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Abstract Details

Clinical and demographic features of LRP4 positive myasthenia gravis patients
Neuromuscular and Clinical Neurophysiology (EMG)
P5 - Poster Session 5 (5:30 PM-6:30 PM)
12-040
To study the demographic,clinical features of anti-lipoprotein related protein4(LRP4) antibody positive myasthenia gravis(MG)patients compared to triple seronegative patients.
Myasthenia gravis has several serological biomarkers.Prior literature showed that 9-18% of patients with negative anti-acetylcholine receptor (AChR) and Muscle Specific Tyrosine kinase (MuSK) antibodies have LRP4 antibodies.There is limited literature looking at the clinical presentation of this group of patients.

After IRB approval for exempt study was obtained,a retrospective chart review was performed on all the patients who are seronegative for AchR and MUSK antibodies,and enrolled in the research study named"Characterization of Agrin/LRP4 Antibody-Positive Myasthenia gravis".Data on the demographic,clinical features was abstracted from the charts.We compared the information on LRP4 positive patients to the negative patients.
Anti-LRP4 antibodies were detected in 4 of 29 patients with double seronegative MG.The mean age of patients with LRP4 antibody was 55.8±7.4 and triple seronegative group (N=25) was 58.7±16.4.The MGFA class at onset of symptoms for LRP4 positive group was MGFA class I in 3 patients,MGFA class II in 1 patient;and 14 of the seronegative patients presented with MGFA I and 11 with MGFA class II.Worst MGFA score remained the same as onset in the LRP4 positive patients,whereas in the seronegative group,2 patients reached MGFA III,15 patients MGFA II and 8 patients remained at MGFA I.All four LRP4 positive patients presented with ocular symptoms at onset whereas in the seronegative group 5 had generalized symptoms at onset,1 patient had shortness of breath and 19 presented with ocular symptoms at onset.Two patients in LRP4 and 19 seronegative patients had CT chest,with all being negative for thymoma.
While the clinical characteristics of more LRP4 positive cases need to be determined,it is possible that majority of them constitute pure ocular symptoms based on our observation.Over the median follow up of 3.5 years duration,MGFA class remained stable in this group.
Authors/Disclosures
Siva P. Pesala, MD (West Virginia University)
PRESENTER 		No disclosure on file
Mazen M. Dimachkie, MD, FAAN (University of Kansas Medical Center) Dr. Dimachkie has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Sanofi. Dr. Dimachkie has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Octapharma. Dr. Dimachkie has received personal compensation in the range of $500-$4,999 for serving as a Consultant for ArgenX. Dr. Dimachkie has received personal compensation in the range of $0-$499 for serving as a Consultant for Catalyst. Dr. Dimachkie has received personal compensation in the range of $0-$499 for serving as a Consultant for Takeda. Dr. Dimachkie has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Medlink. Dr. Dimachkie has received personal compensation in the range of $500-$4,999 for serving as a Consultant for CSL Behring. Dr. Dimachkie has received personal compensation in the range of $0-$499 for serving as a Consultant for Astellas. Dr. Dimachkie has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Janssen. Dr. Dimachkie has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Dianthus. Dr. Dimachkie has received personal compensation in the range of $0-$499 for serving as a Consultant for TACT / Treat NMD. Dr. Dimachkie has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Cabaletta Bio. Dr. Dimachkie has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Clinical Neurological Society of America, Inc . Dr. Dimachkie has received personal compensation in the range of $0-$499 for serving as a Consultant for Ig Society, Inc. Dr. Dimachkie has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Abcuro. Dr. Dimachkie has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Amicus. Dr. Dimachkie has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Horizon/Immunovant. Dr. Dimachkie has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for EMD Serono. Dr. Dimachkie has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Ipsen. Dr. Dimachkie has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Nuvig. Dr. Dimachkie has received personal compensation in the range of $500-$4,999 for serving as a Consultant for 好色先生 (AAN). Dr. Dimachkie has received personal compensation in the range of $500-$4,999 for serving as a Consultant for American Association of Neuromuscular and Electrodiagnostic Medicine (AANEM). Dr. Dimachkie has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Annexon Biosciences. Dr. Dimachkie has received personal compensation in the range of $500-$4,999 for serving as a Consultant for AstraZeneca. Dr. Dimachkie has received personal compensation in the range of $0-$499 for serving as a Consultant for Candid Therapeutics. Dr. Dimachkie has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Creyon Bio, Inc.. Dr. Dimachkie has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Nuvig Therapeutics. Dr. Dimachkie has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for UCB Biopharma. Dr. Dimachkie has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Vertex Pharmaceuticals. Dr. Dimachkie has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Fortrea. Dr. Dimachkie has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Kriya Therapeutics. The institution of Dr. Dimachkie has received research support from NIH. The institution of Dr. Dimachkie has received research support from The Myositis Association. The institution of Dr. Dimachkie has received research support from Biosensics. Dr. Dimachkie has received intellectual property interests from a discovery or technology relating to health care. Dr. Dimachkie has received intellectual property interests from a discovery or technology relating to health care. Dr. Dimachkie has received intellectual property interests from a discovery or technology relating to health care. Dr. Dimachkie has received publishing royalties from a publication relating to health care. Dr. Dimachkie has received publishing royalties from a publication relating to health care.
No disclosure on file
No disclosure on file
Melanie Glenn, MD No disclosure on file
Constantine Farmakidis, MD, FAAN Dr. Farmakidis has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB. Dr. Farmakidis has received personal compensation in the range of $500-$4,999 for serving as a Consultant for J&J. Dr. Farmakidis has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Muscular Dystrophy Association.
Omar Jawdat, MD (The University of Kansas Medical Center) Dr. Jawdat has nothing to disclose.
Duaa Jabari, MD (The University of Kansas Medical Center) Dr. Jabari has nothing to disclose.
Andrew Heim Andrew Heim has nothing to disclose.
No disclosure on file
Richard J. Barohn, MD, FAAN (University of Missouri) Dr. Barohn has received personal compensation in the range of $50,000-$99,999 for serving as a Consultant for NuFactor. The institution of Dr. Barohn has received research support from FDA OPD R01. Dr. Barohn has received intellectual property interests from a discovery or technology relating to health care.
Mamatha Pasnoor, MD, FAAN Dr. Pasnoor has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Argenx BVBA. Dr. Pasnoor has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Janssen Pharmaceutical. Dr. Pasnoor has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Immunovant. Dr. Pasnoor has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB. Dr. Pasnoor has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Annexon. Dr. Pasnoor has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Grifols. Dr. Pasnoor has received personal compensation in the range of $500-$4,999 for serving as a Consultant for amgen.