Acquired neuromyotonia in thymoma-associated myasthenia gravis: a clinical and serological study

被引：19

作者：

Gastaldi, M. ^{[1
]}

De Rosa, A. ^{[2
]}

Maestri, M. ^{[2
]}

Zardini, E. ^{[1
]}

Scaranzin, S. ^{[1
]}

Guida, M. ^{[2
]}

Borrelli, P. ^{[3
]}

Ferraro, O. E. ^{[3
]}

Lampasona, V. ^{[4
]}

Furlan, R. ^{[5
]}

Irani, S. R. ^{[6
]}

Waters, P. ^{[6
]}

Lang, B. ^{[6
]}

Vincent, A. ^{[7
]}

Marchioni, E. ^{[8
]}

Ricciardi, R. ^{[2
]}

Franciotta, D. ^{[1
]}

机构：

[1] IRCCS Mondino Fdn, Neuroimmunol Lab, Pavia, Italy

[2] Univ Pisa, Dept Clin & Expt Med, Neurol Unit, Pisa, Italy

[3] Univ Pavia, Unit Biostat & Clin Epidemiol, Pavia, Italy

[4] Ist Sci San Raffaele, Genom Unit Diag Human Pathol, Div Genet & Cell Biol, Milan, Italy

[5] Ist Sci San Raffaele, Div Neurosci, INSPE, Milan, Italy

[6] Univ Oxford, Nuffield Dept Clin Neurosci, Oxford Autoimmune Neurol Grp, Oxford, England

[7] Univ Oxford, Nuffield Dept Clin Neurosci, Oxford, England

[8] IRCCS Mondino Fdn, Neuroncol & Neuroinflammat Unit, Pavia, Italy

来源：

EUROPEAN JOURNAL OF NEUROLOGY | 2019年 / 26卷 / 07期

关键词：

autoantibodies; DCC antibodies; myasthenia gravis; neuromyotonia; paraneoplastic neurological syndrome; thymoma; UNC5A antibodies; LIMBIC ENCEPHALITIS; RECEPTOR ANTIBODIES; K+ CHANNELS; AUTOANTIBODIES; CLASSIFICATION; MANAGEMENT; SURVIVAL; CASPR2; TUMORS; DCC;

D O I：

10.1111/ene.13922

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Background and purpose Acquired neuromyotonia can occur in patients with thymoma, alone or in association with myasthenia gravis (MG), but the clinical prognostic significance of such comorbidity is largely unknown. The clinico-pathological features were investigated along with the occurrence of neuromyotonia as predictors of tumour recurrence in patients with thymoma-associated myasthenia. Methods A total number of 268 patients with thymomatous MG were studied retrospectively. Patients with symptoms of spontaneous muscle overactivity were selected for autoantibody testing using immunohistology for neuronal cell-surface proteins and cell-based assays for contactin-associated protein 2 (CASPR2), leucine-rich glioma inactivated 1 (LGI1), glycine receptor and Netrin-1 receptor antibodies. Neuromyotonia was diagnosed according to the presence of typical electromyography abnormalities and/or autoantibodies against LGI1/CASPR2. Results Overall, 33/268 (12%) MG patients had a thymoma recurrence. Five/268 (2%) had neuromyotonia, four with typical autoantibodies, including LGI1 (n = 1), CASPR2 (n = 1) or both (n = 2). Three patients had Netrin-1 receptor antibodies, two with neuromyotonia and concomitant CASPR2+LGI1 antibodies and one with spontaneous muscle overactivity without electromyography evidence of neuromyotonia. Thymoma recurrence was more frequent in those with (4/5, 80%) than in those without (28/263, 10%, P < 0.001) neuromyotonia. Neuromyotonia preceded the recurrence in 4/5 patients. In univariate analysis, predictors of thymoma recurrence were age at thymectomy [odds ratio (OR) 0.95, 95% confidence interval (CI) 0.93-0.97], Masaoka stage >= IIb (OR 10.73, 95% CI 2.38-48.36) and neuromyotonia (OR 41.78, 95% CI 4.71-370.58). Conclusions De novo occurrence of neuromyotonia in MG patients with previous thymomas is a rare event and may herald tumour recurrence. Neuronal autoantibodies can be helpful to assess the diagnosis. These observations provide pragmatic risk stratification for tumour vigilance in patients with thymomatous MG.

引用

页码：992 / 999

页数：8

共 31 条

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