Electromyography↗

D-penicillamine-induced myositis in rheumatoid arthritis.

D-penicillamine, an agent still used in the treatment of rheumatoid arthritis (RA) may produce inflammatory myopathy or myositis. Some reported cases are documented with muscle biopsy. We report a 34-year old female, receiving the drug for more than 4 years, who consulted us with recently developed proximal muscle pain and weakness. EMG-findings were typical for inflammatory muscle disease; muscle enzymes remained normal. D-penicillamine was stopped and she was started on prednisolone with rapid improvement. The EMG-findings, with follow-up within three months, proved to be a good diagnostic tool, in the absence of laboratory muscle enzymes abnormalities.

Adult↗

Glucocorticoid-sensitive hereditary inclusion body myositis.

We report a hereditary muscle disorder with features of inclusion body myositis (IBM) in two adult sisters with slowly progressive asymmetrical muscle weakness. The findings of light microscopic and ultrastructural investigations of muscle biopsy specimens were consistent with a diagnosis of IBM. Both patients improved and stabilized on immunosuppressive treatment with corticosteroids and azathioprine. This differentiates our patients from other sporadic and familial cases of IBM. Clinical and histological features are described and compared with those of other previously reported families with IBM.

Female↗

[Myositis proliferans. Differential cervical space-occupying lesion diagnosis].

Myositis proliferans is a rare and benign pseudosarcomatous tumour of soft tissue. In most cases it is found in the region of the shoulder and arm and its occurrence in the region of the neck is described in literature in 16 cases in all. For the ear, nose and throat specialist it is an important differential diagnosis in contrast to malignant tumours. In this report we present the cases of two patients, who were suffering from painful cervical swelling that had been increasing for some days. The problems inherent in the diagnosis of this illness are discussed. Due to its rapid growth the tumour is generally falsely diagnosed and radically removed. Therefore, in appropriate case history, this possibility should be considered and excluded in order to prevent the patient from unnecessary radical operation. In addition to clinical symptoms, imaging and trial excision are suitable.

Diagnosis, Differential↗

[Ocular myositis].

We report a young woman presenting with "painful diplopia" caused by inflammation of external eye muscles affecting both eyes sequentially. Orbital MRI disclosed swelling, signal hyperintensity and enhancement of isolated eye muscles. Corticosteroid treatment led to a complete remission within a few days. The compiled data of a literature review enclosing 52 sufficiently documented patients diagnosed as orbital myositis is reported with respect to clinical features, laboratory findings, associated disease, value of imaging procedures (CT,MRI). Therapeutic aspects and differential diagnosis are reviewed.

Adult↗

[New insights in pathogenesis and therapy of sporadic inclusion body myositis (s-IBM)].

Sporadic inclusion body myositis (s-IBM) is a chronic progressive inflammatory myopathy which occurs preferentially in older patients. Histologic hallmarks are rimmed vacuoles and eosinophilic cytoplasmatic inclusions. The etiology is still unknown, but different pathogenetic mechanisms such as slow virus infection, autoimmunopathogenesis, myonuclear alterations, and mitochondrial defects have been implicated. A relation to neurodegenerative disorders and prion diseases has also been suggested. There is a poor response if any to immunosuppressive therapy. Stabilization of disease progression was shown only by intravenous immunoglobulin (IVIG) therapy. Future findings in the field of s-IBM pathogenesis may result in better therapeutic options.

Aged↗