Abstract：
Stroke-like episodes (SLEs) are defined as acute onset of neurological symptoms mimicking a stroke and radiological lesions non-congruent to vascular territory. We aimed to analyze the acute clinical and radiological features of SLEs to determine their pathophysiology.
We performed a monocenter retrospective analysis of 120 SLEs in 60 children over a 20-year period. Inclusion criteria were compatible clinical symptoms and stroke-like lesions on brain magnetic resonance imaging (MRI; performed for all 120 events) with focal hyperintensity on diffusion-weighted imaging in a non-vascular territory.
Three groups were identified: children with mitochondrial diseases (n = 22) involving mitochondrial DNA mutations (55%) or nuclear DNA mutations (45%); those with other metabolic diseases or epilepsy disorders (n = 22); and those in whom no etiology was found despite extensive investigations (n = 16). Age at first SLE was younger in the group with metabolic or epilepsy disorders (18 months vs. 128 months; p < 0.0001) and an infectious trigger was more frequent (69% vs. 20%; p = 0.0001). Seizures occurred in 75% of episodes, revealing 50% episodes of SLEs and mainly leading to status epilepticus (90%). Of the 120 MRI scans confirming the diagnosis, 28 were performed within a short and strict 48-h period and were further analyzed to better understand the underlying mechanisms. The scans showed primary cortical hyperintensity (n = 28/28) with decreased apparent diffusion coefficient in 52% of cases. Systematic hyperperfusion was found on spin labeling sequences when available (n = 18/18).
Clinical and radiological results support the existence of a vicious circle based on two main mechanisms: energy deficit and neuronal hyperexcitability at the origin of SLE.
We performed a monocenter retrospective analysis of 120 SLEs in 60 children over a 20-year period. Inclusion criteria were compatible clinical symptoms and stroke-like lesions on brain magnetic resonance imaging (MRI; performed for all 120 events) with focal hyperintensity on diffusion-weighted imaging in a non-vascular territory.
Three groups were identified: children with mitochondrial diseases (n = 22) involving mitochondrial DNA mutations (55%) or nuclear DNA mutations (45%); those with other metabolic diseases or epilepsy disorders (n = 22); and those in whom no etiology was found despite extensive investigations (n = 16). Age at first SLE was younger in the group with metabolic or epilepsy disorders (18 months vs. 128 months; p < 0.0001) and an infectious trigger was more frequent (69% vs. 20%; p = 0.0001). Seizures occurred in 75% of episodes, revealing 50% episodes of SLEs and mainly leading to status epilepticus (90%). Of the 120 MRI scans confirming the diagnosis, 28 were performed within a short and strict 48-h period and were further analyzed to better understand the underlying mechanisms. The scans showed primary cortical hyperintensity (n = 28/28) with decreased apparent diffusion coefficient in 52% of cases. Systematic hyperperfusion was found on spin labeling sequences when available (n = 18/18).
Clinical and radiological results support the existence of a vicious circle based on two main mechanisms: energy deficit and neuronal hyperexcitability at the origin of SLE.
摘要：
目的：中风样发作（SLE）被定义为模仿中风和放射学病变与血管区域不一致的神经系统症状的急性发作。我们旨在分析SLE的急性临床和放射学特征，以确定其病理生理学。
方法：我们在20年间对60名儿童的120个SLE进行了单中心回顾性分析。纳入标准是在非血管区域的弥散加权成像中，在脑磁共振成像（MRI；对所有120例事件进行）上具有局灶性高强度的兼容临床症状和中风样病变。
结果：确定了三组：线粒体疾病儿童（n=22），涉及线粒体DNA突变（55％）或核DNA突变（45％）；患有其他代谢疾病或癫痫疾病的儿童（n=22）；以及尽管进行了广泛调查但未发现病因的儿童（n=16）。在患有代谢或癫痫疾病的组中，第一次SLE的年龄较年轻（18个月与128个月；p<0.0001)，感染触发更频繁(69%vs.20%；p=0.0001）。癫痫发作发生在75%的发作中，显示50％的SLE发作，主要导致癫痫持续状态（90％）。在确认诊断的120次核磁共振扫描中,28在短而严格的48小时内进行，并进一步分析以更好地了解潜在的机制。扫描显示原发性皮质高强度（n=28/28），在52％的病例中表观扩散系数降低。当可用时,在自旋标记序列上发现系统性过度灌注(n=18/18)。
结论：临床和放射学结果支持基于两种主要机制的恶性循环的存在：SLE起源的能量缺乏和神经元过度兴奋。
方法：我们在20年间对60名儿童的120个SLE进行了单中心回顾性分析。纳入标准是在非血管区域的弥散加权成像中，在脑磁共振成像（MRI；对所有120例事件进行）上具有局灶性高强度的兼容临床症状和中风样病变。
结果：确定了三组：线粒体疾病儿童（n=22），涉及线粒体DNA突变（55％）或核DNA突变（45％）；患有其他代谢疾病或癫痫疾病的儿童（n=22）；以及尽管进行了广泛调查但未发现病因的儿童（n=16）。在患有代谢或癫痫疾病的组中，第一次SLE的年龄较年轻（18个月与128个月；p<0.0001)，感染触发更频繁(69%vs.20%；p=0.0001）。癫痫发作发生在75%的发作中，显示50％的SLE发作，主要导致癫痫持续状态（90％）。在确认诊断的120次核磁共振扫描中,28在短而严格的48小时内进行，并进一步分析以更好地了解潜在的机制。扫描显示原发性皮质高强度（n=28/28），在52％的病例中表观扩散系数降低。当可用时,在自旋标记序列上发现系统性过度灌注(n=18/18)。
结论：临床和放射学结果支持基于两种主要机制的恶性循环的存在：SLE起源的能量缺乏和神经元过度兴奋。
