MG-QoL-15 scores in treated myasthenia gravis: Experience from a university hospital in India
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Background: Myasthenia gravis (MG) is an autoimmune disorder with a chronic fluctuating course. Quality of life (QoL) is affected by physical restrictions due to disease-related symptoms and effects of long-term treatment.Purpose: The purpose of this study was to assess QoL in a cohort of MG with stable disease course on optimal therapy.Materials and Methods: MG-QoL-15 was applied prospectively to 50 subjects of MG from India.Results: Mean MG-QoL-15 was 10.34 (standard deviation: 9.4; range: 0–32). Mean MG-QoL-15 scores for subjects with Myasthenia Gravis Foundation of America (MGFA) grades I, II, and III/IV were 3.54, 9.4, and 15.94, respectively. QoL scores correlated significantly with the MGFA grade. Age, gender, thymectomized status, thymoma, and steroid therapy did not affect QoL scores. All patients with MGFA grade I scored "0" or "1" in almost all items of MG-QoL-15. Seven and 11 patients with MGFA grades III/IV reported a significant affection (scores "3" or "4") due to "trouble using my eyes" and "plan around MG," respectively; and five subjects were "frustrated by MG." None of the subjects, irrespective of their MGFA grade, reported significant difficulty in getting around public spaces due to MG, or had "trouble in performing personal grooming." One subject each reported significant "trouble driving due to MG" or felt that "MG limits ability to enjoy hobbies and fun activities."Conclusion: This is the first study from India to assess QoL in MG using MG-QoL-15. Increased disease severity was reflected in worse QoL. MG-QoL-15 is a simple, quick, and user-friendly tool. Longitudinal changes in the QoL scores may be required to determine its utility in the Indian context.Keywords:
Affect
Thymectomy
Antibody titer
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To explore the clinical characteristics and outcome of myasthenia gravis with and without thymoma after operation.Two hundred and forty-three patients with myasthenia gravis surgically treated in our department from 1978 to 2003 were studied retrospectively. The clinical characteristics, complication, remission and survival were compared between myasthenia gravis with and without thymoma.The patients of myasthenia gravis with thymoma were significantly older (t = 6.138, P = 0.000), had shorter duration of symptom (t = 3.783, P = 0.000), and also had higher myasthenia crisis rates after operation (chi(2) = 64.77, P = 0.000) than those of myasthenia gravis without thymoma. No differences of Osserman classification was found between the two groups (chi(2) = 7.678, P = 0.104). The complete remission rates and partial remission rates of myasthenia gravis with thymoma were significantly lower than those of myasthenia gravis without thymoma at 1 and 3 years (P = 0.049, P = 0.000; P = 0.015, P = 0.010), but no differences at 5 year (P = 0.457; P = 0.699). The survival rates of MG with thymoma were lower than that of MG without thymoma (Log-rank = 18.58, P = 0.000).The clinical characteristics are different between myasthenia gravis with and without thymoma. The remission of symptom of myasthenia gravis with thymoma is worse than that of myasthenia gravis without thymoma in the near future, but is similar in the long future. The death rates of MG with thymoma is significantly higher than that of MG without thymoma.
Thymectomy
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Thymus Neoplasm
Thymectomy
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Antibodies to acetylcholine receptor were found in 3 of 11 patients with a thymoma removed by operation but without myasthenia gravis. Because masthenia gravis may appear after removal of the thymoma, detection of antiacetylcholine receptor antibodies may have predictive value.
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Thymus Neoplasm
Ocular myasthenia
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Thymectomy
Mediastinal tumor
Anterior mediastinum
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MUCH has been written concerning the relation of myasthenia gravis to the thymus. There are fewer articles, however, describing myasthenia gravis with thymoma, and fewer still on its relation to malignant thymoma. In 1942 only 5 such cases had been collected.1 Castleman and Norris2 stated that thymoma occurred in 10 to 15 per cent of cases of myasthenia gravis and that "if implantation connotes malignancy, then the thymoma may be carcinomatous in a very small percentage of cases (between 5–10%)." Most authors agree with this figure of the association of thymoma with myasthenia gravis3 4 5 6; in 1951 an estimated 130 . . .
Thymus Neoplasm
Thymectomy
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Ocular myasthenia
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Ryanodine receptor (RyR) antibodies are detected in about 50% of patients with myasthenia gravis who have a thymoma. The RyR is a calcium release channel involved in the mechanism of excitation-contraction coupling in striated muscle. In this study the severity of myasthenia gravis assessed by a five point disability score was compared between 12 patients with myasthenia gravis, a thymoma, and RyR antibodies and 10 patients with myasthenia gravis and a thymoma but without such antibodies. Symptoms of myasthenia gravis were significantly more severe in patients with RyR antibodies. The mean (SD) disability scores were 3.7(0.5) in patients with antibodies and 2.7 (0.9) in those without at peak of illness, (p = 0.01) and 3.4(1.4) v 1.6(0.7) at the end of an average observation period of five years (p = 0.002). The number of deaths due to myasthenia gravis was five of 12 RyR antibody positive patients, and none of 10 RyR antibody negative patients (p = 0.04). RyR antibody levels correlated positively with severity of myasthenia gravis. The presence of circulating RyR antibodies seems to be associated with a severe form of thymoma associated myasthenia gravis.
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Myasthenia gravis is a disease that affects the neuromuscular junction causing abnormal weakness,
fatigue, and muscle decay. Loss or dysfunction of acetylcholine receptors occurs mostly due to the
presence of antibodies to nicotinic acetylcholine receptors (NAChR). The thymus and thymoma
have a special role in the pathophysiology of myasthenia gravis due to the role of the thymus in
the maturation and distribution of lymphocytes where cross-reactivity and consequent
autoimmunity to NAChR can occur. The most commonly used diagnostic method for myasthenia
gravis is the measurement of antibody levels to NAChR which is a highly specific test for the
diagnosis of myasthenia gravis. CT is the method of choice in the diagnosis of thymoma. Due to
the importance of the thymus in the pathophysiology of myasthenia gravis the disease can be
treated surgically, by removing the thymus. If there is thymoma present, to plan the operation,
Masaoka and TNM classification are used. Methods that can be used to remove the thymus /
thymoma are transsternal thymectomy, transcervical thymectomy, video-assisted thoracoscopic
surgery (VATS) and robotic surgery. Some of the factors influencing the prognosis after
thymectomy are age and sex, presence of thymoma, surgical technique, extent of resection and
severity and duration of symptoms of myasthenia gravis. New methods of thymectomy are
minimally invasive and with rapid recovery, and most patients show remission of the disease after
removal of thymus.
Thymectomy
Pathophysiology
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