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Status dystonicus in adult patients with anti-N-methyl-D-1 aspartate-acid receptor encephalitis
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Zhang, Yan, Cui, Lili, Chen, Weibi, Huang, Huijin, Liu, Gang, Su, Yingying and Boltze, Johannes (2023) Status dystonicus in adult patients with anti-N-methyl-D-1 aspartate-acid receptor encephalitis. Journal of Neurology, 270 . pp. 2693-2701. doi:10.1007/s00415-023-11599-0 ISSN 0340-5354.
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WRAP-Status-dystonicus-adult-patients-anti-N-methyl-d-aspartate-acid-receptor-23.pdf - Accepted Version Embargoed item. Restricted access to Repository staff only until 22 February 2024. Contact author directly, specifying your specific needs. - Requires a PDF viewer. Download (907Kb) |
Official URL: https://doi.org/10.1007/s00415-023-11599-0
Abstract
Objective
Status dystonicus (SD) is a severe movement disorder (MD) and has rarely been recognized in anti-N-methyl-D-aspartate-acid receptor (NMDAR) encephalitis, particularly in adult patients. We aim to explore the clinical characteristics and outcome of SD in anti-NMDAR encephalitis.
Methods
Patients with anti-NMDAR encephalitis admitted to Xuanwu Hospital from July 2013 to December 2019 were prospectively enrolled. SD was diagnosed based on the patients’ clinical manifestations and video EEG monitoring. Outcome was evaluated 6 and 12 months after enrollment using the modified Ranking Scale (mRS).
Results
A total of 172 patients with anti-NMDAR encephalitis, including 95 males (55.2%) and 77 females (44.8%) with a median age of 26 years (interquartile range 19 to 34) were enrolled. Eighty patients (46.5%) presented with movement disorder (MD), 14 of whom suffered from SD, which manifested as chorea (14/14, 100%), orofacial dyskinesia (12/14, 85.7%), generalized dystonia (8/14, 57.1%), tremor (8/14, 57.1%), stereotypies (5/14, 35.7%), and catatonia (1/14, 7.1%) of the trunk and limbs. All SD patients exhibited disturbed consciousness and central hypoventilation, requiring intensive care. SD patients also had high cerebrospinal fluid NMDAR antibody titers, a higher proportion of ovarian teratoma, higher mRS scores upon enrollment, longer duration to recover, and poorer outcomes at 6 (P < 0.05) but not at 12 months as compared to non-SD patients.
Conclusion
SD is not uncommon in anti-NMDAR encephalitis patients and relates to the severity and worse short-term outcome of the anti-NMDAR encephalitis. Early recognition of SD and timely treatment is important to shorten the time needed for recuperation.
Item Type: | Journal Article | ||||||||
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Subjects: | R Medicine > RC Internal medicine | ||||||||
Divisions: | Faculty of Science, Engineering and Medicine > Science > Life Sciences (2010- ) | ||||||||
Library of Congress Subject Headings (LCSH): | Movement disorders , Neurological emergencies , Encephalitis , Movement disorders -- Treatment | ||||||||
Journal or Publication Title: | Journal of Neurology | ||||||||
Publisher: | Springer Medizin | ||||||||
ISSN: | 0340-5354 | ||||||||
Official Date: | May 2023 | ||||||||
Dates: |
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Volume: | 270 | ||||||||
Page Range: | pp. 2693-2701 | ||||||||
DOI: | 10.1007/s00415-023-11599-0 | ||||||||
Status: | Peer Reviewed | ||||||||
Publication Status: | Published | ||||||||
Reuse Statement (publisher, data, author rights): | “This version of the article has been accepted for publication, after peer review (when applicable) but is not the Version of Record and does not reflect post-acceptance improvements, or any corrections. The Version of Record is available online at: https://doi.org/10.1007/s00415-023-11599-0 Use of this Accepted Version is subject to the publisher’s Accepted Manuscript terms of use https://www.springernature.com/gp/open-research/policies/acceptedmanuscript-terms”. | ||||||||
Access rights to Published version: | Restricted or Subscription Access | ||||||||
Date of first compliant deposit: | 22 February 2023 | ||||||||
RIOXX Funder/Project Grant: |
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