Wolf M, Wolf C, Weber M-A
Abteilung für Neuroradiologie, Universitätsklinikum Heidelberg, Im Neuenheimer Feld 400, 69120, Heidelberg, Deutschland.
Neurologie, Reha-Zentrum Gernsbach, Gernsbach, Deutschland.
Radiologe. 2017 Dec;57(12):1038-1051. doi: 10.1007/s00117-017-0316-x.
Neurogenic myopathies are primary diseases of the nervous system, which secondarily result in denervation of the target musculature. The spectrum of potential causes is manifold ranging from acute traumatic injuries and chronic compression to neurodegenerative, inflammatory, metabolic and neoplastic processes.
The medical history, clinical neurological examination, and electrophysiological tests including electromyography and nerve conduction studies are crucial in diagnosing neuropathic myopathies. Electromyography is the gold standard for diagnosing muscle denervation.
Additional imaging methods and magnetic resonance imaging (MRI) in particular, are capable of contributing valuable information. The MRI examination of denervated musculature shows edema, an increase in the apparent diffusion coefficient (ADC) and hyperperfusion. Chronic denervation results in fatty degeneration and atrophy of affected muscles, which are also detectable by MRI.
Although the MRI findings in muscle denervation are relatively unspecific, they show a high sensitivity, comparable to electromyography. Dedicated MR neurography may often visualize the underlying lesion(s) of the innervating nerve(s).
Besides high sensitivity, comparable to electromyography, MRI is capable of evaluating muscles which are inaccessible for needle electromyography. Due to its non-invasive character, MRI is ideal for follow-up examinations.
The use of MRI is often a meaningful addition to the diagnostics of neurogenic myopathies. The extent and distribution pattern of muscular alterations often provide information on the localization of the causative nerve damage. A correct diagnosis or at least a narrowing down of possible differential diagnoses can often be achieved using MRI.
神经源性肌病是神经系统的原发性疾病,继而导致靶肌肉组织的失神经支配。潜在病因范围广泛,从急性创伤性损伤和慢性压迫到神经退行性、炎症性、代谢性和肿瘤性病变。
病史、临床神经学检查以及包括肌电图和神经传导研究在内的电生理测试对于诊断神经性肌病至关重要。肌电图是诊断肌肉失神经支配的金标准。
其他成像方法,尤其是磁共振成像(MRI),能够提供有价值的信息。失神经支配肌肉组织的MRI检查显示水肿、表观扩散系数(ADC)增加和血流灌注增加。慢性失神经支配导致受影响肌肉的脂肪变性和萎缩,MRI也可检测到这些变化。
尽管肌肉失神经支配的MRI表现相对缺乏特异性,但它们显示出与肌电图相当的高灵敏度。专用的磁共振神经造影术通常可以显示支配神经的潜在病变。
除了与肌电图相当的高灵敏度外,MRI还能够评估针电极肌电图无法触及的肌肉。由于其非侵入性,MRI是随访检查的理想选择。
MRI的使用通常是对神经源性肌病诊断的有意义补充。肌肉改变的范围和分布模式通常可提供有关致病神经损伤部位的信息。使用MRI通常可以实现正确诊断或至少缩小可能的鉴别诊断范围。