Department of Neurology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Japan.
Department of Neurology, Obihiro Kosei General Hospital, Japan.
Intern Med. 2024 Nov 1;63(21):2903-2912. doi: 10.2169/internalmedicine.3275-23. Epub 2024 Mar 18.
Objective In 2022, Wenning et al. proposed the Movement Disorder Society Criteria (MDS criteria) for the Diagnosis of Multiple System Atrophy (MSA). These criteria were expected to provide useful alternatives to the second consensus statement. We examined trends in these diagnostic criteria. Methods We used patient data registered with the Hokkaido Rare Disease Consortium for Multiple System Atrophy, which has been recruiting patients with MSA through medical facilities in Hokkaido since November 2014. Patients were evaluated according to the MDS criteria based on neurological examinations and imaging findings at three separate times: the first evaluation, the time of enrollment (diagnosis), and the most recent evaluation (final evaluation). Results The MDS criteria were examined in 68 of 244 patients enrolled between November 2014 and July 2022. At the initial evaluation, the classifications were as follows: clinically established (n=27; 39.7%); clinically probable (n=13; 19.1%); possible prodromal (n=12; 17.6%); and negative [did not meet criteria (n=16; 23.5%)]. At the time of diagnosis, the classifications were as follows: clinically established (n=45; 66.2%); clinically probable (n=12; 17.6%); possible prodromal (n=4; 5.9%); and negative (n=7; 10.3%). At the final evaluation, the classifications were as follows: clinically established (n=52; 76.5%); clinically probable (n=9; 13.2%); possible prodromal (n=2; 2.9%); and negative (n=5; 7.4%). Conclusion We were able to clarify the changes in the criteria values and transition of patients due to the clarification of imaging and supportive findings in the MDS criteria.
目的 2022 年,Wenning 等人提出了多系统萎缩(MSA)的运动障碍学会标准(MDS 标准)。这些标准有望为第二份共识声明提供有用的替代方案。我们研究了这些诊断标准的趋势。
方法 我们使用了北海道多系统萎缩罕见病联合会登记的患者数据,该联合会自 2014 年 11 月以来一直在北海道的医疗机构中招募 MSA 患者。患者根据 MDS 标准进行评估,该标准基于三次单独的神经检查和影像学发现:第一次评估、入组时(诊断)和最近一次评估(最终评估)。
结果 在 2014 年 11 月至 2022 年 7 月期间入组的 244 名患者中,有 68 名患者接受了 MDS 标准的检查。在初始评估时,分类如下:临床确诊(n=27;39.7%);临床可能(n=13;19.1%);可能前驱期(n=12;17.6%);和阴性[不符合标准(n=16;23.5%)]。在诊断时,分类如下:临床确诊(n=45;66.2%);临床可能(n=12;17.6%);可能前驱期(n=4;5.9%);和阴性(n=7;10.3%)。在最终评估时,分类如下:临床确诊(n=52;76.5%);临床可能(n=9;13.2%);可能前驱期(n=2;2.9%);和阴性(n=5;7.4%)。
结论 我们能够通过 MDS 标准中影像学和支持性发现的澄清,阐明标准值的变化和患者的转变。