Abstract Details

Title Small Fiber Neuropathy with Ganglioside Antibody: A Case Report

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) P7 - Poster Session 7 (5:00 PM-6:00 PM)

Poster/Presentation
Number 11-004

Objective To report a case of small fiber neuropathy associated with an elevated GD1a antibody titer and treatment with intravenous immunoglobulin (IVIG)

Background Cryptogenic small fiber neuropathy (SFN) is a vexing clinical problem often with limited treatment options besides symptomatic control. Immune SFN typically presents with acute or subacute onset, non-length dependent pathology, female gender, and often association with various autoantibodies. A number of immune mechanisms have been proposed, but the role of autoantibodies is controversial. Previous studies have shown an association between SFN and TS-HDS, FGFR-3, Plexin D1, and other autoantibodies, and the role of these antibodies in identifying immunotherapy-responsive SFN is currently under investigation. Other reports have shown responsiveness to IVIG, steroids, or plasmapheresis in SFN related to sarcoidosis, Sjogren syndrome, vasculitis, and post-COVID SFN.

Design/Methods NA

Results A 58-year-old female reported a subacute onset of lower extremity pain, numbness and tingling. On examination the patient’s Utah Early Neuropathy Scale (UENS) score was 12/42. Patient-reported questionnaires revealed the following: SFN-Rasch Overall Disability Scale (SFN-RODS)=58/64, SFN-Symptom Inventory Questionnaire (SFN-SIQ)=11/39, SFN-Screening List (SFN-SL)=18/84 (>11 sensitive for SFN). EMG was normal, but sweat gland nerve fiber density was low in 2 sites, and QSART was abnormal in her distal leg. IgM-GD1a antibodies were present at a titer of 7000 (normal <2000); no other neuropathy cause was identified. After 6 months of IVIG treatment the UENS score was 1/42, SFN-RODS was 61/64, and SFN-SIQ was 13/39. The patient opted to discontinue IVIG treatment as symptoms had improved.

Conclusions This case of SFN with elevated GD1a autoantibody, and subjective and objective improvement on IVIG, highlights the importance of checking for autoantibodies in SFN. Anti- GD1a may be a useful biomarker in screening and managing patients with immune-mediated SFN and may be useful to predict response to immunotherapy, but further studies are needed.

Authors/Disclosures
Maricel Gener, Medical Student PRESENTER	Ms. Gener has nothing to disclose.
Lawrence A. Zeidman, MD, FAAN (Endeavor Health Northshore Department of Neurology)	Dr. Zeidman has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Johnson & Johnson. Dr. Zeidman has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Argenx. Dr. Zeidman has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Pfizer. Dr. Zeidman has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Smith Blake Hill LLC. The institution of Dr. Zeidman has received research support from Octapharma. Dr. Zeidman has received publishing royalties from a publication relating to health care.

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