NEuroMuscular Omnicentre, Fondazione Serena Onlus, Piazza Ospedale Maggiore 3, 20162 Milano, Italy; Neurorehabilitation Unit, University of Milan, Italy.
NEuroMuscular Omnicentre, Fondazione Serena Onlus, Piazza Ospedale Maggiore 3, 20162 Milano, Italy.
Clin Neurophysiol. 2021 Jul;132(7):1564-1571. doi: 10.1016/j.clinph.2021.02.394. Epub 2021 Apr 7.
This study examined neurophysiological (NI), split-hand (SI) and split-leg (SLI) index in patients with amyotrophic lateral sclerosis (ALS), and their correlation with functional status, disease duration, staging and survival.
Eighty-two patients underwent nerve conduction study to analyze NI, SI and SLI. Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R), disease progression rate (ΔFS), Milano-Torino (MiToS) and King's staging systems, Forced Vital Capacity (FVC), and survival data were collected.
Both NI and SI indices were significantly associated with ALSFRS-R, MiToS, King's and FVC. Slow progressor patients (ΔFS < 0.5) reported a significantly higher NI and SI values compared to both normal (0.5 ≤ ΔFS < 1.00) and fast progressors (ΔFS ≥ 1.0). After dichotomizing patients in slow progressors (ΔFS < 0.5) and not-slow progressors (ΔFS ≥ 0.5), a combination of SI index and disease duration revealed to be the best prediction model to discriminate patients in accordance with their disease progression (c-index: 0.92), leading to a new prognostic index: the 'Split-Hand prognostic index' (SHpi).
SI and NI are correlated with functional status and FVC. SHpi index could represent an useful tool to discriminate patients in accordance with their disease progression.
These data provide novel evidence of neurophysiological indices as promising biomarkers in ALS.
本研究旨在检测肌萎缩侧索硬化症（ALS）患者的神经生理（NI）、手部分离（SI）和腿部分离（SLI）指数，并分析其与功能状态、疾病持续时间、分期和生存的相关性。
82 名患者接受神经传导研究以分析 NI、SI 和 SLI。收集肌萎缩侧索硬化功能评定量表修订版（ALSFRS-R）、疾病进展率（ΔFS）、米兰-都灵（MiToS）和金氏分期系统、用力肺活量（FVC）和生存数据。
NI 和 SI 指数与 ALSFRS-R、MiToS、金氏和 FVC 均显著相关。与正常（0.5≤ΔFS<1.00）和快速进展者（ΔFS≥1.0）相比，进展缓慢（ΔFS<0.5）的患者报告的 NI 和 SI 值明显更高。将患者分为进展缓慢（ΔFS<0.5）和非进展缓慢（ΔFS≥0.5）后，SI 指数和疾病持续时间的组合被证明是区分患者疾病进展的最佳预测模型（C 指数：0.92），导致新的预后指数：“手部分离预后指数”（SHpi）。
SI 和 NI 与功能状态和 FVC 相关。SHpi 指数可作为一种有用的工具，根据疾病进展来区分患者。
这些数据为神经生理指数作为 ALS 有前途的生物标志物提供了新的证据。
