Unusual association of amyotrophic lateral sclerosis and myasthenia gravis: A dysregulation of the adaptive immune system?

Maria del Mar Amador, Nadia Vandenberghe, Nawel Berhoune, Jean Philippe Camdessanché, Sophie Gronier, Emilien Delmont, Claude Desnuelle, Pascal Cintas, Sophie Pittion, Sarah Louis, Sophie Demeret, Timothée Lenglet, Vincent Meininger, François Salachas, Pierre François Pradat, Gaëlle Bruneteau

Research output: Contribution to journalArticlepeer-review

16 Citations (Scopus)


Myasthenia gravis is an autoimmune disorder affecting neuromuscular junctions that has been associated with a small increased risk of amyotrophic lateral sclerosis (ALS). Here, we describe a retrospective series of seven cases with a concomitant diagnosis of ALS and myasthenia gravis, collected among the 18 French reference centers for ALS in a twelve year period. After careful review, only six patients strictly met the diagnostic criteria for both ALS and myasthenia gravis. In these patients, limb onset of ALS was reported in five (83%) cases. Localization of myasthenia gravis initial symptoms was ocular in three (50%) cases, generalized in two (33%) and bulbar in one (17%). Median delay between onset of the two conditions was 19 months (6-319 months). Anti-acetylcholine receptor antibodies testing was positive in all cases. All patients were treated with riluzole and one had an associated immune-mediated disease. In the one last ALS case, the final diagnosis was false-positivity for anti-acetylcholine receptor antibodies. The co-occurrence of ALS and myasthenia gravis is rare and requires strict diagnostic criteria. Its demonstration needs thoughtful interpretation of electrophysiological results and exclusion of false positivity for myasthenia gravis antibody testing in some ALS cases. This association may be triggered by a dysfunction of adaptive immunity.

Original languageEnglish
Pages (from-to)342-346
Number of pages5
JournalNeuromuscular Disorders
Issue number6
Publication statusPublished (in print/issue) - 1 Jun 2016


  • Amyotrophic lateral sclerosis
  • Immunology
  • Myasthenia gravis
  • Neuromuscular transmission


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