The role of skin biopsy in differentiating small-fiber neuropathy from ganglionopathy

V. Provitera, C. H. Gibbons, G. Wendelschafer-Crabb, V. Donadio, D. F. Vitale, A. Loavenbruck, A. Stancanelli, G. Caporaso, R. Liguori, N. Wang, L. Santoro, W. R. Kennedy, M. Nolano

Research output: Contribution to journalArticle

Abstract

Background and purpose: We aimed to test the clinical utility of the leg:thigh intraepidermal nerve-fiber (IENF) density ratio as a parameter to discriminate between length-dependent small-fiber neuropathy (SFN) and small-fiber sensory ganglionopathy (SFSG) in subjects with signs and symptoms of small-fiber pathology. Methods: We retrospectively evaluated thigh and leg IENF density in 314 subjects with small-fiber pathology (173 with distal symmetrical length-dependent SFN and 141 with non-length-dependent SFSG). A group of 288 healthy subjects was included as a control group. The leg:thigh IENF density ratio was calculated for all subjects. We used receiver operating characteristic curve analyses to assess the ability of this parameter to discriminate between length-dependent SFN and SFSG, and the decision curve analysis to estimate its net clinical benefit. Results: In patients with neuropathy, the mean IENF density was 14.8 ± 6.8/mm at the thigh (14.0 ± 6.9/mm in length-dependent SFN and 15.9 ± 6.7/mm in patients with SFSG) and 7.5 ± 4.5/mm at the distal leg (5.4 ± 3.2/mm in patients with length-dependent SFN and 10.1 ± 4.6/mm in patients with SFSG). The leg:thigh IENF density ratio was significantly (P < 0.01) lower in patients with length-dependent SFN (0.44 ± 0.23) compared with patients with SFSG (0.68 ± 0.28). The area under the curve of the receiver operating characteristic analysis to discriminate between patients with length-dependent SFN and SFSG was 0.79. The decision curve analysis demonstrated the clinical utility of this parameter. Conclusions: The leg:thigh IENF ratio represents a valuable tool in the differential diagnosis between SFSG and length-dependent SFN.

Original languageEnglish
Pages (from-to)848-853
Number of pages6
JournalEuropean Journal of Neurology
Volume25
Issue number6
DOIs
Publication statusPublished - Jun 1 2018

Keywords

  • immunohistochemistry
  • intraepidermal nerve fibers
  • neuropathic pain
  • sensory ganglionopathy
  • skin biopsy
  • small-fiber neuropathy

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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    Provitera, V., Gibbons, C. H., Wendelschafer-Crabb, G., Donadio, V., Vitale, D. F., Loavenbruck, A., Stancanelli, A., Caporaso, G., Liguori, R., Wang, N., Santoro, L., Kennedy, W. R., & Nolano, M. (2018). The role of skin biopsy in differentiating small-fiber neuropathy from ganglionopathy. European Journal of Neurology, 25(6), 848-853. https://doi.org/10.1111/ene.13608