OBJECTIVE Transthyretin familial amyloid polyneuropathy (TTR-FAP) is an aggressive hereditary neuropathy characterized by sensory and autonomic dysfunction. There are numerous reports of TTR-FAP misdiagnosed and treated as chronic inflammatory demyelinating polyneuropathy (CIDP), leading to delayed diagnosis, risk of iatrogenic adverse events and increased socio-economic costs. Quantitative sudomotor function measured by electrochemical skin conductance (ESC) appears to be a sensitive test in TTR-FAP. We aimed to evaluate the performance of ESC in differentiating TTR-FAP from CIDP. METHODS Thirty-eight patients with genetically confirmed hereditary TTR amyloidosis and 26 with definite CIDP according to the EFNS/PNS guidelines and negative TTR-FAP genetic testing were involved in this study. We compared the ESC for feet and hands measured by Sudoscan for each patient. RESULTS ESC (µS) was significantly lower in TTR-FAP for both hands (72 vs 45, p < 0.0001) and feet (77 vs 35, p < 0.0001). Feet ESC < 64 µS had a 89% sensitivity and a 96% specificity to differentiate between CIDP and TTR-FAP. CONCLUSION Sudoscan is a fast, non-invasive and easy to perform test, able to distinguish CIDP and TTR-FAP patients with good sensitivity and specificity. SIGNIFICANCE Sudoscan can be helpful in distinguishing between CIDP and TTR-FAP.

中文翻译：

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定量促汗试验有助于区分转甲状腺素蛋白家族性淀粉样多发性神经病和慢性炎症性脱髓鞘性多发性神经病

目的 甲状腺素运载蛋白家族性淀粉样多发性神经病 (TTR-FAP) 是一种侵袭性遗传性神经病，其特征是感觉和自主神经功能障碍。有大量报告称 TTR-FAP 被误诊为慢性炎症性脱髓鞘性多发性神经病 (CIDP)，导致诊断延迟、医源性不良事件风险和社会经济成本增加。通过电化学皮肤电导 (ESC) 测量的定量促汗功能似乎是 TTR-FAP 中的敏感测试。我们旨在评估 ESC 在区分 TTR-FAP 与 CIDP 方面的性能。方法 38 名遗传性 TTR 淀粉样变性患者和 26 名根据 EFNS/PNS 指南明确 CIDP 且 TTR-FAP 基因检测阴性的患者参与了这项研究。我们比较了 Sudoscan 为每位患者测量的脚和手的 ESC。结果 在 TTR-FAP 中，双手（72 对 45，p < 0.0001）和脚（77 对 35，p < 0.0001）的 ESC (μS) 显着降低。脚 ESC < 64 µS 具有 89% 的灵敏度和 96% 的特异性来区分 CIDP 和 TTR-FAP。结论 Sudoscan 是一种快速、无创且易于执行的测试，能够以良好的敏感性和特异性区分 CIDP 和 TTR-FAP 患者。意义 Sudoscan 有助于区分 CIDP 和 TTR-FAP。非侵入性且易于执行的测试，能够以良好的敏感性和特异性区分CIDP和TTR-FAP患者。意义 Sudoscan 有助于区分 CIDP 和 TTR-FAP。非侵入性且易于执行的测试，能够以良好的敏感性和特异性区分CIDP和TTR-FAP患者。意义 Sudoscan 有助于区分 CIDP 和 TTR-FAP。