The DOI matches its Crossref record; 60 of the 61 references it cites were found in a registry or an index.
Small fiber neuropathy: Diagnosis, causes, and treatment
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.
Overview
Integrity signals
Registry
Cites
Statistics
Verification
Claims the paper makes
Claims other papers make about this one
Each is checked against this paper’s own text: does it say what the citing paper says it does?
In the citing paper
“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]
Citations
Cites
Pharmacotherapy for neuropathic pain in adults: a systematic review and meta-analysis
Finnerup, N. B., Attal, N., Haroutounian, S., McNicol, E., Baron, R., Dworkin, R. H., Gilron, I., Haanpää, M., Hansson, P., Jensen, T. S., et al. (2015) · The Lancet Neurology · doi:10.1016/s1474-4422(14)70251-0 · Cited by 3,980
Bouhassira, D., Attal, N., Alchaar, H., Boureau, F., Brochet, B., Bruxelle, J., Cunin, G., Fermanian, J., Giniès, P., Grun-Overdyking, A., et al. (2005) · Pain · doi:10.1016/j.pain.2004.12.010 · Cited by 2,834
Tesfaye, S., Boulton, A. J. M., Dyck, P. J., Freeman, R., Horowitz, M., Kempler, P., Lauria, G., Malik, R. A., Spallone, V., Vinik, A., et al. (2010) · Diabetes Care · doi:10.2337/dc10-1303 · Cited by 2,690
Prevalence of chronic pain with neuropathic characteristics in the general population
Bouhassira, D., Lantéri‐Minet, M., Attal, N., Laurent, B., & Touboul, C. (2007) · Pain · doi:10.1016/j.pain.2007.08.013 · Cited by 1,827
Lauria, G., Hsieh, S., Johansson, O., Kennedy, W. R., Léger, J., Mellgren, S. I., Nolano, M., Merkies, I. S. J., Polydefkis, M. J., Smith, A. G., et al. (2010) · European Journal of Neurology · doi:10.1111/j.1468-1331.2010.03023.x · Cited by 887
Abbott, C. A., Malik, R. A., van Ross, E. R. E., Kulkarni, J., & Boulton, A. J. M. (2011) · Diabetes Care · doi:10.2337/dc11-1108 · Cited by 868
The diagnostic criteria for small fibre neuropathy: from symptoms to neuropathology
Devigili, G., Tugnoli, V., Penza, P., Camozzi, F., Lombardi, R., Melli, G., Broglio, L., Granieri, E., & Lauria, G. (2008) · Brain · doi:10.1093/brain/awn093 · Cited by 796
Gain of function Na V 1.7 mutations in idiopathic small fiber neuropathy
Faber, C. G., Hoeijmakers, J. G. J., Ahn, H., Cheng, X., Han, C., Choi, J., Estación, M., Lauria, G., Vanhoutte, E. K., Gerrits, M. M., et al. (2011) · Annals of Neurology · doi:10.1002/ana.22485 · Cited by 554
Surrogate Markers of Small Fiber Damage in Human Diabetic Neuropathy
Quattrini, C., Tavakoli, M., Jeziorska, M., Kallinikos, P. A., Tesfaye, S. H., Finnigan, J., Marshall, A. G., Boulton, A. J. M., Efron, N., & Malik, R. A. (2007) · Diabetes · doi:10.2337/db07-0285 · Cited by 516
A Gain-of-Function Mutation in TRPA1 Causes Familial Episodic Pain Syndrome
Kremeyer, B., Lopera, F. J., Cox, J. J., Momin, A., Rugiero, F., Marsh, S., Woods, C. G., Jones, N. G., Paterson, K. J., Fricker, F. R., et al. (2010) · Neuron · doi:10.1016/j.neuron.2010.04.030 · Cited by 447
Hansson, P. T., Bačkonja, M., & Bouhassira, D. (2007) · Pain · doi:10.1016/j.pain.2007.03.030 · Cited by 379
Painful and painless channelopathies
Bennett, D. L. H., & Woods, C. G. (2014) · The Lancet Neurology · doi:10.1016/s1474-4422(14)70024-9 · Cited by 350
An In Vivo Confocal Masked Study on Corneal Epithelium and Subbasal Nerves in Patients with Dry Eye
del Castillo, J. M. B., Wasfy, M. A., Fernandez, C. A., & Garcia-Sanchez, J. (2004) · Investigative Ophthalmology & Visual Science · doi:10.1167/iovs.04-0251 · Cited by 298
Sweat testing to evaluate autonomic function
Illigens, B. M. W., & Gibbons, C. H. (2009) · Clinical Autonomic Research · doi:10.1007/s10286-008-0506-8 · Cited by 255
Intraepidermal nerve fiber density and its application in sarcoidosis
Bakkers, M., Merkies, I. S. J., Lauria, G., Devigili, G., Penza, P., Lombardi, R., Hermans, M. C. E., van Nes, S. I., Baets, M. H. D., & Faber, C. G. (2009) · Neurology · doi:10.1212/wnl.0b013e3181bacf05 · Cited by 253
Erythropoietin both protects from and reverses experimental diabetic neuropathy
Bianchi, R., Büyükakıllı, B., Brines, M. L., Savino, C., Cavaletti, G., Oggioni, N., Lauria, G., Borgna, M., Lombardi, R., Çimen, B., et al. (2004) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0307823100 · Cited by 247
Painful sensory polyneuropathy associated with impaired glucose tolerance
Singleton, J. R., Smith, A. G., & Bromberg, M. B. (2001) · Muscle & Nerve · doi:10.1002/mus.1136 · Cited by 241
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 229
Small fibre neuropathy in sarcoidosis
Hoitsma, E., Marziniak, M., Faber, C. G., Reulen, J. P. H., Sommer, C., Baets, M. H. D., & Drent, M. (2002) · The Lancet · doi:10.1016/s0140-6736(02)08912-2 · Cited by 220
Selvarajah, D., Cash, T., Davies, J., Sankar, A., Rao, G., Grieg, M., SHILLO, P. R., Gandhi, R. A., Wilkinson, I. D., & Tesfaye, S. H. (2015) · PLoS ONE · doi:10.1371/journal.pone.0138224 · Cited by 180
Pharmacotherapy of neuropathic pain
Attal, N., & Bouhassira, D. (2015) · Pain · doi:10.1097/01.j.pain.0000460358.01998.15 · Cited by 174
Non-length dependent small fibre neuropathy/ganglionopathy
Gorson, K. C., Herrmann, D. N., Thiagarajan, R., Brannagan, T. H., Chin, R. L., Kinsella, L. J., & Ropper, A. H. (2007) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp.2007.128801 · Cited by 165
Tavee, J. O., Karwa, K. A., Ahmed, Z. A., Thompson, N. R., Parambil, J. G., & Culver, D. A. (2017) · Respiratory Medicine · doi:10.1016/j.rmed.2017.03.011 · Cited by 159
Tavakoli, M., Marshall, A. G., Thompson, L. A., Kenny, M., Waldek, S., Efron, N., & Malik, R. A. (2009) · Muscle & Nerve · doi:10.1002/mus.21383 · Cited by 154
The diagnostic utility of Sudoscan for distal symmetric peripheral neuropathy
Smith, A. G., Lessard, M. K., Reyna, S. P., Doudova, M., & Singleton, J. R. (2014) · Journal of Diabetes and its Complications · doi:10.1016/j.jdiacomp.2014.02.013 · Cited by 152
Painful small-fiber neuropathy in Sjogren syndrome
Chai, J. Y. H., Herrmann, D. N., Stanton, M., Barbano, R. L., & Logigian, E. L. (2005) · Neurology · doi:10.1212/01.wnl.0000176034.38198.f9 · Cited by 152
Peripheral Neuropathy in Primary Sjögren Syndrome
Gøransson, L. G., Herigstad, A., Tjensvoll, A. B., Harboe, E., Mellgren, S. I., & Omdal, R. (2006) · Archives of Neurology · doi:10.1001/archneur.63.11.1612 · Cited by 152
Brines, M. L., Dunne, A. N., van Velzen, M., Proto, P. L., Östenson, C., Kirk, R. I., Petropoulos, I. N., Javed, S., Malik, R. A., Cerami, A., et al. (2014) · Molecular Medicine · doi:10.2119/molmed.2014.00215 · Cited by 145
Small fibers, large impact: Quality of life in small‐fiber neuropathy
Bakkers, M., Faber, C. G., Hoeijmakers, J. G. J., Lauria, G., & Merkies, I. S. J. (2013) · Muscle & Nerve · doi:10.1002/mus.23910 · Cited by 143
Venlafaxine for neuropathic pain in adults
Gallagher, H. C., Gallagher, R. M., Butler, M., Buggy, D. J., & Henman, M. C. (2015) · Cochrane Database of Systematic Reviews · doi:10.1002/14651858.cd011091.pub2 · Cited by 136
Skin biopsy in the management of peripheral neuropathy
Sommer, C., & Lauria, G. (2007) · The Lancet Neurology · doi:10.1016/s1474-4422(07)70172-2 · Cited by 136
The metabolic syndrome and neuropathy: Therapeutic challenges and opportunities
Callaghan, B. C., & Feldman, E. L. (2013) · Annals of Neurology · doi:10.1002/ana.23986 · Cited by 126
Peripheral Neuropathies Associated With Primary Sjögren Syndrome
Sène, D., Jallouli, M., Lefaucheur, J., Saadoun, D., Costedoat-Chalumeau, N., Maisonobe, T., Diemert, M., Musset, L., Haroche, J., Pietté, J. C., et al. (2011) · Medicine · doi:10.1097/md.0b013e31820fd2d1 · Cited by 118
Self-citationDiagnosis of small fiber neuropathy: A comparative study of five neurophysiological tests
Lefaucheur, J. P., Wahab, A., Planté‐Bordeneuve, V., Sène, D., Ménard‐Lefaucheur, I., Rouie, D., Tebbal, D., Salhi, H., Créange, A., Zouari, H. G., et al. (2015) · Neurophysiologie Clinique · doi:10.1016/j.neucli.2015.09.012 · Cited by 115
Self-citationHeij, L. R., Niesters, M., Swartjes, M., Hoitsma, E., Drent, M., Dunne, A. N., Grutters, J. C., Vogels, O. J. M., Brines, M. L., Cerami, A., et al. (2012) · Molecular Medicine · doi:10.2119/molmed.2012.00332 · Cited by 114
Causes of neuropathy in patients referred as “idiopathic neuropathy”
Farhad, K., Traub, R., Ruzhansky, K. M., & Brannagan, T. H. (2015) · Muscle & Nerve · doi:10.1002/mus.24969 · Cited by 104
Culver, D. A., Dahan, A., Bajorunas, D. R., Jeziorska, M., van Velzen, M., Aarts, L. P. H. J., Tavee, J. O., Tannemaat, M. R., Dunne, A. N., Kirk, R. I., et al. (2017) · Investigative Ophthalmology & Visual Science · doi:10.1167/iovs.16-21291 · Cited by 104
Idiopathic distal small fiber neuropathy
Gorson, K. C., & Ropper, A. H. (2009) · Acta Neurologica Scandinavica · doi:10.1111/j.1600-0404.1995.tb00150.x · Cited by 103
Dahan, A., Dunne, A. N., Swartjes, M., Proto, P. L., Heij, L. R., Vogels, O. J. M., van Velzen, M., Sarton, E. Y., Niesters, M., Tannemaat, M. R., et al. (2013) · Molecular Medicine · doi:10.2119/molmed.2013.00122 · Cited by 96
Choi, J., Zhang, L., Dib‐Hajj, S. D., Han, C., Tyrrell, L., Lin, Z., Wang, X., Yang, Y., & Waxman, S. G. (2009) · Experimental Neurology · doi:10.1016/j.expneurol.2008.12.012 · Cited by 91
Non‐length dependent small fiber neuropathy. A prospective case series
Gemignani, F., Giovanelli, M., Vitetta, F., Santilli, D., Bellanova, M. F., Brindani, F., & Marbini, A. (2010) · Journal of the Peripheral Nervous System · doi:10.1111/j.1529-8027.2010.00252.x · Cited by 74
Kemp, H. I., Petropoulos, I. N., Rice, A. S. C., Vollert, J., Maier, C., Sturm, D., Schargus, M., Pető, T., Hau, S., Chopra, R., et al. (2017) · JAMA Ophthalmology · doi:10.1001/jamaophthalmol.2017.1703 · Cited by 74
Sjögren Syndrome-Associated Small Fiber Neuropathy
Sène, D., Cacoub, P., Authier, F., Haroche, J., Créange, A., Saadoun, D., Amoura, Z., Guillausseau, P.-J., & Lefaucheur, J. (2013) · Medicine · doi:10.1097/md.0000000000000005 · Cited by 71
Self-citationSouayah, N., Chin, R. L., Brannagan, T. H., Latov, N., Green, P. H. R., Kokoszka, A., & Sander, H. W. (2008) · European Journal of Neurology · doi:10.1111/j.1468-1331.2008.02305.x · Cited by 70
Kizawa, M. (2006) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp.2005.084533 · Cited by 64
Sousa, D. (2006) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp.2006.091074 · Cited by 61
Severe polyneuropathy in tangier disease mimicking syringomyelia or leprosy
Gibbels, E., Schaefer, H. E., Runne, U., Schr�der, J. M., Haupt, W. F., & Assmann, G. (1985) · Journal of Neurology · doi:10.1007/bf00313867 · Cited by 56
Glucose intolerance, metabolic syndrome, and neuropathy
Cortez, M. M., Singleton, J. R., & Smith, A. G. (2014) · Handbook of clinical neurology · doi:10.1016/b978-0-444-53480-4.00009-6 · Cited by 53
Efficacy of Venlafaxine in Neuropathic Pain: A Narrative Review of Optimized Treatment
Trouvin, A., Perrot, S., & Lloret‐Linares, C. (2017) · Clinical Therapeutics · doi:10.1016/j.clinthera.2017.05.347 · Cited by 53
Cregg, R., Cox, J. J., Bennett, D., Wood, J. N., & Werdehausen, R. (2014) · British Journal of Pharmacology · doi:10.1111/bph.12788 · Cited by 48
Small fiber neuropathy following vaccination for rabies, varicella or Lyme disease
Souayah, N., Ajroud‐Driss, S., Sander, H. W., Brannagan, T. H., Hays, A. P., & Chin, R. L. (2009) · Vaccine · doi:10.1016/j.vaccine.2009.09.077 · Cited by 35
Impact of pain on cognitive functions in primary Sjögren syndrome with small fiber neuropathy
Indart, S., Hugon, J., Guillausseau, P. J., Gilbert, A., Dumurgier, J., Paquet, C., & Sène, D. (2017) · Medicine · doi:10.1097/md.0000000000006384 · Cited by 28
Self-citationFamilial amyloid polyneuropathy: When does it stop to be asymptomatic and need a treatment?
Adams, D., Beaudonnet, G., Adam, C., Lacroix, C., Théaudin, M., Cauquil, C. A., & Labeyrie, C. (2016) · Revue Neurologique · doi:10.1016/j.neurol.2016.08.007 · Cited by 26
Sène, D., Authier, F., Amoura, Z., Cacoub, P., & Lefaucheur, J. P. (2010) · La Revue de Médecine Interne · doi:10.1016/j.revmed.2010.07.022 · Cited by 23
Self-citationNeuropathies douloureuses et atteinte des petites fibres
Lefaucheur, J. P. (2014) · Revue Neurologique · doi:10.1016/j.neurol.2014.10.008 · Cited by 21
Munday, W. R., McNiff, J. M., Watsky, K. L., DiCapua, D. B., & Galan, A. (2015) · Journal of Cutaneous Pathology · doi:10.1111/cup.12484 · Cited by 16
Sturm, D., Schmidt‐Wilcke, T., Greiner, T., Maier, C., Schargus, M., Tegenthoff, M., & Vorgerd, M. (2016) · Case Reports in Neurology · doi:10.1159/000446538 · Cited by 16
Franques, J., Sahuc, P., Dussol, B., Pénaranda, G., Swiader, L., Froissart, R., Attarian, S., Stavris, C., Chiche, L., & Pouget, J. (2017) · Revue Neurologique · doi:10.1016/j.neurol.2017.05.007 · Cited by 11
Finsterer, J., Wanschitz, J., Quasthoff, S., Iglseder, S., Löscher, W. N., & Grisold, W. (2017) · Acta Neurologica Scandinavica · doi:10.1111/ane.12758 · Cited by 11
Small-Fiber Neuropathy/Neuronopathy Associated With Celiac Disease
Brannagan, T. H., Hays, A. P., Chin, S. S., Sander, H. W., Chin, R. L., Magda, P., Green, P. H. R., & Latov, N. (2005) · Archives of Neurology · doi:10.1001/archneur.62.10.1574 · Cited by 5
Research works printed but not linked: 1
- Sene, 2010, [Small fibre neuropathy: diagnostic approach and therapeutic issues, and its association with primary Sjogren's syndrome], Rev Med Interne, 31, 677
Cited by
Sorted by citations
Basic mechanisms of peripheral nerve injury and treatment via electrical stimulation
Chu, X.-L., Song, X.-Z., Li, Q., Li, Y.-R., He, F., Gu, X.-S., & Ming, D. (2022) · Neural Regeneration Research · doi:10.4103/1673-5374.335823 · Cited by 164
Vitamin B-6-Induced Neuropathy: Exploring the Mechanisms of Pyridoxine Toxicity
Hadtstein, F., & Vrolijk, M. F. (2021) · Advances in Nutrition · doi:10.1093/advances/nmab033 · Cited by 120
Small-fiber neuropathy definition, diagnosis, and treatment
Basantsova, N. Y., Starshinova, A. A., Dori, A., Zinchenko, Y. S., Yаblonskiy, P. K., & Shoenfeld, Y. (2019) · Neurological Sciences · doi:10.1007/s10072-019-03871-x · Cited by 81
Current View of Diagnosing Small Fiber Neuropathy
Raasing, L. R. M., Vogels, O. J. M., Veltkamp, M., van Swol, C. F. P., & Grutters, J. C. (2020) · Journal of Neuromuscular Diseases · doi:10.3233/jnd-200490 · Cited by 68
Hereditary transthyretin-related amyloidosis
Finsterer, J., Iglseder, S., Wanschitz, J., Topakian, R., Löscher, W. N., & Grisold, W. (2018) · Acta Neurologica Scandinavica · doi:10.1111/ane.13035 · Cited by 64
Metadata
- 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)
- 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
- Locations
- Joint Bone Spine (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Physiology (medical) › Pain Mechanisms and Treatments
- Health Sciences › Medicine › Clinical 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
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review
