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PubMed1982

Acetylcholine receptor and thymus in experimental autoimmune myasthenia gravis and experimental myositis.

This study was attempted to obtain information about biological properties of junctional acetylcholine receptor (AChR) and extrajunctional AChR, and about nerve influences on muscles AChRs under the pathological conditions of experimental myasthenia and myositis. Experimental autoimmune myasthenia gravis (EAMG) was induced in Wistar rats by immunizations with AChR purified from the electric organ of Narke Japonica without using Freund's complete adjuvant experimental myositis by immunization with rat muscle extract depleted of AChR. Thirty-five days after the initial immunization, unilateral dissection of the ischiadic nerve was performed in all immunized rats. Contents of AChR in both hind limb muscles were measured by double immunoprecipitation assay method 15 days after the experimental denervation. In the control animals the amount of AChR extractable from innervated muscles was 2.7 +/- 0.5 (mean +/- s.d.) pmole/g muscle and increased about 10-fold 15 days after the denervation (30 +/- 7.9). In rats with EAMG, AChR contents was reduced in both denervated (1.1 +/- 1.0) and innervated muscles (1.3 +/- 0.9). In experimental myositis, the increase of muscle AChR was impaired in denervated muscles (2.4 +/- 0.6), but AChR contents was not reduced in innervated muscles (2.7 +/- 0.9). These results suggest that nerves may influence AChR metabolism, keeping numbers of AChR constant even in inflammatory condition. In addition, germinal centre formation in thymic medulla was detected in EAMG rats.

Animals↗
PubMedDate not supplied

[Diagnostic significance of scleroderma and myositis-associated autoantibodies].

In more than 95% of patients with systemic sclerosis and in about 60% of patients suffering from idiopathic inflammatory myopathies autoantibodies directed at different nuclear or cytoplasmic antigens can be detected with different methods. Scleroderma-associated autoantibodies can be visualized as antinuclear antibodies (ANA) by immunofluorescence assays using cultured monolayer cells. In case of a negative ANA result the diagnosis of systemic sclerosis is unlikely. In individual patients the different autoantibodies (against DNA topoisomerase I (Scl-70), centromeric antigens, fibrillarin, To (Th), RNA polymerases, NOR-90, U1-nRNP, PM-Scl, Ku) are mutually exclusive. They can be detected early in the course of diseases, most often are persistent, and are closely associated with immunogenetic markers. They are characteristic for distinct subsets of patients homogeneous in clinical manifestations as well as in disease outcome. Myositis-associated autoantibodies are directed to nuclear (about 60% of myositis patients; PM-Scl, Mi-2) or cytoplasmic antigens (about 35-40%; Jo-1 and other aminoacyl-tRNA-synthetases, signal recognition particle (SRP), KJ and others) and likewise are related to distinct clinical, prognostic, and immunogenetic traits leading to the description of characteristic antibody-based syndromes. Based on published results and on our own investigations, the diagnostic potential of scleroderma- and myositis-associated antibodies is evaluated and a new classification of systematic myositic and sclerodermatous disease is proposed.

Antibodies, Antinuclear↗
PubMed1994

Idiopathic inflammatory myopathies: inclusion-body myositis, polymyositis, and dermatomyositis.

In this review, the main emphasis is on new advances concerning sporadic inclusion-body myositis and hereditary inclusion-body myopathy. Polymyositis and dermatomyositis are reviewed briefly. Hypotheses are presented regarding the possible cause and significance of abnormally accumulated beta-amyloid protein, two other epitopes of beta-amyloid precursor protein, hyperphosphorylated tau, alpha 1-antichymotrypsin, ubiquitin, and prion protein in sporadic inclusion-body myositis and hereditary inclusion-body myopathy. Because most of those proteins are also accumulated at the neuromuscular junction, "junctionalization" of other muscle fiber nuclei is a possibility. Attention is given to the fact that vacuolated muscle fibers in hereditary inclusion-body myopathy may represent early changes because they are virtually free of congophilic amyloid deposit but, like sporadic inclusion-body myositis, contain large accumulations of beta-amyloid protein and prion.

Aged↗
PubMed1994

Asymptomatic myositis ossificans of the medial pterygoid muscles: a case report.

Traumatic myositis ossificans, also known as myositis ossificans circumscripta or fibrodysplasia ossificans circumscripta, is a form of dystrophic calcification leading to heterotopic ossification of intramuscular connective tissue. This is usually due to a single severe injury or repeated minor injuries to muscle, although cases without a history of injury have been reported. Heterotopic ossification is rare in the orofacial region, especially in the medial pterygoid muscles. A case of medial pterygoid myositis ossificans with unique computed tomography findings is described.

Aged↗
PubMed1993

[Osteogenetic cells in myositis ossificans].

We have used the lectins Arachis hypogaea (PNA), Canavalia ensiformis (Con A) and Triticum vulgaris (WGA) for the examination of bone forming cells in 8 variously differentiated cases of myositis ossificans. The development of myositis ossificans could be characterized with the appearance of a WGA binding cell population. In this cell population the lectin binding spots may be found in the perinuclear cytoplasm, possibly in the Golgi complex. The lectin binding of the bone forming cells has disappeared in the ripe myositis ossificans. We think that the WGA binding cell population may be equal with the progenitor cells, wandering in form the stroma ot the bone marrow and that the ability of WGA binding may be a characteristic developmental marker of these cells.

Adult↗
PubMed1994

[Magnetic resonance imaging in the diagnosis of myositis ossificans circumscripta].

A retrospective study of 5 cases, 4 of myositis ossificans circumscripta (MOC) and 1 of non-ossificans myositis, is presented. The clinical presentation was a painful soft-tissue swelling, and the final diagnosis was established by biopsy in 3 cases and clinical evolution in 2 cases. Different types of imaging techniques were performed: 4 MRI, 2 arteriographies, 3 Tc 99m scintigraphies, 3 US, 3 CT and plain film radiographs for all patients. MOC is a benign process (as opposed to myositis ossificans progressiva, which is an inherited pathology with a fatal prognosis) with 3 phases of evolution: an acute or pseudo-inflammatory phase, a sub-acute or pseudo-tumoral phase and a chronic phase with a spontaneous healing. The radiologic diagnostic findings are dependent of the phase of the disease. The calcifications are seen earlier with scintigraphy than plain films, with US being less helpful. A heterogenous mass and calcifications are seen with CT. MRI allows the characterization of oedema during the acute phase and sometimes can exclude a malignant process. MRI is the best method for an early diagnosis, the differential diagnosis and to follow the evolution.

Acute Disease↗
PubMed1993

Focal myositis: a localized form of polymyositis?

We describe an additional case of focal myositis presenting as a painful pseudotumor of the lower leg. Surgical biopsy showed typical features of myositis; the disease improved spontaneously, without clinical or biological abnormalities. Focal myositis can remain a localized process, but in some cases it can become an authentic polymyositis. These generalized forms are associated early with elevated erythrocyte sedimentation rate and/or muscular enzymes.

Adult↗
PubMed1993

[Localized nodular myositis--a variation of polymyositis].

A case of focal nodular myositis is reported. The patient was a 37-year old male with no prior medical history. He had three episodes of focal nodular myositis over a three month period, recovering completely after each attack. During the last attack there were three lesions in the left quadriceps and one in the biceps muscle of the left arm. Histologically there was severe myositis. After the last attack he has been well for 12 months without sequelae.

Adult↗
PubMed1995

Thallium-201 accumulation in myositis ossificans and in juxta-articular ossification.

We present the findings on 201Tl and 99mTc-MDP scintigraphy in three patients suffering from heterotopic ossification (two patients presenting with myositis ossificans and one patient presenting with juxta-articular ossification in combination with myositis ossificans). Since resection of the lesions has to be delayed until stabilization, 99mTc-MDP is often used as a parameter of lesional activity, although it is not optimal. For this clinical problem, we evaluated 201Tl scintigraphy as a marker of metabolic activity. In addition to the well-documented uptake of 99mTc-MDP, marked accumulation of 201Tl was observed in all heterotopic ossification sites. Hence, our results support the use of 201Tl scintigraphy in the therapeutic management and monitoring of conditions associated with ectopic ossification. On the other hand, although myositis ossificans is sometimes clinically, radiographically and even histologically confused with extraosseous osteogenic sarcoma, 201Tl accumulation may not be a helpful factor in the differential diagnosis due to the presence of tracer accumulation in both disorders.

Adult↗
PubMed1996

[Inclusion body myositis].

Inclusion body myositis has been recently recognized as a clinical entity although its exact definition remains uncertain. Initially considered to be an inflammatory dermatomyositis, inclusion body myositis can actually take on three specific forms: disseminated muscle atrophy and weakness, pseudopolymyositis, or pseudo-degenerative disease. Inclusion body myositis is different from non-inflammatory neuromuscular diseases with vacuoles. Abnormal deposits are seen within the muscle fiber may contain amyloid substance, beta-amyloid precursor, ubiquitin, antichymotrypsin, protein tau, apolipoprotein E and even prions. The signification of these deposits is unknown. Deletions in mitochondrial DNA have been demonstrated but do not appear to play a causal role. More and more hereditary forms are being recognized and certain may be related to an abnormality in chromosome 9.

Amyloid beta-Peptides↗
PubMed1996

[Granulomatous idiopathic myositis at pediatric onset. Description of a case and a review of literature].

The authors report a case of granulomatous myositis in a child (the cases in literature begin between 13 and 72 years). Our case is peculiar because the histological report of granulomatous myositis was accompanied by an important rise in serum CPK level without any symptoms of muscular disease. The serum CPK level, for 18 months, fluctuated between 4.190 and 22.960 UI/L while no symptom occurred. The authors complete the article with a review of the literature about the granulomatous myositis.

Adolescent↗
PubMed1996

[Pseudotumor in the forearm caused by focal myositis. A rare form of idiopathic inflammatory myopathy].

Focal myositis is a rare form of idiopathic inflammatory myopathy. We describe an additional case of focal myositis in a 54-years-old woman presenting as a painful pseudotumor of the left forearm. Muscle enzymes were normal but generalized electromyographic abnormalities were found. We consider that focal myositis is a heterogeneous clinical syndrome, and the cases showing only focal involvement can be different from those that evolve into polymyositis.

Diagnosis, Differential↗
PubMed1998

Magnetic resonance imaging in focal myositis.

We describe 4 cases of focal myositis of the lower leg in which magnetic resonance imaging (MRI) was useful and sensitive in clearly revealing the detailed anatomic extent of muscle change and in guiding muscle biopsy. We also observed a correlation between MRI findings and severity of both clinical and histological manifestations, suggesting that MRI may provide prognostic criteria in focal myositis. MRI may be a helpful, noninvasive test for the followup of focal myositis, in assessing disease progression or resolution.

Adult↗
PubMed1998

Acute myositis following autologus bone marrow transplantation (BMT).

A 4-year-old boy with acute myeloid leukemia developed acute myositis associated with refractory thrombocytopenia one month after autologous bone marrow transplantation (BMT). Clinical, electromyographic and biohumoral features were consistent with the diagnosis of myositis. The patient responded to corticosteroids, and 39 months after BMT he is in complete remission and has regained good muscle function. Although we could not determine with certainty the specific pathophysiologic mechanism of this complication, it should be pointed out that acute myositis can occur in the early post-BMT period.

Acute Disease↗
PubMed1998

['Inclusion body'-myositis].

In 3 patients, a 72-year-old man, a 62-year-old man and a 73-year-old woman with weakness of respectively the quadriceps femoris, the finger flexors and the pharyngeal muscles, the diagnosis of 'inclusion body myositis' was made. This is a rare, slowly progressive skeletal muscle disorder which is more common in men and after the age of fifty. The activity of serum creatine kinase is often 2-5 times the highest normal value. The electromyogram pattern is myopathic, but can also display neuropathic changes (exclusively). Inclusion body myositis is often misdiagnosed, which can lead to an inappropriate treatment or approach. A frozen muscle biopsy is needed to make cryostat sections for demonstration of myositis with rimmed vacuoles.

Aged↗
PubMedDate not supplied

Bilateral proptosis caused by orbital myositis. A case report.

Proptosis, unilateral or bilateral, is a frequent indication for medical evaluation and orbital computed tomography. The most common cause of proptosis in adults and children is inflammatory disorders. Orbital myositis is a subgroup of the orbital pseudotumor syndrome in which one or more of the extraocular muscles are primarily infiltrated by an inflammatory process. Computed tomography and ultrasonography showed enlargement of one or more extraocular muscles in orbital myositis. In our case, we observed clinically and by orbital computed tomography, evidence of isolated bilateral extraocular muscle swelling after upper respiratory tract infection. Proptosis and other findings were spontaneously and completely resolved after twenty days. Because proptosis caused by orbital myositis is extremely rate in children and there is limited information in the literature, this case was reported.

Acute Disease↗
PubMed1998

[Myositis caused by hydroxyurea in a patient with chronic myelogenous leukemia].

A 59-year-old man with chronic myelogenous leukemia (CML) had a white-blood-cell (WBC) count of 55,400/microliter when admitted in July 1997, and was placed on oral hydroxyurea (HU) of 1,500 mg/day. Treatment with 600 MU/day of interferon alpha (IFN alpha) was started on August 5. HU was discontinued when the patient's WBC count dropped to 8,100/microliter on August 18. However, HU was resumed about a month later, after his WBC count increased to 10,100/microliter, but discontinued when the patient started to complain of chills, high fever, and bilateral femoral pain. HU treatment was initiated again one week later, after the patient's WBC count had begun rising but ceased again after he experienced chills, high fever, and bilateral femoral pain. The patient's myogenetic enzymes were found to be increasing the following day, and a lymphocyte stimulation test (LST) with HU showed a high stimulation index of 41.7%. The elevation of myogenetic enzymes and the results of the LST suggested that myositis due to HU was the cause of the patient's clinical manifestations. His myositis spontaneously disappeared after HU was discontinued. Although the patient is no longer receiving HU, IFN alpha has brought his CML under control. To our knowledge, this is the first reported case of myositis caused by HU.

Antineoplastic Agents↗
PubMed1998

[Diagnostic problems of circumscribed myositis ossificans: presentation of an atypical case].

The authors report the case of a 22-year-old female suffering from thigh pain for 5 months. The first diagnosis considered was circumscribed myositis ossificans but the duration of symptoms and the radiological aspect mimicking some malignant features led to consider parosteal sarcoma as a possible diagnosis. Surgical resection was therefore requested and histological analysis confirmed the diagnosis of circumscribed myositis ossificans. This case emphasizes the difficulty in differentiating circumscribed myositis ossificans from a slowly growing tumor as both can be associated with increasing pain and a malignant radiological aspect. A surgical approach is therefore frequently necessary for definitive diagnosis and treatment.

Adult↗