Konig, Nicole, Stetefeld, Henning R., Dohmen, Christian, Mergenthaler, Philipp, Kohler, Siegfried, Schonenberger, Silvia, Bosel, Julian, Lee, De-Hyung, Gerner, Stefan T. ORCID: 0000-0001-6020-8290, Huttner, Hagen B., Schneider, Hauke ORCID: 0000-0002-9641-0922, Reichmann, Heinz, Fuhrer, Hannah, Berger, Benjamin, Zinke, Jan, Alberty, Anke, Kleiter, Ingo, Schneider-Gold, Christiane, Roth, Christian, Dunkel, Juliane, Steinbrecher, Andreas, Thieme, Andrea, Schlachetzki, Felix, Linker, Ralf A., Angstwurm, Klemens, Meisel, Andreas and Neumann, Bernhard (2021). MuSK-antibodies are associated with worse outcome in myasthenic crisis requiring mechanical ventilation. J. Neurol., 268 (12). S. 4824 - 4834. 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). Muscle-specific kinase-antibodies (MuSK-ABs) are detected in similar to 6% of MG, but data on outcome of MuSK-MCs are still lacking. We made a subgroup analysis of patients who presented with MC with either acetylcholine-receptor-antibody positive MG (AchR-MG) or MuSK-MG between 2006 and 2015 in a retrospective German multicenter study. We identified 19 MuSK-AB associated MCs in 15 patients and 161 MCs in 144 patients with AchR-ABs only. In contrast to patients with AchR-AB, MuSK-AB patients were more often female (p = 0.05, OR = 2.74) and classified as Myasthenia Gravis Foundation of America-class IV before crisis (p = 0.04, OR = 3.25). MuSK-AB patients suffer more often from multiple chronic disease (p = 0.016, OR = 4.87) and were treated more invasively in terms of plasma exchanging therapies (not significant). The number of days of mechanical ventilation (MV) (43.0 +/- 53.1 vs. 17.4 +/- 18; p < 0.0001), days on an intensive care unit (ICU) (45.3 +/- 49.5 vs. 21.2 +/- 19.7; p < 0.0001), and hospital-length of stay (LOS) (55.9 +/- 47.6 vs. 28.8 +/- 20.9 days; p < 0.0001) were significantly increased in MuSK-MC. Remarkable is that these changes were mainly due to patients with MusK-ABs only, whereas patients' outcome with both antibodies was similar to AchR-MCs. Furthermore, our data showed a shortened duration of MV after treatment with plasma exchanging therapies compared to treatment with intravenous immunoglobulin in MuSK-MCs. We conclude that MuSK-AB-status is associated with a longer need of MV, ICU-LOS, and hospital-LOS in MC, and therefore recommend early initiation of a disease-specific therapy.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Konig, NicoleUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Stetefeld, Henning R.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Dohmen, ChristianUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Mergenthaler, PhilippUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kohler, SiegfriedUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schonenberger, SilviaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Bosel, JulianUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Lee, De-HyungUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gerner, Stefan T.UNSPECIFIEDorcid.org/0000-0001-6020-8290UNSPECIFIED
Huttner, Hagen B.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schneider, HaukeUNSPECIFIEDorcid.org/0000-0002-9641-0922UNSPECIFIED
Reichmann, HeinzUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Fuhrer, HannahUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Berger, BenjaminUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Zinke, JanUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Alberty, AnkeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kleiter, IngoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schneider-Gold, ChristianeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Roth, ChristianUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Dunkel, JulianeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Steinbrecher, AndreasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Thieme, AndreaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schlachetzki, FelixUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Linker, Ralf A.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Angstwurm, KlemensUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Meisel, AndreasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Neumann, BernhardUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-603829
DOI: 10.1007/s00415-021-10603-9
Journal or Publication Title: J. Neurol.
Volume: 268
Number: 12
Page Range: S. 4824 - 4834
Date: 2021
Publisher: SPRINGER HEIDELBERG
Place of Publication: HEIDELBERG
ISSN: 1432-1459
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
GRAVIS; AUTOANTIBODIES; MORTALITYMultiple languages
Clinical NeurologyMultiple languages
URI: http://kups.ub.uni-koeln.de/id/eprint/60382

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