Abstract：
Encephalopathy is part of the clinical triad of Susac syndrome, but a detailed understanding of the neurocognitive and neuropsychiatric profile of this condition is lacking. Existing literature indicates that cognitive deficits range in severity from subtle to profound. Executive function and short-term recall are affected frequently. Psychiatric manifestations may be absent or may include anxiety, mood disorders or psychosis. If psychiatric phenomena develop during the disease course, it can be hard to disentangle whether symptoms directly relate to the pathology of Susac syndrome or are secondary to treatment-related side effects. In this article, we review what is known about the cognitive and psychiatric morbidity of Susac syndrome and identify areas where knowledge is deficient. Importantly, we also provide a framework for future research, arguing that better phenotyping, understanding of pathophysiology, evaluation of treatments on cognitive and psychiatric outcomes, and longitudinal data capture are vital to improving patient outcomes.
摘要：
脑病是Susac综合征临床三联征的一部分，但是缺乏对这种情况的神经认知和神经精神病学特征的详细了解。现有文献表明，认知缺陷的严重程度从微妙到深刻。执行功能和短期召回经常受到影响。精神病表现可能不存在或可能包括焦虑，情绪障碍或精神病。如果精神病现象在疾病过程中发展，很难弄清楚症状是否与Susac综合征的病理直接相关，还是继发于治疗相关的副作用。在这篇文章中,我们回顾了有关Susac综合征的认知和精神病发病率的已知信息，并确定了知识不足的领域。重要的是,我们还为未来的研究提供了一个框架，认为更好的表型，对病理生理学的理解，对认知和精神病学结果的治疗评估，纵向数据采集对改善患者预后至关重要。
