Finsterer Josef, Aliyev Rahim
Krankenanstalt Rudolfstiftung, Messerli Institute, Vienna, Austria.
Department of Neurology and Clinical Neurophysiology, Azerbaijan State Advanced Training Institute for Doctors named after A. Aliyev, Baku, Azerbaijan.
J Neurol Sci. 2020 May 15;412:116726. doi: 10.1016/j.jns.2020.116726. Epub 2020 Feb 7.
One of the most frequent cerebral lesions in mitochondrial disorders(MIDs) on imaging is the stroke-like lesion(SLL) clinically manifesting as stroke-like episode (SLE, metabolic stroke). This review aims at discussing recent advances concerning the presentation, diagnosis, and treatment of SLLs.
Systematic literature review using appropriate search terms.
SLLs are the hallmark of MELAS but occasionally occur in other MIDs. SLLs are best identified on multimodal, cerebral MRI. SLLs may present as uni-/multilocular, symmetric/asymmetric, cortical/subcortical, supra-/infratentorial condition, initially resembling a cytotoxic edema and later a vasogenic edema, or a variable mix between them. SLLs run through an acute and a chronic stage. The acute stage is characterised by a progressively expanding lesion over days, weeks, or months, showing up as increasing hyperintensity on T2/FLAIR, DWI, and PWI and by hyperperfusion, that does not conform to a vascular territory. ADC maps are initially hypointens to become hyperintens during the course. More rarely, a variable mixture of hyper- and hypointensities may be found. The chronic stage is characterised by hypoperfusion, gadolinium enhancement, and regression of hyperintensities to various endpoints. SLLs originate from an initial cortical lesion due to focal metabolic breakdown, which either remains stable or expands within the cortex or to subcortical areas. Some SLLs show spontaneous reversibility (fleeing cortical lesions) suggesting that neuronal/glial damage does not reach the threshold of irreversible cell death.
SLLs are a unique feature of various MIDs in particular MELAS. SLLs are dynamic and change their appearance over time. SLLs are accessible to treatment.
线粒体疾病（MIDs）影像学上最常见的脑部病变之一是类卒中病变（SLL），临床上表现为类卒中发作（SLE，代谢性卒中）。本综述旨在讨论有关SLL的表现、诊断和治疗的最新进展。
使用适当的检索词进行系统文献综述。
SLL是线粒体脑肌病伴乳酸血症和卒中样发作（MELAS）的标志，但偶尔也会出现在其他MIDs中。SLL在多模态脑磁共振成像（MRI）上最易识别。SLL可表现为单房/多房、对称/不对称、皮质/皮质下、幕上/幕下病变，最初类似细胞毒性水肿，随后为血管源性水肿，或两者的可变混合。SLL经历急性期和慢性期。急性期的特征是病变在数天、数周或数月内逐渐扩大，在T2/液体衰减反转恢复序列（FLAIR）、扩散加权成像（DWI）和灌注加权成像（PWI）上表现为高信号增强，且呈高灌注，不符合血管分布区域。表观扩散系数（ADC）图最初为低信号，病程中变为高信号。更罕见的情况下，可能会发现高信号和低信号的可变混合。慢性期的特征是低灌注、钆增强以及高信号向不同终点的消退。SLL起源于局灶性代谢分解导致的初始皮质病变，该病变要么保持稳定，要么在皮质内或向皮质下区域扩展。一些SLL表现出自发性可逆性（游走性皮质病变），提示神经元/胶质细胞损伤未达到不可逆细胞死亡的阈值。
SLL是各种MIDs尤其是MELAS的独特特征。SLL是动态的，其外观随时间变化。SLL可进行治疗。
