{"doi":"10.1002/mus.26985","title":"Clinical features of <scp>LRP4</scp>/agrin‐antibody–positive myasthenia gravis: A multicenter study","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.","journal":"Muscle & Nerve","year":2020,"id":55939,"datarank":0.0,"base_score":0.0,"endowment":0.0,"self_citation_contribution":0.0,"citation_network_contribution":0.0,"self_endowment_contribution":0.0,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":89,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.9534,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2020-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":290618,"name":"Brandy Quarles","orcid":null,"position":1,"is_corresponding":false},{"id":289855,"name":"Jin‐Xiu Pan","orcid":"0000-0003-4013-1390","position":2,"is_corresponding":false},{"id":289856,"name":"Yu Zheng","orcid":"0000-0003-2317-6511","position":3,"is_corresponding":false},{"id":270885,"name":"James F. 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Ubogu","orcid":"0000-0002-8307-9994","position":22,"is_corresponding":false},{"id":226508,"name":"James B. Caress","orcid":"0000-0002-5814-6083","position":23,"is_corresponding":false},{"id":289861,"name":"Mamatha Pasnoor","orcid":"0000-0001-7424-4719","position":24,"is_corresponding":false},{"id":289862,"name":"Hongyan Xu","orcid":"0000-0001-7291-0458","position":25,"is_corresponding":false},{"id":289863,"name":"Lin Mei","orcid":"0000-0001-5772-1229","position":26,"is_corresponding":false},{"id":262662,"name":"Michael H. Rivner","orcid":"0000-0002-6135-035X","position":0,"is_corresponding":true}],"reference_count":43,"raw_metadata":null,"created_at":"2026-07-18T21:05:29.672878Z","pmid":"32483837","pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}