Finsterer Josef, Scorza Fulvio A
Neurology & Neurophysiology Center, Vienna, Austria.
Disciplina de Neurociência, Escola Paulista de Medicina/Universidade Federal de São Paulo/. (EPM/UNIFESP), São Paulo, Brasil.
Acta Neurol Scand. 2022 May;145(5):493-503. doi: 10.1111/ane.13591. Epub 2022 Feb 7.
Small fiber neuropathy (SFN) is a peripheral nervous system disease due to affection of A-delta or C-fibers in a proximal, distal, or diffuse distribution. Selective SFN (without large fiber affection) manifests with pain, sensory disturbances, or autonomic dysfunction. Though uniform diagnostic criteria are unavailable, most of them request typical clinical features and reduced intra-epidermal nerve fiber density on proximal or distal skin biopsy. Little consensus has been reached about the treatment of SFN, why this narrative review aims at summarizing and discussing treatment options for SFN. Treatment of SFN can be classified as symptomatic, pathophysiologic, or causal. Prerequisites for treating SFN are an established diagnosis, knowledge about the symptoms and signs, and the etiology. Pain usually responds to oral/intravenous pain killers, antidepressants, anti-seizure drugs, or topical, transdermal specifications. Some of the autonomic disturbances respond favorably to symptomatic treatment. SFN related to Fabry disease or hATTR are accessible to pathogenesis-related therapy. Immune-mediated SFN responds to immunosuppression or immune-modulation. Several of the secondary SFNs respond to causal treatment of the underlying disorder. In conclusion, treatment of SFN relies on a multimodal concept and includes causative, pathophysiologic, and symptomatic measures. It strongly depends on the clinical presentation, diagnosis, and etiology, why it is crucial before initiation of treatment to fix the diagnosis and etiology. Due to the heterogeneous clinical presentation and multi-causality, treatment of SFN should be individualized with the goal of controlling the underlying cause, alleviating pain, and optimizing functionality.
小纤维神经病变(SFN)是一种周围神经系统疾病,由于A-δ纤维或C纤维在近端、远端或弥漫性分布中受到影响所致。选择性SFN(无大纤维受累)表现为疼痛、感觉障碍或自主神经功能障碍。尽管尚无统一的诊断标准,但大多数标准要求具备典型的临床特征以及近端或远端皮肤活检时表皮内神经纤维密度降低。关于SFN的治疗,目前尚未达成太多共识,因此本叙述性综述旨在总结和讨论SFN的治疗选择。SFN的治疗可分为对症治疗、病理生理治疗或病因治疗。治疗SFN的前提是明确诊断、了解症状和体征以及病因。疼痛通常对口服/静脉用止痛药、抗抑郁药、抗癫痫药或局部、经皮制剂有反应。一些自主神经功能障碍对对症治疗反应良好。与法布里病或遗传性转甲状腺素蛋白淀粉样变相关的SFN可采用发病机制相关治疗。免疫介导的SFN对免疫抑制或免疫调节有反应。一些继发性SFN对潜在疾病的病因治疗有反应。总之,SFN的治疗依赖于多模式概念,包括病因治疗、病理生理治疗和对症治疗措施。这很大程度上取决于临床表现、诊断和病因,因此在开始治疗前明确诊断和病因至关重要。由于临床表现的异质性和多因果关系,SFN的治疗应个体化,目标是控制潜在病因、减轻疼痛并优化功能。