Child ; Humans ; Male ; Myositis ; diagnosis ; drug therapy ; pathology

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 ; Case Report ; Human ; Male ; Muscles/pathology ; Myositis, Inclusion Body/*pathology/physiopathology

Adult ; Female ; Humans ; Lymphoma, T-Cell ; pathology ; physiopathology ; Lymphoma, T-Cell, Cutaneous ; pathology ; physiopathology ; Myositis ; physiopathology

Inflammatory myopathies are comprised of three major subsets, polymyositis, dermatomyositis and inclusion body myositis. Although their etiology is unclear, each group retains its characteristic clinical, immunopathologic features. In polymyositis, a CD8+ T-cell mediated cytotoxicity against muscle fibers has emerged as the main pathologic event, whereas in dermatomyositis complement-mediated injury by antibody may be the primary pathology. There has been several reports on polymyositis internationally but we could find only a few reports in Korea. We report here a 8-year old female patient admitted with a stuporous mentality. After coughing and fever for 3 days, she got myalgia, abruptly developed gross hematuria and dyspnea. After admission, she showed weak self respiration and exclussively elevated muscle enzyme in blood chemistry. In muscle biopsy, lymphocytic infiltrations were found in the fascicles without endomysial fibrosis and these lymphocytes were composed of T lymphocytes on immunohistochemical stain. She received two infusions of intravenous immunoglobulin(1g/kg/day), and showed dramatic improvement in symptoms and signs.
Biopsy ; Chemistry ; Child ; Cough ; Dermatomyositis ; Dyspnea ; Female ; Fever ; Fibrosis ; Hematuria ; Humans ; Immunoglobulins* ; Korea ; Lymphocytes ; Myalgia ; Myositis ; Myositis, Inclusion Body ; Pathology ; Polymyositis* ; Respiration ; Stupor ; T-Lymphocytes

Inflammatory myopathies are comprised of three major subsets, polymyositis, dermatomyositis and inclusion body myositis. Although their etiology is unclear, each group retains its characteristic clinical, immunopathologic features. In polymyositis, a CD8+ T-cell mediated cytotoxicity against muscle fibers has emerged as the main pathologic event, whereas in dermatomyositis complement-mediated injury by antibody may be the primary pathology. There has been several reports on polymyositis internationally but we could find only a few reports in Korea. We report here a 8-year old female patient admitted with a stuporous mentality. After coughing and fever for 3 days, she got myalgia, abruptly developed gross hematuria and dyspnea. After admission, she showed weak self respiration and exclussively elevated muscle enzyme in blood chemistry. In muscle biopsy, lymphocytic infiltrations were found in the fascicles without endomysial fibrosis and these lymphocytes were composed of T lymphocytes on immunohistochemical stain. She received two infusions of intravenous immunoglobulin(1g/kg/day), and showed dramatic improvement in symptoms and signs.
Biopsy ; Chemistry ; Child ; Cough ; Dermatomyositis ; Dyspnea ; Female ; Fever ; Fibrosis ; Hematuria ; Humans ; Immunoglobulins* ; Korea ; Lymphocytes ; Myalgia ; Myositis ; Myositis, Inclusion Body ; Pathology ; Polymyositis* ; Respiration ; Stupor ; T-Lymphocytes

PURPOSE: Neurogenic myositis ossificans (NMO) in patients with traumatic spinal cord or brain injuries can cause severe joint ankylosis or compromise neurovascularture. The purpose of this study was to evaluate the clinical and radiological outcomes of and review considerations relevant to surgical resection of NMO of the hip joint. MATERIALS AND METHODS: Six patients (9 hips) underwent periarticular NMO resection between 2015 and 2017. The medical records of these patients were retrospectively reviewed. Preoperative computed tomography including angiography was performed to determine osteoma location and size. Improvement in hip motion allowing sitting was considered the sole indicator of a successful surgery. The anterior approach was used in all patients. The ranges of motion (ROM) before and after surgery were compared. RESULTS: The mean time from accident to surgery was 3.6 years. Average ROM improved from 24.3°(flexion and extension) to 98.5°(flexion and extension) after surgery, and improvement was maintained at the last follow-up. No commom complications (e.g., deep infection, severe hematoma, deep vein thrombosis) occurred in any patient. Improvement in ROM in one hip in which surgical resection was performed 10 years after the accident was not satisfactory owing to the pathologic changes in the joint. CONCLUSION: Surgical excision of periarticular NMO of the hip joint can yield satisfactory results, provided that appropriate preoperative evaluation is performed. Early surgical intervention yields satisfactory results and may prevent the development of intra-articular pathology.
Angiography ; Ankylosis ; Brain Injuries ; Follow-Up Studies ; Hematoma ; Hip Joint ; Hip ; Humans ; Joints ; Medical Records ; Myositis Ossificans ; Myositis ; Osteoma ; Pathology ; Retrospective Studies ; Spinal Cord ; Veins

Here we report a case of the classical inclusion body myositis. The muscle pathology in a 61-year-old male patient with slowly progressive proximal muscle weakness and atrophy revealed basophilic rimmed vacuoles on light microscope and intracytoplasmic filamentous inclusions with membranous whorls through electron microscope. He did not respond to steroid therapy.
Atrophy ; Basophils ; Humans ; Inclusion Bodies* ; Male ; Middle Aged ; Muscle Weakness ; Myositis, Inclusion Body* ; Pathology ; Vacuoles

Child, Preschool ; Diagnosis, Differential ; Humans ; Male ; Myositis Ossificans ; diagnosis ; diagnostic imaging ; pathology ; physiopathology ; Prognosis ; Tomography, X-Ray Computed

Humans ; Infant, Newborn ; Leg ; diagnostic imaging ; pathology ; Magnetic Field Therapy ; methods ; Male ; Myositis Ossificans ; diagnostic imaging ; pathology ; therapy ; Tomography, X-Ray Computed

The patient was a 55-year-old man who was admitted to hospital with "progressive myalgia and weakness for 4 months, and exacerbated for 1 month". Four months ago, he presented with persistent shoulder girdle myalgia and elevated creatine kinase (CK) at routine physical examination, which fluctuated from 1 271 to 2 963 U/L after discontinuation of statin treatment. Progressive myalgia and weakness worsened seriously to breath-holding and profuse sweating 1 month ago. The patient was post-operative for renal cancer, had previous diabetes mellitus and coronary artery disease medical history, had a stent implanted by percutaneous coronary intervention and was on long-term medication with aspirin, atorvastatin and metoprolol. Neurological examination showed pressure pain in the scapularis and pelvic girdle muscles, and V- grade muscle strength in the proximal extremities. Strongly positive of anti-HMGCR antibody was detected. Muscle magnetic resonance imaging (MRI) T2-weighted image and short time inversion recovery sequences (STIR) showed high signals in the right vastus lateralis and semimembranosus muscles. There was a small amount of myofibrillar degeneration and necrosis, CD4 positive inflammatory cells around the vessels and among myofibrils, MHC-Ⅰ infiltration, and multifocal lamellar deposition of C5b9 in non-necrotic myofibrils of the right quadriceps muscle pathological manifestation. According to the clinical manifestation, imageological change, increased CK, blood specific anti-HMGCR antibody and biopsy pathological immune-mediated evidence, the diagnosis of anti-HMGCR immune-mediated necrotizing myopathy was unequivocal. Methylprednisolone was administrated as 48 mg daily orally, and was reduced to medication discontinuation gradually. The patient's complaint of myalgia and breathlessness completely disappeared after 2 weeks, the weakness relief with no residual clinical symptoms 2 months later. Follow-up to date, there was no myalgia or weakness with slightly increasing CK rechecked. The case was a classical anti-HMGCR-IMNM without swallowing difficulties, joint symptoms, rash, lung symptoms, gastrointestinal symptoms, heart failure and Raynaud's phenomenon. The other clinical characters of the disease included CK as mean levels >10 times of upper limit of normal, active myogenic damage in electromyography, predominant edema and steatosis of gluteus and external rotator groups in T2WI and/or STIR at advanced disease phase except axial muscles. The symptoms may occasionally improve with discontinuation of statins, but glucocorticoids are usually required, and other treatments include a variety of immunosuppressive therapies such as methotrexate, rituximab and intravenous gammaglobulin.
Male ; Humans ; Middle Aged ; Autoantibodies ; Myositis/diagnosis* ; Autoimmune Diseases ; Muscle, Skeletal/pathology* ; Hydroxymethylglutaryl-CoA Reductase Inhibitors/therapeutic use* ; Necrosis/pathology* ; Muscular Diseases/drug therapy*