Seronegative myasthenic crisis: a multicenter analysis

Mergenthaler P, Stetefeld HR, Dohmen C, Kohler S, Schoenenberger S, Boesel J, Gerner S, Huttner H, Schneider H, Reichmann H, Fuhrer H, Berger B, Zinke J, Alberty A, Kleiter I, Schneider-Gold C, Roth C, Dunkel J, Steinbrecher A, Thieme A, Lee DH, Linker RA, Angstwurm K, Meisel A, Neumann B (2022)

Publication Type: Journal article

Publication year: 2022

Journal

Journal of Neurology Springer Verlag (Germany)

DOI: 10.1007/s00415-022-11023-z

Abstract

Myasthenic crisis (MC) is a life-threatening condition for patients with myasthenia gravis (MG). Seronegative patients represent around 10–15% of MG, but data on outcome of seronegative MCs are lacking. We performed a subgroup analysis of patients who presented with MC with either acetylcholine-receptor-antibody-positive MG (AChR-MG) or seronegative MG between 2006 and 2015 in a retrospective German multicenter study. We identified 15 seronegative MG patients with 17 MCs and 142 AChR-MG with 159 MCs. Seronegative MCs were younger (54.3 ± 14.5 vs 66.5 ± 16.3 years; p = 0.0037), had a higher rate of thymus hyperplasia (29.4% vs 3.1%; p = 0.0009), and were more likely to be female (58.8% vs 37.7%; p = 0.12) compared to AChR-MCs. Time between diagnosis of MG and MC was significantly longer in seronegative patients (8.2 ± 7.6 vs 3.1 ± 4.4 years; p < 0.0001). We found no differences in duration of mechanical ventilation (16.2 ± 15.8 vs 16.5 ± 15.9 days; p = 0.94) and length of stay at intensive care unit (17.6 ± 15.2 vs 17.8 ± 15.4 days; p = 0.96), or in-hospital mortality (11.8% vs. 10.1%; p = 0.69). We conclude that MC in seronegative MG affects younger patients after a longer period of disease, but that crisis treatment efficacy and outcome do not differ compared to AChR-MCs.

Authors with CRIS profile

Stefan Gerner Department of Psychiatry and Psychotherapy Hagen Huttner Medizinische Fakultät

Involved external institutions

Universität zu Köln DE Germany (DE) HELIOS Kliniken DE Germany (DE) Universität Regensburg DE Germany (DE) Charité - Universitätsmedizin Berlin DE Germany (DE) Universitätsklinikum Heidelberg DE Germany (DE) Technische Universität Dresden DE Germany (DE) Albert-Ludwigs-Universität Freiburg DE Germany (DE) Universitätsklinikum Jena DE Germany (DE) Kliniken Maria Hilf GmbH DE Germany (DE) Ruhr-Universität Bochum (RUB) DE Germany (DE) DRK-Kliniken Nordhessen DE Germany (DE)

How to cite

APA:

Mergenthaler, P., Stetefeld, H.R., Dohmen, C., Kohler, S., Schoenenberger, S., Boesel, J.,... Neumann, B. (2022). Seronegative myasthenic crisis: a multicenter analysis. Journal of Neurology. https://doi.org/10.1007/s00415-022-11023-z

MLA:

Mergenthaler, Philipp, et al. "Seronegative myasthenic crisis: a multicenter analysis." Journal of Neurology (2022).

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