Evidence map›Paper›PMID 41376410›Full record

ArticleJournal of the peripheral nervous system : JPNS2025

Blood 1-Deoxysphingolipid Levels Are Associated With Epidermal Denervation in Small Fiber Neuropathy.

Luisa Kreß, Caren Meyer Zu Altenschildesche, Nadine Egenolf, Claudia Sommer, Thorsten Hornemann, Nurcan Üçeyler

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Article in Journal of the peripheral nervous system : JPNS, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

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1citing papers in PubMed
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1 · What the graph read from it

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3 · Its place in the literature

Who cites it

1 citing paper in PubMed.

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4 · The record

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5 · Who and what money

Authors and funding

6 authors.

Luisa KreßDepartment of Neurology, University Hospital Würzburg, Würzburg, Germany.
Caren Meyer Zu AltenschildescheDepartment of Neurology, University Hospital Würzburg, Würzburg, Germany.
Nadine EgenolfDepartment of Neurology, University Hospital Würzburg, Würzburg, Germany.
Claudia SommerDepartment of Neurology, University Hospital Würzburg, Würzburg, Germany.
Thorsten HornemannDepartment of Biochemistry, University of Zurich, Zurich, Switzerland.
Nurcan ÜçeylerDepartment of Neurology, University Hospital Würzburg, Würzburg, Germany.

Funding

Deutsche Forschungsgemeinschaft UE171-15/1Deutsche Forschungsgemeinschaft UE171-3/1European Joint Program on Rare Diseases EJP RD+SNF 32ER30_187505Schweizerischer Nationalfonds zur Förderung der Wissenschaftlichen Forschung SNF 310030_215134
6 · The paper itself

Abstract

BACKGROUND AND

aimsDysfunctional sphingolipid metabolism leads to nerve fiber degeneration, particularly of small caliber Aδ and C fibers, in hereditary sensory and autonomic neuropathies. We aimed to investigate the association of blood 1-deoxysphingolipid (1-deoxySL) profiles and skin denervation in idiopathic small fiber neuropathy (SFN).

methodsSeventy-five patients with idiopathic SFN were recruited prospectively. Patients underwent a skin punch biopsy at the lower leg and upper thigh for intraepidermal nerve fiber density (IENFD) quantification. IENFD was correlated with individual blood 1-deoxySL levels, amino acid profiles, and determinants of glucose and lipoprotein metabolism.

resultsMedian distal IENFD in SFN patients was 5.3 fibers/mm. In 38/75 (51%) patients, IENFD was ≤ 5.3 fibers/mm (i.e., "low fiber density," LFD), while in 37/75 (49%) patients, IENFD was > 5.3 fibers/mm (i.e., "high fiber density," HFD). 1-deoxySL was higher in patients with LFD compared to HFD (p < 0.05). Fasting plasma glucose was also higher in patients with LFD than in patients with HFD (p < 0.01), while HDL was lower in patients with LFD compared to HFD (p < 0.001).

interpretation1-deoxySL, lipoprotein metabolism, and glucose levels are associated with the extent of epidermal denervation in SFN, supporting the role of this metabolic axis in small nerve fiber pathology.

Indexed as

EpidermisSmall Fiber NeuropathySphingolipidsAdultAgedFemaleHumansMaleMiddle AgedNerve FibersProspective StudiesSphingolipidsepidermal denervationlipoproteinssmall fiber neuropathysphingolipids

Identifiers

PMID41376410
PMCPMC12696513

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