皮层兴奋性可将 ALS 与假性疾病区分开来。

Cortical excitability distinguishes ALS from mimic disorders.

机构信息

Sydney Medical School Westmead, University of Sydney, Australia.

出版信息

Clin Neurophysiol. 2011 Sep;122(9):1860-6. doi: 10.1016/j.clinph.2010.12.062. Epub 2011 Mar 5.

DOI:10.1016/j.clinph.2010.12.062

PMID:21382747

Abstract

OBJECTIVE

The diagnosis of amyotrophic lateral sclerosis (ALS) relies on stringent clinical criteria, resulting in diagnostic delay and inevitably the institution of appropriate therapy. Cortical hyperexcitability, as assessed by the novel threshold tracking transcranial magnetic stimulation (TTTMS) technique, appears as an early feature of ALS. Consequently, the present study assessed the diagnostic utility of threshold tracking TMS and developed algorithms to aid the diagnosis of ALS.

METHODS

Prospective studies were undertaken on a cohort of 156 consecutive patients with neuromuscular symptoms (104 ALS and 52 lower motor neuron syndrome, non-ALS syndrome, NALS) and 62 healthy controls.

RESULTS

Short-interval intracortical inhibition (SICI) was significantly reduced in ALS patients (2.4 ± 0.9%) compared to NALS (8.7 ± 0.8%, P<0.0001) and controls (10.6 ± 0.8%, P < 0.0001). The MEP amplitude and intracortical facilitation were increased, while the cortical silent period duration was reduced in ALS, all indicative of cortical hyperexcitability. Analysis of receiver operating characteristic curves suggested that threshold tracking TMS distinguished ALS from NALS, with averaged (area under curve 0.76, P < 0.0001) and peak SICI 3 ms (area under curve 0.73, P<0.0001) being the most robust diagnostic markers.

CONCLUSIONS

The presence of cortical hyperexcitability distinguishes ALS from mimic disorders.

SIGNIFICANCE

The threshold tracking TMS techniques may prove useful as a diagnostic investigation for ALS.

摘要

目的

肌萎缩侧索硬化症（ALS）的诊断依赖于严格的临床标准，这导致了诊断的延迟，不可避免地需要进行适当的治疗。皮质兴奋性增高，如新型阈跟踪经颅磁刺激（TTTMS）技术评估，是 ALS 的早期特征。因此，本研究评估了阈跟踪 TMS 的诊断效用，并开发了算法来辅助 ALS 的诊断。

方法

前瞻性研究纳入了 156 例连续的神经肌肉症状患者（104 例 ALS 和 52 例下运动神经元综合征，非 ALS 综合征，NALS）和 62 例健康对照。

结果

与 NALS（8.7 ± 0.8%，P<0.0001）和对照组（10.6 ± 0.8%，P<0.0001）相比，ALS 患者的短程皮质内抑制（SICI）明显降低（2.4 ± 0.9%）。MEP 幅度和皮质内易化增加，而 ALS 患者的皮质静息期持续时间缩短，均提示皮质兴奋性增高。ROC 曲线分析表明，阈跟踪 TMS 可将 ALS 与 NALS 区分开来，平均（曲线下面积 0.76，P<0.0001）和峰值 SICI 3ms（曲线下面积 0.73，P<0.0001）是最具稳健性的诊断标志物。