Early diagnosis of ALS: The search for signs of denervation in clinically normal muscles

Early diagnosis of ALS: The search for signs of denervation in clinically normal muscles
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DOI:
10.1016/j.jns.2007.07.008
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发表时间:
2007-12-15
影响因子:
4.4
通讯作者:
Zwarts, Machiel J.
Zwarts, Machiel J.
中科院分区:
医学3区
文献类型:
--
作者:
Blijham, Paul J.;Schelhaas, H. Jurgen;Zwarts, Machiel J.

文献摘要

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目的和方法:我们前瞻性地研究了肌萎缩侧索硬化症(ALS)的早期诊断是否可以通过在临床和肌电图未受影响的区域的肌肉中显示去神经支配的迹象来促进。肌纤维传导速度(MFCV)测定在18例患者中的诊断ALS被认为是,但没有建立超过临床可能的ALS水平,根据修订后的埃尔埃斯科里亚标准。从同一肌肉获得肌肉活检,以证明神经源性变化。该研究遵循的准则,从STARD initiation.Results和结论:结果进行了分析,就最终诊断。平均随访16个月后,9例患者发生了可能或明确的ALS。MFCV异常诊断ALS的敏感性为89%。肌肉活检证实失神经支配是MFCV异常的原因。我们的结论是,MFCV可用于检测失神经支配的肌肉,显示没有下运动神经元疾病的临床或肌电图的迹象,因此可能有助于早期诊断可能的实验室支持的ALS。(c)2007年爱思唯尔B。V.保留所有权利。
Aim and methods: We prospectively investigated whether early diagnosis of amyotrophic lateral sclerosis (ALS) could be facilitated by demonstrating signs of denervation in a muscle of a clinical and electromyographical unaffected region. Muscle fibre conduction velocity (MFCV) was determined in 18 patients in whom the diagnosis ALS was considered but not established beyond a level of clinically possible ALS according to the revised El Escorial criteria. A muscle biopsy was obtained from the same muscle, to demonstrate neurogenic changes. The study followed the guidelines from the STARD initiative.Results and conclusion: Results were analysed with respect to the final diagnosis. After a mean follow-up of 16 months, 9 patients developed probable or definite ALS. Sensitivity of abnormal MFCV for developing ALS was 89%. Muscle biopsy confirmed that denervation was the cause of abnormal MFCV. We concluded that MFCV can be used to detect denervation in muscles that show no clinical or electromyographical signs of lower motor neuron disease, and thus may contribute to early diagnosis of probable laboratory-supported ALS. (c) 2007 Elsevier B. V. All rights reserved.