Milone reports receiving research funding from a Mayo Medical center benefactor and a discretionary fund from the Department of Neurology, Mayo Medical center for unrelated research projects, and an honorarium to serve as associated editor of Neurology Genetics. 0.003 ?Ptosis6 (25)0 0.003 ?Neck flexor weakness8 (33)24 (63) 0.002 ?Neck extensor weakness2 (9)8 (21)0.19?Hamstrings weaker than quadriceps6 (25)15 (40)0.2Myocarditis, (%)8 (33)0 0.001 HyperCKemia, (%)15 (68)a37 (100)a,b 0.001 ?CK 1000?IU/l (%)10 (45)c37 (100) 0.007 ?Peak CK, median (range) IU/l686 (28C7279)6456 (1151C29 000) 0.001 Aldolase elevation, (%)7/11 (64)16/16 (100) 0.009 ?With normal SAR407899 HCl CK, (%)2/7 (29)d0HMGCR-IgG positive, (%)0/1320/38 SAR407899 HCl (52) 0.001 SRP-IgG positive, (%)0/134/38 (10) 0.001 Other SAR407899 HCl myositis-specific antibodies0/112/29e0.24Myositis Rabbit Polyclonal to CBX6 associated antibodies3/116/29f0.35AChR binding antibody positive, (%)3/17 (18)1/3 (33)g0.5AChR modulating antibody positive, (%)2/17 (12)1/3 (33)g0.4Striational antibody positive, (%)8/17 (47)1/19 (5)g 0.002 Paraneoplastic antibody positive, (%)3/17 (18)h0/18 0.001 Lymphopenia, (%)18/24(75)7/34 (20) 0.001 Absolute lymphocyte count, median (range; 109/l)0.72 (0.05C3.5)1.38 (0.56C3.4) 0.001 Elevated TSH, (%)4/24 (19)12/28 (42)0.14AST or ALT elevation, (%)15/24 (70)32/35 (91) 0.003 Open in a separate window AChR = acetylcholine receptor; ALT = amino alanine transferase; AST = aspartate amino transferase; CK = creatinine kinase; HMGCR = 3-hydroxy-3-methylglutaryl-coenzyme-A reductase; ICIAM = immune checkpoint inhibitor-associated myositis; IMNM = immune-mediated necrotizing myopathy; NS = not significant; SRP, transmission acknowledgement particle; TSH, thyroid stimulating hormone. All strong values indicate valueonline. Competing interest Dr. Liewluck reports receiving a discretionary fund from the Department of Neurology, Mayo Medical center for unrelated research projects. Dr. Milone reports receiving research funding from a Mayo Medical center benefactor and a discretionary fund from the Department of Neurology, Mayo Medical center for unrelated research projects, and an honorarium to serve as associated editor of Neurology Genetics. Dr. Zekeridou reports a patent on PDE10A-IgG as a biomarker of paraneoplastic neurological autoimmunity. Drs. Shelly, Triplett, Pinto and Diehn statement no disclosure. Supplementary Material fcaa181_Supplementary_DataClick here for additional data file.(18K, docx) Glossary CK = creatine kinaseCTCAE = Common Terminology Criteria for Adverse Events versionHMGCR = 3-Hydroxy-3-Methylglutaryl-CoA ReductaseICIAM = immune checkpoint inhibitor-associated myopathyIMNM = immune-mediated necrotizing myopathyirAEs = immune-related adverse eventsIVMP = intravenous SAR407899 HCl methylprednisolone;PD-1 = programmed cell death-1RNS = repetitive nerve stimulationSFEMG = single-fibre EMGSRP = transmission recognition particle.
