1/144. hyperthyroidism of Graves' disease: evidence for only unilateral involvement of the thyroid gland in a 31-year-old female patient.hyperthyroidism of Graves' disease (Morbus Basedow) is known to involve the thyroid gland in toto, unlike Graves' ophthalmopathy which clinically may either be unilateral or bilateral. We report a 31-year-old Caucasian female patient who presented with unilateral goiter and clinical and laboratory evidence for hyperthyroidism. High-resolution ultrasonography of the thyroid gland revealed a morphology indicative of an autoimmune thyroid disease strictly limited only to the right lobe. 123I-scintiscanning showed a homogenous but several fold increased uptake of the radionuclide in the right lobe of the thyroid gland, whereas the uptake in the left lobe did not differ from the uptake in normal controls. Cytology of the fine needle aspirate of the right lobe revealed a remarkable inflammatory background mainly by presence of lymphocytes, a finding which was not seen in the cytology of the left lobe. Furthermore, both serum antibodies to TSH-receptors and thyroid peroxidase were significantly increased. Consequently, hyperthyroidism of Graves' disease with the involvement of only one lobe of the thyroid gland was diagnosed.- - - - - - - - - - ranking = 1keywords = ophthalmopathy (Clic here for more details about this article) |
2/144. Apparent cure of Graves-Basedow disease after sibling allogeneic bone marrow transplantation.Evidence that allogeneic bone marrow transplantation (BMT) can cure or alter the course of intractable autoimmune diseases comes from both extensive experimental work in animal models and anecdotal case reports in humans. We describe a female patient diagnosed as having severe aplastic anaemia (SAA), hyperthyroidism and ophthalmopathy of Graves-Basedow disease who received a BMT from her histocompatible sister. Fifty-three months after BMT, complete remission of hyperthyroidism and ocular signs persists. The SAA is cured and she is free of any chronic graft-versus-host disease (GVHD). In the early post-BMT period, PCR analysis of bone marrow and peripheral blood cells confirmed a complete chimerism of donor origin. Thus, it is plausible to attribute the resolution of the patient's thyroid hyperfunction and opththalmopathy to the replacement of the host immune system.- - - - - - - - - - ranking = 1keywords = ophthalmopathy (Clic here for more details about this article) |
3/144. Graves' disease and thyroid associated ophthalmopathy triggered by 131I treatment of non-toxic goiter.A 39-year-old Danish woman was treated with an oral dose of 400 MBq 131I for a non-toxic goiter with compression symptoms. serum anti-TPO and TSH receptor antibodies were negative before radioiodine therapy. The patient developed hyperthyroidism four months after treatment and ophthalmopathy after another three months. TSH receptor stimulating and blocking antibodies, eye muscle IgA and IgG were measured and an increase in stimulating TSH receptor antibodies were found concomitant with development of hyperthyroidism. IgG class antibodies against porcine eye muscle antigens and eye muscle fibroblasts were elevated and unchanged during the follow-up. A significant increase in IgA class antibodies against porcine eye muscle antigens was seen concomitant with development of ophthalmopathy. It is speculated that 131I has led to an exacerbation of eye muscle as well as TSH receptor stimulating antibodies and possibly leading to the development of ophthalmopathy, or at least is associated with it.- - - - - - - - - - ranking = 7keywords = ophthalmopathy (Clic here for more details about this article) |
4/144. Orbital volume expansion of dysthyroid ophthalmopathy by surgical placement of lateral rim implants: a case study.PURPOSE: To measure the increase in orbital volume expansion effected by surgical placement of a lateral rim implant. methods: Computed tomography was used to obtain 1-mm axial sections of a normal human cadaver skull. A computer program was used to measure the orbital area of each section and integrate the sum of the areas to obtain the total orbital volume. Following placement of a lateral orbital rim implant, this procedure was repeated to obtain the new orbital volume. RESULTS: The orbital volume increased from 22.2 cm3 to 23.6 cm3 (an increase of 6%) following placement of the lateral orbital rim implant. CONCLUSION: Surgical placement of a lateral orbital rim implant can be an effective method of orbital volume expansion in dysthyroid ophthalmopathy.- - - - - - - - - - ranking = 5keywords = ophthalmopathy (Clic here for more details about this article) |
5/144. Toxic adenoma and papillary thyroid carcinoma in a patient with Graves' disease.A case of a very rare association of toxic adenoma and papillary carcinoma with Graves' disease is presented. A 34-year-old woman developed Graves' disease with mild ophthalmopathy. An ultrasound revealed diffuse thyroid enlargement with a hypoechoic pattern and a hypoechoic nodule with regular edges of 1.6 cm in diameter at the lower pole of the left lobe. A thyroid 131I scintiscan showed a diffuse and homogeneous 131I distribution. The 131I uptake (RAIU) was elevated. One year later, while still on a low dose of methimazole, the patient had a recurrence of hyperthyroidism following an iodine load from a contrast agent. A further thyroid ultrasound confirmed the previously described pattern but showed a new hypoechoic nodule of 1.1 cm with irregular edges in the left lobe. A thyroid 131I scintiscan this time demonstrated a hyperactive area localised in the larger nodule and a lower diffuse uptake of the remaining tissue. Because of the worsening of the symptoms of hyperthyroidism, the patient had a left lobectomy. On histological examination, the larger nodule was well encapsulated and showed the characteristics of a hyperfunctioning follicular adenoma. The smaller nodule was a typically unencapsulated papillary carcinoma. Several other microfoci of papillary carcinoma were also found in the adjacent tissue. Completion of thyroidectomy was therefore performed, followed by 131I ablative therapy and thyroxine suppressive treatment. This observation suggests that the chronic abnormal stimulation of the thyroid gland by the thyroid-stimulating antibody (TSAb) may facilitate the neoplastic transformation of the thyrocytes in individuals with a critical genetic background.- - - - - - - - - - ranking = 1keywords = ophthalmopathy (Clic here for more details about this article) |
6/144. The role of restricted motility in determining outcomes for vertical strabismus surgery in Graves' ophthalmology.OBJECTIVE: To identify factors predictive of operative success or failure for vertical muscle surgery performed in patients with Graves' ophthalmopathy. DESIGN: Prospective noncomparative case series. PARTICIPANTS: Thirty-one consecutive patients with Graves' ophthalmopathy who demonstrated vertical ocular motor imbalance, with or without simultaneous horizontal muscle imbalance. INTERVENTION: Vertical extraocular muscle surgery performed either in isolation or in association with horizontal muscle surgery. MAIN OUTCOME MEASUREMENTS: Vertical limitations of extraocular muscles were correlated with preoperative hypertropia. Stepwise linear regression was used to determine the significant predictors of postoperative hypertropia in primary gaze. Logistic analysis was used to estimate the probability of surgical failure (>5 diopters) on the basis of preoperative parameters. RESULTS: The amount of preoperative hypertropia was negatively correlated with total restriction of vertical ductions (r = -0.52, P < 0.01). Preoperative hypertropia was positively correlated with asymmetry in muscle restriction between the two eyes (r = 0.67, P < 0.0001). The best predictor of preoperative hypertropia was the difference between restriction of the contralateral opposing recti, namely the right superior rectus, and the left inferior rectus, as well as the right inferior rectus and the left superior rectus (r = 0.74, P < 0.0001). Restriction of the contralateral opposing recti was also the most significant predictor of surgical success (postoperative hypertropia < 5 prism diopters). CONCLUSIONS: Surgery tailored to address restriction of ductions, specifically the difference between contralateral opposing recti, is likely to improve the success of initial surgery beyond that based primarily on the magnitude of the vertical deviation.- - - - - - - - - - ranking = 2keywords = ophthalmopathy (Clic here for more details about this article) |
7/144. Graves' disease and low-output cardiac dysfunction: implications for autoimmune disease in endomyocardial biopsy tissue from eleven patients.Classic high-output thyrotoxic heart disease is generally considered a direct effect of thyroid hormone. In contrast, the cause of the less common low-output heart failure is generally unknown. The aim of this study was to retrospectively evaluate available endomyocardial biopsy tissue from patients with coexistent Graves' disease and idiopathic low-output heart failure and determine whether the biopsy features were consistent with an autoimmune process. The study group consisted of 11 patients whose mean age was 47 years when they were diagnosed with hyperthyroidism and 52 years when diagnosed with cardiac dysfunction. Right ventricular endomyocardial biopsy tissue revealed severe lymphocytic myocarditis in a patient with severe ophthalmopathy and showed borderline myocarditis in a patient without ophthalmopathy. biopsy tissues from 6 other patients showed appreciable myocyte hypertrophy and interstitial fibrosis, consistent with dilated cardiomyopathy. Two patients had nondiagnostic biopsy specimens, and 1 patient had features suggestive of arrhythmogenic right ventricular dysplasia. In conclusion, for the 11 patients with Graves' disease and unexplained systolic dysfunction, only 2 (18%) had lymphocytic infiltrates consistent with an autoimmune process. Thus, among patients with Graves' disease, most cases of low-output cardiac dysfunction appear to be due to causes other than an active autoimmune inflammatory process.- - - - - - - - - - ranking = 2keywords = ophthalmopathy (Clic here for more details about this article) |
8/144. Elephantiasic pretibial myxedema: insight into and a hypothesis regarding the pathogenesis of the extrathyroidal manifestations of Graves' disease.The basis for the extrathyroidal manifestations of Graves' ophthalmopathy (GO) and dermopathy are not well understood. We describe immunohistochemical studies on the skin of a patient with an extreme, elephantiasic form of Graves' dermopathy that developed after periods of prolonged standing with dependent edema. Excision of part of the lesion with subsequent skin grafting from a normal donor site resulted in recurrence of the disease at the original site as well as in development of disease at the donor site. A murine monoclonal antibody reacted with the thyrotropin receptor (TSHR) or a cross-reacting protein in fibroblast-like cells in the patient's upper dermis and, surprisingly, with dermal cells from unaffected individuals. The patient's dermis containing lymphoid follicles comprising B cells and CD3 , CD4 T cells, with few CD8 T cells. CD21 cells (most likely follicular dendritic cells) were also present in the dermis. Based on past and present observations, we raise an unifying hypothesis to explain the diverse extrathyroidal manifestations of Graves' disease and their apparent lack of association with TSHR autoantibodies. As opposed to the present concept that these phenomena relate to site-specific properties on preadipocytes or fibroblasts, we suggest that clinically evidence GO and dermopathy are primarily caused by local factors (particularly in the orbit) superimposed on a systemic, low-grade connective tissue inflammation.- - - - - - - - - - ranking = 1keywords = ophthalmopathy (Clic here for more details about this article) |
9/144. Measurement of venous outflow pressure in the central retinal vein to evaluate intraorbital pressure in Graves' ophthalmopathy: a preliminary report.PURPOSE. To evaluate the intraorbital pressure in patients with Graves' ophthalmopathy (GO) in relation to the intraocular pressure (IOP) and proptosis and to find out whether optic nerve compression is predictable. methods. The venous outflow pressure (VOP) in the central retinal vein was measured by the perviously described technique of oculodynamometry.1 Since the central retinal vein passes through the orbit, the VOP cannot be lower than the intraorbital pressure if outflow is to be guaranteed. The IOP was measured either in primary position or with slight chin elevation to avoid restriction of the globe. Fifty-seven patients underwent a complete ophthalmologic examination, including VOP measurements, Hertel exophthalmometry and visual fields. RESULTS. The IOP in primary position ranged between 10 and 29 mmHg and in most (n=54) cases the VOP was 0-4 mmHg higher than the IOP. These patients had neither scotomas nor visual deterioration during an observation period of up to 2 years. In those cases (n=3) where the difference between IOP and VOP was 35 mmHg, the patients developed scotomas and visual deterioration and had to be treated (high-dose steroids or orbital decompression). The elevation in VOP did not correlate with the degree of proptosis. In one unilateral case, treatment of high IOP (32 mmHg) with dorzolamide drops led to a decrease in visual acuity of two lines, inferior field depression and relative afferent pupillary defect. The difference between IOP and VOP was 10 mmHg. Stopping treatment normalized visual function, the IOP rose to its original level and the difference between IOP and VOP was 4 mmHg. CONCLUSION. The increased IOP in GO is not caused by primary glaucoma but by elevated intraorbital pressure. The difference between IOP and VOP must be <5 mmHg to guarantee normal perfusion. We interpret these findings to suggest that loss of visual acuity and visual field defects may not only be caused by optic nerve compression at the apex but also by deterioration of optic nerve head perfusion.- - - - - - - - - - ranking = 5keywords = ophthalmopathy (Clic here for more details about this article) |
10/144. time course of 21-hydroxylase antibodies and long-term remission of subclinical autoimmune adrenalitis after corticosteroid therapy: case report.Subclinical Addison's disease is characterized by the presence of adrenal autoantibodies (ACA) and steroid 21-hydroxylase autoantibodies (21OHAb) with or without adrenal function failure. In our previous longitudinal study some patients with high titers of ACA and at stage 2 of subclinical adrenocortical failure showed disappearance of ACA with recovery of normal adrenocortical function after corticosteroid treatment for Graves' ophthalmopathy. To investigate whether corticosteroid-induced modification of the adrenal autoimmune markers can also involve 21OHAb and to evaluate whether the remission of subclinical adrenocortical failure can persist over a long period of time, we followed-up for 100 months the levels of 21OHAb and ACA as well as the metabolic markers of adrenal function in one patient with Graves' ophthalmopathy and at stage 2 of subclinical adrenocortical failure before and after corticosteroid therapy. A 34-yr-old woman with Graves' disease and active ophthalmopathy who was found to be positive for ACA and to have high PRA, low aldosterone levels, and normal basal ACTH and cortisol levels, but impaired cortisol response to ACTH was studied. The patient was treated with oral corticosteroid therapy for 6 months. After corticosteroid therapy, 21OHAb, initially positive, became negative in concomitance with the disappearance of ACA and the restoration of normal adrenal function. The disappearance of both 21OHAb and ACA and their prolonged absence during the follow-up suggest that corticosteroid treatment can induce long-term remission of subclinical adrenal insufficiency and prevent the onset of the clinical phase of the disease. Our pilot study may pave the way to future trials aimed at preventing the onset of the clinical signs of Addison's disease in ACA/21OHAb-positive patients.- - - - - - - - - - ranking = 3keywords = ophthalmopathy (Clic here for more details about this article) |
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