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Acute gastrocnemius myositis. Another extraintestinal manifestation of Crohn's disease.

OBJECTIVE: To present the first case in Australia of skeletal muscle inflammation as a manifestation of Crohn's disease. CLINICAL FEATURES: A 50-year-old man was admitted to hospital with a two-week history of bloody diarrhoea, weight loss of 3 kg, soreness of his calf and thigh muscles, and night sweats. A clinical diagnosis of acute Crohn's gastrocnemius myositis was made. Muscle biopsy was not performed. INTERVENTION AND OUTCOME: He was prescribed hydrocortisone by the intravenous route, which was replaced by prednisone by mouth. The myositis resolved completely within 48 hours. CONCLUSION: Corticosteroids are an effective treatment for this rare extraintestinal manifestation of Crohn's disease. Invasive investigations can be reserved for those who fail to respond.

Administration, Oral↗

[Two cases of orbital myositis with monoclonal gammopathy of undetermined significance].

PURPOSE: We report two cases of idiopathic orbital myositis with monoclonal gammopathy. CASE: Case one was a 47-year-old man, who had bilateral swelling of the extraocular muscles and impairment of the left optic nerve. Case two was a 27-year-old woman, who had bilateral proptosis. An immunological test showed that both patients had monoclonal gammopathy, and they were diagnosed as having monoclonal gammopathy of undetermined significance(MGUS). RESULTS: In case one, the patient achieved remission with steroid pulse therapy followed by administration of high doses of a steroid. In case two, because of repeated recurrence, the patient was treated with steroid pulse therapy and then radiation therapy to achieve final remission. CONCLUSION: We need to pay attention in the diagnosis of orbital myositis to distinguish MGUS. Such patients have an atypical clinical course and are resistant to ordinary steroid therapy.

Adult↗

Radiation therapy for idiopathic orbital myositis: two case reports and literature review.

We report two cases of idiopathic orbital myositis treated with radiation therapy (RT). Both patients visited our hospital with complaints of orbital pain on eye movement and eyelid swelling. There was no history of thyroid disease or trauma, and no signs of infection, neoplasm, or collagen disease. The laboratory investigations, including a thyroid function test, showed no abnormalities in either woman. They were initially treated with a combination of corticosteroids (CS) and nonsteroidal anti-inflammatory drugs (NSAIDs), however, they became dependent on or refractory to oral CS therapy, and received 20 Gy in 10 fractions of RT to the orbit. Their symptoms decreased immediately, and both patients were able to reduce the dosage of oral CS after RT. One patient could completely withdraw oral CS thereafter. However, they both experienced recurrence at eight months after RT, and have been receiving oral CS to control their symptoms. RT at doses of 20 Gy in 10 fractions for patients with idiopathic orbital myositis appears to be effective in palliating symptoms, but long-term control is not satisfactory.

Administration, Oral↗

Bilateral ocular myositis as a late complication of dermatomyositis.

The association of bilateral ocular myositis with primary inflammatory muscle disease is rare. We describe a 30-year-old man with a 2-month history of eyelid edema, erythema, and orbital pain, occurring in the course of previously undiagnosed and untreated dermatomyositis (DM). Although ocular myositis is a very rare manifestation of DM, it might be overlooked by clinicians who are not aware of this complication.

Adult↗

Rehabilitation of myositis ossificans in the brachialis muscle.

Theories on the etiology and management of myositis ossificans vary greatly. In this article, possible causative factors and treatment options are reviewed. We present a successful treatment regimen for myositis ossificans of the brachialis muscle in a 15-year-old football player. The treatment program emphasizes using joint mobilization and eccentric strengthening early in rehabilitation. The patient regained full elbow range of motion after nine weeks of this rehabilitation program. Follow-up at nine months revealed nearly complete maturation of the heterotopic bone formation, full pain-free range of motion, normal strength, and a safe return to athletic competition.

Journal Article↗

Tick inoculation in an eyelid region: report on five cases with one complication of the orbital myositis associated with Lyme borreliosis.

PURPOSE: To determine the frequency and dependence of Lyme borreliosis after tick infestation in the eyelid region. MATERIAL AND METHODS: Five patients after tick inoculation were investigated by immunofluorescence assays for IgM and IgG system). Ophthalmologic evaluation of myositis was supported with MRI, laboratory, and internal clinical investigations. RESULTS: Four children showed negative Borrelia serology after a bite from a tick. In one case the left abducens nerve palsy was found, which was diagnosed in MRI as a thickened left lateral rectus muscle. The diagnosis of myositis with positive Borrelia burgdorferi serology was consistent with Lyme borreliosis. Other laboratory examinations were negative. The symptoms were reduced after treatment with ceftriaxon. CONCLUSIONS: Lyme borreliosis was found in one in five patients after tick infestation in the eyelid region. Antibiotic prophylaxis against Lyme borreliosis with ampicillin is recommended for children after a tick bite.

Animals↗

The spectrum of myositis and rhabdomyolysis associated with bacterial infection.

OBJECTIVE: (1) To describe the clinical and radiographic features of 6 patients with myositis or rhabdomyolysis associated with bacterial infection. (2) To analyze the role of computed tomography (CT) in myositis associated with bacterial infection. METHODS: Review of cases treated by the authors with literature review. RESULTS: Two patients had classical pyomyositis with Staphylococcus aureus as the etiologic agent. One patient had pyomyositis with Enterobacter cloacae (the first reported to our knowledge), 2 had myositis/fasciitis (one due to Clostridium perfringens and one due to S. aureus), and one had fatal toxic rhabdomyolysis in association with C. perfringens bacteremia without evidence of gas gangrene. No patient had a completely normal CT scan of affected muscles, but CT scans in 3 patients failed to show abscesses that were subsequently discovered at surgery, while in another patient CT scanning falsely suggested a large abscess that was not present at surgery. CONCLUSION: Infection associated muscle involvement represents a spectrum of clinical manifestations that include pyomyositis, myonecrosis, fasciitis/myositis, and toxic rhabdomyolysis. Diagnosis may be delayed by the often mild clinical presentation. CT scanning alone may be unreliable in distinguishing muscle abscess from swollen muscle unless combined with CT guided needle biopsy.

Adult↗

[A family case of HAM and HTLV-I carrier including two sisters presenting with myositis].

We reported a family case of HAM and HTLV-I carrier including two sisters presenting with myositis. Both the 65-year-old elder sister and her 53-year-old younger sister initially noted lumbago and developed difficulty in walking several years later. Neurologic examination revealed muscle weakness of the proximal parts of the upper and lower limbs (especially the latter). Mild hyperreflexia in all extremities, urinary disturbance and constipation were detected in the elder sister, while only urinary disturbance and constipation were seen in the younger. Anti-HTLV-I antibody in both serum and CSF was positive in both sisters. Muscle biopsy specimens showed features of myositis with perivascular, perimysial and endomysial lymphocyte infiltration. Although an association between HTLV-I and polymyositis had not yet been established, we think that our cases support such an association.

Aged↗

Adult Still's disease and inflammatory myositis.

Although myalgias occur often in adult onset Still's disease (AOSD), inflammatory myositis is rare. We describe a 41-year-old female with AOSD, who had clinical, enzymatic, electromyographic and pathological evidence of myositis, fulfilling the diagnostic criteria for polymyositis. To our knowledge, this exceptional association has only once been reported before.

Adult↗

[A case of orbital myositis with blepharoptosis demonstrating the swelling of superior levator palpebrae muscle on magnetic resonance images].

A 51-year-old woman developed diplopia and left blepharoptosis with an insidious onset. On orbital MRI, the extraocular and a left superior levator palpebrae muscles were swollen and enhanced by gadolinium, suggesting orbital myositis. The left blepharoptosis was improved two weeks and the swelling of superior levator palpebrae muscle one month after prednisolone administration. This is the first report that the lesion of superior levator palpebrae muscle was demonstrated by MRI in orbital myositis.

Administration, Oral↗

[Orbital myositis].

Orbital myositis is a subgroup of the nonspecific inflammatory syndrome or orbital pseudotumor and is characterized by a primary inflammation of extraocular muscles. The authors describe a 70-year-old patient with acute proptosis, ocular pain and right ophthalmoplegia, whose orbital computed tomographic scan showed enlargement of the homolateral extraocular muscles. Clinical presentation and complementary tests were compatible with the diagnosis of orbital myositis however, because of the particular aspects, which included retinal central vein occlusion, optic nerve lesion, distension of the superior ophthalmic vein and the homolateral cavernous sinus, the differential diagnosis with cavernous sinus pathology and thyroid ophthalmopathy was considered. The importance of a rapid diagnosis and treatment is stressed.

Aged↗

Inclusion body myositis: investigation of the mumps virus hypothesis by polymerase chain reaction.

Inclusion body myositis (IBM) is a distinctive form of chronic inflammatory myopathy characterized pathologically by the finding of rimmed vacuoles and 15-18nm microtubular filamentous inclusions in muscle fiber nuclei and cytoplasm. The observation that these filaments resembled nucleocapsids of the paramyxovirus group and showed immunoreactivity with mumps virus (MV) antibodies has led to a long-standing postulate that IBM may be a "slow" mumps infection. We searched for the presence of MV RNA in 34 muscle biopsies (17 frozen and 17 paraffin-embedded) from 18 patients with IBM and 43 control biopsies (mainly from patients with other forms of inflammatory myopathy) using a polymerase chain reaction (PCR). The MV PCR was shown to be sensitive and specific for MV strains (including J-L) and the integrity of muscle RNA extracts was confirmed by PCR detection of constitutive Ableson tyrosine kinase mRNA. MV RNA was not found in any biopsy from the IBM group nor any of the control cases. Our results therefore do not support the mumps hypothesis for IBM.

Adult↗

Immune-mediated conditions and antibodies associated with sporadic inclusion body myositis.

We reviewed 99 patients with sporadic inclusion body myositis (IBM), searching for a coexisting autoimmune disease, other conditions with altered immune function, or the presence of autoantibodies. Thirteen patients had one or more of 11 diseases with altered immune function. Forty-three patients had elevated titers of one or more of nine different, albeit nondisease-specific, autoantibodies. Twenty-five patients had dysproteinemia or dysproteinuria. We conclude that IBM is frequently associated with systemic immune disorders or nonspecific autoantibodies. Although aging may explain some of these phenomena, an altered immune function need to be considered in the pathogenesis of IBM.

Autoantibodies↗

Proteomic analysis of inclusion body myositis.

Sporadic inclusion body myositis (IBM) is the most frequently acquired inflammatory myopathy of late adult life, yet its diagnostic criteria and pathogenesis remain poorly defined. Because effective treatment is lacking, research efforts have intensified to identify specific markers for this debilitating disorder. In this study, proteomic analysis of 4 cases of sporadic IBM was compared with 5 cases of inflammatory myopathy without clinicopathologic features of IBM to distinguish the IBM-specific proteome. Proteins were separated by 2-dimensional polyacrylamide gel electrophoresis and profiled by mass spectrometric sequencing. Expression of most proteins remained unchanged; however, 16 proteins were upregulated and 6 proteins were downregulated in IBM compared with cases of non-IBM inflammatory myopathy. These IBM-specific proteins included apolipoprotein A-I, amyloid beta precursor protein, and transthyretin, which have been associated with amyloidosis; superoxide dismutase, enolase, and various molecular chaperones indicate perturbations in detoxification, energy metabolism, and protein folding, respectively. The IBM-downregulated proteins mainly serve as carriers for muscle contraction and other normal muscle functions. We further applied Western blot and immunohistochemistry to verify the proteomic findings. This study validates proteomics as a powerful tool in the study of muscle disease and indicates a unique pattern of protein expression in IBM.

Aged↗

Inclusion body myositis. A corticosteroid-resistant idiopathic inflammatory myopathy.

In seven patients with slowly progressive muscle weakness, inclusion body myositis (IBM) was diagnosed on biopsy. None had stigmata of collagen-vascular disease or malignancy. Serum creatine kinase levels were mildly or moderately increased. The six patients treated with prednisone did not improve. Needle electromyography showed a "myopathic" pattern in all patients, but four also had diffuse neurogenic changes with normal nerve conductions. Histologic study of muscle showed a mixture of small rounded fibers varying in size, atrophic angulated fibers forming small groups, and hypertrophic fibers. Variable amounts of inflammation, necrosis, and regeneration were seen in all specimens. All showed numerous intracytoplasmic vacuoles lined with purple-blue granules. Electron microscopy showed membranous whorls and masses of abnormal filaments measuring 14 to 18 nm in diameter. Although IBM seems to be a distinct type of inflammatory myopathy, its etiology and pathogenesis are not clear.

Adult↗

Spectrum of inclusion body myositis.

The clinical, laboratory, and biopsy features are described for a large group of patients with inclusion body myositis (IBM) (15 men and four women; mean age, 63 years). A quantitative histopathologic analysis of muscle biopsy specimens revealed less fiber necrosis and endomysial and perivascular inflammation in IBM than in polymyositis (PM) and dermatomyositis, but a more frequent occurrence of dark-angular and hypertrophied fibers. Rimmed vacuoles were present in 3.4% of all fibers and 15- to 18-nm filaments were identified in the biopsy specimens of nine of 11 patients. A panel of monoclonal antibodies immunoreactive with lymphocytes and cells of monocyte/macrophage lineage suggested that the inflammatory reaction in IBM was similar to that in PM (but not dermatomyositis) and mediated by cellular immune responses. These studies confirm the clinical and histopathologic distinctions between IBM and chronic PM, and that differentiation between these disorders is often difficult.

Adult↗

Amyloid filaments in inclusion body myositis. Novel findings provide insight into nature of filaments.

Inclusion body myositis (IBM) represents a serious debilitating disease of muscle without identifiable cause or treatment. Muscle biopsy specimens have characteristic rimmed vacuoles, varying degrees of inflammation, and, most importantly, cytoplasmic and intranuclear filamentous inclusions of unknown composition. Fresh-frozen sections of muscle biopsy specimens from 24 IBM cases were stained with Congo red dye (pH, 10.5 to 11.0). Control biopsy specimens included polymyositis, dermatomyositis, hereditary vacuolar myopathies of unknown cause, acid maltase deficiency, distal myopathy, oculopharyngeal dystrophy, and chloroquine myopathy. Sections were also immunostained with antibody to transthyretin, human P component, and immunoglobulin light chains. In the vacuolated fibers in IBM, amyloidogenic green-birefringent deposits were seen. Some deposits were delicate and wispy appearing, and others were plaque-like. The size of deposits varied, measuring 1 x 2 to 8 microns, and rarely up to 20 microns in length. The number of amyloid-positive fibers correlated with the number of vacuolated fibers. Similar deposits were seen in one case of distal myopathy and one hereditary vacuolar myopathy. Other control cases were negative for amyloid deposits. Antibody staining for known amyloidogenic proteins was negative. This study demonstrates that the filaments in IBM share properties with amyloid proteins. The location implies that this amyloid material is formed intracellularly, rather than having a systemic derivation. The association of amyloid deposits with autophagic vacuoles in IBM raises the likely possibility that the filaments represent a modification of a normal protein within an acidic degradative vacuolar compartment. An alternative possibility, considering the shared properties of IBM filaments and prions (which include size and amyloidogenic properties), is that IBM represents a human prion disease.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Orbital myositis after upper respiratory tract infection.

Two cases of isolated unilateral extraocular muscle myositis after antecedent upper respiratory tract infections are reported. Pain, inflammation over the involved muscle, restricted motility, and an enlarged muscle on computerized axial tomography scan are the findings. This is differentiated from conjunctival inflammatory disease and neurologic disorders. The treatment is orally administered steroids.

Adult↗