Langlois V, Bedat Millet A-L, Lebesnerais M, Miranda S, Marguet F, Benhamou Y, Marcorelles P, Lévesque H
Service de médecine interne et maladies infectieuses, CH Le Havre, 29, avenue Pierre-Mendès, 76290 Montivilliers, France; U1096, service de médecine interne, Normandie univ, UNIROUEN, 76000 Rouen, France.
Département de neurophysiologie, CHU de Rouen, 1, rue de Germont, 76031 Rouen, France.
Rev Med Interne. 2018 Feb;39(2):99-106. doi: 10.1016/j.revmed.2017.03.013. Epub 2017 Apr 11.
Small fiber neuropathy (SFN) is still unknown. Characterised by neuropathic pain, it typically begins by burning feet, but could take many other expression. SFN affects the thinly myelinated Aδ and unmyelinated C-fibers, by an inherited or acquired mechanism, which could lead to paresthesia, thermoalgic disorder or autonomic dysfunction. Recent studies suggest the preponderant role of ion channels such as Nav1.7. Furthermore, erythromelalgia or burning mouth syndrome are now recognized as real SFN. Various aetiologies of SFN are described. It could be isolated or associated with diabetes, impaired glucose metabolism, vitamin deficiency, alcohol, auto-immune disease, sarcoidosis etc. Several mutations have recently been identified, like Nav1.7 channel leading to channelopathies. Diagnostic management is based primarily on clinical examination and demonstration of small fiber dysfunction. Laser evoked potentials, Sudoscan, cutaneous biopsy are the main test, but had a difficult access. Treatment is based on multidisciplinary management, combining symptomatic treatment, psychological management and treatment of an associated etiology.
小纤维神经病变(SFN)的病因仍不明确。其特征为神经性疼痛,通常始于足部灼痛,但也可能有许多其他表现形式。SFN通过遗传或后天机制影响薄髓鞘Aδ纤维和无髓鞘C纤维,这可能导致感觉异常、热痛觉障碍或自主神经功能障碍。最近的研究表明离子通道如Nav1.7起主要作用。此外,红斑性肢痛症或灼口综合征现在被认为是真正的SFN。文中描述了SFN的各种病因。它可能是孤立的,也可能与糖尿病、糖代谢受损、维生素缺乏、酒精、自身免疫性疾病、结节病等相关。最近已鉴定出几种突变,如导致通道病的Nav1.7通道。诊断管理主要基于临床检查和小纤维功能障碍的证明。激光诱发电位、Sudoscan、皮肤活检是主要检查方法,但难以实施。治疗基于多学科管理,结合对症治疗、心理管理和相关病因的治疗。