Clinical features of LRP4/agrin-antibody–positive myasthenia gravis: A multicenter study

Michael H. Rivner, Brandy M. Quarles, Jin Xiu Pan, Zheng Yu, James F. Howard, Andrea Corse, Mazen M. Dimachkie, Carlayne Jackson, Tuan Vu, George Small, Robert P. Lisak, Jerry Belsh, Ikjae Lee, Richard J. Nowak, Vanessa Baute, Stephen Scelsa, J. Americo Fernandes, Zachary Simmons, Andrea Swenson, Richard BarohnR. Bhavaraju Sanka, Clifton Gooch, Eroboghene Ubogu, James Caress, Mamatha Pasnoor, Hongyan Xu, Lin Mei

Research output: Contribution to journalArticle

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.

Original languageEnglish (US)
JournalMuscle and Nerve
DOIs
StateAccepted/In press - 2020

Keywords

  • LRP4
  • agrin
  • clinical features
  • myasthenia gravis
  • neuromuscular transmission disorders
  • seronegative myasthenia gravis

ASJC Scopus subject areas

  • Physiology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience
  • Physiology (medical)

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    Rivner, M. H., Quarles, B. M., Pan, J. X., Yu, Z., Howard, J. F., Corse, A., Dimachkie, M. M., Jackson, C., Vu, T., Small, G., Lisak, R. P., Belsh, J., Lee, I., Nowak, R. J., Baute, V., Scelsa, S., Fernandes, J. A., Simmons, Z., Swenson, A., ... Mei, L. (Accepted/In press). Clinical features of LRP4/agrin-antibody–positive myasthenia gravis: A multicenter study. Muscle and Nerve. https://doi.org/10.1002/mus.26985