Abstract

Background

Quantitative sensory testing (QST) has been one of the neurophysiological tools used for follow-up and disease progression assessment in ATTRv amyloidosis. We aimed to detect the utility of QST in identifying subclinical neuropathic involvement in ATTRV30M amyloidosis carriers.

Methods

A cohort of ATTRV30M amyloidosis carriers were assessed with vibratory (VDT) and cooling (CDT) detection thresholds and heat pain responses. Subjects were divided into asymptomatic carriers (Group 1), paucisymptomatic carriers (Group 2) and stage 1 ATTRv-PN patients (Group 3). Nonparametric statistics were used for group comparisons.

Results

A total of 207 ATTRV30M amyloidosis carriers (83 males) were included. Of these, 113 subjects were asymptomatic and 94 symptomatic carriers. In asymptomatic carriers, CDT and Heat Pain (HP 5.0 and HP 0.5) were significantly lower when compared to both group of symptomatic carriers (p ≤ 0.005). In Group 3, VDT, CDT and HP 5.0 were significantly higher, when compared to Group 2 (p < 0.05).

Conclusions

QST, in particular CDT, HP 5 and HP 0.5 modalities, seems a good tool to identify subclinical neuropathy in ATTRv amyloidosis carriers, with CDT showing a higher sensitivity to detect and early neuropathic involvement.

中文翻译：

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定量感觉测试：识别 ATTRV30M 淀粉样变性患者亚临床神经病变的好工具？

摘要

背景

定量感觉测试（QST）已成为用于 ATTRv 淀粉样变性随访和疾病进展评估的神经生理学工具之一。我们的目的是检测 QST 在识别 ATTRV30M 淀粉样变性携带者亚临床神经病变方面的效用。

方法

通过振动 (VDT) 和冷却 (CDT) 检测阈值以及热痛反应对 ATTRV30M 淀粉样变性携带者队列进行评估。受试者分为无症状携带者（第 1 组）、少症状携带者（第 2 组）和 1 期 ATTRv-PN 患者（第 3 组）。非参数统计用于组间比较。

结果

总共包括 207 名 ATTRV30M 淀粉样变性携带者（83 名男性）。其中，113 名受试者无症状，94 名有症状携带者。在无症状携带者中，CDT 和热痛（HP 5.0 和 HP 0.5）显着低于两组有症状携带者 ( p  ≤ 0.005)。与第 2 组相比，第 3 组的 VDT、CDT 和 HP 5.0 显着更高 ( p  < 0.05)。

结论

QST，特别是 CDT、HP 5 和 HP 0.5 模式，似乎是识别 ATTRv 淀粉样变性携带者亚临床神经病变的良好工具，CDT 对检测和早期神经病变参与表现出更高的敏感性。