Journal article
Clinical features ofLRP4/agrin-antibody-positive myasthenia gravis: A multicenter study
Muscle & nerve, Vol.62(3), pp.333-343
09/01/2020
DOI: 10.1002/mus.26985
PMCID: PMC7496236
PMID: 32483837
Abstract
Introduction Our aim in this study was to identify the prevalence and clinical characteristics of LRP4/agrin-antibody-positive double-seronegative myasthenia gravis (DNMG). Methods DNMG patients at 16 sites in the United States were tested for LRP4 and agrin antibodies, and the clinical data were collected. Results Of 181 DNMG patients, 27 (14.9%) were positive for either low-density lipoprotein receptor-related protein 4 (LRP4) or agrin antibodies. Twenty-three DNMG patients (12.7%) were positive for both antibodies. More antibody-positive patients presented with generalized symptoms (69%) compared with antibody-negative patients (43%) (P <= .02). Antibody-positive patients' maximum classification on the Myasthenia Gravis Foundation of America (MGFA) scale was significantly higher than that for antibody-negative patients (P <= .005). Seventy percent of antibody-positive patients were classified as MGFA class III, IV, or V compared with 39% of antibody-negative patients. Most LRP4- and agrin-antibody-positive patients (24 of 27, 89%) developed generalized myathenia gravis (MG), but with standard MG treatment 81.5% (22 of 27) improved to MGFA class I or II during a mean follow-up of 11 years. Discussion Antibody-positive patients had more severe clinical disease than antibody-negative patients. Most DNMG patients responded to standard therapy regardless of antibody status.
Details
- Title: Subtitle
- Clinical features ofLRP4/agrin-antibody-positive myasthenia gravis: A multicenter study
- Creators
- Michael H. Rivner - Prevention InstituteBrandy M. Quarles - Prevention InstituteJin-Xiu Pan - Case Western Reserve UniversityZheng Yu - Case Western Reserve UniversityJames F. Howard - University of North Carolina at Chapel HillAndrea Corse - Johns Hopkins UniversityMazen M. Dimachkie - University of KansasCarlayne Jackson - The University of Texas Health Science Center at San AntonioTuan Vu - University of South FloridaGeorge Small - Allegheny General HospitalRobert P. Lisak - Wayne State UniversityJerry Belsh - Rutgers, The State University of New JerseyIkjae Lee - University of Alabama at BirminghamRichard J. Nowak - Yale Univ, Dept Neurol, New Haven, CT USAVanessa Baute - Wake Forest UniversityStephen Scelsa - Yeshiva UniversityJ. Americo Fernandes - University of Nebraska Medical CenterZachary Simmons - Pennsylvania State UniversityAndrea Swenson - University of IowaRichard Barohn - University of KansasR. Bhavaraju Sanka - The University of Texas Health Science Center at San AntonioClifton Gooch - University of South FloridaEroboghene Ubogu - University of Alabama at BirminghamJames Caress - Wake Forest UniversityMamatha Pasnoor - University of KansasHongyan Xu - Prevention InstituteLin Mei - Case Western Reserve University
- Resource Type
- Journal article
- Publication Details
- Muscle & nerve, Vol.62(3), pp.333-343
- DOI
- 10.1002/mus.26985
- PMID
- 32483837
- PMCID
- PMC7496236
- NLM abbreviation
- Muscle Nerve
- ISSN
- 0148-639X
- eISSN
- 1097-4598
- Publisher
- Wiley
- Number of pages
- 11
- Grant note
- 5R01NS090083-06 / National Institutes of Health; United States Department of Health & Human Services; National Institutes of Health (NIH) - USA
- Language
- English
- Date published
- 09/01/2020
- Academic Unit
- Neurology
- Record Identifier
- 9984302206002771
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