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NEUROLOGY 2009;73:1142-1148
© 2009 American Academy of Neurology

Intraepidermal nerve fiber density and its application in sarcoidosis

M. Bakkers, MD, I.S.J. Merkies, MD, PhD, G. Lauria, MD, G. Devigili, MD, P. Penza, MD, R. Lombardi, PhD, M. C.E. Hermans, MD, S. I. van Nes, MD, M. De Baets, MD, PhD and C. G. Faber, MD, PhD

From the Department of Neurology (M.B., I.S.J.M., M.C.E.H., C.G.F.), Maastricht University Medical Center, The Netherlands; Department of Neurology (I.S.J.M.), Spaarne Hospital, The Netherlands; Department of Neurology (I.S.J.M., S.I.v.N.), Erasmus Medical Center Rotterdam, The Netherlands; Neuromuscular Diseases Unit (G.L., P.P., R.L.), IRCCS Foundation, "Carlo Besta" National Neurological Institute, Milan, Italy; Neurologic Clinic (G.D.), University of Ferrara, Italy; and School for Mental Health and Neuroscience (M.D.B.), Maastricht, The Netherlands.

Address correspondence and reprint requests to Dr. Mayienne Bakkers, Department of Neurology, Maastricht University Medical Centre, PO Box 5800, 6202 AZ, Maastricht, The Netherlands m.bakkers{at}mumc.nl

Background: Intraepidermal nerve fiber density (IENFD) is considered a good diagnostic tool for small fiber neuropathy (SFN).

Objectives: To assess stratified normative values for IENFD and determine the reliability and validity of IENFD in sarcoidosis.

Methods: IENFD was assessed in 188 healthy volunteers and 72 patients with sarcoidosis (n = 58 with SFN symptoms, n = 14 without SFN symptoms). Healthy controls were stratified (for age and sex), resulting in 6 age groups (20–29, 30–39, ... up to ≥70 years) containing at least 15 men and 15 women. A skin biopsy was taken in each participant 10 cm above the lateral malleolus and analyzed in accordance with the international guidelines using bright-field microscopy. Interobserver/intraobserver reliability of IENFD was examined. In the patients, a symptoms inventory questionnaire (SIQ; assessing SFN symptoms) and the Vickrey Peripheral Neuropathy Quality-of-Life Instrument-97 (PNQoL-97) were assessed to examine the discriminative ability of normative IENFD values.

Results: There was a significant age-dependent decrease of IENFD values in healthy controls, with lower densities in men compared with women. Good interobserver/intraobserver reliability scores were obtained ({kappa} values ≥0.90). A total of 21 patients with sarcoidosis had a reduced IENFD score (<5th percentile; 19 [32.8%] in patients with SFN symptoms, 2 [14.3%] in patients without SFN symptoms). The validity of the normative IENFD values was demonstrated by distinguishing between the SIQ scores and various PNQoL-97 values for the different patient groups.

Conclusion: This study provides clinically applicable distal intraepidermal nerve fiber density normative values, showing age- and sex-related differences.

Abbreviations: IENFD = intraepidermal nerve fiber density; MUMC = Maastricht University Medical Center; NS = not significant; PNQoL-97 = Vickrey Peripheral Neuropathy Quality-of-Life Instrument-97; SFN = small fiber neuropathy; SIQ = symptoms inventory questionnaire.

Supplemental data at www.neurology.org

Supported by a grant from the "Profileringsfonds" of the Maastricht University Medical Center, The Netherlands (project number 062053). This fund has had no role in the design and conduct of the study, nor in the collection, management, analysis, or interpretation of the data, in preparation, review, or approval of the manuscript.

Disclosure: Author disclosures are provided at the end of the article.

Received December 30, 2008. Accepted in final form July 16, 2009.






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