Boekestein Werner A, Kleine Bert U, Hageman G, Schelhaas Helenius J, Zwarts Machiel J
Department of Neurology, Donders Institute for Brain, Cognition and Behaviour, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands.
Amyotroph Lateral Scler. 2010 Dec;11(6):497-501. doi: 10.3109/17482961003777462. Epub 2010 Jun 11.
The Awaji Commission recently proposed a modification of the electrodiagnostic criteria for ALS. We assessed whether the Awaji recommendations improve the sensitivity of the early diagnosis of ALS. In a retrospective study we reviewed clinical and neurophysiological data for 213 patients who visited our motor neuron disease outpatient clinic between October 2006 and December 2008. Using the El Escorial criteria, 51 patients were diagnosed with definite or probable ALS, 14 with probable laboratory-supported ALS, and 28 with possible ALS. An alternative diagnosis was present in 120 patients. Applying the Awaji recommendations, 66 patients were diagnosed with either definite or probable ALS, and 27 with possible ALS. Of the 14 patients diagnosed with probable laboratory-supported ALS, eight switched to probable ALS and six to possible ALS using the Awaji recommendations; none of the patients with an ALS mimic was diagnosed with ALS according to the Awaji recommendations. In conclusion, the new criteria for ALS do not result in a loss of specificity and can potentially improve the sensitivity by 16%. However, this diagnostic improvement appears eliminated if patients with probable laboratory-supported ALS - due to UMN signs in one region - should be categorized as possible ALS.
淡路委员会最近提议修改肌萎缩侧索硬化症（ALS）的电诊断标准。我们评估了淡路建议是否能提高ALS早期诊断的敏感性。在一项回顾性研究中，我们回顾了2006年10月至2008年12月期间到我们运动神经元疾病门诊就诊的213例患者的临床和神经生理学数据。根据埃尔埃斯科里亚尔标准，51例患者被诊断为确诊或疑似ALS，14例为疑似实验室支持的ALS，28例为可能的ALS。120例患者有其他诊断。应用淡路建议，66例患者被诊断为确诊或疑似ALS，27例为可能的ALS。在14例被诊断为疑似实验室支持的ALS患者中，根据淡路建议，8例转为疑似ALS，6例转为可能的ALS；根据淡路建议，没有一例ALS疑似患者被诊断为ALS。总之，新的ALS标准不会导致特异性丧失，并且有可能将敏感性提高16%。然而，如果由于一个区域出现上运动神经元体征而被诊断为疑似实验室支持的ALS患者应归类为可能的ALS，那么这种诊断上的改善似乎就会消失。
