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Ocular Myasthenia Gravis Associated with Autoimmune Thyroid Disease

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Ocular Myasthenia Gravis Associated with Autoimmune Thyroid Disease Case Report OCULAR MYASTHENIA GRAVIS ASSOCIATED WITH AUTOIMMUNE THYROID DISEASE Aylin Yaman, M.D.* / Hakan Yaman, M.D., M.S.** * Departm ent o f neurology, İsparta State Hospital, İsparta, Turkey. ** Fam ily Physician, School o f Medicine, Suleyman Dem irel University, İsparta, Turkey. ABSTRACT pathologies is a rare condition but should be taken into consideration. The coincidence of thyroid ophthalmopathy and ocular myasthenia gravis is a defined but rather rare condition. Key Words: Myasthenia gravis, Graves’ disease, Ophthalmopathy, Ocular involvement, A 30-year-old female patient was admitted with Thyrotoxicosis, Thymectomy complaints of weight loss, sweating, palpitation and tremor of both hands for about 4 months. She was diagnosed as having Graves' disease INTRODUCTION with elevated thyroid hormone levels, diffuse hyperplastic thyroid gland with increased iodine, Graves’ disease and myasthenia gravis are both 131 uptake and positive antithyroid antibodies. autoimmune diseases and the coexistence of Two months after the diagnosis of thyroid these two diseases is well recognised. disease, she developed diplopia and in the Myasthenia Gravis is more frequent in patients following days, bilateral ptosis. Orbital magnetic with thyroid disease. It is seen in less than one resonance imaging showed that the lateral and percent of patients with Graves’ disease, and inferior rectus muscles were thickened indicating approximately five percent of myasthenic the infiltration of those muscles. She had positive patients are thyrotoxic at some time (1). response to the prostigmine test and elevated Moreover thyroid diseases occur more frequently acetylcholine receptor antibody levels supporting in close relatives of patients with myasthenia the diagnosis of myasthenia gravis. She had gravis than in the general population (2). undergone tymectomy and the pathology Antithyroglobulin and antimicrosomal antibodies specimen revealed thymic hyperplasia. have been reported to be present in 11% and 27% of myasthenic patients, respectively (3). This case demonstrates the comorbidity of Conversely, 51% of thyrotoxic non-myasthenic thyroid ophthalmoplegia and ocular myasthenia patients have been shown to have circulating gravis. Ocular involvement by this two different antibodies against thyroid, smooth muscle and (Accepted 2 September, 2001) Marmara Medical Journal 2001 ;14(4):241 -244 Correspondance to: Hakan Yaman, M.D. - Family Physician, School ot Medicine, Suleyman Demirel University, İsparta. Turkey e.mail address: [email protected] 241 Aylin Yaman, et al thymus gland (3). If these two clinical conditions coexist in a patient, both present with their characteristic signs and symptoms. Here we present a case of thyroid ophthalmopathy and isolated ocular myasthenia gravis. CASE REPORT A thirty-year-old, right-handed woman was admitted with the complaints of weight loss, sweating, palpitation and tremulousness continuing for about 4 months. The patient was F ig .2 : Downward gaze showing the restriction of the right eye diagnosed as having Graves’ disease. The findings were: diffuse enlarged thyroid gland, elevated thyroid hormone levels, positive exceeded the limbus 3mm and the pupilla was antithyroid antibodies, and increased iodine 131 covered by the median line. uptake. Propylthiouracil 300 mg/day was started. Two months after the diagnosis of thyroid The routine laboratory investigations including disease she developed diplopia on downward blood glucose level, erythrocyte sedimentation and lateral gazes. Three weeks later, bilateral rate, electrolytes, kidney and liver function tests ptosis was added to the symptoms. The severity were normal. Complete hemogram was of her ptosis fluctuated during day time. Her consistent with mild iron deficiency anemia. medical history revealed that, she had had a Thyroid function tests revealed free T3: 10,19 cesarian section 10 months before the pmol/L (Normal values:3,5-6,5), free T4: 31,84 appearence of the symptoms. pmol/L (Normal values:11,5-23,2), TSH: 0,01 mlU/ml (Normal values:0,35-5,5). Investigation of The patient was examined three days after the thyroid antibodies revealed Anti-M: 1020 IU/ml onset of ptosis. In physical examination, she had (Normal values:0-50), Anti-Tg: 17,7 IU/ml a diffusely palpable thyroid gland and (Normal values:0-50), Tr Ab: 49 IU/ml (Normal exophthalmos of both eyes (right eye 24mm values:0-15). Hertel, left eye 22mm Hertel). The neurological examination revealed bilateral ptosis Magnetic resonance imaging (MRI) of the globes predominantly on the right side (Fig. 1). Diplopia showed increased intensity and thickening of occurred during horizontal and downward gazes. both lateral rectus, right medial rectus, right The right globe was restricted during downward superior rectus and left inferior rectus muscles, gaze (Fig. 2). The severity of ptosis was indicating the infiltration of these muscles, which increased after sustained upward gaze. Both lids was consistent with thyroid ophthalmopathy. Ptosis showed significant improvement after the intramuscular injection of 1,5 mg neostigmine methylsulphate, but the restriction of eye movements did not change. Repetitive stimulation of the right ulnar nerve-adductor digiti minimi muscle and facial nerve-nasalis muscle revealed no decrement. Acetylcholine receptor antibody level was found to be 1,30 nmol/L, which was more than two times higher than the normal levels (Normal values:0-0,50). Thorax computed tomography revealed a mass lesion of 6x3x2 cm located in the thymus zone. F ig .i s Primary position showing bilateral ptosis After two months of propylthiouracil treatment, the patient became euthyroid. Pyridostigmine 60 242 Ocular myasthenia gravis mg four times daily was added for the treatment frequency of OMG in AITD could be that these of ptosis. In spite of medication, exophthalmos disorders have a common genetic background and exophthalmic ophthalmoplegia were not (9). relieved, but ptosis responded to the treatment. After two sessions of plasmapheresis, the patient In three-quarters of patients with both conditions, was operated on. Total thyroidectomy and thyrotoxic symptoms occur before or extended thymectomy were performed. concurrently with those of myasthenia. Our Pathological examination was relevant with patient had thyrotoxicosis symptoms seven thymic hyperplasia. After the operation, the months before her ptosis developed (10). patient’s medication included 60 mg pyridostigmine four times daily and 0,15 mg The ocular changes in Graves' disease may levothyroxine sodium daily. Under this include exophthalmos, periorbital oedema, lid medication, her ophthalmoparesis continued, but lag, chemosis and ophthalmoplegia. the ptosis was fairly controlled. Exophthalmic ophthalmoplegia may be unilateral, but it is usually bilateral. The extraocular muscles most commonly involved DISCUSSION are the superior and lateral recti (11). In our patient, the downward movement of the right eye Myasthenia gravis (MG) is an autoimmune was restricted and the patient was complaining of disease characterized by impaired diplopia during downward and horizontal gazes. neuromuscular transmission due to circulating Her diplopia and ophthalmoparesis did not antiacetylcholine receptor autoantibodies The change after neostigmine injection, while her clinical expression of MG varies, ranging from a ptosis improved visibly. The MRI findings mild localized disease such as ocular MG (OMG) showing increased intensity and thickening of the to a severe generalized disease (GMG) (4). extraocular eye muscles were consistent with thyroid ophthalmopathy. Patients with MG may have evidence of coexisting autoimmune thyroid diseases (AITD), Two thirds of the patients with both disorders which include autoimmune thyroiditis and show improvement in myasthenia gravis after Graves’ disease (5). Epidemiological studies treatment for thyroid disease. In other patients, showed that AITD occur in approximately 5-10% however, the treatment of thyroid disease has no of MG patients, whereas a fairly low incidence of effect on myasthenic symptoms (1). The MG, (0.2%) has been reported in patients with treatment of hyperthyroidism has not improved AITD (6). The clinical presentation of MG the myasthenic symptoms in our patient, since associated with AITD is frequently restricted to her need for anticholinesterase did not decrease. the eye muscles. GMG was found to be more Some authors state that, thymectomy may have frequent in patients without thyroid disease. Also, positive effects on the clinical condition of both thymic abnormalities were less frequent in MG myasthenia (12) and ATID (13). Our patient did associated with AITD than in MG without thyroid not show an obvious improvement after autoimmunity. Although this is not true for the thymectomy. patient who had thymic hyperplasia, a greater frequency of thyroid antibodies in OMG, when In conclusion, MG associated with AITD has a compared to GMG, has been reported (4). mild clinical expression characterized by preferential involvement of the eye muscles. This The reason for the association of AITD with OMG is consistent with the hypothesis that OMG and is unknown, but several hypotheses may be GMG are separate diseases with different considered. First, OMG and GMG might actually spectra of associated diseases and different represent separate diseases (7) with different immunogenetic back-grounds. The association spectra of associated diseases. Second, an of OMG with AITD might also be due to common immunological
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