1/31. Pseudo-myasthenia gravis and thymic hyperplasia in Graves' disease.BACKGROUND: Diagnostic confusion between thyroid disease and myasthenia gravis (MG) can arise because the two may have similar clinical features, and also because of the more frequent coexistence of these autoimmune disorders in the same individual. In MG, autoantibodies directed against the acetylcholine receptor result in muscle weakness. Thymic pathology is well recognized in MG, with thymic hyperplasia frequent in early onset MG and thymoma more common in later onset MG. In Graves' disease, autoantibodies against thyroid antigens result in hyperthyroidism. A seldom-recognized feature of Grave's disease is the occurrence of an enlarged thymus (thymic hyperplasia) on chest CT, or of thymic lymphoid hyperplasia pathologically. CASE STUDY: This report describes a case in which the discovery of a mediastinal mass during imaging of the thyroid, and the presence of myasthenic-like symptoms, in a patient with Graves' disease prompted investigations into whether the patient also had MG. RESULTS: Despite symptoms which strongly suggested MG, subsequent investigations did not confirm the diagnosis, and treatment of Grave's lead to a resolution of the symptoms and regression of the thymic enlargement seen on CT. CONCLUSIONS: The case study highlighted clinical similarities between Grave's disease and myasthenia gravis which might cause diagnostic confusion, and also the investigations which are useful in order to differentiate the two diseases. In addition to common clinical features, the autoimmune diseases Grave's disease and myasthenia gravis may both produce radiological thymic enlargement.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/31. Paraneoplastic pemphigus associated with Castleman tumor, myasthenia gravis and bronchiolitis obliterans.BACKGROUND: Cases of paraneoplastic pemphigus (PNP) have been reported associated with various lymphoproliferative malignancies and benign Castleman tumors, with the most severe course and fatal outcome seen in patients with bronchiolitis obliterans. OBJECTIVE: The aim was to establish immunologic associations by coexistence of Castleman tumor, myasthenia gravis, and bronchiolitis obliterans and to evaluate the treatment modalities. methods: Clinical studies included computed tomography of the mediastinum, computed tomography and magnetic resonance imaging of the abdominal cavity, and quantitative electromyography. Direct and indirect immunofluorescence on various substrates, immunoblot analysis, immunoprecipitation, and specific enzyme-linked immunosorbent assay using recombinant desmogleins (Dsg) were performed as immunologic assays. RESULTS: Direct and indirect immunofluorescence including rat bladder showed intercellular antibodies. immunoblotting disclosed antibodies to envoplakin (210 kd protein) and periplakin (190 kd protein); in addition, immunoprecipitation detected antibodies to desmoplakin I (250 kd protein). Antibodies to Dsg3 (pemphigus vulgaris antigen) were detected by specific enzyme-linked immunosorbent assay. myasthenia gravis was controlled by drugs; however, mucocutaneous changes were not fully responsive to corticosteroids and cyclophosphamide pulses, cyclosporine, and intravenous immunoglobulins. The surgical removal of Castleman tumor did not change the course of the disease. The fatal outcome was the result of bronchiolitis obliterans that occurred after the surgery and was only transitionally controlled by plasmapheresis. CONCLUSION: This is the first case of paraneoplastic pemphigus associated with Castleman tumor, myasthenia gravis, and bronchiolitis obliterans. Despite a benign character of the tumor the patient died, as do all patients with bronchiolitis obliterans. Massive plasmapheresis has only a transient effect. We confirmed the presence of antibodies to Dsg 3, in addition to the set of specific paraneoplastic pemphigus antibodies against various proteins of plakin family.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
3/31. Coexistence of epilepsy, myasthenia gravis and psoriasis vulgaris.We report the case of a 36-year-old Chinese man with a history of complex partial seizure of temporal lobe origin since the age of 12 years, superimposed by myasthenia gravis since the age of 27 years and psoriasis vulgaris since the age of 29 years. With an eight-year follow-up, the above three diseases remained without complete remission. Anticonvulsant therapy (phenytoin and trimethadione) caused drug-induced myasthenia gravis, which should gradually disappear after discontinuing the drugs. However, the myasthenic symptoms and serum acetylcholine receptor antibody persisted following the discontinuation of phenytoin in our patient. myasthenia gravis and psoriasis are both autoimmune diseases and correlate with specific human histocompatibility antigens. This suggests a close connection between these two diseases. The coexistence of epilepsy, myasthenia gravis and psoriasis vulgaris has not been previously reported, and to the best of our knowledge, our patient is the first reported case. The relationship among these three diseases requires further investigation.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
4/31. Familial autoimmune myasthenia gravis.Familial Autoimmune myasthenia gravis (FAMG) is rarely reported. We present a mother and son with late-onset mild to moderate ocular disease, low acetylcholine receptor antibody titre and the absence of a thymoma. Both responded well to low doses of anticholinesterase. HLA typing revealed that they did not share the usual hla antigens or haplotypes with that previously reported in Caucasian and Chinese sporadic myasthenia gravis. Chinese FAMG may be associated with hla antigens different from that of sporadic MG.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
5/31. Induction of myasthenia gravis, myositis, and insulin-dependent diabetes mellitus by high-dose interleukin-2 in a patient with renal cell cancer.interleukin-2 is an effective agent against renal cell carcinoma and melanoma, but it has been associated with autoimmune sequelae such as hypothyroidism and vitiligo. A 64-year-old man with non-insulin-dependent diabetes and metastatic renal cell carcinoma developed insulin-dependent diabetes after his first cycle of therapy with high-dose (HD) interleukin-2. After additional therapy with interleukin-2, the patient developed generalized myasthenia gravis (MG) and polymyositis, both of which responded to treatment with corticosteroids and plasmapheresis. To investigate the role of IL-2 in the development of these autoimmune complications, autoantibody titers were assayed from serum obtained before and after IL-2 treatment and after treatment with corticosteroids plus plasmapheresis. Before IL-2 treatment, the patient had antibodies directed against insulin, islet cell antigens, and striated muscle. acetylcholine receptor antibody levels were normal before starting IL-2. After treatment with IL-2, the patient developed acetylcholine receptor binding antibodies and exhibited an increase in the striated muscle antibody titer from 1:40 to 1:160. Recovery from the MG and polymyositis was associated with substantial decreases in the acetylcholine receptor and striated muscle antibody titers. These findings suggest that HD IL-2 accelerated the progression of latent autoimmune diabetes and myositis in this patient whose tolerance to islet cell antigens and striated muscle had already been broken and precipitated a break in tolerance to the acetylcholine receptor resulting in the development of MG. This case demonstrates the importance of prompt recognition of IL-2-induced MG and shows how this complication can be successfully managed with aggressive therapy.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
6/31. Cancer-associated retinopathy associated with invasive thymoma.PURPOSE: To report a case of cancer-associated retinopathy associated with invasive thymoma. DESIGN: Interventional case report. METHOD: A 41-year-old Japanese woman was observed between February 1998 and May 2001. Ophthalmologic examinations and systemic examinations were performed. The patient received treatment including corticosteroid pulse therapy, plasmapheresis, and thymectomy. RESULTS: The patient developed progressive visual dysfunction including bilateral visual acuity loss, concentric contraction of visual fields, and color vision loss. In both eyes, retinal vessel attenuation and retinal pigment epithelium degeneration were observed with fundus ophthalmoscopy and fluorescein angiography. Response in electroretinogram was reduced, suggesting both rod and cone dysfunction. Autoantibody against 23-kD cancer-associated retinopathy (CAR) antigen (antirecoverin antibody) was detected in the patient's serum. A mediastinal tumor that was histopathologically diagnosed as invasive thymoma was detected and was surgically resected. During more than 3 years of follow-up, no other malignancy was detected despite extensive systemic evaluation. The patient also suffered from subclinical myasthenia gravis. Although temporary improvement of visual function was observed after treatment with steroid pulse therapy and plasmapheresis' light perception of each eye was lost in the end. CONCLUSIONS: The patient was diagnosed as having CAR. Invasive thymoma was considered to be the causative tumor because there had been no evidence that suggested other systemic malignancy during more than 3 years of follow-up.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
7/31. Anti-MuSK myasthenia gravis presenting with purely ocular findings.BACKGROUND: Antibodies to a muscle-specific receptor tyrosine kinase (MuSK) have been found in approximately 40% of patients with generalized myasthenia gravis who are seronegative for the antiacetylcholine receptor antibody. Many of the patients with anti-MuSK antibodies have prominent oculobulbar symptoms or weakness of the neck and respiratory muscles, but patients with ocular myasthenia have not been described. OBJECTIVE: To report a case of ocular myasthenia due to anti-MuSK antibodies. PATIENT: A young woman with ocular myasthenia and antibodies to MuSK. RESULTS: Anti-MuSK antibody was detected by radioimmunoassay using highly purified MuSK recombinant antigen. CONCLUSION: Ocular myasthenia gravis is a presentation of the anti-MuSK antibody syndrome.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
8/31. A case of multiple sclerosis associated with defective neuromuscular transmission.A 38-year-old woman developed relapsing disseminated sclerosis associated with myasthenic syndrome. The cerebrospinal fluid showed 12 lymphocytes per 1 mul and paretic colloidal curve. The serum contained antibody against smooth muscle antigen (titre 1 : 10) but no antibody against nuclear antigen or VDRL antigen. Repetitive stimulation of the right ulnar nerve at 35 c/sec. demonstrated typical decrements between 70 and 72%. The prostigmine test showed significant diminishing of decrements. The possible immunopathological mechanism of myasthenic syndrome in multiple sclerosis was briefly discussed.- - - - - - - - - - ranking = 3keywords = antigen (Clic here for more details about this article) |
9/31. Immune studies in human immunodeficiency virus infection with myasthenia gravis: a case report.A patient with human immunodeficiency virus infection had cellular and humoral immune responses studied longitudinally from the onset of generalized myasthenia gravis. Progressive decline in CD4 , CD45R and CD4 , CDw29 T-cells, cellular immune responses to alloantigen and mitogen stimulation, and acetylcholine receptor antibody titers were associated with clinical improvement of all myasthenic symptoms.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
10/31. Thymic lymphoepitheliomas and skeletal muscle expressing common antigen(s).Rabbit antiserum to a citric acid extract of human skeletal muscle (CA) stained epithelial thymoma cells as well as skeletal muscle. Thymomas from two myasthenia gravis (MG) patients showing no circulating anti-CA antibodies prior to thymectomy were also stained by the antiserum. Thus, in these patients as well, the thymoma and skeletal muscle possess common antigens. The rabbit and the human antibodies most probably reacted with different antigens, apparently located close to each other in the cell membrane. The reason why anti-CA antibodies cannot be detected in serum from a few MG patients with a thymoma may be that the thymoma-associated antigen is not present in vivo in these cases, or that an inhibiting factor blocks the antibody synthesis. Both patients developed anti-CA antibodies post-operatively, which favours the latter explanation.- - - - - - - - - - ranking = 7keywords = antigen (Clic here for more details about this article) |
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