Advances in diagnosis and management of distal sensory polyneuropathies.
Advances in diagnosis and management of distal sensory polyneuropathies.
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远端感觉多发性神经病的诊断和治疗进展。
DOI：
10.1136/jnnp-2021-328489
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发表时间：
2023
期刊：
影响因子：
--
通讯作者：
Simon,NeilG
中科院分区：
文献类型：
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作者：
Silsby,Matthew;Feldman,EvaL;Dortch,RichardD;Roth,Alison;Haroutounian,Simon;Rajabally,YusufA;Vucic,Steve;Shy,MichaelE;Oaklander,AnneLouise;Simon,NeilG
远端感觉多神经病变（DSP）的特征是长度依赖，感觉为主的症状和体征，包括潜在的致残对称慢性疼痛，刺痛和平衡不良。一些患者也有或发展自主神经异常或运动受累，这取决于主要受影响的是大髓鞘纤维还是小纤维。虽然发病率很高，但诊断和治疗可能具有挑战性。虽然经典的糖尿病和中毒原因已经得到了很好的认识，但也有越来越多的不同的关联，包括免疫功能障碍、风湿病和神经退行性疾病。尽管进行了彻底的评估，但大约一半的病例最初被认为是特发性的，但通常，随着新症状的出现或检测的进展，例如采用遗传方法，病因后来才会出现。改进和标准化DSP指标，正如已经完成的运动神经病，将允许在临床纵向跟踪自然病史和治疗反应。标准化表现型可以推进研究，并促进潜在疗法的试验，这一点迄今为止还很落后。这篇综述更新了最近的进展，并总结了目前特定治疗的证据。
Distal sensory polyneuropathy (DSP) is characterised by length-dependent, sensory-predominant symptoms and signs, including potentially disabling symmetric chronic pain, tingling and poor balance. Some patients also have or develop dysautonomia or motor involvement depending on whether large myelinated or small fibres are predominantly affected. Although highly prevalent, diagnosis and management can be challenging. While classic diabetes and toxic causes are well-recognised, there are increasingly diverse associations, including with dysimmune, rheumatological and neurodegenerative conditions. Approximately half of cases are initially considered idiopathic despite thorough evaluation, but often, the causes emerge later as new symptoms develop or testing advances, for instance with genetic approaches. Improving and standardising DSP metrics, as already accomplished for motor neuropathies, would permit in-clinic longitudinal tracking of natural history and treatment responses. Standardising phenotyping could advance research and facilitate trials of potential therapies, which lag so far. This review updates on recent advances and summarises current evidence for specific treatments.