Autoantibodies in thymona-associated myasthenia gravis with myositis or neuromyotonia

Åse Mygland, Angela Vincent, John Newsom-Davis, Henry Kaminski, Francesco Zorzato, Mark Agius, Nils E. Gilhus, Johan A. Aarli

Research output: Contribution to journalArticle

85 Citations (Scopus)

Abstract

Background: About 50% of patients with thymoma have paraneoplastic myasthenia gravis (MG). Myositis and myocarditis or neuromyotonia (NMT) will also develop in some. Patients with thymoma-associated MG produce autoantibodies to a variety of neuromuscular antigens, particularly acetylcholine receptor (AChR), titin, skeletal muscle calcium release channel (ryanodine receptor [RyR]), and voltage-gated potassium channels (VGKC). Objective: To examine whether neuromuscular autoantibodies in patients with thymoma correlate with specific clinical syndromes. Methods: Serum and plasma samples from 19 patients with thymoma-associated MG, of whom 5 had myositis and 6 had NMT, underwent testing for antibodies to AChR, titin, RyR, and VGKC. Results: Antibodies to AChR and titin were found in 19 and 17 patients, respectively. Antibodies to RyR correlated with the presence of myositis (P = .03); they were found in all 5 patients with myositis and in only 1 patient with NMT, but also in 4 of 8 patients with neither disease. Antibodies to VGKC were found in 4 patients with NMT, 1 of 3 patients undergoing testing for myositis, and 2 of 7 patients undergoing testing with neither comorbidity. Presence of RyR antibodies correlated with high levels of titin antibodies. Conclusions: The results appear to distinguish partially between 3 groups of patients with thymoma-associated MG: the first with RyR antibodies and myositis or myocarditis, the second with NMT without RyR antibodies, and the third without RyR antibodies, myositis, or NMT. Differences in the thymoma may underlie these pathologic associations.

Original languageEnglish (US)
Pages (from-to)527-531
Number of pages5
JournalArchives of Neurology
Volume57
Issue number4
StatePublished - Apr 2000
Externally publishedYes

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Isaacs Syndrome
Myositis
Myasthenia Gravis
Autoantibodies
Ryanodine Receptor Calcium Release Channel
Thymoma
Connectin
Antibodies
Cholinergic Receptors
Myocarditis
Voltage-Gated Potassium Channels

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Mygland, Å., Vincent, A., Newsom-Davis, J., Kaminski, H., Zorzato, F., Agius, M., ... Aarli, J. A. (2000). Autoantibodies in thymona-associated myasthenia gravis with myositis or neuromyotonia. Archives of Neurology, 57(4), 527-531.

Autoantibodies in thymona-associated myasthenia gravis with myositis or neuromyotonia. / Mygland, Åse; Vincent, Angela; Newsom-Davis, John; Kaminski, Henry; Zorzato, Francesco; Agius, Mark; Gilhus, Nils E.; Aarli, Johan A.

In: Archives of Neurology, Vol. 57, No. 4, 04.2000, p. 527-531.

Research output: Contribution to journalArticle

Mygland, Å, Vincent, A, Newsom-Davis, J, Kaminski, H, Zorzato, F, Agius, M, Gilhus, NE & Aarli, JA 2000, 'Autoantibodies in thymona-associated myasthenia gravis with myositis or neuromyotonia', Archives of Neurology, vol. 57, no. 4, pp. 527-531.
Mygland Å, Vincent A, Newsom-Davis J, Kaminski H, Zorzato F, Agius M et al. Autoantibodies in thymona-associated myasthenia gravis with myositis or neuromyotonia. Archives of Neurology. 2000 Apr;57(4):527-531.
Mygland, Åse ; Vincent, Angela ; Newsom-Davis, John ; Kaminski, Henry ; Zorzato, Francesco ; Agius, Mark ; Gilhus, Nils E. ; Aarli, Johan A. / Autoantibodies in thymona-associated myasthenia gravis with myositis or neuromyotonia. In: Archives of Neurology. 2000 ; Vol. 57, No. 4. pp. 527-531.
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AU - Zorzato, Francesco

AU - Agius, Mark

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N2 - Background: About 50% of patients with thymoma have paraneoplastic myasthenia gravis (MG). Myositis and myocarditis or neuromyotonia (NMT) will also develop in some. Patients with thymoma-associated MG produce autoantibodies to a variety of neuromuscular antigens, particularly acetylcholine receptor (AChR), titin, skeletal muscle calcium release channel (ryanodine receptor [RyR]), and voltage-gated potassium channels (VGKC). Objective: To examine whether neuromuscular autoantibodies in patients with thymoma correlate with specific clinical syndromes. Methods: Serum and plasma samples from 19 patients with thymoma-associated MG, of whom 5 had myositis and 6 had NMT, underwent testing for antibodies to AChR, titin, RyR, and VGKC. Results: Antibodies to AChR and titin were found in 19 and 17 patients, respectively. Antibodies to RyR correlated with the presence of myositis (P = .03); they were found in all 5 patients with myositis and in only 1 patient with NMT, but also in 4 of 8 patients with neither disease. Antibodies to VGKC were found in 4 patients with NMT, 1 of 3 patients undergoing testing for myositis, and 2 of 7 patients undergoing testing with neither comorbidity. Presence of RyR antibodies correlated with high levels of titin antibodies. Conclusions: The results appear to distinguish partially between 3 groups of patients with thymoma-associated MG: the first with RyR antibodies and myositis or myocarditis, the second with NMT without RyR antibodies, and the third without RyR antibodies, myositis, or NMT. Differences in the thymoma may underlie these pathologic associations.

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