Axonal polyneuropathy is the main feature of hereditary transthyretin amyloidosis (ATTRv). Nerve morphological abnormalities have been reported, but longitudinal changes have never been assessed. We performed a prospective widespread nerve ultrasound evaluation and nerve cross-sectional area (CSA) was compared with baseline data in both ATTRv patients and pre-symptomatic carriers. Thirty-eight subjects were evaluated (mean follow-up 17.1 months), among them 21 had polyneuropathy while 17 were pre-symptomatic carriers. CSA significantly increased at brachial plexus in both groups (p = 0.008 and p = 0.012) pointing to progressive brachial plexus enlargement as a longitudinal biomarker of both disease progression and disease occurrence in pre-symptomatic carriers.

中文翻译：

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遗传性转甲状腺素蛋白淀粉样变性的进行性臂丛神经增大。

轴索性多发性神经病是遗传性转甲状腺素蛋白淀粉样变性（ATTRv）的主要特征。已经报告了神经形态学异常，但从未评估过纵向变化。我们进行了一项前瞻性广泛的神经超声评估，并将神经横截面积 (CSA) 与 ATTRv 患者和症状前携带者的基线数据进行了比较。对 38 名受试者进行了评估（平均随访 17.1 个月），其中 21 名患有多发性神经病，而 17 名是症状前携带者。两组的臂丛神经 CSA 均显着增加（p = 0.008 和 p = 0.012），表明进行性臂丛扩大是症状前携带者疾病进展和疾病发生的纵向生物标志物。