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[Possibilities for the demonstration and differentiation of eye muscle changes in endocrine orbitopathy and ocular myositis using nuclear magnetic resonance tomography].

Nuclear magnetic resonance (NMR) tomography is a new imaging technique which at present is still not very common in ophthalmology. Improvement of contrast and resolution by use of surface coils produces high-quality pictures which open up completely new possibilities in orbital diagnosis. This article explains the procedure and describes its use in some cases of Graves' disease and ocular myositis. Differential diagnostic possibilities in orbital diseases are discussed.

Adult↗

[Myositis ossificans following extensive burns].

A report on a case of myositis ossificans following 50-60% burns (second to third-degree) on the trunk and extremities with periarticular localization of the ossification at the hip and knee joints. In terms of its extent and localization the ossification is primarily similar to post-paraplegic ossification of soft tissues.

Burns↗

[Polyneuritis and myositis in Trypanosoma gambiense infection].

During a four-week trip to Nigeria a 54-year-old German developed a fever of 39 degrees C. Later on he had lymphadenopathy, pretibial oedema, dyspnoea and weight loss. After 16 weeks a wreath-like pale pink skin rash, increased pulse rate with pulse deficit and hepatosplenomegaly were noted. Abnormal laboratory findings were an increased blood sedimentation rate (95 mm), raised immunoglobulin M (483 mg/dl), haemoglobin of 12.0 g/dl, mean corpuscular volume of 76 fl and Borrelia IgM antibody titre of 1:512. The electrocardiogram was suggestive of myocarditis: the cardiac symptoms were controlled with digoxin and verapamil. The patient's general condition deteriorated while he was receiving antibiotic treatment with tetracycline and penicillin. Cerebrospinal fluid (CSF) showed an increased cell count (39/microliters) and albumin (0.98 g/dl). There was a mild, predominantly proximal, tetraplegia which--on the basis of electromyographic and biopsy findings--was thought to be due to polyneuritis and myositis. At this stage blood smear and CSF examination revealed Trypanosoma. He thereupon received suramin (1.0 g) and prednisolone (120 mg down to 40 mg) daily, to which melarsoprol was added after 6 days (0.5 ml up to 5.0 ml daily for 36 days). Almost all symptoms then regressed within 6 weeks.

Animals↗

[Diagnostic and therapeutic aspects of myositis ossificans (author's transl)].

Diagnosis of localised myositis ossificans is difficult in the initial stage. A history of trauma is frequently missing. Clinical symptoms point in the direction of osteomyelitis or soft tissue abscess. Sonotomography is useful for localisation and delineation of the lesion. Biopsy is the only reliable method to establish the diagnosis. Conservative treatment is recommended until the process is inactive. Radiographic follow-up will demonstrate the expected massive calcification and scintigraphic follow-up at 6-monthly intervals will eventually prove the metabolic inactivity of the lesion necessary for successful operation. Surgical removal of the calcified tumor will become necessary in cases of continued pain or significant functional limitations of the involved extremity.

Biopsy↗

Neutrophilic myositis as an extracutaneous manifestation of neutrophilic dermatosis.

Neutrophilic dermatoses are characterized histologically by a sterile infiltration of neutrophils throughout the dermis. The condition may affect various internal organs, notably the lungs, the digestive tract, and the joints, whereas muscle impairment is considered to be extremely rare. We describe the case of a patient with pyoderma gangrenosum in whom severe sterile neutrophilic myositis developed as the first manifestation of an acute myelogenous leukemia.

Aged↗

Fractured heterotopic bone in myositis ossificans traumatica.

OBJECTIVE: To discuss the case of a patient with an acute fracture occurring through preexisting, quiescent, posttraumatic heterotopic bone formation of the gastrocnemius muscle. The lesion demonstrated a previously unreported pattern of healing. This case serves to demonstrate an infrequent event with a rare and heretofore unreported sequella. CLINICAL FEATURES: A 54-year-old man with preexisting, mature heterotopic bone within the left gastrocnemius muscle experienced reinjury to the site. The preexisting heterotopic bone was a result of a sporting injury that had occurred 32 years previously. At the time of the new injury, the patient experienced immediate, severe pain. Radiographs demonstrated a nondisplaced oblique fracture through the preexisting heterotopic bone. INTERVENTION AND OUTCOME: The patient was treated with protective wrapping and physiotherapy modalities. Because only the heterotopic bone was involved, the leg was not placed in a cast. Follow-up radiographs obtained 14 weeks later showed callus formation about the fractured site and evidence of union. CONCLUSION: Fractures occurring through mature heterotopic bone formations, such as those seen in myositis ossificans traumatica, are infrequent; callus formation with union of the fractured fragments is an even rarer occurrence.

Fractures, Bone↗

MyoD expression restores defective myogenic differentiation of human mesoangioblasts from inclusion-body myositis muscle.

Inflammatory myopathies (IM) are acquired diseases of skeletal muscle comprising dermatomyositis (DM), polymyositis (PM), and inclusion-body myositis (IBM). Immunosuppressive therapies, usually beneficial for DM and PM, are poorly effective in IBM. We report the isolation and characterization of mesoangioblasts, vessel-associated stem cells, from diagnostic muscle biopsies of IM. The number of cells isolated, proliferation rate and lifespan, markers expression, and ability to differentiate into smooth muscle do not differ among normal and IM mesoangioblasts. At variance with normal, DM and PM mesoangioblasts, cells isolated from IBM, fail to differentiate into skeletal myotubes. These data correlate with lack in connective tissue of IBM muscle of alkaline phosphatase (ALP)-positive cells, conversely dramatically increased in PM and DM. A myogenic inhibitory basic helix-loop-helix factor B3 is highly expressed in IBM mesoangioblasts. Indeed, silencing this gene or overexpressing MyoD rescues the myogenic defect of IBM mesoangioblasts, opening novel cell-based therapeutic strategies for this crippling disorder.

Alkaline Phosphatase↗

Inclusion body myositis-like phenotype induced by transgenic overexpression of beta APP in skeletal muscle.

Inclusion body myositis (IBM), the most common age-related muscle disease in the elderly population, is an incurable disorder leading to severe disability. Sporadic IBM has an unknown etiology, although affected muscle fibers are characterized by many of the pathobiochemical alterations traditionally associated with neurodegenerative brain disorders such as Alzheimer's disease. Accumulation of the amyloid-beta peptide, which is derived from proteolysis of the larger amyloid-beta precursor protein (betaAPP), seems to be an early pathological event in Alzheimer's disease and also in IBM, where in the latter, it predominantly occurs intracellularly within affected myofibers. To elucidate the possible role of betaAPP mismetabolism in the pathogenesis of IBM, transgenic mice were derived in which we selectively targeted betaAPP overexpression to skeletal muscle by using the muscle creatine kinase promoter. Here we report that older (>10 months) transgenic mice exhibit intracellular immunoreactivity to betaAPP and its proteolytic derivatives in skeletal muscle. In this transgenic model, selective overexpression of betaAPP leads to the development of a subset of other histopathological and clinical features characteristic of IBM, including centric nuclei, inflammation, and deficiencies in motor performance. These results are consistent with a pathogenic role for betaAPP mismetabolism in human IBM.

Amyloid beta-Protein Precursor↗

Sporadic inclusion body myositis correlates with increased expression and cross-linking by transglutaminases 1 and 2.

Sporadic inclusion body myositis (SIBM) is characterized by vacuolar degeneration of muscle fibers and intrafiber clusters of paired helical filaments with abnormal amyloid deposition. Because of their potential involvement in other degenerative disorders, we have examined the expression of transglutaminases (TGases) in normal and SIBM tissues. We report that at least two different enzymes, the ubiquitous TGase 2 as well as the TGase 1 enzyme, are present in muscle tissues. However, in comparison with normal tissue, the expression of TGases 1 and 2 was increased 2.5- and 4-fold in SIBM, accompanied by about a 20-fold higher total TGase activity. By immunohistochemical staining, in normal muscle, TGase 2 expression was restricted to some endomysial connective tissue elements, whereas TGase 1 and beta-amyloid proteins were not detectable. In SIBM muscle, both TGases 1 and 2 as well as amyloid proteins were brightly expressed and co-localized in the vacuolated muscle fibers, but none of these proteins colocalized with inflammatory cell markers. Next, we isolated high molecular weight insoluble proteins from SIBM muscle tissue and showed that they were cross-linked by about 6 residues/1000 residues of the isopeptide bond. Furthermore, by amino acid sequencing of solubilized tryptic peptides, they contain amyloid and skeletal muscle proteins. Together, these findings suggest that elevated expression of TGases 1 and 2 participate in the formation of insoluble amyloid deposits in SIBM tissue and in this way may contribute to progressive and debilitating muscle disease.

Amyloid beta-Protein Precursor↗

Air bag injury as a cause of inflammatory myofibroblastic pseudotumour of the subglottic larynx progressing to myositis ossificans.

Inflammatory myofibroblastic pseudotumour (IMFPT) is a rare condition. The clinical presentation can be indistinguishable from that of a malignant neoplasm. The most frequently affected organ is the lung; a much less common site is the larynx and subglottic involvement is particularly rare. Trauma is purportedly one potential aetiological factor, although there have been no previous reports of IMFPT occurring in the larynx secondary to external laryngeal trauma. We present a case of IMFPT of the subglottis which occurred subsequent to external trauma of the neck sustained during air bag inflation in a road traffic accident. This mechanism has not been previously reported. Furthermore, the lesion progressed uniquely to show myositis ossificans-like maturation over time. The patient was ultimately treated by means of complete local excision.

Accidents, Traffic↗

Clinical features, laboratory findings and differential diagnosis of benign acute childhood myositis.

We report clinical and laboratory data from 32 children with benign acute childhood myositis (BACM), children who presented with calf tenderness and gait abnormality. Laboratory evidence of a viral infection was evident in 23 patients, while serum creatine phosphokinase was uniformly increased (558 to 6800 U/L). Twenty-five patients (78.1%) were given a diagnosis other than BACM by their general practitioner or paediatrician. All patients made a rapid recovery within one week. We conclude that BACM should be encountered among the main causes of sudden-onset gait abnormality in young children.

Child↗

Myositis and scleritis associated with Behcet's disease: an atypical presentation.

We report a case of an 18-year-old female, who was diagnosed as having systemic Behcet's disease with chief complaints of vision loss in the right eye and redness and pain in both eyes. In addition to an anterior necrotizing scleritis, she had several attacks of retrobulbar neuritis and lateral rectus myositis. She responded well to i.v. and topical prednisolone and her subsequent attacks were treated with i.v. cyclophosphamide (500 mg on presentation and on Days 7, 10, 12, 15, and 20; the cycle was repeated every 20 days). She benefited on this treatment and her vision improved dramatically. Her final acuity of 20/50 was thought to be due to presumed previous attacks of optic neuritis and anterior segment inflammation. During the five-month follow-up period, she had no other attacks. Ischemia and inflammation are common in Behcet's disease and sometimes thrombosis and vasculitic changes may cause a decrease in vision. In this case, the immunosuppressive therapy helped attenuate the inflammatory vasculitic episodes during the disease course.

Adolescent↗

Streptococcal myositis. A report of two cases.

Two patients are described with acute streptococcal myositis. One of them died after a brief duration of illness in multiple organ failure; the other survived extensive muscular damage complicated by diffuse intravascular coagulation, acute renal failure, adult respiratory distress syndrome, bronchopneumonia, Pseudomonas septicaemia and probably streptococcal toxic shock syndrome. Both patients received nonsteroidal antiphlogistics, purportedly involved in the pathogenesis of this syndrome. Based on a mouse model, clindamycin would seem to be the antibiotic of choice.

Adult↗

Autoantibodies against alanyl-tRNA synthetase and tRNAAla coexist and are associated with myositis.

The sera of six patients with autoimmune disease, predominantly myositis with pulmonary fibrosis, contain antibodies of the PL-12 specificity. These autoantibodies react with both protein and RNA components of human cells. The protein has a subunit molecular mass of 110 kD, and the RNA comprises a group of bands in the tRNA size class. Aminoacylation experiments identify the antigens as alanyl-tRNA synthetase and its corresponding tRNAs, tRNAAla. Anti-tRNA antibody can be absorbed out without depleting antisynthetase activity, showing that the antigens are recognized independently by separable antibodies that coexist in these sera. The concurrence of separate antibodies to the two components suggests that the autoimmune response may be mounted against the charging enzyme-tRNA complex. However, the antisynthetase antibody fails to coprecipitate tRNA with the enzyme, suggesting that the antibody reacts with its target only when it is not complexed with tRNA.

Adult↗

Histidyl-tRNA synthetase and asparaginyl-tRNA synthetase, autoantigens in myositis, activate chemokine receptors on T lymphocytes and immature dendritic cells.

Autoantibodies to histidyl-tRNA synthetase (HisRS) or to alanyl-, asparaginyl-, glycyl-, isoleucyl-, or threonyl-tRNA synthetase occur in approximately 25% of patients with polymyositis or dermatomyositis. We tested the ability of several aminoacyl-tRNA synthetases to induce leukocyte migration. HisRS induced CD4(+) and CD8(+) lymphocytes, interleukin (IL)-2-activated monocytes, and immature dendritic cells (iDCs) to migrate, but not neutrophils, mature DCs, or unstimulated monocytes. An NH(2)-terminal domain, 1-48 HisRS, was chemotactic for lymphocytes and activated monocytes, whereas a deletion mutant, HisRS-M, was inactive. HisRS selectively activated CC chemokine receptor (CCR)5-transfected HEK-293 cells, inducing migration by interacting with extracellular domain three. Furthermore, monoclonal anti-CCR5 blocked HisRS-induced chemotaxis and conversely, HisRS blocked anti-CCR5 binding. Asparaginyl-tRNA synthetase induced migration of lymphocytes, activated monocytes, iDCs, and CCR3-transfected HEK-293 cells. Seryl-tRNA synthetase induced migration of CCR3-transfected cells but not iDCs. Nonautoantigenic aspartyl-tRNA and lysyl-tRNA synthetases were not chemotactic. Thus, autoantigenic aminoacyl-tRNA synthetases, perhaps liberated from damaged muscle cells, may perpetuate the development of myositis by recruiting mononuclear cells that induce innate and adaptive immune responses. Therefore, the selection of a self-molecule as a target for an autoantibody response may be a consequence of the proinflammatory properties of the molecule itself.

Amino Acyl-tRNA Synthetases↗

Blinded, placebo-controlled trial of antiparasitic drugs for trichinosis myositis.

There is no consensus on the benefits of treatment with any specific anthelminthic compound on muscle-stage trichinosis. A double-blind, placebo-controlled comparison was done of 3 antiparasitic drugs during an outbreak of trichinosis in Chiangrai Province, northern Thailand. Forty-six adults were randomized to receive 10 days of oral treatment with mebendazole (200 mg twice a day), thiabendazole (25 mg/kg twice a day), fluconazole (400 mg initially, then 200 mg daily), or placebo. All patients received treatment to eradicate adult intestinal worms. Trichinella spiralis infection was proved parasitologically in 19 (41%) of 46 patient and by serodiagnosis in all cases. Significantly more patients improved after treatment with mebendazole (12/12) and thiabendazole (7/7) than after treatment with placebo (6/12; P<.05) or fluconazole (6/12). Muscle tenderness resolved in more patients treated with thiabendazole and mebendazole than in those treated with placebo (P<.05). However, 30% of volunteers could not tolerate the side effects of thiabendazole. In summary, Trichinella myositis responds to thiabendazole and to mebendazole.

Adolescent↗

Sporadic inclusion body myositis in a patient with human T cell leukemia virus type 1-associated myelopathy.

Sporadic inclusion body myositis is a disease of unknown pathogenesis in which a viral etiology has long been suspected. We report a case that occurred in a patient with human T cell leukemia virus type 1-associated myelopathy. The diagnosis was confirmed by histopathological studies of the deltoid muscle. Nucleic acids amplification and in situ hybridization indicated the presence of integrated proviral DNA and viral mRNA transcripts in the lesions.

Adult↗