Gwathmey Kelly Graham
Continuum (Minneap Minn). 2017 Oct;23(5, Peripheral Nerve and Motor Neuron Disorders):1411-1436. doi: 10.1212/CON.0000000000000518.
This article describes the methods of diagnosis and management of the sensory-predominant polyneuropathies. To simplify the approach to this category of patients, sensory-predominant polyneuropathies are divided broadly into either small fiber (or pain-predominant) neuropathies and large fiber (or ataxia-predominant) neuropathies, of which the sensory neuronopathies (dorsal root ganglionopathies) are highlighted.
Physicians can now easily perform skin biopsies in their offices, allowing access to the gold standard pathologic diagnostic tool for small fiber neuropathies. Additional diagnostic techniques, such as corneal confocal microscopy, are emerging. Recently, small fiber neuropathies have been associated with a broader spectrum of diseases, including fibromyalgia, sodium channel mutations, and voltage-gated potassium channel antibody autoimmune disease.
Despite advances in diagnosing small fiber neuropathies and sensory neuronopathies, many of these neuropathies remain refractory to treatment. In select cases, early identification and treatment may result in better outcomes. "Idiopathic" should be a diagnosis of exclusion and a thorough investigation for treatable causes pursued.
本文描述了以感觉为主的多发性神经病的诊断和管理方法。为简化对这类患者的诊疗方法,以感觉为主的多发性神经病大致分为小纤维(或疼痛为主型)神经病和大纤维(或共济失调为主型)神经病,其中突出介绍了感觉神经元病(背根神经节病)。
医生现在可以在办公室轻松进行皮肤活检,从而能够使用小纤维神经病的金标准病理诊断工具。其他诊断技术,如角膜共聚焦显微镜检查,也正在兴起。最近,小纤维神经病与更广泛的疾病相关,包括纤维肌痛、钠通道突变和电压门控钾通道抗体自身免疫性疾病。
尽管在诊断小纤维神经病和感觉神经元病方面取得了进展,但许多此类神经病仍难以治疗。在某些情况下,早期识别和治疗可能会带来更好的结果。“特发性”应作为排除性诊断,并应彻底调查可治疗的病因。