Abstract Details

Title Characterization of Clinical and Paraclinical Features Associated with TS-HDS Autoantibody Seropositivity

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) C10 - Immune-mediated Neuropathies (2:20 PM-2:25 PM)
P2 - Poster Session 2 (9:00 AM-3:00 PM)

Poster/Presentation
Number 080

Objective To evaluate neuropathy phenotypes and clinical outcomes associated with trisulfated heparin disaccharide (TS-HDS) autoantibodies.

Background TS-HDS autoantibody has been reported as a biomarker of immune-mediated neuropathy. However, studies evaluating the clinical associations of this autoantibody are limited.

Design/Methods Electronic medical records were reviewed to identify TS-HDS autoantibody seropositive patients and characterize their clinical and electrodiagnostic findings.

Results Seventy-seven TS-HDS-IgM seropositive (titer range 9000-350,000) patients were identified (50 females; median age of onset was 48 years (range 9-83 years). Eleven patients were also positive for FGFR3-IgG (titer range 4000-19,000). 70% (54/77) had clinical/paraclinical evidence of neuropathy (54/77, 70% of TS-HDS-IgM alone; 10/11, 91% of TS-HDS-IgM with coexisting FGFR3-IgG). The managing physician characterized an immune-mediated neuropathy in 30% (23/77) and 54% (6/11) of the TS-HDS-IgM only and TS-HDS-IgM with coexisting FGFR3-IgG seropositive patients, respectively. Small fiber neuropathy presented in 58% (45/77) and 63% (7/11) of TS-HDS-IgM only, and both antibodies seropositive patients, respectively. Length-dependent neuropathy was the most common neuropathy phenotype amongst TS-HDS IgM (43/54, 80%) and dual seropositive cases (7/11, 63%). Forty-one (53%) patients received immunotherapy, predominantly: IVIG (n=37), IV solumedrol (n=7), oral prednisone (n=14), and mycophenolate mofetil (n=12). Among these, 43% (15/35) with TS-HDS-IgM seropositivity alone had improvement in inflammatory neuropathy cause and treatment (INCAT) disability score or modified Rankin Scale (mRS), while 33% (2/6) of patients with dual seropositivity had INCAT and mRS improvement. TS-HDS-IgM titers had low discriminative ability to identify immunotherapy response with an AUC of 0.621.

Conclusions Neuropathy associations and clinical phenotypes amongst TS-HDS-IgM seropositive cases are variable. Furthermore, only a minority of cases are immunotherapy responsive, limiting the value of this biomarker in identifying immune-mediated neuropathies.

Authors/Disclosures
Pitcha Chompoopong, MD (University of Minnesota) PRESENTER	Dr. Chompoopong has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Astrazeneca. Dr. Chompoopong has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Alnylam.
Divyanshu Dubey, MD, FAAN (Mayo Clinic) PRESENTER	The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Argenx. The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Arialys. The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB . Dr. Dubey has received research support from Department of Defense . Dr. Dubey has received research support from Department of Defense . Dr. Dubey has received research support from UCB. Dr. Dubey has received research support from David J. Tomassoni ALS Research Grant Program . Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care.
Mohamed M. Rezk, MD (utmb)	Dr. Rezk has nothing to disclose.
Igal Mirman, MD (UTMCK)	Dr. Mirman has nothing to disclose.
Sarah E. Berini, MD (Mayo Clinic)	Dr. Berini has nothing to disclose.
Christopher J. Klein, MD, FAAN (Mayo Clinic)	Dr. Klein has received personal compensation in the range of $500-$4,999 for serving as a Consultant for NMD Pharma.
John R. Mills, MD, PhD (Mayo Clinic)	The institution of Dr. Mills has received research support from Werfen Diagnostics. Dr. Mills has received intellectual property interests from a discovery or technology relating to health care.

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