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Progressive external ophthalmoplegia and myositis.

We reported a senile male patient with progressive external ophthalmoplegia (PEO) and myositis. The ophthalmoplegia was severe, but other neuromuscular features were nearly normal. Muscle enzymes in serum were moderately elevated. Autoimmune, endocrinological or malignant diseases were not observed during the previous 4 years. Pathology of non-weak limb muscles biopsied twice was consistent with active inflammatory myopathy. The ragged-red or cytochrome c oxidase-negative fibers, which are a hallmark of mitochondrial myopathy with PEO, were not increased in comparison with age-matched control muscles. Analysis of mitochondrial DNA in muscle by the Southern blot method did not reveal any deletions. It was concluded that the inflammatory myopathy, myositis clinically localized at the ocular muscles, is an important and distinct disorder in PEO.

Aged↗

Intravenous immunoglobulin therapy for refractory myositis.

The efficacy of polyvalent intravenous immunoglobulin therapy (IVIG) was evaluated in three patients with refractory myositis. Patients refractory to conventional therapy, such as corticosteroid administration, corticosteroid or cyclophosphamide pulse therapy [2 with dermatomyositis (DM), 1 with polymyositis (PM)] were treated with IVIG (0.4 g/kg daily) for 5 days. Clinical improvement was apparent within 1-2 months after IVIG and persisted for 19-23 months (DM patients) and 12 months (PM patient). No adverse effects were observed. Thus, IVIG may be considered an effective therapy for refractory myositis.

Adult↗

Myositis as a manifestation of chronic graft-versus-host disease.

We report a 22-year-old man who had myositis in the course of chronic graft-versus-host disease after bone marrow transplantation for acute monocytic leukemia. The distribution of muscular involvement was different from idiopathic polymyositis. Muscular atrophy and weakness were noted in the distal muscles as well as in the proximal muscles of the upper extremities but there was little weakness in the proximal muscles of the lower extremities. However, histological and immunohistochemical study of the biceps brachii muscle showed findings similar to those of idiopathic polymyositis. It was suggested that myositis can be a manifestation of chronic GVHD caused by a cellular immune reaction by donor T cells.

Adult↗

Familial inclusion body myositis: a report on two Japanese sisters.

Familial occurrence of inclusion body myositis is extremely rare, and only a few cases in Western countries have been reported. In these reports, a strong association of this disease with DR3 (DRB1*0301/0302) and the efficacy of immunosuppressants suggested that an immune pathomechanism is involved in the disease. We, for the first time, report two Japanese sisters who suffered myopathy clinicopathologically similar to inclusion body myositis. One sister received corticosteroid and azathioprine and the therapy relieved dysphagia. Both of our patients had DR15(2)/4 (DRB1*1502/0405), suggesting a distinct genetic association with the disease in the Japanese population.

Aged↗

Focal myositis in monozygotic twins.

Focal myositis is a rare disease with unknown etiology and a broad spectrum. Here, we present two cases in monozygotic twins who complained of recurrent pain of their calves and showed histological signs of inflammation and MRI image compatible with the diagnosis of focal myositis. The occurrence of twin cases not living in the same household suggests a genetic susceptibility to the disease.

Adult↗

Effective treatment with low-dose methotrexate pulses of a child of mixed connective tissue disease with severe myositis refractory to corticosteroid.

A 13-year-old girl with mixed connective tissue disease (MCTD) was described. She visited our hospital with recurrent parotid gland swelling, arthritis, and myositis. Sclerodactyly and Raynaud's phenomenon were also defined, and the laboratory findings of high titers of antinuclear antibody (speckled type), positive anti-RNP antibody, positive rheumatoid factor, and hypergammaglobulinemia suggested the diagnosis of MCTD associated with Sjögren syndrome. The muscle weakness and the increased levels of CK prompted us to examine the muscle biopsy and to perform the electromyography, both of which suggested severe muscle inflammation. The siarography and lip biopsy indicated definitively the association of Sjögren syndrome. Corticosteroid therapy including methyl-prednisolone pulses was started, but the effects were limited. The addition of low-dose methotrexate effectively lowered the levels of CK, and gradually improved the muscle strength. Thus, low-dose methotrexate therapy is recommended to the patients with MCTD who have severe myositis refractory to corticosteroid.

Adolescent↗

Proliferative myositis in an infant: report of a case with electron microscopic observations.

Proliferative myositis presented in a 7-month-old female infant as a poorly circumscribed mass growing rapidly within the interscapular skeletal muscle. Muscle fascicles and individual fibers were dissected by an inflammatory infiltrate of lymphocytes, plasma cells, and histiocytes immersed in a basophilic intercellular substance. Numerous ganglionlike giant cells were present and had ultrastructural features suggestive of histiocytic origin. The possible relation to other rapidly growing lesions that appear in soft tissues is discussed. This probably represents the first reported case of proliferative myositis in a pediatric patient.

Female↗

Myositis (fasciitis) ossificans in an infant.

Myositis (fasciitis) ossificans developed following minor trauma to the soft tissues of the posterior aspect of the right knee of a 5-month-old female infant. The clinical and pathological features of myositis ossificans are reviewed and the differential diagnosis of soft tissue ossification in early childhood is discussed.

Female↗

Histologic, physical and chemical investigation of myositis ossificans traumatica.

Histologic, physical and chemical analyses of the excised bony mass from a case of myositis ossificans traumatica were carried out. X-ray diffraction showed that the newly formed bone was an intermediate fluor-hydroxylapatite. Infrared spectroscopy and chemical analysis supported this finding. Contrary to articular free bodies the mineral part of the bony mass from the myositis ossificans investigated was formed most probably in a basic environment.

Adult↗

Inclusion body myositis--a case report.

Inclusion body myositis is a rare myopathy that clinically resembles a chronic polymyositis and histopathologically is characterized by the presence of rimmed vacuoles containing ultrastructural cytoplasmic degradation products with filamentous intranuclear and cytoplasmic inclusions. Since clinical features are not uniform, histopathologic and ultrastructural studies are necessary to confirm the diagnosis. We report a typical case of inclusion body myositis with histopathologic and ultrastructural study. The patient was a 31 year old male who presented with progressive weakness of both forearms, hands and lower extremities for 10 years.

Adult↗

Neutrophilic myositis without cutaneous involvement as the first manifestation of acute myeloid leukemia.

Muscle involvement in acute febrile neutrophilic dermatosis is uncommon. Herein, we report a case of acute febrile neutrophilic myositis, without cutaneous involvement, as the first manifestation of acute myeloid leukemia. The patient was a 35-year-old male, referred due to painful swelling of the left upper arm and fever. The overlying skin looked normal, and a muscle biopsy revealed dense infiltrates, predominantly composed of mature neutrophils, edema and tissue necrosis. All culture reports were negative, and he was finally diagnosed as having acute febrile neutrophilic myositis, associated with acute myeloid leukemia. Corticosteroid treatment resulted in the progressive regression of the fever, myalgia and swelling.

Adult↗

An outbreak of acute eosinophilic myositis attributed to human Sarcocystis parasitism.

Seven members of a 15-man U.S. military team that had operated in rural Malaysia developed an acute illness consisting of fever, myalgias, bronchospasm, fleeting pruritic rashes, transient lymphadenopathy, and subcutaneous nodules associated with eosinophilia, elevated erythrocyte sedimentation rate, and elevated levels of muscle creatinine kinase. Sarcocysts of an unidentified Sarcocystis species were found in skeletal muscle biopsies of the index case. Albendazole ameliorated symptoms in the index case; however, his symptoms persisted for more than 5 years. Symptoms in 5 other men were mild to moderate and self-limited, and 1 team member with laboratory abnormalities was asymptomatic. Of 8 team members tested for antibody to Sarcocystis, 6 were positive; of 4 with the eosinophilic myositis syndrome who were tested, all were positive. We attribute this outbreak of eosinophilic myositis to accidental tissue parasitism by Sarcocystis.

Acute Disease↗

The myocarditis of systemic lupus erythematosus: association with myositis.

Five patients with clinically overt myocarditis in the setting of systemic lupus erythematosus were analyzed in terms of associated clinical and serologic features. Myositis and antibodies to nuclear ribonucleoprotein (RNP) were present in all. A retrospective review in 140 consecutive patients with systemic lupus erythematosus, including three of these five, showed a highly significant association of myocarditis with myositis (P less than 0.0005). The presence of antibodies to RNP in this small group did not attain statistical significance (P less than or equal to 0.10). The pathologic findings in the one patient who died showed similar patterns of inflammation in both cardiac and skeletal muscle, suggesting the possibility of a generalized inflammatory process directed against striated muscle. Furthermore, although anti-RNP antibodies were found uniformly in these patients, their significance remains to be defined.

Adolescent↗

Myositis and death in bobwhites, Colinus virginianus (L.), due to hemorrhagic cysts of a haemosporozoan of undetermined taxonomic status.

An epizootic of myositis and death in pen-reared bobwhites occurred at a hunting club in California. The myositis was caused by myriads of elongate protozoan cysts. The cysts were in various stages of development and when mature, contained spherical zoites 1 micron in diameter. Sinuous compartments were present in all cysts. The walls of the compartments were composed of a material similar to the cyst walls. Mature cysts were filled with blood. The histologic and ultrastructural morphology of the parasite revealed the parasite to be a haemosporozoan. The parasite has numerous similarities to both Akiba caulleryi (Mathis and Leger, 1909) and organisms that cause aberrant leucocytozoonosis in other species of birds. Further studies are needed to determine if the parasite is a part of the normal parasite fauna of quail or if it represents a parasite in an aberrant host.

Animals↗

Inclusion body myositis long after dermatomyositis: a report of two cases.

Dermatomyositis, polymyositis, and inclusion body myositis are rare illnesses which appear to be distinct in clinical and pathologic features, pathogenesis, natural history, and response to therapy. We report two patients who first developed dermatomyositis, and then, after a disease-free interval of many years, developed inclusion body myositis. This may have useful therapeutic implications for patients with dermatomyositis whose illness bocomes refractory to treatment.

Adult↗

Reversible renal failure and myositis caused by phenytoin hypersensitivity.

A 38-year old woman receiving phenytoin (diphenylhydantoin) noticed maculopapular erythema as the first manifestation of a syndrome that included acute renal failure and myositis in addition to fever, lymphadenopathy, exfoliative dermatitis, and hepatitis. Prednisolone sodium phosphate therapy resulted in resolution of this hypersensitivity reaction, and she recovered from renal insufficiency. The occurrence of renal failure and myositis in the spectrum of phenytoin hypersensitivity reactions is discussed, and the importance of a morbilliform rash in a patient receiving phenytoin is emphasized.

Acute Kidney Injury↗

[Myositis in the presence of slightly elevated creatine kinase values].

The clinical picture with, among other things, muscular swelling, fever and nocturnal sweating in three males aged 30, 37 and 52 years, suggested a neoplasm and sepsis. Ultimately, they were found to suffer from focal myositis, localized nodular myositis and polymyositis, respectively. The ESR and leukocyte counts were increased, the serum creatinine kinase (CK) activity was normal or slightly increased. The symptoms decreased after prednisone treatment. Both localized and generalized inflammatory muscular diseases may be present without the serum CK activity being raised.

Adult↗