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Small fiber neuropathy(SFNP)

MedGen UID:
478336
Concept ID:
C3276706
Disease or Syndrome
Synonym: SFNP
SNOMED CT: Small fiber neuropathy (709489006); Small nerve fiber neuropathy (709489006); SFNP - Small fiber neuropathy (709489006)
 
Monarch Initiative: MONDO:0800207
OMIM®: 133020; 603415

Definition

Small fiber neuropathy is a condition characterized by severe pain attacks that typically begin in the feet or hands. As a person ages, the pain attacks can affect other regions. Some people initially experience a more generalized, whole-body pain. The attacks usually consist of pain described as stabbing or burning, or abnormal skin sensations such as tingling or itchiness. In some individuals, the pain is more severe during times of rest or at night. The signs and symptoms of small fiber neuropathy usually begin in adolescence to mid-adulthood.

Individuals with small fiber neuropathy cannot feel pain that is concentrated in a very small area, such as the prick of a pin. However, they have an increased sensitivity to pain in general (hyperalgesia) and experience pain from stimulation that typically does not cause pain (allodynia). People affected with this condition may also have a reduced ability to differentiate between hot and cold. However, in some individuals, the pain attacks are provoked by cold or warm triggers.

Some affected individuals have urinary or bowel problems, episodes of rapid heartbeat (palpitations), dry eyes or mouth, or abnormal sweating. They can also experience a sharp drop in blood pressure upon standing (orthostatic hypotension), which can cause dizziness, blurred vision, or fainting.

Small fiber neuropathy is considered a form of peripheral neuropathy because it affects the peripheral nervous system, which connects the brain and spinal cord to muscles and to cells that detect sensations such as touch, smell, and pain. [from MedlinePlus Genetics]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSmall fiber neuropathy

Professional guidelines

PubMed

Tavee J, Brannagan TH 3rd, Lenihan MW, Muppidi S, Kellermeyer L, D Donofrio P; AANEM
Muscle Nerve 2023 Oct;68(4):356-374. Epub 2023 Jul 11 doi: 10.1002/mus.27922. PMID: 37432872
Cheshire WP, Freeman R, Gibbons CH, Cortelli P, Wenning GK, Hilz MJ, Spies JM, Lipp A, Sandroni P, Wada N, Mano A, Ah Kim H, Kimpinski K, Iodice V, Idiáquez J, Thaisetthawatkul P, Coon EA, Low PA, Singer W
Clin Neurophysiol 2021 Feb;132(2):666-682. Epub 2020 Dec 22 doi: 10.1016/j.clinph.2020.11.024. PMID: 33419664
Devigili G, Cazzato D, Lauria G
Expert Rev Neurother 2020 Sep;20(9):967-980. Epub 2020 Jul 23 doi: 10.1080/14737175.2020.1794825. PMID: 32654574

Recent clinical studies

Etiology

Liampas A, Parperis K, Erotocritou MF, Nteveros A, Papadopoulou M, Moschovos C, Akil M, Coaccioli S, Hadjigeorgiou GM, Hadjivassiliou M, Zis P
Eur J Neurol 2023 Jan;30(1):255-265. Epub 2022 Sep 23 doi: 10.1111/ene.15555. PMID: 36086910Free PMC Article
Kazamel M, Stino AM, Smith AG
Muscle Nerve 2021 Mar;63(3):285-293. Epub 2020 Oct 24 doi: 10.1002/mus.27086. PMID: 33098165
Devigili G, Cazzato D, Lauria G
Expert Rev Neurother 2020 Sep;20(9):967-980. Epub 2020 Jul 23 doi: 10.1080/14737175.2020.1794825. PMID: 32654574
Siao P, Kaku M
Semin Neurol 2019 Oct;39(5):519-530. Epub 2019 Oct 22 doi: 10.1055/s-0039-1694747. PMID: 31639835
Terkelsen AJ, Karlsson P, Lauria G, Freeman R, Finnerup NB, Jensen TS
Lancet Neurol 2017 Nov;16(11):934-944. doi: 10.1016/S1474-4422(17)30329-0. PMID: 29029847

Diagnosis

Strand N, Wie C, Peck J, Maita M, Singh N, Dumbroff J, Tieppo Francio V, Murphy M, Chang K, Dickerson DM, Maloney J
Curr Pain Headache Rep 2022 Jun;26(6):429-438. Epub 2022 Apr 6 doi: 10.1007/s11916-022-01044-8. PMID: 35384587
Finsterer J, Scorza FA
Acta Neurol Scand 2022 May;145(5):493-503. Epub 2022 Feb 7 doi: 10.1111/ane.13591. PMID: 35130356
Kazamel M, Stino AM, Smith AG
Muscle Nerve 2021 Mar;63(3):285-293. Epub 2020 Oct 24 doi: 10.1002/mus.27086. PMID: 33098165
Devigili G, Cazzato D, Lauria G
Expert Rev Neurother 2020 Sep;20(9):967-980. Epub 2020 Jul 23 doi: 10.1080/14737175.2020.1794825. PMID: 32654574
Zhou L
Semin Neurol 2019 Oct;39(5):570-577. Epub 2019 Oct 22 doi: 10.1055/s-0039-1688977. PMID: 31639840

Therapy

Cohen Tervaert JW, Martinez-Lavin M, Jara LJ, Halpert G, Watad A, Amital H, Shoenfeld Y
Autoimmun Rev 2023 May;22(5):103287. Epub 2023 Feb 3 doi: 10.1016/j.autrev.2023.103287. PMID: 36738954
Strand N, Wie C, Peck J, Maita M, Singh N, Dumbroff J, Tieppo Francio V, Murphy M, Chang K, Dickerson DM, Maloney J
Curr Pain Headache Rep 2022 Jun;26(6):429-438. Epub 2022 Apr 6 doi: 10.1007/s11916-022-01044-8. PMID: 35384587
Finsterer J, Scorza FA
Acta Neurol Scand 2022 May;145(5):493-503. Epub 2022 Feb 7 doi: 10.1111/ane.13591. PMID: 35130356
Kazamel M, Stino AM, Smith AG
Muscle Nerve 2021 Mar;63(3):285-293. Epub 2020 Oct 24 doi: 10.1002/mus.27086. PMID: 33098165
Sopacua M, Hoeijmakers JGJ, Merkies ISJ, Lauria G, Waxman SG, Faber CG
J Peripher Nerv Syst 2019 Mar;24(1):19-33. Epub 2019 Jan 8 doi: 10.1111/jns.12298. PMID: 30569495

Prognosis

Chan ACY, Kumar S, Tan G, Wong HY, Ong JJY, Chandra B, Huang H, Sharma VK, Lai PS
Muscle Nerve 2023 Apr;67(4):259-271. Epub 2022 Nov 30 doi: 10.1002/mus.27752. PMID: 36448457
Liampas A, Parperis K, Erotocritou MF, Nteveros A, Papadopoulou M, Moschovos C, Akil M, Coaccioli S, Hadjigeorgiou GM, Hadjivassiliou M, Zis P
Eur J Neurol 2023 Jan;30(1):255-265. Epub 2022 Sep 23 doi: 10.1111/ene.15555. PMID: 36086910Free PMC Article
Johnson SA, Shouman K, Shelly S, Sandroni P, Berini SE, Dyck PJB, Hoffman EM, Mandrekar J, Niu Z, Lamb CJ, Low PA, Singer W, Mauermann ML, Mills J, Dubey D, Staff NP, Klein CJ
Neurology 2021 Nov 30;97(22):e2236-e2247. Epub 2021 Oct 27 doi: 10.1212/WNL.0000000000012894. PMID: 34706972Free PMC Article
Kazamel M, Stino AM, Smith AG
Muscle Nerve 2021 Mar;63(3):285-293. Epub 2020 Oct 24 doi: 10.1002/mus.27086. PMID: 33098165
Zhou L
Semin Neurol 2019 Oct;39(5):570-577. Epub 2019 Oct 22 doi: 10.1055/s-0039-1688977. PMID: 31639840

Clinical prediction guides

Johnson SA, Shouman K, Shelly S, Sandroni P, Berini SE, Dyck PJB, Hoffman EM, Mandrekar J, Niu Z, Lamb CJ, Low PA, Singer W, Mauermann ML, Mills J, Dubey D, Staff NP, Klein CJ
Neurology 2021 Nov 30;97(22):e2236-e2247. Epub 2021 Oct 27 doi: 10.1212/WNL.0000000000012894. PMID: 34706972Free PMC Article
Baum P, Toyka KV, Blüher M, Kosacka J, Nowicki M
Int J Mol Sci 2021 Oct 7;22(19) doi: 10.3390/ijms221910835. PMID: 34639176Free PMC Article
Cheshire WP, Freeman R, Gibbons CH, Cortelli P, Wenning GK, Hilz MJ, Spies JM, Lipp A, Sandroni P, Wada N, Mano A, Ah Kim H, Kimpinski K, Iodice V, Idiáquez J, Thaisetthawatkul P, Coon EA, Low PA, Singer W
Clin Neurophysiol 2021 Feb;132(2):666-682. Epub 2020 Dec 22 doi: 10.1016/j.clinph.2020.11.024. PMID: 33419664
Kazamel M, Stino AM, Smith AG
Muscle Nerve 2021 Mar;63(3):285-293. Epub 2020 Oct 24 doi: 10.1002/mus.27086. PMID: 33098165
Tavee J, Culver D
Curr Opin Pulm Med 2019 Sep;25(5):533-538. doi: 10.1097/MCP.0000000000000597. PMID: 31365388

Recent systematic reviews

Tayebi A, Samimisedeh P, Jafari Afshar E, Mahmoudnia S, Milan N, Ayati A, Madady A, Rastad H
BMC Neurol 2023 Dec 11;23(1):437. doi: 10.1186/s12883-023-03486-y. PMID: 38082244Free PMC Article
Chan ACY, Kumar S, Tan G, Wong HY, Ong JJY, Chandra B, Huang H, Sharma VK, Lai PS
Muscle Nerve 2023 Apr;67(4):259-271. Epub 2022 Nov 30 doi: 10.1002/mus.27752. PMID: 36448457
Liampas A, Parperis K, Erotocritou MF, Nteveros A, Papadopoulou M, Moschovos C, Akil M, Coaccioli S, Hadjigeorgiou GM, Hadjivassiliou M, Zis P
Eur J Neurol 2023 Jan;30(1):255-265. Epub 2022 Sep 23 doi: 10.1111/ene.15555. PMID: 36086910Free PMC Article
Haroutounian S, Todorovic MS, Leinders M, Campagnolo M, Gewandter JS, Dworkin RH, Freeman R
Muscle Nerve 2021 Feb;63(2):170-177. Epub 2020 Sep 29 doi: 10.1002/mus.27070. PMID: 32989823
Finsterer J, Iglseder S, Wanschitz J, Topakian R, Löscher WN, Grisold W
Acta Neurol Scand 2019 Feb;139(2):92-105. Epub 2018 Oct 23 doi: 10.1111/ane.13035. PMID: 30295933

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