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24.07.2019 | Original Communication

Clinical characteristics of autoimmune disorders in the central nervous system associated with myasthenia gravis

verfasst von: Kimitoshi Kimura, Yoichiro Okada, Chihiro Fujii, Kenichi Komatsu, Ryosuke Takahashi, Sadayuki Matsumoto, Takayuki Kondo

Erschienen in: Journal of Neurology | Ausgabe 11/2019

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Abstract

Myasthenia gravis (MG) is occasionally associated with autoimmune diseases in the central nervous system (CNS), such as neuromyelitis optica spectrum disorder (NMOSD), multiple sclerosis (MS), Morvan syndrome, and anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. Here, we report five original cases associated with autoimmune disorders in the CNS among 42 patients with MG in a single tertiary hospital in Japan (11.9%). In four of these five cases, the second disease developed when the preceding disease was unstable. Accurate diagnosis of the newly developing disease may be difficult in such cases, because some neurological symptoms can be seen in both disorders. This implies the great importance of recognizing the possible co-occurrence of MG and disorders in the CNS. In addition, a comprehensive review of the literature revealed distinct clinical characteristics depending on the associated disease in the CNS, including thymic pathology and temporal relationship between MG and associated CNS disorders. Notably, NMOSD usually develops after the onset of MG and thymectomy, in clear contrast to MS. Thymoma is highly prevalent among patients with Morvan syndrome, in contract to cases with NMOSD and MS. The analysis of clinical characteristics, representing the first such investigation to the best of our knowledge, suggests different pathogeneses of these autoimmune diseases in the CNS, and provides significant implications for clinical practice.

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Torres-Vega E, Mancheno N, Cebrian-Silla A, Herranz-Perez V, Chumillas MJ, Moris G, Joubert B, Honnorat J, Sevilla T, Vilchez JJ, Dalmau J, Graus F, Garcia-Verdugo JM, Bataller L (2017) Netrin-1 receptor antibodies in thymoma-associated neuromyotonia with myasthenia gravis. Neurology 88(13):1235–1242. https://doi.org/10.1212/WNL.0000000000003778 CrossRefPubMedPubMedCentral

Titel: Clinical characteristics of autoimmune disorders in the central nervous system associated with myasthenia gravis
verfasst von: Kimitoshi Kimura
Yoichiro Okada
Chihiro Fujii
Kenichi Komatsu
Ryosuke Takahashi
Sadayuki Matsumoto
Takayuki Kondo
Publikationsdatum: 24.07.2019
Verlag: Springer Berlin Heidelberg
Erschienen in: Journal of Neurology / Ausgabe 11/2019
Print ISSN: 0340-5354
Elektronische ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-019-09461-3

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