Small fiber neuropathy: diagnosis, causes, and treatment

Sène, Damien

doi:10.1016/j.jbspin.2017.11.002

Cited by 113Pain Mechanisms and TreatmentsPeripheral Neuropathies and DisordersHereditary Neurological Disorders

Small fiber neuropathy: diagnosis, causes, and treatment

Sène, Damien·2018·Joint Bone Spine

DOI 10.1016/j.jbspin.2017.11.002

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Abstract

Small fiber neuropathy, which affects the sensory Aδ and C fibers, is now a major diagnostic and therapeutic challenge. Nearly 7% of the general population have chronic neuropathic pain responsible for severe quality-of-life impairments. Awareness must therefore be raised among clinicians of the somatosensory and autonomic symptoms that can reveal small fiber neuropathy, appropriate diagnostic investigations, most common causes, and best treatment options for each patient profile. To help achieve this goal, the present review article discusses the clinical presentation of neuropathic pain and paresthesia and/or autonomic dysfunction due to involvement of nerves supplying exocrine glands and smooth muscle; normal findings from standard electrophysiological investigations; most informative diagnostic tests (epidermal nerve fiber density in a skin biopsy, laser-evoked potentials, heat- and cold-detection thresholds, electrochemical skin conductance); main causes, which consist chiefly of metabolic diseases (diabetes mellitus, glucose intolerance), dysimmunity syndromes (Sjögren's syndrome, sarcoidosis, monoclonal gammopathy), and genetic abnormalities (familial amyloidosis due to a transthyretin mutation, Fabry disease, sodium channel diseases); and the available symptomatic and etiological treatments.

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Cardiovascular dysautonomia and a range of other functional symptoms commonly reported in hEDS/HSD are also considered typical manifestations of small-fibre neuropathy.
“Interestingly, cardiovascular dysautonomia and a range of other functional symptoms commonly reported in hEDS/HSD are also considered typical manifestations of small-fibre neuropathy [31].”
From Severity classes in adults with hypermobile Ehlers–Danlos syndrome/hypermobility spectrum disorders: a pilot study of 105 Italian patients (2019) · cites this paper as [31]

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Devigili, G., Cazzato, D., & Lauria, G. (2020) · Expert Review of Neurotherapeutics · doi:10.1080/14737175.2020.1794825 · Cited by 87 · see citing works
Small fiber neuropathy: a common and important clinical disorder
Hoitsma, E., Reulen, J. P. H., Baets, M. H. D., Drent, M., Spaans, F., & Faber, C. G. (2004) · Journal of the Neurological Sciences · doi:10.1016/j.jns.2004.08.012 · Cited by 228 · see citing works
[Small Fiber Neuropathy with Inadequate Response to Medical Therapy: Diagnosis and Treatment of Small Fiber Neuropathy].
Suzuki, C. (2022) · PubMed · doi:10.11477/mf.1416202088

Authors: Sène, D.
Affiliations: Assistance Publique – Hôpitaux de Paris (FR)
Corresponding author: Sène, Damien
ORCID: Sène, Damien
Venue: Joint Bone Spine
Publisher: Elsevier BV
Published: 2018-10
Volume: 85
Issue: 5
Pages: 553-559
ISSN: 1297-319X (ISSN-L)
DOI: 10.1016/j.jbspin.2017.11.002
OpenAlex: W2770269036
Cited by: 113
References: 61
Citations by year: 2026: 6 · 2025: 11 · 2024: 16 · 2023: 18 · 2022: 22 · 2021: 16 · 2020: 15 · 2019: 7 · 2018: 2
FWCI: 0.81
Type: journal article
Language: en
Open access: closed
Licence: www.elsevier.com/tdm/userlicense/1.0/ (tdm)
www.elsevier.com/legal/tdmrep-license (tdm)
Locations: Joint Bone Spine (publishedVersion)
PubMed (publishedVersion)
Topics: Health Sciences › Medicine › Physiology › Pain Mechanisms and Treatments
Health Sciences › Medicine › Neurology › Peripheral Neuropathies and Disorders
Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Hereditary Neurological Disorders
Keywords: small fiber neuropathy, chronic neuropathic pain, epidermal nerve fiber density, laser-evoked potentials, electrochemical skin conductance, autonomic dysfunction, paresthesia, diabetes mellitus, impaired glucose tolerance, Sjögren's syndrome, sarcoidosis, monoclonal gammopathy, familial amyloidosis, transthyretin mutations, Fabry disease, neuropathic pain treatment
MeSH terms: Small Fiber Neuropathy, Analgesics, Antidepressive Agents, Biopsy, Needle, Electromyography, Female, Humans, Immunohistochemistry, Male, Neurologic Examination, Pain Measurement, Prognosis, Sarcoidosis, Sjogren's Syndrome, Treatment Outcome, Risk Assessment, Chronic Pain

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Small fiber neuropathy: diagnosis, causes, and treatment

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