Department of Neurology, David Geffen, UCLA School of Medicine, Los Angeles, California.
LAC Olive View, UCLA Medical Center, Sylmar, California.
Acta Neurol Scand. 2019 Jan;139(1):82-85. doi: 10.1111/ane.13027. Epub 2018 Oct 8.
To assess the utility of Magnetic Resonance Spectroscopy (MRS) as a biomarker of response to L-arginine in mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS).
To describe a case of MELAS treated with L-arginine that showed improvement clinically and on serial MRS METHODS: MRS was performed on a 1.5-Tesla scanner to evaluate a MELAS patient before, during, and after intravenous (IV) L-arginine therapy for the treatment of stroke-like episodes. L-arginine was infused at a dose of 500 mg/kg daily for 7 days followed by oral arginine therapy.
The patient had clinical improvement after treatment with IV L-arginine. MRS performed before, during, and after treatment with IV L-arginine showed significant improvement in brain lactate and increase in the N-acetylaspartate/Choline (NAA/Cho) ratio compared to pre-treatment baseline.
Serial MRS imaging showed significant improvement in lactate peaks and NAA/Cho ratios that corresponded with clinical improvement after L-arginine therapy. Given this correlation between radiologic and clinical improvement, MRS may be a useful biomarker assessing response to treatment in MELAS.
评估磁共振波谱（MRS）作为线粒体脑肌病伴高乳酸血症和卒中样发作（MELAS）对精氨酸反应的生物标志物的效用。
描述一例接受精氨酸治疗的 MELAS 患者，该患者在静脉（IV）精氨酸治疗卒中样发作期间和之后在临床上和连续 MRS 上均有改善。
在 1.5T 扫描仪上进行 MRS，以评估 MELAS 患者在静脉内（IV）精氨酸治疗卒中样发作之前、期间和之后的情况。精氨酸以 500mg/kg 的剂量每天输注 7 天，然后进行口服精氨酸治疗。
该患者在接受 IV 精氨酸治疗后临床症状改善。与治疗前基线相比，在接受 IV 精氨酸治疗之前、期间和之后进行的 MRS 显示脑内乳酸水平显著改善，N-乙酰天冬氨酸/胆碱（NAA/Cho）比值增加。
连续 MRS 成像显示乳酸峰和 NAA/Cho 比值显著改善，与 L-精氨酸治疗后的临床改善相对应。鉴于放射学和临床改善之间的这种相关性，MRS 可能是评估 MELAS 治疗反应的有用生物标志物。
