Lefaucheur J-P, Wahab A, Planté-Bordeneuve V, Sène D, Ménard-Lefaucheur I, Rouie D, Tebbal D, Salhi H, Créange A, Zouari H, Ng Wing Tin S
EA 4391, faculté de médecine de Créteil, université Paris-Est de Créteil, 8, avenue du Général-Sarrail, 94010 Créteil, France; Réseau Amylose-Henri-Mondor, hôpital Henri-Mondor, Assistance publique-Hôpitaux de Paris, 51, avenue de Lattre-de-Tassigny, 94010 Créteil, France; Service de physiologie-explorations fonctionnelles, hôpital Henri-Mondor, Assistance publique-Hôpitaux de Paris, 51, avenue de Lattre-de-Tassigny, 94010 Créteil, France.
Service de physiologie-explorations fonctionnelles, hôpital Henri-Mondor, Assistance publique-Hôpitaux de Paris, 51, avenue de Lattre-de-Tassigny, 94010 Créteil, France.
Neurophysiol Clin. 2015 Dec;45(6):445-55. doi: 10.1016/j.neucli.2015.09.012. Epub 2015 Nov 17.
The diagnosis of small fiber neuropathy (SFN) is a challenge for clinical neurophysiology. Conventional nerve conduction studies are inappropriate for this purpose and therefore various neurophysiological tests have been proposed. In this study, we compared the diagnostic value of five of these tests in 87 patients with clinically definite (n=33) or possible (n=54) SFN related to amyloid neuropathy secondary to transthyretin gene mutation or monoclonal gammopathy (n=30), primary Sjögren's syndrome (n=20), Fabry's disease (n=2), or unknown cause (n=35). Neurophysiological tests included quantitative sensory testing with determination of warm and cold detection thresholds (WDT, CDT), recording of laser-evoked potentials (LEP) and sympathetic skin responses (SSRs), and measurement of electrochemical skin conductance (ESC) using Sudoscan(®) device. All tests were performed at the four extremities (hands and feet). All patients with clinically definite SFN and 70% of the patients with possible SFN had at least one abnormal test. The LEP was the most sensitive test (altered in 79% of the patients with at least one abnormal test), followed by ESC (61%), WDT (55%), SSR (41%), and CDT (32%). The combination of LEP, assessing A-delta sensory fibers, WDT, assessing sensory C fibers, and ESC, assessing autonomic C fibers, appears a relevant approach for the diagnosis of SFN. Compared to SSR and CDT, these three tests, LEP, WDT, and ESC, had a significantly better diagnostic sensitivity and their combination further improved diagnostic accuracy.
小纤维神经病变（SFN）的诊断对临床神经生理学而言是一项挑战。传统的神经传导研究不适用于此目的，因此人们提出了各种神经生理学测试方法。在本研究中，我们比较了其中五项测试对87例临床确诊（n = 33）或可能（n = 54）患有SFN患者的诊断价值，这些患者的SFN与转甲状腺素蛋白基因突变或单克隆丙种球蛋白病继发的淀粉样神经病变相关（n = 30）、原发性干燥综合征（n = 20）、法布里病（n = 2）或病因不明（n = 35）。神经生理学测试包括定量感觉测试，测定温暖和寒冷检测阈值（WDT、CDT），记录激光诱发电位（LEP）和交感皮肤反应（SSR），以及使用Sudoscan®设备测量电化学皮肤电导（ESC）。所有测试均在四肢（手和脚）进行。所有临床确诊的SFN患者以及70%可能患有SFN的患者至少有一项测试异常。LEP是最敏感的测试（在至少一项测试异常的患者中，79%出现改变），其次是ESC（61%）、WDT（55%）、SSR（41%）和CDT（32%）。评估A-δ感觉纤维的LEP、评估感觉C纤维的WDT以及评估自主C纤维的ESC相结合，似乎是诊断SFN的一种有效方法。与SSR和CDT相比，这三项测试，即LEP、WDT和ESC，具有显著更高的诊断敏感性，它们的组合进一步提高了诊断准确性。
