Department of Neurology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, 466-8550, Japan.
Division of Advanced Medicine, Nagoya University Hospital, 65 Tsurumai-cho, Showa-ku, Nagoya, 466-8560, Japan.
J Neurol. 2021 Oct;268(10):3835-3844. doi: 10.1007/s00415-021-10537-2. Epub 2021 Apr 2.
To clarify whether serum neurofilament light chains (NfLs) serve as a biomarker of axonal damage in patients with chronic inflammatory demyelinating polyneuropathy (CIDP), especially in patients with anti-neurofascin 155 (NF155) antibodies.
The Simoa system was used to examine serum NfL levels from 58 patients with CIDP, including 13 anti-NF155 antibody-positive patients, and from 14 age- and sex-matched healthy individuals. Serum NfL levels were evaluated before and after treatment in eight patients with anti-NF155 antibodies. Clinical features, electrophysiological findings, and cerebrospinal fluid (CSF) protein levels, were evaluated. The pathological features of sural nerves from 40 patients were also examined.
Serum NfL levels were significantly higher in patients with CIDP than in healthy individuals (median 29.63 vs. 7.71 pg/mL, p < 0.001) and were correlated with both modified Rankin Scale scores (r = 0.584, p < 0.001) and CSF protein levels (r = 0.432, p = 0.001). The NfL levels of anti-NF155 antibody-positive patients were higher than those of antibody-negative patients (p = 0.005). Serum NfL levels were negatively correlated with compound muscle action potential amplitudes of the tibial nerves (r = - 0.404, p = 0.004) and positively correlated with the degree of active axonal degeneration in the pathological findings (r = 0.485, p = 0.001). In the antibody-positive group, NfL levels and antibody titers decreased after treatment in all examined patients.
Serum NfL correlated with pathological indices of axonal degeneration, and may serve as a biomarker that reflects active axonal damage of CIDP.
阐明血清神经丝轻链（NfL）是否可作为慢性炎症性脱髓鞘性多发性神经病（CIDP）患者轴索损伤的生物标志物，特别是抗神经节苷脂 155（NF155）抗体阳性患者。
采用 Simoa 系统检测 58 例 CIDP 患者（包括 13 例抗 NF155 抗体阳性患者）和 14 名年龄和性别匹配的健康个体的血清 NfL 水平。对 8 例抗 NF155 抗体阳性患者进行了治疗前后的血清 NfL 水平评估。评估了临床特征、电生理发现和脑脊液（CSF）蛋白水平。还检查了 40 例患者的腓肠神经病理特征。
CIDP 患者的血清 NfL 水平明显高于健康个体（中位数 29.63 比 7.71 pg/ml，p<0.001），与改良 Rankin 量表评分（r=0.584，p<0.001）和 CSF 蛋白水平（r=0.432，p=0.001）均相关。抗 NF155 抗体阳性患者的 NfL 水平高于抗体阴性患者（p=0.005）。血清 NfL 水平与腓肠神经复合肌肉动作电位幅度呈负相关（r=-0.404，p=0.004），与病理发现的活跃轴索变性程度呈正相关（r=0.485，p=0.001）。在抗体阳性组中，所有患者治疗后 NfL 水平和抗体滴度均下降。
血清 NfL 与轴索变性的病理指标相关，可能是反映 CIDP 活跃轴索损伤的生物标志物。
