Department of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden.
Department of Neurology, Karolinska University Hospital, Stockholm, Sweden.
Eur J Neurol. 2022 Sep;29(9):2810-2822. doi: 10.1111/ene.15428. Epub 2022 Jun 20.
To investigate the diagnostic and prognostic value of axonal injury biomarkers in patients with inflammatory polyneuropathies.
Neurofilament light chain (NfL) and total tau (T-tau) were measured in the cerebrospinal fluid (CSF) and plasma in 41 patients with Guillain-Barré syndrome (GBS), 32 patients with chronic inflammatory demyelinating polyneuropathy (CIDP), 10 with paraproteinemia-related demyelinating polyneuropathy (PDN), and 8 with multifocal motor neuropathy (MMN), in comparison with 39 disease-free controls and 59 other controls. Outcome was measured with the GBS-disability score (GBS-ds) or Inflammatory Neuropathy Cause and Treatment (INCAT) disability score.
Neurofilament light chain levels in CSF and plasma were higher in GBS, CIDP, and PDN vs. disease-free controls. Patients with MMN had higher NfL levels in plasma vs. disease-free controls, but lower levels in CSF and plasma vs. patients with amyotrophic lateral sclerosis (ALS). T-tau levels in plasma were higher in GBS, CIDP, PDN, and MMN vs. all control groups. Neurofilament light chain levels in CSF and plasma in patients with GBS correlated with GBS-ds, as higher levels were associated with inability to run after 6 and 12 months. NfL levels in CSF and plasma in CIDP did not correlate significantly with outcome.
Acute and chronic inflammatory neuropathies are associated with an increase in levels of NfL in CSF and plasma, but NfL is validated as a prognostic biomarker only in GBS. NfL could be used in differentiating patients with MMN from ALS. T-tau in plasma is a novel biomarker that could be used in a diagnostic assessment of patients with acute and chronic inflammatory polyneuropathies.
研究轴索损伤生物标志物在炎性多神经病患者中的诊断和预后价值。
在 41 例格林-巴利综合征(GBS)患者、32 例慢性炎症性脱髓鞘性多发性神经病(CIDP)患者、10 例与副蛋白血症相关的脱髓鞘性多发性神经病(PDN)患者和 8 例多发性运动神经病(MMN)患者的脑脊液(CSF)和血浆中测量神经丝轻链(NfL)和总 tau(T-tau),并与 39 例无病对照者和 59 例其他对照者进行比较。采用 GBS 残疾评分(GBS-ds)或炎症性神经病病因和治疗(INCAT)残疾评分来评估预后。
与无病对照组相比,CSF 和血浆中的 NfL 水平在 GBS、CIDP 和 PDN 中均升高。与无病对照组相比,MMN 患者的血浆 NfL 水平升高,但 CSF 和血浆中的 NfL 水平均低于肌萎缩侧索硬化症(ALS)患者。与所有对照组相比,GBS、CIDP、PDN 和 MMN 患者的血浆 T-tau 水平升高。GBS 患者 CSF 和血浆中的 NfL 水平与 GBS-ds 相关,因为较高的水平与 6 个月和 12 个月后无法跑步有关。CIDP 患者 CSF 和血浆中的 NfL 水平与预后无显著相关性。
急性和慢性炎性神经病患者的 CSF 和血浆 NfL 水平升高,但 NfL 仅在 GBS 中被验证为预后生物标志物。NfL 可用于区分 MMN 患者和 ALS 患者。T-tau 在血浆中是一种新的生物标志物,可用于急性和慢性炎性多神经病患者的诊断评估。