The Severity of Myasthenia Gravis Correlates With the Serum Concentration of Titin and Ryanodine Receptor Antibodies

Romi, Fredrik; Skeie, Geir; Aarli, Johan A.; Gilhus, Nils Erik

doi:10.1001/archneur.57.11.1596

Cited by 95 publications

(84 citation statements)

References 34 publications

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“…In our opinion, the age at MG onset is a more important factor than the age at thymectomy [7, 23, 32]. …”

Section: Discussionmentioning

confidence: 99%

“…While the concentration of AChR antibodies is highest in early-onset MG [7], the occurrence of titin and RyR antibodies is highest in late-onset MG [23, 33, 34]and is accompanied by a more severe disease in late-onset MG [22, 23, 35, 36]. Histologically, thymic hyperplasia is seen in thymuses from early-onset MG patients, while thymuses from late-onset MG patients are atrophic [28, 37, 38, 39].…”

Section: Discussionmentioning

confidence: 99%

“…Titin antibodies were detected by ELISA using purified titin fragment MGT-30 as the antigen [22, 23]. …”

Section: Methodsmentioning

confidence: 99%

“…RyR antibodies were also detected by ELISA using pc2 Ry1 fusion protein as the antigen [23, 24]. RyR antibodies were also confirmed by Western blot using methods previously described [24, 25].…”

Section: Methodsmentioning

confidence: 99%

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Thymectomy in Nonthymoma Early-Onset Myasthenia gravis in Correlation with Disease Severity and Muscle Autoantibodies

Romi

Gilhus

Varhaug³

et al. 2003

Eur Neurol

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Objective: To study the clinical effect of thymectomy in a well-defined early-onset MG subgroup and to correlate it to MG severity, the presence of circulating muscle autoantibodies, and the need for pharmacological treatment in a long-term setting. Methods: Fifty-two consecutive AChR antibody-positive early-onset MG patients (34 thymectomized and 18 nonthymectomized) were included. Severity was assessed and the pharmacological treatment monitored on a yearly basis, starting from the year of MG onset, for 5, 10, 15, and 20 consecutive years; AChR, titin, and RyR antibodies were assayed. Results: In the four follow-up groups, MG severity was significantly higher in nonthymectomized compared to thymectomized MG patients. The postthymectomy MG improvement was significant and persistent. There were 21/34 remissions in thymectomized patients and only 4/18 in the nonthymectomized group. Patients with initially high or low AChR antibody concentration had a similar thymectomy outcome. Only 6 patients had titin antibodies, and none had RyR antibodies. Conclusion: The present study indicates a benefit of thymectomy in early-onset MG. The muscle autoantibody concentration does not influence the outcome of thymectomy in early-onset MG.

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“…In our opinion, the age at MG onset is a more important factor than the age at thymectomy [7, 23, 32]. …”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

“…Titin antibodies were detected by ELISA using purified titin fragment MGT-30 as the antigen [22, 23]. …”

Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

See 2 more Smart Citations

Thymectomy in Nonthymoma Early-Onset Myasthenia gravis in Correlation with Disease Severity and Muscle Autoantibodies

Romi

Gilhus

Varhaug³

et al. 2003

Eur Neurol

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“…Whatever the initiating events, they lead to dysregulated T cell activity and subsequent autoantibody production [10] . Polymorphisms in some immune-related and nonimmune-related genes are associated with increased susceptibility to MG.…”

Section: Risk Factorsmentioning

confidence: 99%

Intercommunication between the Neuroendocrine and Immune Systems: Focus on Myasthenia Gravis

Mays

Butts

2011

Neuroimmunomodulation

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Crosstalk exists between the nervous, endocrine, and immune systems, and perturbations in these interactions have been associated with disease. This includes production of neuroendocrine factors that alter immune system activity and increase susceptibility to or severity of immune-related conditions, such as myasthenia gravis (MG) – a T-cell-dependent, B-cell-mediated autoimmune disorder. MG results from impairment of transmission to the neuromuscular junction and involves the thymus – especially in early-onset disease, but the exact mechanism by which the thymus impacts disease is unclear. MG afflicts millions of individuals worldwide each year, and both men and women can develop symptoms. However, prevalence and age of onset differs between men and women. Women exhibit higher incidence and earlier age of onset compared to men, and disease fluctuates during pregnancy. This suggests that sex hormones play a role in influencing disease outcome. In this review, we will consider what is known about the manifestation of MG, theories on how different forms of MG are influenced or alleviated by steroid hormones, current treatment options, and what measures could be important to consider in the future.

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Striational Antibodies in Myasthenia Gravis

Romi¹,

Skeie²,

Gilhus³

et al. 2005

Arch Neurol

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140

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yasthenia gravis is an autoimmune disease caused, in most cases, by antibodies attaching to the acetylcholine receptor. Some myasthenia gravis patients have antibodies that bind in a cross-striational pattern to skeletal and heart muscle tissue sections (striational antibodies). These antibodies react with epitopes on the muscle proteins titin and ryanodine receptor, are found mainly in sera of patients with thymoma and lateonset myasthenia gravis, and may correlate with myasthenia gravis severity. Their presence may predict an unsatisfactory outcome after thymectomy. The detection of titin and ryanodine receptor antibodies provides more specific clinical information than the immunofluorescent demonstration of striational antibodies.

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The Severity of Myasthenia Gravis Correlates With the Serum Concentration of Titin and Ryanodine Receptor Antibodies

Cited by 95 publications

References 34 publications

Thymectomy in Nonthymoma Early-Onset Myasthenia gravis in Correlation with Disease Severity and Muscle Autoantibodies

Thymectomy in Nonthymoma Early-Onset Myasthenia gravis in Correlation with Disease Severity and Muscle Autoantibodies

Intercommunication between the Neuroendocrine and Immune Systems: Focus on Myasthenia Gravis

Striational Antibodies in Myasthenia Gravis

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