Small Fiber Neuropathy with Ganglioside Antibody: A Case Report (P7-11.004)

Document Type

Conference Proceeding

Publication Date

4-8-2025

Publication Title

Neurology

Keywords

autoantibody, biological marker, ganglioside antibody, immunoglobulin M, plexin, steroid, antibody titer, case report, clinical article, conference abstract, coronavirus disease 2019, drug therapy, electromyogram, electromyography, female, human, neuropathy, paresthesia, plasmapheresis, questionnaire, sarcoidosis, Sjoegren syndrome, small fiber neuropathy, therapy, vasculitis

Abstract

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: SFNRasch 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 SFNSIQ 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.

Volume

104

Issue

7_Supplement_1

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