Cases reported "Vision Disorders"

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1/14. Treatment of paraneoplastic visual loss with intravenous immunoglobulin: report of 3 cases.

    BACKGROUND: Paraneoplastic visual loss is an autoimmune disorder believed to be caused by the remote effects of cancer on the retina (cancer-associated retinopathy [CAR]) or optic nerve. Both disorders may result in rapid and complete blindness. Spontaneous recovery of vision has not been reported. The serum of patients with CAR contains autoantibodies against recoverin, enolase, or unidentified retinal proteins. autopsy examination results of eyes of blind patients with CAR show complete absence of the retinal neurons involved in phototransduction. Corticosteroids and plasmapheresis are the only treatment options previously described. OBJECTIVE: To treat paraneoplastic visual loss. DESIGN AND methods: Three patients with metastatic cancer developed rapidly progressive loss of vision. The first patient had visual acuity of hand movements in each eye before intravenous immunoglobulin treatment. The second patient had visual acuity of light perception in both eyes. The third patient's visual acuity was 20/400 OD and 20/20 OS. Diagnostic tests included magnetic resonance imaging of the head and cytologic examination of the cerebrospinal fluid to exclude metastasis as the cause of visual loss and then an electroretinogram and serum tests for autoantibodies against retinal antigens to confirm the clinical diagnosis of CAR. patients 1 and 2 were treated with intravenous immunoglobulin (400 mg/kg per day) for 5 days; however, patient 3 received only a single dose due to adverse effects consisting of shortness of breath and itching. RESULTS: Within 24 hours of taking the first dose of intravenous immunoglobulin, the visual acuity of patient 1 improved from hand movements only in both eyes to 20/50 OD and 20/200 OS. After the third day of treatment, visual acuity in the left eye further improved to 20/40. Even with the improved acuity, Goldmann visual field perimetry results showed poor responses in both eyes. However, 2 weeks later there was marked visual field improvement, and visual acuity was maintained at 20/50 OD and 20/40 OS. Patient 2 had no improvements and continued to have light perception in both eyes. Patient 3 had improvements in visual field defects but remained 20/400 OD and 20/20 OS. CONCLUSION: Intravenous immunoglobulin may be another treatment option offered to patients with paraneoplastic visual loss in addition to corticosteroids or plasmapheresis because a review of the medical literature has shown no spontaneous improvements of visual function without treatment.
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2/14. Two cases of primary open angle glaucoma with serum autoantibody against retinal ganglion cells.

    PURPOSE: To present two cases of primary open angle glaucoma (POAG) with serum autoantibody against retinal 25 kDa or 50 kDa antigen. methods: Ocular examinations were performed on a 60-year-old man (case 1) and a 74-year-old woman (case 2). We also performed serological and immunological examinations including Western blot analysis and immunocytochemical analysis. RESULTS: In Western blot analysis, they were found to have serum autoantibody against retinal 25 kDa or 50 kDa antigen, and immunocytochemical analysis revealed that retinal ganglion cell layers were specifically labeled by each patient's IgG. CONCLUSION: We report two interesting cases of POAG with serum autoantibody against retinal 25 kDa or 50 kDa antigen.
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keywords = antigen
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3/14. Epithelioid haemangioendothelioma of the sphenoid bone.

    The authors report a case of cranial EH occurred in the left sphenoid bone that was totally excised without transfusion. A 26-year-old woman presented with a 1-year history of progressing exophthalmos in the left eye. A cranial X-ray showed a mixed osteolytic and sclerotic expansile mass lesion in the left sphenoid bone. Neuroradiologic imaging study revealed a left temporosphenoidal extra-axial expansile mass lesion with heterogeneous enhancement after contrast enhancement associated with destruction and erosion of the temporal and sphenoid bones. The cerebral parenchyma and orbital contents were compressed without any sign of infiltration. Bone scan showed hot uptake in the left orbital region. Angiogram demonstrated marked neoplastic angiogenesis from the middle meningeal artery and other branches of left external carotid artery, for which preoperative embolisation was tried in vain. Cranio-orbito-zygomatic approach was undergone in two stages because of the patient's religious belief (patient was Jehovah's witness) and profuse bleeding during the first surgery. Two weeks after initial operation, second surgery was carried out with total excision of the residual mass in the left orbital ridge. Histopathological examination revealed typical epithelioid cell cords or nests in myxoid stroma with a positive immunoreactivity to factor viii-related antigen. The authors report an unusual case of EH involving sphenoid and temporal bone in a young woman. Preoperative embolisation is thought to be absolutely essential before removal. Because there is no convincing data to advise radiation/chemotherapy, total resection and close follow-up may be reasonable.
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4/14. The photoreceptor cell-specific nuclear receptor is an autoantigen of paraneoplastic retinopathy.

    OBJECTIVES: To report a novel antibody associated with paraneoplastic retinopathy and to characterize the retinal autoantigen. methods: immunohistochemistry of rat and human tissues was used to identify antiretinal antibodies. Serologic screening of a bovine retinal cDNA expression library was performed to clone the target antigen. RESULTS: A 72-year-old woman presented with a 6-month history of progressive visual loss, bilateral central scotomas, light flashes, and night blindness. visual acuity was 20/40 OD and 20/30 OS. There was generalized loss of retinal pigment and narrow arterioles; discs were normal in appearance. The electroretinogram showed no response. Chest computed tomograph scan demonstrated a right lung mass; biopsy revealed poorly differentiated carcinoma. The patients' serum contained antibodies that immunolabeled nuclei of cells of the outer--and to a lesser extent, the inner--nuclear layer of the adult rat retina. No reactivity was identified with nonretinal adult human or rat tissues. Reactivity was seen in the developing rat embryo. Serologic screening of a bovine retinal library resulted in the isolation of three overlapping clones, encoding a protein highly homologous to the human photoreceptor cell-specific nuclear receptor gene product. CONCLUSIONS: The target antigen of an antibody associated with paraneoplastic retinopathy is the photoreceptor cellspecific nuclear receptor, a member of a conserved family of nuclear receptors involved in photoreceptor cell development or maintenance.
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ranking = 7
keywords = antigen
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5/14. 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.
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6/14. An unusual symptom of lidocaine allergy. Report of a case.

    The incidence of lidocaine allergy is rare. A confirmed case of antigen/antibody reaction is presented here. It is further distinguished by the unusual presence of blurred vision in the patient.
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7/14. Birdshot retinochoroidopathy in monozygotic twins.

    Birdshot retinochoroidopathy is a rare ocular disorder which was named and delineated as a separate clinical entity by Ryan & Maumenee in 1980. We diagnosed birdshot retinochoroidopathy in a monozygotic pair of twins, who were affected with a time interval of 12 years, respectively. These are the first with birdshot retinochoroidopathy to be reported from the Nordic countries and the first report on this disorder in monozygotic twins. Due to night-blindness, visual field defects and a severely affected electroretinogram one of our cases initially was diagnosed as a choroidoretinal dystrophy. Birdshot retinochoroidopathy should be kept in mind as a differential diagnosis in retinitis pigmentosa-like disorders with widespread choroidal involvement. Our cases substantiated the evidence of a strong correlation with the presence of HLA-A29 antigen.
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keywords = antigen
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8/14. Metastasis of prostatic adenocarcinoma to the sphenoid sinus.

    The presentation, diagnosis, and management of prostatic adenocarcinoma metastatic to the sphenoid sinus are reviewed. We present a case report with a review of the literature. A 67-year-old man with a history of prostatic adenocarcinoma presented with gradual left visual loss. magnetic resonance imaging revealed a lesion of the left orbital apex with extension into the ipsilateral sphenoid sinus. Operative biopsy of the lesion was significant for adenocarcinoma of the prostate. When an otolaryngologist encounters a mass in the sphenoid sinus, he or she needs to consider a diverse differential diagnosis. In evaluating possible causes, a history of malignancies should be elicited. Furthermore, the pathophysiology and potential routes of metastatic disease should be assessed for these primary neoplasms. Having a high level of suspicion for metastatic disease from specific primary sites will help guide the pathological evaluation. As in this clinical scenario of a patient with a history of prostatic adenocarcinoma, appropriate analysis would entail sending specimens for immunohistochemical staining, such as prostate-specific antigen and prostate-specific acid phosphatase. Correct diagnosis is crucial, as these patients may achieve remission and prolonged survival with irradiation and/or hormonal therapy.
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9/14. Autoimmune basis for visual paraneoplastic syndrome in patients with small-cell lung carcinoma.

    Unexplained neurological disorders sometimes develop in patients with cancer, even when the primary tumour or its metastases have not invaded the central nervous system. Visual dysfunction in patients with small-cell carcinoma of the lung is one of these paraneoplastic syndromes and is associated with serum auto-antibodies which react with specific subsets of retinal neurons. These sera have now been shown to react with cell lines derived from small-cell carcinoma. The antibodies recognise a small number of antigens shared by retinal and tumour cells, suggesting that an autoimmune response may be triggered by the tumour.
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10/14. Disseminated intravascular coagulopathy.

    disseminated intravascular coagulation (DIC) is not a disease but a pathological process with widespread thrombus formation in small vessels; it occurs in many systemic conditions that stimulate the intravascular clotting mechanism. There may be widespread tissue involvement, and any tissue in the body may be affected, especially in the kidney, brain, liver, heart, and lungs. This abnormal coagulation is now commonly referred to as disseminated intravascular coagulopathy. It is prone to occur in obstetrical complications, in cancer, after transplantations, and where there has been tissue damage, such as burning, crushing, and surgery, all of which release thromboplastin into the circulation. It may also occur in Gram negative bacterial systemic infections, in antigen-antibody reactions, and in thrombotic thrombocytopenic purpura. When the eye is involved, the thrombi occur in the choriocapillaris, and are usually limited to the submacular and peripapillary choroid. The anterior parts of the eye generally escape involvement. Visual symptoms may be early, and may be due to central choroidopathy or to secondary retinal detachment.
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