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Congenital myopathy with abundant ring fibres, rimmed vacuoles and inclusion body myositis-type inclusions.

We report a 17-year-old girl with an unusual neuromuscular disorder characterised by slowly progressive proximal muscle weakness whose muscle biopsy showed multiple ring fibres and numerous rimmed vacuoles as well as intracytoplasmic and intranuclear inclusions of the inclusion body myositis-type. The clinical features of the presented case, manifested by the onset of the disease in early childhood, delayed motor development, short stature, lordosis and joint contractures were suggestive of congenital myopathy. The coexistence of ring fibres, rimmed vacuoles and inclusion-body myositis-type inclusions in a child with congenital myopathy has not been previously reported.

Adolescent↗

[Myositis of the sternocleidomastoid muscle as a result of arthritis of the sternoclavicular joint].

This article describes a rare case of a myositis of the sternocleidomastoid muscle due to an acute septic arthritis of the sternoclavicular joint. A 51-year-old male in reduced condition was admitted to the Department of Otorhinolaryngology with a one-week history of a painless swelling of the sternocleidomastoid muscle associated with recurrent fever. Physical examination was otherwise unremarkable. An intravenous antibiotic therapy was initiated without improvement of the process, accordingly a specific infection or a malignancy was ruled out by biopsy. The positron emission tomography and magnetic resonance imaging finally revealed the diagnosis of a septic arthritis of the sternoclavicular joint as the causal focus of the infection. After an extensive surgical debridement and under continued antibiotic therapy the patient made an uneventful recovery. The acute septic arthritis of the sternoclavicular joint is an uncommon infection with an insidious onset and is often unrecognized until it spreads to adjacent structures leading to serious complications. This case emphasizes that a myositis of sternocleidomastoid muscle can be caused by an ascending infection due to an acute septic arthritis of the sternoclavicular joint. Clinical course, diagnosis and treatment of this uncommon disease are reviewed in this article.

Acute Disease↗

[Myositis ossificans of the neck in a 47 year old patient].

BACKGROUND: Myositis ossificans is a heterotopic ossification of the soft tissue, which leads in progredient cases to functional disorder. Cervical localization is a rare entity. The clinical symptoms are more or less unspecific. Indicating is a foregoing trauma of the affected region. CASE REPORT: We report about a 47 year old male patient with a dorsocervical mass, which had caused dysfunction. He was operated after the diagnosis had been histologically and radiologically confirmed. CONCLUSIONS: In case of cervical tumors with radiological evidence of partial calcification differential diagnosis should keep in mind myositis ossificans next to tuberculosis or sarcoma. Surgery and postoperative prophylaxis with Indometacin is the therapy of choice.

Biopsy↗

[Acute and chronic orbital myositis].

Ocular muscle diseases fall into two distinct groups: acute or subacute exophthalmic myositis and chronic oligosymptomatic myositis. The diagnosis can only be established with certainty by means of B-scan sonography and CT scanning; these examinations demonstrate the irregular thickening of one or several eye muscles. The disease is apparently primitive; in some cases concomitant autoimmune factors may be found. The course is spontaneous, with continual recurrence, and may have sequelae in the form of a definitive exophthalmos or an acquired retraction syndrome. It responds remarkably well to corticotherapy and, if necessary, immunosuppressors; in general, these lead to rapid and - if treatment is started in time - total regression of the disease.

Acute Disease↗

Acute limb ischemia secondary to myositis-induced compartment syndrome in a patient with human immunodeficiency virus infection.

Myositis, while uncommon, develops more frequently in patients with human immunodeficiency virus infection. We report a case of acute lower leg ischemia caused by myositis in such a patient. Urgent four-compartment fasciotomy of the lower leg was performed, which decompressed the compartmental hypertension and reversed the arterial ischemia. This case underscores the importance of recognizing compartment syndrome as a cause of acute limb ischemia.

Acute Disease↗

Cryptococcal myositis: a case report and review of the literature.

Only rare cases of cryptococcal myositis have been previously reported in the literature. All of these cases have occurred in the setting of human immunodeficiency virus (HIV) infection. We report a case of cryptococcal myositis diagnosed premortem on a needle biopsy in a heart transplant patient undergoing immunosuppressive therapy.

Aged↗

Cryptococcus neoformans myositis without dissemination.

We report a case of isolated cryptococcal myositis involving the paraspinal muscle without evidence of disseminated disease in a patient with a large B-cell lymphoma diagnosed at the time of presentation. Biopsy of the muscle involved grew a pure culture of Cryptococcus neoformans and periodic acid-Schiff staining showed numerous budding yeast consistent with Cryptococcus spp. The patient responded to systemic antifungal therapy with complete resolution of his infection. We also present a review of 5 previously published cases of cryptococcal myositis.

Adult↗

Benign acute childhood myositis in Kuwait.

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.

Acute Disease↗

An autopsy case of aggressive CD30+ extra-nodal NK/T-cell lymphoma initially manifested with granulomatous myositis.

This study reports an autopsy case of a 53 year-old male with rapidly progressive extra-nodal NK/T-cell lymphoma accompanied with unusual clinical and pathologic features. He was initially presented with localized swelling and tenderness in the right lower extremity and the biopsy from the calf muscle was interpreted as granulomatous myositis masquerizing lymphoma. The biopsy from erythematous skin lesion of trunk showed infiltration of medium sized atypical lymphoid cells with relatively plump cytoplasm and immunophenotype of CD30+, CD56+/- and surface CD3-, which lead to the diagnosis of CD30+ anaplastic large cell lymphoma. About 2 months later, nasal obstruction was developed and the nasal biopsy was done. After confirmation of EBV infection, he was finally diagnosed as extra-nodal NK/T-cell lymphoma with peculiar immunophenotype of CD3 dim+ and CD30+. Despite the chemotherapy, he was going rapidly downhill and died of respiratory and multi-organ failure 8 months after the onset of soft tissue lesion. At autopsy, disseminated angiocentric lymphoma was found all over the internal organs including the brain. This case emphasizes that extra-nodal NK/T-cell lymphoma should be considered as a cause of granulomatous myositis and can express CD30 positivity and CD3 weak positivity, which are unusual but rarely predominant feature of NK/T-cell lymphoma.

CD3 Complex↗

Proliferative myositis in a two-year-old child.

A 2-year-old boy had intermittent pain and mottling of the left thigh. Swelling of the vastus lateralis developed, and a biopsy revealed proliferative myositis. The pathogenesis is unknown, but trauma and ischemia have been implicated. The rarity of proliferative myositis in children, and the possibility of mistaking the lesion for a sarcoma make the case noteworthy.

Child, Preschool↗

Sporadic inclusion body myositis: pilot study on the effects of a home exercise program on muscle function, histopathology and inflammatory reaction.

OBJECTIVE: To evaluate the safety and effect of a home training program on muscle function in 7 patients with sporadic inclusion body myositis. DESIGN: The patients performed exercise 5 days a week over a 12-week period. METHODS: Safety was assessed by clinical examination, repeated muscle biopsies and serum levels of creatine kinase. Muscle strength was evaluated by clinical examination, dynamic dynamometer and by a functional index in myositis. RESULTS: Strength was not significantly improved after the exercise, however none of the patients deteriorated concerning muscle function. The histopathology was unchanged and there were no signs of increased muscle inflammation or of expression of cytokines and adhesion molecules in the muscle biopsies. Creatine kinase levels were unchanged. A significant decrease was found in the areas that were positively stained for EN-4 (a marker for endothelial cells) in the muscle biopsies after training. CONCLUSION: The home exercise program was considered as not harmful to the muscles regarding muscle inflammation and function. Exercise may prevent loss of muscle strength due to disease and/or inactivity.

Aged↗

Anti-threonyl-tRNA synthetase, a second myositis-related autoantibody.

An autoantibody known as PL-7 was found in the serum of four patients with myositis and one with a systemic lupus erythematosus-like syndrome. The PL-7 antigen is an 80,000 dalton polypeptide that coprecipitates with transfer RNA. In aminoacylation reactions, PL-7 IgG inhibited the charging of tRNA with threonine but had little or no effect on charging with other amino acids. Experimental antibodies raised against purified threonyl-tRNA synthetase recognized the same 80,000 dalton polypeptide, but tRNA was not coprecipitated. We conclude that PL-7 antibody is directed at threonyl-tRNA synthetase, and that different antigenic sites are recognized by the human and experimental autoantibodies. Our findings emphasize the link between myositis and autoimmunity to tRNA-related structures.

Adult↗

Myositis due to Pleistophora (Microsporidia) in a patient with AIDS.

Microsporidia are obligate intracellular protozoa that parasitize both vertebrates and invertebrates and are now recognized as important pathogens in individuals infected with human immunodeficiency virus type 1 (HIV-1). We describe the clinical and morphological features of a case of pleistophora (microsporidian) myositis in a patient with AIDS and delineate the stages of the microsporidian life cycle, as visualized by light and electron microscopy. We discuss significant aspects of microsporidian infections in humans and of myopathy attributable to other causes in HIV-1-infected individuals. As far as we know, ours is only the second reported case of microsporidian myositis and the first reported case in a patient with documented HIV-1 infection.

AIDS-Related Opportunistic Infections↗

Calcitonin for myositis ossificans.

A case of myositis ossificans, following a hemiparesis due to a cerebral haemorrhage and treated with synthetic slamon calcitonin, is described. An improvement in joint range and a cessation of further ectopic calcification was seen but, because of the remitting nature of myositis ossificans itself, the therapeutic role of calcitonin in this case is unproven.

Calcitonin↗

Mycophenolate mofetil treatment in resistant myositis.

OBJECTIVES: To assess the efficacy and tolerability of mycophenolate mofetil (MMF) in six patients with myositis refractory to conventional immunosuppressive therapy. METHODS: Six patients were identified from hospital notes. All had previously failed to respond to other immunosuppressive treatments. Efficacy was measured as changes in muscle strength, creatine kinase (CK) levels and prednisolone dose. RESULTS: The mean age of the group was 49.8 +/- 9.1 yrs, 6 (100%) were female and Caucasian. Patients had failed to respond to a median of 3 (range 1-3) immunosuppressive drugs. They received MMF for a mean of 22.3 +/- 18.9 months with a mean MMF dose of 1.6 +/- 0.5 g/day. The mean initial prednisolone dose was 13.7 +/- 7.7 mg and the mean follow up dose was 8.5 +/- 4.9 mg/day (P = 0.03). CK levels were reduced from mean 2395 IU/l +/- 1202.8 to 746.6 +/- 555.8 IU/l (P = 0.03). CONCLUSION: Our data demonstrate that MMF may be effective in myositis, previously unresponsive to conventional immunosuppressive drugs.

Adult↗

Inclusion body myositis. An electrophysiologic study.

Inclusion body myositis is a rare and slowly progressive myositis associated with cytoplasmic inclusions and fibrillar nuclear material. These histopathologic findings are of unknown significance. The clinical presentation of IBM has marked similarities to that of chronic polymyositis with proximal greater than distal weakness and muscle wasting more pronounced in the lower than upper extremities. In contrast to polymyositis, however, relatively few individuals report neck flexor weakness or dysphagia. Corticosteroid treatment is usually ineffective. The clinical, histopathologic and electrophysiologic findings in a patient with IBM are presented. Of particular interest in this report is the detailed motor unit recruitment frequency data. A number of previous IBM reports fail to mention specific electrophysiologic data or present evidence suggestive of a possible combined neuropathic and myopathic disease. Recruitment intervals of 150 ms or greater in combination with decreased motor unit duration and amplitudes in the involved muscles imply a myopathic pathophysiology. These findings are discussed in relation to electrophysiologic data from previously reported cases.

Electromyography↗

Therapy for myositis.

The management of inflammatory myopathy is a challenge. Although corticosteroids remain the initial therapeutic choice in most instances, their side effects and lack of efficacy are such that alternative modes of therapy are frequently sought. The identification of serum autoantibodies associated with more severe forms of myositis has prompted new classification schemes and has led to more aggressive approaches with combination therapy, using corticosteroids, immunosuppressive drugs, or other immune-modulating agents. Non-corticosteroid agents that provide some benefit include methotrexate, azathioprine, chlorambucil, and perhaps cyclophosphamide. Cyclosporine has been increasingly studied with encouraging results, and intravenous immunoglobulin has demonstrated some benefit for myositis patients. Plasma exchange and leukapheresis were of no proven benefit in a randomized, double-blind trial.

Adrenal Cortex Hormones↗

Cellular aspects of myositis.

Polymyositis, dermatomyositis, and inclusion-body myositis are characterized by muscle cell infiltration and specific alterations on or within muscle fibers. Infiltrating immune cells (ie, T or B lymphocytes, macrophages, and natural killer cells) have distinctive distributions in these conditions: increased presence of CD8+/MHC I-restricted T lymphocytes at endomysial sites in polymyositis and more B than T lymphocytes perivascularly in muscles of dermatomyositis patients. Muscle-infiltrating T lymphocytes mainly express alpha beta T cell receptors (TCRs) in polymyositis; they also express TCRs characterized by oligoclonal V beta repertoire, with a consensus motif indicating a conventional antigen as target of the immune attack. In inclusion-body myositis, TCRs with oligoclonal V beta also are found, but no consensus motif has been identified, suggesting possible superantigen involvement in lymphocyte recruitment. Sequence analysis of TCRs in these lymphocytes has provided insight into the probable nature of the antigenic stimulus and into recruitment of these cells to the inflammation sites. T cell- or natural killer cell-mediated cytotoxic agents have been characterized by messenger RNA or protein expression in these inflammatory myopathies, and the roles of other cytokines in the inflammation processes have been determined. In vivo and in vitro studies on muscle cells have assessed their functions as target cells or antigen-presenting cells. Combined molecular and cellular immunology studies on effector and target cells are expected to clarify the pathogenetic mechanisms underlying these inflammatory myopathies in the near future.

Humans↗