Introduction

Idiopathic systemic capillary leak syndrome (ISCLS) is a rare cryptogenic disorder characterized by recurrent hemoconcentration, hypoalbuminemia, edema, and hypotension due to extravascular fluid leakage. This is the first report that details uncommon extensive leukoencephalopathy caused by ISCLS upon a neuropathological investigation.

Case report

A 68-year-old female had recurrent episodes of hemoconcentration, hypoalbuminemia, and generalized edema and was diagnosed with ISCLS. After 9 years, brain magnetic resonance imaging (MRI) incidentally revealed extensive leukoencephalopathy without neurological deficits. Thorough examinations ruled out other disorders, and the cerebral involvement due to ISCLS was finally diagnosed. Three years later, she developed an acute-onset coma and status epilepticus together with hypotension and hemoconcentration, which were compatible with ISCLS recurrence. Electroencephalogram and MRI were correlated with a seizure arising from the left hemisphere. Extensive leukoencephalopathy did not show notable changes for 3 years. Although treatment for ISCLS recurrence temporally improved hemoconcentration and consciousness, consciousness worsened again by marked edema of the left hemisphere, and she died of cerebral herniation. A brain autopsy revealed straggly perivascular plasma leakage around the small vessels of the deep white matter, which supported that the leukoencephalopathy was caused by ISCLS. Widespread myelin pallor and decreased axonal density with sparse astrogliosis and microgliosis were observed in the cerebral white matter and corresponded with a chronic change in the MRI.

Conclusion

Current radiological and pathological observations revealed that frequent perivascular leakages could cause chronic leukoencephalopathy, were linked with the development of systemic capillary leakage in ISCLS, and provided insights into the mysterious pathophysiology.

中文翻译：

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与特发性毛细血管渗漏综合征相关的广泛性白质脑病：一例神经病理学报告

介绍

特发性系统性毛细血管渗漏综合征（ISCLS）是一种罕见的隐源性疾病，其特征是由于血管外液体渗漏而引起的经常性血液浓缩，低白蛋白血症，水肿和低血压。这是第一份通过神经病理学调查详细介绍由ISCLS引起的罕见广泛性白质脑病的报告。

案例报告

一名68岁的女性反复出现血液浓缩，低白蛋白血症和全身性水肿，并被诊断为ISCLS。9年后，脑磁共振成像（MRI）偶然发现广泛的白质脑病，而没有神经功能缺损。彻底的检查排除了其他疾病，最终诊断出由于ISCLS引起的大脑受累。三年后，她出现了急性发作性昏迷和癫痫持续状态，并伴有低血压和血液浓缩，与ISCLS复发相适应。脑电图和MRI与左半球发作有关。广泛性白质脑病3年未显示明显变化。尽管对ISCLS的复发治疗暂时改善了血液浓度和意识，左半球明显水肿再次使意识恶化，她死于脑疝。脑部解剖显示，深部白质小血管周围弥漫着血管周围血浆渗漏，这表明白脑病是由ISCLS引起的。在脑白质中观察到广泛的髓鞘苍白和轴突密度降低以及稀疏的星形胶质细胞增生和微胶质细胞增生，这与MRI的慢性变化相对应。

结论

当前的放射学和病理学观察表明，频繁的血管周围渗漏可能导致慢性白质脑病，与ISCLS中系统性毛细血管渗漏的发展有关，并提供了对神秘病理生理学的见解。