Frequent atrophic groups with mixed-type myofibers is distinctive to motor neuron syndromes
Frequent atrophic groups with mixed-type myofibers is distinctive to motor neuron syndromes
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DOI：
10.1002/mus.20755
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发表时间：
2007-07-01
期刊：
影响因子：
3.4
通讯作者：
Pestronk, Alan
中科院分区：
文献类型：
--
作者：
Baloh, Robert H.;Rakowicz, Wojtek;Pestronk, Alan
本研究旨在确定运动神经元疾病的肌肉去神经支配模式是否有显著特征。我们首先比较了肌萎缩侧索硬化症（ALS）或肯尼迪病患者的肌肉活检与其他原因的去神经。萎缩肌纤维组，个别组包含纤维类型I和11，经常发生在运动神经元疾病，但不是其他原因的去神经支配。然后，我们确定了11个额外的肌肉活检与频繁萎缩组含有混合纤维类型。病历审查显示，10例患者最终诊断为运动神经元疾病或ALS，1例患者患有多灶性运动神经病。我们的结论是，肌肉活检可能有诊断效用的早期运动神经元疾病的过程中。常见的萎缩性肌群中含有混合纤维类型的肌活检模式应提示运动神经元综合征或运动神经病的诊断。
This study was performed to determine whether there are distinctive features to the pattern of muscle denervation in motor neuron disease. We first compared muscle biopsies from patients with amyotrophic lateral sclerosis (ALS) or Kennedy's disease with other causes of denervation. Groups of atrophic muscle fibers, with individual groups containing both fiber types I and 11, occurred frequently in motor neuron disease but not other causes of denervation. We then identified 11 additional muscle biopsies with frequent atrophic groups containing mixed fiber types. Chart review revealed that 10 patients had a final diagnosis of motor neuron disease or ALS and one had multifocal motor neuropathy. We conclude that muscle biopsy may have diagnostic utility early in the course of motor neuron disease. The muscle biopsy pattern of frequent atrophic groups containing mixed fiber types should suggest a diagnosis of a motor neuron syndrome or motor neuropathy.