Cases reported "Myositis, Inclusion Body"

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1/4. Report of a patient with inclusion body myositis and CD8 chronic lymphocytic leukaemia--post-mortem analysis of muscle and brain.

    We report a 73-year-old woman with sporadic inclusion body myositis (s-IBM) and a T-cell chronic lymphocytic leukaemia (T-CLL). The s-IBM diagnosis was based on clinical symptoms and muscle biopsy showing inflammatory infiltrates and rimmed vacuoles with 15 18 nm diameter tubulofilamentous inclusions on ultrastructural examination. The inflammatory infiltrates consisted of CD8 t-lymphocytes and macrophages. The diagnosis of a CD8 T-CLL was based on peripheral blood samples and bone marrow aspiration. The postmortem analysis of skeletal muscle showed fascicular atrophy, which may support a neurogenic component in s-IBM and the analysis of the brain showed only a few diffuse plaques in different cortical regions and occasionally neuritic plaques. A pathophysiological analogy between s-IBM and Alzheimer's has been suggested on the basis of similarities in protein accumulation in muscle of s-IBM patients and brain of Alzheimer's patients. However, we were unable to detect any changes suggestive of Alzheimer's disease in the brain of the s-IBM patient presented here.
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keywords = macrophage, bone
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2/4. aluminum phagocytosis in quadriceps muscle following vaccination in children: relationship to macrophagic myofasciitis.

    Macrophagic myofasciitis (MMF) is a rare, seemingly emerging entity among adult patients in france. We encountered two children with the first two cases of MMF in north america. A 5-year-old male with chronic intestinal pseudo-obstruction required nighttime parenteral nutrition. Abnormal pupillary reflexes and urinary retention suggested a diffuse dysautonomia, which prompted a neurological diagnostic work-up. A 3-year-old child had developmental delay and hypotonia. Both children received age-appropriate immunizations. quadriceps muscle biopsy from each child showed the typical patchy, cohesive centripetal infiltration of alpha-1-antitrypsin , alpha-1-antichymotrypsin , CD68 , PAS , CD1a-, S-100-, factor xiii- granular macrophages with adjacent myofiber atrophy, dilated blood vessels, and mild endomysial and perimysial fibrosis. No myonecrosis was observed and no discrete granulomas were seen. A single aluminum peak was demonstrated on energy dispersive X-ray microanalysis. The etiology of the clinical symptoms in these cases and in cases reported as MMF remains intriguing. Despite numerous stains to demonstrate organisms, most infectious causes leading to macrophage activation were ruled out. These cases are being reported to increase awareness of this condition and to encourage a systematic epidemiologic and clinicopathologic study in north america.
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ranking = 1.9961823246857
keywords = macrophage
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3/4. Pediatric macrophagic myofasciitis associated with motor delay.

    BACKGROUND: Macrophagic myofasciitis (MMF) is a rare inflammatory myopathy characterized by accumulation of perifascicular macrophages without muscle fiber necrosis. Few sporadic pediatric cases have been described, and MMF is recognized as a possible reaction to intramuscular injections of aluminum-containing vaccines. The association of MMF and motor delay is unclear in the pediatric population. We report the clinical evaluation and follow-up of 4 young children with MMF and review of 4 cases previously reported of sporadic, pediatric MMF to better determine the possible association of sporadic MMF in children presenting with motor delay. patients AND methods: Described our 4 case reports in which we observed children presenting for evaluation of motor delay with unrevealing clinical and laboratory evaluations for common causes of motor delay and histopathological evaluations consistent with macrophagic myofasciitis. Muscle data was obtained by quadriceps muscle biopsy. RESULTS: Clinical presentations were similar in all children and were characterized by motor delay, hypotonia, and failure to thrive with an unrevealing evaluation for central nervous system disease, congenital, and mitochondrial myopathies. CONCLUSIONS: Our cases and those previously reported in the literature demonstrate MMF should be considered in the evaluation of children with failure to thrive, hypotonia, and muscle weakness, as clinical outcome appears to be favorable.
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ranking = 0.99809116234285
keywords = macrophage
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4/4. Inclusion body myositis in hiv-1 and HTLV-1 infected patients.

    Sporadic inclusion body myositis (IBM) is the most common inflammatory myopathy affecting patients over the age of 50 years. Dysimmune and degenerative aetiologies have been postulated, but viral infections have not been associated with the disease. Two HIV-I (human immunodeficiency virus type 1) infected men and one woman infected with HTLV-1 (human T cell leukaemia virus type 1) developed progressive proximal muscle weakness unrelated to antiretroviral therapy. Their muscle biopsies were studied by light and electron microscopy, by immunocytochemistry to determine the expression of major histocompatibility complex (MHC) molecules and identify the type of infiltrating cells and T cell receptor (TCR) subunits, and by reverse transcription-polymerase chain reaction (RT-PCR) and single or double immunocytochemistry to search for retrovirally infected endomysial cells. The clinical features were consistent with sporadic IBM. The muscle biopsies showed primary endomysial inflammation, red-rimmed vacuoles, amyloid deposits, eosinophilic inclusions, and small round fibres in groups, all diagnostic of IBM. The muscle fibres expressed MHC class-1 antigens and were invaded primarily by CD8 t-lymphocytes preferentially bearing TCR V beta 5.1 and V beta 13 chains. The hiv-1 or HTLV-1 antigens were detected only on endomysial macrophages on or around muscle fibres, but not within the muscle fibres. We conclude that IBM occurs in hiv-1 and HTLV-1 infected individuals and has a clinical, histological and immunological pattern identical to sporadic IBM in the non-retrovirally infected patients. Retroviruses do not directly infect the muscle, but persistent retroviral infections may provide superantigenic stimulation and trigger an endomysial inflammatory response identical to that occurring in sporadic IBM.
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ranking = 0.99809116234285
keywords = macrophage
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