Abstract
Small-fibre neuropathies (SFN) can be defined as diseases of small nerve fibres: thinly myelinated A-δ and unmyelinated C fibres. The clinical manifestations include autonomic symptoms such as dry eyes, dry mouth, dizziness, constipation, bladder incontinence, sexual dysfunction, excessive sweating, and red or white skin discoloration. Patients also have sensory symptoms that are often their main symptom, including pain, pruritus (in about 2/3 of patients), burning, tingling, or numbness. Patient history and physical examination are considered the most important for diagnosing SFN. Diagnosis can be confirmed with quantitative sensory testing (QST) or with skin biopsies, by reduced intraepidermal nerve fibre (IENF) density measurements in the distal leg. SFN are usually idiopathic but may be associated with various diseases, such as metabolic diseases (diabetes), infectious diseases (HIV, hepatitis C infection), inflammatory diseases (Sjögren syndrome), autoimmune diseases, paraneoplastic syndromes, or genetic diseases (Fabry disease). SFN lead to a significant reduction in the overall quality of life. Treatment is based on etiology, and symptomatic treatments (e.g., gabapentin, pregabalin, tricyclic antidepressants) can be used as well.
Keywords
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- AIDS:
-
Acquired immune deficiency syndrome
- BMS:
-
Primary burning mouth syndrome
- DN4:
-
Douleur Neuropathique 4
- HIV:
-
Human immunodeficiency virus
- IENF:
-
Intraepidermal nerve fibre
- NPSI:
-
Neuropathic pain symptom inventory
- PFA:
-
Paraformaldehyde
- PGP9.5:
-
Protein gene product 9.5
- QST:
-
Quantitative sensory testing
- SFN:
-
Small-fibre neuropathies
- TST:
-
Thermoregulatory sweat testing
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Brenaut, E., Misery, L. (2016). Small-Fibre Neuropathies. In: Misery, L., Ständer, S. (eds) Pruritus. Springer, Cham. https://doi.org/10.1007/978-3-319-33142-3_32
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DOI: https://doi.org/10.1007/978-3-319-33142-3_32
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