Ross M A, Miller R G, Berchert L, Parry G, Barohn R J, Armon C, Bryan W W, Petajan J, Stromatt S, Goodpasture J, McGuire D
Neurology. 1998 Mar;50(3):768-72. doi: 10.1212/wnl.50.3.768.
We modified the World Federation of Neurology (WFN) diagnostic criteria for ALS to facilitate early diagnosis and used these criteria for enrollment of ALS patients in a clinical trial. The criteria developed required lower motor neuron (LMN) involvement in at least two limbs and upper motor neuron involvement in at least one region (bulbar, cervical, or lumbosacral). The EMG finding of fibrillation potentials was required for evidence of LMN involvement. Electrodiagnostic studies, neuroimaging, and laboratory studies were also used to exclude disorders that might mimic ALS. Using these criteria, the diagnosis of ALS was made at a mean time of 9.7 months from onset of symptoms, which compares favorably with the 12-month period cited in the literature. Using clinical assessment at completion of the trial, the diagnosis of ALS was believed to be accurate in those patients entered in the trial. However, pathologic confirmation of the diagnosis of ALS was not obtained. Based on our preliminary experience, we propose that these ALS diagnostic criteria will facilitate early diagnosis of ALS. Future studies should prospectively compare these criteria with the WFN criteria currently in use.
我们修改了世界神经病学联合会（WFN）的肌萎缩侧索硬化症（ALS）诊断标准以促进早期诊断，并在一项临床试验中使用这些标准来招募ALS患者。所制定的标准要求至少两个肢体有下运动神经元（LMN）受累，且至少一个区域（延髓、颈段或腰骶段）有上运动神经元受累。需要有肌纤维颤动电位的肌电图表现作为LMN受累的证据。还使用了电诊断研究、神经影像学和实验室研究来排除可能模仿ALS的疾病。使用这些标准，ALS的诊断在症状出现后的平均9.7个月时做出，这与文献中引用的12个月相比更具优势。根据试验结束时的临床评估，认为试验中纳入的那些患者的ALS诊断是准确的。然而，未获得ALS诊断的病理证实。基于我们的初步经验，我们提出这些ALS诊断标准将促进ALS的早期诊断。未来的研究应前瞻性地将这些标准与目前使用的WFN标准进行比较。
