Mergenthaler, Philipp, Stetefeld, Henning R., Dohmen, Christian, Kohler, Siegfried, Schoenenberger, Silvia, Boesel, Julian, Gerner, Stefan T., Huttner, Hagen B., Schneider, Hauke ORCID: 0000-0002-9641-0922, Reichmann, Heinz, Fuhrer, Hannah, Berger, Benjamin ORCID: 0000-0003-2654-2898, Zinke, Jan, Alberty, Anke, Kleiter, Ingo ORCID: 0000-0002-8249-4408, Schneider-Gold, Christiane, Roth, Christian, Dunkel, Juliane, Steinbrecher, Andreas, Thieme, Andrea, Lee, De-Hyung, Linker, Ralf A., Angstwurm, Klemens, Meisel, Andreas ORCID: 0000-0001-7233-5342 and Neumann, Bernhard (2022). Seronegative myasthenic crisis: a multicenter analysis. J. Neurol., 269 (7). S. 3904 - 3912. HEIDELBERG: SPRINGER HEIDELBERG. ISSN 1432-1459

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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.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Mergenthaler, PhilippUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Stetefeld, Henning R.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Dohmen, ChristianUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kohler, SiegfriedUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schoenenberger, SilviaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Boesel, JulianUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gerner, Stefan T.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Huttner, Hagen B.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schneider, HaukeUNSPECIFIEDorcid.org/0000-0002-9641-0922UNSPECIFIED
Reichmann, HeinzUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Fuhrer, HannahUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Berger, BenjaminUNSPECIFIEDorcid.org/0000-0003-2654-2898UNSPECIFIED
Zinke, JanUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Alberty, AnkeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kleiter, IngoUNSPECIFIEDorcid.org/0000-0002-8249-4408UNSPECIFIED
Schneider-Gold, ChristianeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Roth, ChristianUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Dunkel, JulianeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Steinbrecher, AndreasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Thieme, AndreaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Lee, De-HyungUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Linker, Ralf A.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Angstwurm, KlemensUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Meisel, AndreasUNSPECIFIEDorcid.org/0000-0001-7233-5342UNSPECIFIED
Neumann, BernhardUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-695666
DOI: 10.1007/s00415-022-11023-z
Journal or Publication Title: J. Neurol.
Volume: 269
Number: 7
Page Range: S. 3904 - 3912
Date: 2022
Publisher: SPRINGER HEIDELBERG
Place of Publication: HEIDELBERG
ISSN: 1432-1459
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
GRAVIS; MORTALITY; ANTIBODYMultiple languages
Clinical NeurologyMultiple languages
URI: http://kups.ub.uni-koeln.de/id/eprint/69566

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