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首页> 外文期刊>Amyotrophic lateral sclerosis and other motor neuron disorders: Official publication of the World Federation of Neurology, Research Group on Motor Neuron Diseases >Pathophysiological significance of fasciculations in the early diagnosis of ALS
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Pathophysiological significance of fasciculations in the early diagnosis of ALS

机译：纤毛在ALS的早期诊断中的病理生理意义

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In amyotrophic lateral sclerosis (ALS), muscles with preserved strength can have fasciculation potentials (FPs) as the only abnormality. The FPs in strong muscles are predominatly simple and stable, and can often be recruited by a slight voluntary contraction. In weak and atrophic muscles, the FPs are generally complex and unstable, tend to have a slower firing rate and are not recruited by voluntary contraction. Macro-electromyography studies suggest that these FPs are part of a more complex motor unit, as opposed to simple FPs which can represent all the motor unit. FPs driven by transcranial magnetic stimulation (TMS) were observed in 13 ALS patients. TMS-driven fasciculations had a simple morphology and were stable. Complex potentials were never cortically driven. These observations strengthen the conviction that fasciculations in ALS can have different origins. Simple, stable FPs arise proximally and are probably related to excitotoxicity phenomena, while complex, unstable FPs are most likely to originate in distal axonal sprouts that are associated with the reinnervation process. Some ALS patients with fatigue and mild weakness can have profuse fasciculations at an early phase in the evolution of the disease. Patients with denervation localized in one region and diffuse FPs should be strongly suspected of having ALS. With this strategy it should be possible to shorten the diagnosis time.
机译：在肌萎缩性侧索硬化症（ALS）中，强度保持不变的肌肉可能具有束缚电位（FPs）作为唯一异常。强壮肌肉中的FP主要是简单而稳定的，通常可以通过轻微的自愿收缩而募集。在肌肉萎缩和萎缩的肌肉中，FP通常很复杂且不稳定，射击速度较慢，并且不会因自愿收缩而募集。宏观肌电图研究表明，这些FP是更复杂的电机单元的一部分，而不是可以代表所有电机单元的简单FP。在13例ALS患者中观察到了经颅磁刺激（TMS）驱动的FP。 TMS驱动的细纹形态简单且稳定。复杂的潜力从来没有被驱动。这些发现强化了人们的信念，即ALS的迷恋可能有不同的起源。简单，稳定的FPs在近端出现，可能与兴奋性毒性现象有关，而复杂，不稳定的FPs最有可能起源于与神经支配过程相关的远端轴突芽。一些患有疲劳和轻度无力的ALS患者在疾病发展的早期阶段可能会出现大量发作。失神经的患者局限于一个区域，且弥漫性FPs应该强烈怀疑患有ALS。通过这种策略，应该可以缩短诊断时间。

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