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Feasibility of assessing progression of transthyretin amyloid polyneuropathy using nerve conduction studies: Findings from the Transthyretin Amyloidosis Outcomes Survey (THAOS)
Journal of the Peripheral Nervous System ( IF 3.8 ) Pub Date : 2021-04-12 , DOI: 10.1111/jns.12444
Márcia Waddington-Cruz 1 , Yukio Ando 2 , Leslie Amass 3 , Jan Kiszko 3 , Doug Chapman 3 , Yoshiki Sekijima 4 ,
Affiliation  

Patients with transthyretin amyloid polyneuropathy (ATTR-PN) show decreased motor and sensory nerve amplitudes and conduction. Electrophysiological changes over time may be sensitive indicators of progression. This analysis from the Transthyretin Amyloidosis Outcomes Survey (THAOS) assessed longitudinal changes in nerve conduction as signals of neurologic disease progression in patients with hereditary ATTR (ATTRv) amyloidosis. Patients with ATTRv in THAOS with recorded nerve conduction values were included (data cut-off: January 6, 2020); changes in nerve amplitude and velocity over time were assessed. Patients (n = 1389) were 45.0% male; 80.4% were the Val30Met (p.Val50Met) genotype. Mean (SD) age at enrollment was 43.6 (14.5) years; duration of symptoms was 9.3 (6.4) years. Median (10th, 90th percentile) sural nerve amplitude and velocity was 18.0 (4.9, 35.0) μV and 50.7 (41.0, 57.9) m/s; peroneal conduction was 13.0 (4.4, 27.0) μV and 51.0 (41.7, 59.7) m/s, respectively. Median (10th, 90th percentile) percentage change from baseline in sural nerve amplitude was variable, but generally decreased over time from −7.4 (−43.2, 52.4) at year 1 to −14.4 (−76.9, 46.7) at year 8. Percent change from baseline in sural nerve velocity declined similarly: −0.1 (−14.5, 15.3) at year 1 and − 6.4 (−21.3, 10.5) at year 8. The decline was more pronounced in patients with greater disability at baseline. Similar patterns were observed for the peroneal nerve. These data show an association between nerve amplitudes and velocities and disease severity, suggesting progressive deterioration in nerve conduction may be an indicator of ATTRv amyloidosis disease progression.

中文翻译:

使用神经传导研究评估转甲状腺素蛋白淀粉样多发性神经病进展的可行性:转甲状腺素蛋白淀粉样变性结果调查 (THAOS) 的结果

甲状腺素运载蛋白淀粉样多发性神经病 (ATTR-PN) 患者表现出运动和感觉神经振幅和传导降低。随着时间的推移,电生理变化可能是进展的敏感指标。这项来自转甲状腺素蛋白淀粉样变性结果调查 (THAOS) 的分析评估了神经传导的纵向变化,作为遗传性 ATTR (ATTRv) 淀粉样变性患者神经系统疾病进展的信号。纳入 THAOS 中具有记录神经传导值的 ATTRv 患者(数据截止日期:2020 年 1 月 6 日);评估了神经振幅和速度随时间的变化。患者 (n = 1389) 为 45.0% 男性;80.4% 是 Val30Met (p.Val50Met) 基因型。入学时的平均 (SD) 年龄为 43.6 (14.5) 岁;症状持续时间为 9.3 (6.4) 年。中位数(第 10 位,第 90 个百分位)腓肠神经振幅和速度分别为 18.0(4.9、35.0)μV 和 50.7(41.0、57.9)m/s;腓骨传导分别为 13.0 (4.4, 27.0) μV 和 51.0 (41.7, 59.7) m/s。腓肠神经振幅相对于基线的中位数(第 10、90 个百分位数)百分比变化是可变的,但通常随着时间的推移从第 1 年的 -7.4(-43.2、52.4)下降到第 8 年的-14.4(-76.9、46.7)。百分比变化腓肠神经速度与基线相比同样下降:第 1 年为 -0.1 (-14.5, 15.3),第 8 年为 -6.4 (-21.3, 10.5)。基线残疾程度较高的患者下降更为明显。腓神经也观察到了类似的模式。这些数据显示了神经振幅和速度与疾病严重程度之间的关联,
更新日期:2021-06-21
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