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周围神经兴奋性过高综合征和肌萎缩侧索硬化症中的异位冲动产生。

Ectopic impulse generation in peripheral nerve hyperexcitability syndromes and amyotrophic lateral sclerosis.

机构信息

Brain and Mind Centre, Sydney Medical School, University of Sydney, Sydney, NSW, Australia.

St Vincent's Clinical School, University of New South Wales, NSW, Australia.

出版信息

Clin Neurophysiol. 2018 May;129(5):974-980. doi: 10.1016/j.clinph.2018.01.061. Epub 2018 Feb 10.

DOI:10.1016/j.clinph.2018.01.061
PMID:29554580
Abstract

OBJECTIVE

To elucidate differences in the distribution and firing frequency of fasciculations between peripheral nerve hyperexcitability syndromes and amyotrophic lateral sclerosis (ALS) and to explore the generator site of fasciculations.

METHODS

Ultrasound of 14 preselected muscles was performed in patients with peripheral hyperexcitability and ALS. The distribution and firing frequency of fasciculations were calculated. Cortical excitability assessment was also done by threshold tracking transcranial magnetic stimulation.

RESULTS

In total, 518 muscles from 37 peripheral hyperexcitability patients and 756 muscles from 54 ALS patients were examined. Regarding the detection rate, 74% of muscles in ALS patients demonstrated fasciculations, compared with 34% of muscles in peripheral hyperexcitability patients (P < 0.001). The number of unique repeating focal muscle fasciculation movements per muscle and firing frequency of individual fasciculations in ALS were both significantly higher than those in peripheral hyperexcitability (P < 0.001). Furthermore, cortical silent period duration negatively correlated with the number and firing frequency of fasciculations in ALS (P < 0.05). A similar relationship was not evident in peripheral hyperexcitability.

CONCLUSIONS

In ALS patients, fasciculations were more widespread, greater in number and higher in firing frequency than in peripheral hyperexcitability patients.

SIGNIFICANCE

A significant proportion of fasciculations in ALS may be influenced by changes in central excitability.

摘要

目的

阐明神经兴奋性过高综合征和肌萎缩侧索硬化症(ALS)之间肌束震颤的分布和发放频率的差异,并探讨肌束震颤的发生部位。

方法

对周围神经兴奋性过高和 ALS 患者的 14 块预选肌肉进行超声检查。计算肌束震颤的分布和发放频率。还通过阈追踪经颅磁刺激评估皮质兴奋性。

结果

共检查了 37 例周围神经兴奋性过高患者的 518 块肌肉和 54 例 ALS 患者的 756 块肌肉。就检出率而言,ALS 患者 74%的肌肉出现肌束震颤,而周围神经兴奋性过高患者仅有 34%(P<0.001)。ALS 患者每块肌肉的独特重复局灶性肌束震颤运动次数和单个肌束震颤的发放频率均明显高于周围神经兴奋性过高患者(P<0.001)。此外,皮质静息期持续时间与 ALS 患者肌束震颤的数量和发放频率呈负相关(P<0.05)。在周围神经兴奋性过高患者中,这种关系并不明显。

结论

与周围神经兴奋性过高患者相比,ALS 患者的肌束震颤分布更广泛,数量更多,发放频率更高。

意义

ALS 中相当一部分肌束震颤可能受到中枢兴奋性变化的影响。

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