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Abstract Details

Ratio of Distal to Proximal Epidermal Nerve Fiber Density in Small Fiber Neuropathy
Neuromuscular and Clinical Neurophysiology (EMG)
P1 - Poster Session 1 (8:00 AM-9:00 AM)
11-006
To assess the distal-to-proximal IENFD ratio in patients with SFN.

IENFD assessment has become an important tool for diagnosis of small fiber neuropathy (SFN). Distal leg, distal thigh and proximal thigh are the usual sites of biopsy. At least two-site biopsies are performed to assess for length dependency. Age dependent normative data have been established for distal leg. In some patients with paresthesia, although the distal and proximal biopsies are normal, the distal IENFD is much less compared to proximal density. There is limited literature looking at the significance of the ratio of distal-to-proximal biopsy IENFD.

After IRB approval, we retrospectively evaluated charts of subjects who underwent skin biopsy for evaluation of SFN from May 2020 to May 2023 in the neurology department of KUMC. The distal leg, proximal thigh IENFD information was collected, and ratio calculated for all subjects. Subjects were categorized as having possible or probable SFN based on the clinical criteria established by Diabetic Neuropathy Study Group of the European Association for the Study of Diabetes (NEURODIAB). Descriptive and correlation analysis was performed.

Total of 56 charts were reviewed. Female to male ratio was 3:1. Mean age was 50±26. Total number of patients with clinical diagnosis of neuropathy (probable and possible) was 46. IENFD was abnormal in 23, normal in 23 with mean distal to proximal ratio of 0.5 and 0.6 respectively. The mean ratio for patients without clinical diagnosis of neuropathy was 0.9. The 23 patients with clinical diagnosis of neuropathy with negative skin biopsy had significant difference in mean ratio compared to patients without clinical diagnosis of neuropathy using T-test (p 0.02).

The findings of this study suggest that in patients with normal IENFD and clinical suspicion for SFN, the distal-to-proximal ratio may provide additional benefit in making the diagnosis.
Authors/Disclosures
Georgette Dib, MD
PRESENTER
Dr. Dib has nothing to disclose.
Matthew Varon, MD (University of Kansas Medical Center) Dr. Varon has nothing to disclose.
Omar Jawdat, MD (The University of Kansas Medical Center) Dr. Jawdat has nothing to disclose.
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.
Swathy Chandrashekhar, MD (University of Kansas Medical Center) Dr. Chandrashekhar has received publishing royalties from a publication relating to health care.
Jeffrey Statland, MD (University of Kansas Medical Center) Dr. Statland has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Arrowhead. Dr. Statland has received personal compensation in the range of $500-$4,999 for serving as a Consultant for ML Bio. Dr. Statland has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Epic Bio. Dr. Statland has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Armatus . Dr. Statland has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Kate Therapeutics. Dr. Statland has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Vita Therapeutics. Dr. Statland has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Dyne Therapeutics. Dr. Statland has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Avidity . Dr. Statland has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Fulcrum Therapeutics. Dr. Statland has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. Dr. Statland has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Vertex . The institution of Dr. Statland has received research support from NIH. The institution of Dr. Statland has received research support from FSHD Society. The institution of Dr. Statland has received research support from Friends of FSH Research. The institution of Dr. Statland has received research support from FSHD Canada. The institution of Dr. Statland has received research support from MDA.
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.
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.