Acquired neuromyotonia in thymoma-associated myasthenia gravis: a clinical and serological study: European Journal of Neurology

M Gastaldi, A De Rosa, M Maestri, E Zardini, S Scaranzin, M Guida, P Borrelli, OE Ferraro, V Lampasona, R Furlan, SR Irani, P Waters, B Lang, A Vincent, E Marchioni, R Ricciardi, D Franciotta

Research output: Contribution to journalArticle

Abstract

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 
Original languageEnglish
Pages (from-to)992-999
Number of pages8
JournalEur. J. Neurol.
Volume26
Issue number7
DOIs
Publication statusPublished - 2019

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    Gastaldi, M., De Rosa, A., Maestri, M., Zardini, E., Scaranzin, S., Guida, M., Borrelli, P., Ferraro, OE., Lampasona, V., Furlan, R., Irani, SR., Waters, P., Lang, B., Vincent, A., Marchioni, E., Ricciardi, R., & Franciotta, D. (2019). Acquired neuromyotonia in thymoma-associated myasthenia gravis: a clinical and serological study: European Journal of Neurology. Eur. J. Neurol., 26(7), 992-999. https://doi.org/10.1111/ene.13922