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Abstract Details

Interobserver Reliability of Epidermal Nerve Fiber Density Estimation
Peripheral Nerve
P02 - (-)
186
BACKGROUND: Epidermal nerve fiber density (ENFD) analysis has been widely used to qualify and quantify small fiber neuropathy(SFN). The quantification is usually done by trained neurologists following the previously established guidelines. There is limited research looking at the interobserver reliability of ENFD estimation.
DESIGN/METHODS: We performed a prospective study to assess the interobserver reliability in ENFD estimate for SFN. Three millimeter skin biopsies were performed and stained using PGP9.5 pan-axonal marker. Epidermal nerve fibers were counted in the sections obtained using previously published counting rules by two independent observers (staff neurologist and a senior neurology resident). ENFD was estimated by using the length of epidermis (EP). Interobserver reliability was assessed by comparison of the ENFD between 2 observers using the spearman's rank correlation coefficient.
RESULTS: Three sites (distal leg, distal thigh and proximal thigh) skin biopsies from 10 patients, total 30 biopsies, were analyzed. ENFD was estimated by 2 observers. Mean ENFD (observer 1, observer 2) at distal leg 4.11 卤3.27 fibers/mm, 4.25卤 3.49 fibers/mm; distal thigh 6.12 卤1.84 fibers/mm, 6.42卤 2.34 fibers/mm and proximal thigh 10.06 卤3.79 fibers/mm, 10.99卤3.76 fibers/mm. Seven (70%) patients had SFN based on the ENFD estimation, with no change in diagnosis between observers. The correlation coefficient for interobserver reliability was 0.95 (P<0.001).
CONCLUSIONS: This study showed a high level of interobserver reliability in ENFD estimation for diagnosis of SFN. There was no change in the diagnosis based on ENFD estimation by two different observers.
Authors/Disclosures
Omar Jawdat, MD (The University of Kansas Medical Center)
PRESENTER 		Dr. Jawdat has nothing to disclose.
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.
April L. McVey, MD (April L. McVey, MD) No disclosure on file
Regina Berkovich, MD, PhD (Regina Berkovich MD PhD Inc) The institution of Dr. Berkovich has received personal compensation in the range of $50,000-$99,999 for serving as a Consultant for Alexion, Biogen, Genentec, Mallincrodt, Sanofi, Novartis, . Dr. Berkovich has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen, Genentec, Sanofi, Merck . Dr. Berkovich has received personal compensation in the range of $50,000-$99,999 for serving on a Speakers Bureau for Alexion, Biogen, Sanofi, Merck, Mallincrodt . The institution of Dr. Berkovich has received research support from Biogen, Sanofi, Merck.
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.
Alastair Compston, PhD, FRCP No disclosure on file
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.