Department of Neurology, Rabin Medical Center, Zeev Jabotinsky St 39, 49100, Petah Tikva, Israel.
Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
Acta Neurol Belg. 2023 Jun;123(3):1019-1028. doi: 10.1007/s13760-023-02196-z. Epub 2023 Feb 15.
Stroke-like episodes (SLEs) in patients with MELAS syndrome are often initially misdiagnosed as acute ischemic stroke (AIS), resulting in treatment delay. We aimed to determine clinical features that may distinguish SLEs from AISs and explore the benefit of early L-arginine treatment on patient outcomes.
We looked retrospectively for MELAS patients admitted between January 2005 and January 2022 and compared them to an AIS cohort with similar lesion topography. MELAS patients who received L-arginine within 40 days of their first SLE were defined as the early treatment group and the remaining as late or no treatment group.
Twenty-three SLEs in 10 MELAS patients and 21 AISs were included. SLE patients had significantly different features: they were younger, more commonly reported hearing loss, lower body mass index, had more commonly a combination of headache and/or seizures at presentation, serum lactate was higher, and hemiparesis was less common. An SLE Early Clinical Score (SLEECS) was constructed by designating one point to each above features. SLEECS ≥ 4 had 80% sensitivity and 100% specificity for SLE diagnosis. Compared to late or no treatment, early treatment group patients (n = 5) had less recurrent SLEs (total 2 vs. 11), less seizures (14% vs. 25%, p = 0.048), lower degree of disability at first and last follow-up (modified ranking scale, mRS 2 ± 0.7 vs. 4.2 ± 1, p = 0.005; 2 ± 0.7 vs. 5.8 ± 0.5, p < 0.001, respectively), and a lower mortality (0% vs. 80% p = 0.048).
The SLEECS model may aid in the early diagnosis and treatment of SLEs and lead to improved clinical outcomes.
线粒体脑肌病伴高乳酸血症和卒中样发作（MELAS）患者的卒中样发作（SLE）常被误诊为急性缺血性卒中（AIS），导致治疗延误。我们旨在确定可将 SLE 与 AIS 区分开来的临床特征，并探讨早期 L-精氨酸治疗对患者结局的益处。
我们回顾性地查找了 2005 年 1 月至 2022 年 1 月期间收治的 MELAS 患者，并将其与具有相似病变部位的 AIS 队列进行了比较。在首次 SLE 后 40 天内接受 L-精氨酸治疗的 MELAS 患者被定义为早期治疗组，其余患者为晚期或未治疗组。
纳入了 10 例 MELAS 患者的 23 例 SLE 和 21 例 AIS。SLE 患者的特征明显不同：他们更年轻，更常报告听力丧失，体重指数更低，发作时更常同时出现头痛和/或癫痫，血清乳酸水平更高，偏瘫较少见。通过为上述每一个特征指定 1 分，构建了 SLE 早期临床评分（SLEECS）。SLEECS≥4 对 SLE 诊断的敏感性为 80%，特异性为 100%。与晚期或未治疗相比，早期治疗组患者（n=5）SLE 复发次数更少（总复发 2 次 vs. 11 次），癫痫发作更少（14% vs. 25%，p=0.048），首次和最后一次随访时的残疾程度更低（改良 Rankin 量表，mRS 2±0.7 vs. 4.2±1，p=0.005；2±0.7 vs. 5.8±0.5，p<0.001），死亡率更低（0% vs. 80%，p=0.048）。
SLEECS 模型可辅助 SLE 的早期诊断和治疗，并改善临床结局。
