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Myositis ossificans circumscripta in para/tetraplegics.

At the Hospital of Physical Medicine, Hornbaek, we analysed in retrospect 52 cases of myositis ossificans circumscripta (MOC) among 605 patients with para/tetraplegia. MOC proved significantly more common after total than after subtotal spinal cord injuries. MOC was not observed in any case above the motor level of the spinal cord lesion. This neurological relationship appears to "explain" the finding that MOC is more common in para/tetraplegia of traumatic origin (more complete spinal cord lesions) than among those caused by slipped discs or tumours. Twenty-five per cent of the patients ended up with fairly mild and 12% with more severe hip contractures, the latter causing recurrent, contralateral decubital ulcers over the ischial tuberosity and protracted hospitalization. To aid diagnosis, the authors suggest a simple programme for all para/tetraplegics. In our opinion, routine X-ray examination is not necessary. Intensified research into the causative factors and treatment is needed, not only to facilitate these patients' social adaptation, but also for socio-economic reasons.

Adolescent↗

Myositis ossificans progressiva. Clinical and metabolical observations in a case treated with a diphosphonate (EHDP) and surgical removal of ectopic bone.

A 20-year-old woman with a severely crippling myositis ossificans progressiva was treated with a diphosphonate (EHDP), 10-20 mg/kg/day. While being treated with this drug, surgical removal of ectopic bone was performed. Although ectopic calcification recurred postoperatively, considerable functional improvement was achieved. At the highest dosage of EHDP, hypercalcaemia gradually appeared, but was reversible upon cessation of drug treatment. It is probably related to a direct effect of EHDP on the bone.

Adult↗

Regional blood flow in experimental myositis ossificans. A microsphere study in conscious rabbits.

In a recent model for heterotopic bone formation (Michelsson et al. 1980). muscular oedema, swelling and necrosis is seen in the quadriceps muscle of rabbit hind limbs immobilized for at least 2 weeks when, from the second week, the immobilized limb is subjected to daily forcible mobilization lasting about 5 min. According to this model, heterotopic calcification develops gradually from the second week of forcible mobilization and is located in the vastus intermedius region. Between the fourth and fifth week of immobilization and forcible mobilization, heterotopic bone formation is seen in virtually all cases. The histological findings are similar to those in human ectopic bone formation. In the present investigation the labelled microsphere technique was used to study the regional blood flow effects in the early development of myositis ossificans with this model. The results are quite different from those reported by other investigators on immobilization alone and point to a causal relation between regional blood flow and forcible mobilization of the immobilized rabbit hind limb. Prostaglandins as mediators between the traumatic inflammation, a part of the circulatory effects observed and the induction of new bone is suggested.

Animals↗

A brachial plexopathy due to myositis ossificans. Case report and review of the literature.

Myositis ossificans (MO) is a disorder characterized by the intramuscular proliferation of fibroblasts and osteoblasts, with subsequent deposition of bone and cartilage. A typical clinical presentation involves traumatic injury to a young adult, usually localized to the thigh, buttock, or upper arm, with resultant MO and mildly restricted range of motion in adjacent joints. Rarely, MO is associated with peripheral neuropathies involving the radial, median, sciatic, and sural nerves. The authors present an unusual case of MO causing a brachial plexopathy. To their knowledge, this is the first description of such a presentation.

Adult↗

Infectious myositis. A tropical disease steals out of its zone.

Infectious myositis is an acute infection of skeletal muscle that is increasing in prevalence with the increased incidence of HIV disease. Typical presentation is asymmetric swelling of an isolated muscle, with exquisite pain and fever. Results of laboratory studies are usually nonspecific, but magnetic resonance imaging of the affected area suggests the diagnosis. Muscle biopsy and direct identification of the pathogen on tissue culture are required, because the list of potential pathogens is long. With prompt treatment, most patients achieve complete resolution and return to their preinfection level of health.

Adult↗

Atypical presentation of restrictive orbital myositis.

The histories of two patients with restrictive ophthalmopathy are reported in whom the initial diagnosis was delayed primarily due to the relatively acute onset. This form of onset of dysthyroid orbital myositis is unique in our experience and demonstrates the importance of realizing that the restrictive ocular motility disorder of Graves' ophthalmopathy may occur abruptly. By such recognition, one may proceed with the appropriate laboratory investigation and spare the patient invasive procedures.

Aged↗

Disseminated muscular cysticercosis with myositis induced by praziquantel therapy.

Cysticercosis, the infestation with the encysted larval stage of Taenia solium, is increasingly recognized as a major problem in most countries in Latin America, Asia, and Africa, involving mainly the central nervous system. Muscle involvement is also common, although it usually remains asymptomatic. We describe a case of disseminated muscular cysticercosis followed by myositis (fever, diffuse myalgia, weakness of the lower limbs, and inflammatory reaction around dying cysticerci) induced by praziquantel therapy, an event not described previously.

Adult↗

Bcl-2, Bcl-x, and Bax expression by immunohistochemistry in inclusion body myositis: a study of 27 cases.

CONTEXT: Bcl-2, Bcl-x, and Bax are among the variety of proteins that have been described as being involved in the regulation of apoptotic cell death. Bcl-2 and Bcl-x(L) inhibit apoptosis, and Bax is proapoptotic. OBJECTIVE: To evaluate the expression of Bcl-2, Bcl-x, and Bax in inclusion body myositis (IBM).Design.-We examined muscle specimens from 27 patients (17 men, 10 women) with IBM to evaluate Bcl-2, Bcl-x, and Bax expression by immunohistochemistry. RESULTS: Patient ages ranged from 29 to 80 years (mean 62.2 years). All biopsies were marked by endomysial chronic inflammation, muscle fiber necrosis, and regeneration. Rimmed (autophagic) vacuoles were present in all cases. Ragged red fibers were noted in 4 biopsies (15%), and cytochrome oxidase-deficient fibers were found in 10 biopsies (37%). Ultrastructural evidence of intranuclear or cytoplasmic tubulofilamentous inclusions, confirming the diagnosis of IBM, were noted in all cases. Paracrystalline mitochondrial inclusions were seen in 5 biopsies (18.5%). Inflammatory cells stained positively with Bcl-2 in all biopsies, Bax in 26 biopsies (96%), and Bcl-x in 8 biopsies (30%). Degenerating muscle fibers were highlighted with Bax in 24 biopsies (89%), Bcl-2 in 2 biopsies (7%), and Bcl-x in 3 biopsies (11%). Regenerative muscle fibers were noted to stain with Bax in 24 muscles (89%), Bcl-2 in 21 muscles (78%), and Bcl-x in 4 muscles (15%). Rimmed vacuoles were highlighted by Bax in 24 biopsies (89%) and only rarely by Bcl-2 (n = 2, 7%) and Bcl-x (n = 3, 11%). A subsarcolemmal staining pattern was observed in 21 biopsies (78%) with Bax, 6 biopsies (22%) with Bcl-2, and only 1 biopsy (4%) with Bcl-x. CONCLUSIONS: (1) Bax (proapoptotic) immunostaining highlighted most autophagic vacuoles; (2) subsarcolemmal Bax and Bcl-2 immunoreactivity may be associated with mitochondrial defects that are commonly noted in IBM; (3) Bcl-2 and Bax immunoreactivity were not confined to degenerating muscle fibers and in fact appeared to be expressed more commonly in regenerating fibers, suggesting that their expression may be independent of apoptosis in the setting of IBM.

Adult↗

Cyclic eosinophilic myositis and hyperimmunoglobulin-E.

A 40-year-old man had regular cyclic episodes of weight gain and eosinophilic myositis associated with hyperimmunoglobulin-E and hypereosinophilia for 9 years. During the episodes his body weight increased up to 10.8%; eosinophil counts reached 41.3 X 10(-9) cells/L; and IgE levels reached 18 000 IU/mL. These values changed regularly in a definite sequence relative to the clinical state. Attempts to document a parasitic cause were unsuccessful, and several courses of anthelmintic therapy were ineffective. An oral dose of prednisone, 10 mg/d, begun in July 1982 resulted in an immediate lessening of the severity of the episodes and a progressive lengthening of the cycle from 35 to 170 days. No further episodes have occurred since March 1984. The patient is fit and well on prednisone therapy, 12.5 mg on alternate days. This apparently unique syndrome has a benign course and is a cyclic disease involving skeletal muscle as the target organ.

Adult↗

Treatment of inclusion body myositis.

Sporadic inclusion body myositis (s-IBM) is considered the most common muscle disease in patients older than 50 years, with a male predominance. Features of s-IBM include insidious onset, slowly and relentlessly progressive muscle weakness, a characteristic distribution and atrophy of both the proximal and distal muscle groups, and resistance to immunosuppressive drugs. The most characteristic pathologic feature is vacuolar degeneration of muscle fibers accompanied by intrafiber congophilia and clusters ("tangles") of paired-helical filaments, containing phosphorylated tau. The response of s-IBM to immunotherapy remains controversial. Some reports emphasized partial improvement in early stages of the disease. However, the lack of clear response to corticosteroids and immunosuppressive therapies, the deterioration of clinical strength despite suppression of inflammation but increasing number of fibers with vacuoles and amyloid deposits under therapy, and the accumulation of "Alzheimer-characteristic" proteins in vacuolated muscle fibers suggest that s-IBM may be a degenerative rather than an auto-immune inflammatory myopathy, and a secondary inflammation response.

Humans↗

[Lockjaw in progressive myositis ossificans].

Frequency, symptomatology, course and therapy of myositis ossificans progressiva are described by means of literature references and personal observation in a patient. The patient was born in 1935 and the disease manifested itself for the first time in 1956 in the right sternocleidomastoid muscle. Despite surgery total trismus could not be prevented and later-on muscles and connective tissue of other areas ossified, too.

Adult↗

Myositis and interstitial lung disease associated with autoantibody to a transfer RNA-related protein Wa.

We describe 2 patients with myositis and interstitial lung disease with the autoantibody to Wa antigen, a 48-kDa transfer RNA-related protein. In contrast to the previous description of anti-Wa antibody, our patients lacked systemic sclerosis-related features, but had clinical features consistent with those associated with antibodies to aminoacyl-transfer RNA synthetases. The difference in clinical presentation between our patients and patients with systemic sclerosis may be explained by recognition of different epitopes on the Wa antigen.

Aged↗

High-dose vitamin C therapy for inclusion body myositis.

OBJECTIVES: The efficiency of high-dose vitamin C therapy for inclusion body myositis (IBM) was assessed. SUBJECTS & METHODS: The subjects were five patients with IBM confirmed pathologically. After the intravenous administration of 40 mg/kg vitamin C five times/week for four weeks, muscle weakness was found to improve in three cases. The average muscle score improved from 8.1 to 8.8, from 7.0 to 8.1 and from 6.2 to 6.8. Magnetic resonance imaging (MRI) demonstrated a reduction in the size of T2 high lesions and gadolinium enhancement in the thigh muscles in one case. Based on our findings, high-dose vitamin C therapy is considered to be effective in some cases of IBM.

Aged↗

[Inclusion body myositis associated with sacroidosis: a report of 3 cases].

Inclusion body myositis (IBM) is a severe form of idiopathic inflammatory myopathy. A predominantly T CD8+ lymphocytic infiltrate, with focally non-necrotizing muscular fiber invasion, and rimmed-vacuoles are specific histological signs. A few cases of IBM associated with other dysimmune diseases have been reported, but only once with systemic sarcoidosis. We report three cases of muscular sarcoidosis associated with IBM. This very uncommon observation suggests that major complex of histocompatibility, soluble factors, cytokines and adhesion molecules could be involved. Our cases are a novel example of associated dysimmune diseases.

Biopsy↗

[Progressive myositis ossificans. Stone man].

Myositis ossificans progressiva (MOP) is a rare hereditary connective tissue disease, genetically inherited as an autossomal dominant trait with complete penetrance but variable expression. The onset usually takes place during childhood and progressive involvement of the spinal cord and proximal extremities leads to immobilization and articular dysfunction. We present a case of a 29-year-old woman with the typical features of MOP and review the pathogenesis, clinical manifestations and treatment options of this rare disease.

Adult↗

Inclusion body myositis associated with hepatitis C virus infection.

Inclusion body myositis (IBM) is a chronic progressive inflammatory myopathy in elders. Three patients with chronic hepatitis C developed IBM. Their muscle biopsies were examined by polymerase chain reaction (PCR) for two patients and immunohistochemistry for three patients. The hepatitis C virus (HCV)-RNA genome was detected in one of the two patients. The degenerated and atrophic muscle fibers were immunoreactive for an anti-HCV antibody in all three patients. Immunoreactivity for an anti-HCV antibody was not apparent in the muscle specimen from an IBM patient without HCV infection. Our findings suggest that HCV infection is related to the pathogenesis in some cases of IBM.

Aged↗

[A case of eosinophilic myositis proven by immunohistochemistry using antibodies against eosinophilic granule protein].

A 50-year-old man had been well until three months earlier, when he felt general fatigue, and cutaneous rash with itching. Thereafter a general muscular weakness developed and the patient could not walk for a month. Four weeks before referral to our hospital, he had high fever and could not role over in the bed. On admission, the patient was able to walk. He had no skin rash. Neurologically, he showed mild weakness in proximal muscles. Hematologic examination showed mild eosinophilia and serum creatine kinase was mildly elevated. Needle electromyogram revealed a diffuse myogenic pattern in extremities. Eosinophilic myositis was diagnosed by a biopsy of the left calf muscle showing mild infiltration of eosinophilis which was identified using antibodies against eosinophilic granule protein.

Autoantibodies↗

[Myositis ossificans and other ossifying lesions of the soft tissues].

Knowledge of the clinical history and typical imaging characteristics of myositis ossificans is important as identification of this entity will avoid unnecessary and invasive work-up. Conversely, the radiologist must pay attention to any atypical clinical or imaging finding, which will warrant further investigations.

Diagnosis, Differential↗