Department of Medicine,Division of Neurology, Dalhousie University,Halifax Infirmary, 1796 Summer Street, Halifax, NS, B3H 3A7, Canada.
Department of Neurology, Dartmouth-Hitchcock Medical Center and Geisel School of Medicine at Dartmouth, One Medical Center Drive, Lebanon, NH, 03766, USA.
Curr Pain Headache Rep. 2021 Mar 18;25(4):25. doi: 10.1007/s11916-021-00939-2.
Provide an overview of the current diagnosis, pathophysiology, and treatment of Susac's syndrome (SuS), with special emphasis on summarizing what is currently known about headache as a symptom of disease activity.
The most recent literature in SuS has focused on furthering the understanding of the underlying pathology and efficacy of treatments for SuS. The importance of early recognition to facilitate timely treatment and avoid long-term disability has been highlighted. Headache, the most common symptom experienced by patients with SuS, can occur up to 6 months in advance of other symptoms, and exacerbations of headache can herald increased disease activity. Susac's syndrome (SuS) is a rare disorder classically characterized by triad of encephalopathy, branch retinal artery occlusion (BRAO), and sensory neuronal hearing loss (SNHL). The full triad is uncommon at initial presentation, which can confound efforts to make timely diagnosis and treatment decisions. Headache is the most common symptom in SuS, is often an early feature, and can help separate SuS from other diagnoses in the differential. However, the features and management of the headache associated with SuS have not been systematically defined in the literature.
提供 Susac 综合征（SuS）的当前诊断、病理生理学和治疗概述，特别强调总结目前已知的关于疾病活动的头痛作为症状的情况。
SuS 的最新文献重点在于进一步了解潜在的病理生理学，并评估 SuS 的治疗效果。早期识别的重要性已被强调，这有助于及时治疗和避免长期残疾。头痛是 SuS 患者最常见的症状，可在其他症状出现前 6 个月发生，头痛加剧可能预示着疾病活动增加。Susac 综合征（SuS）是一种罕见的疾病，其特征为三联征：脑病、视网膜分支动脉阻塞（BRAO）和感觉神经元性听力损失（SNHL）。在初始表现时，完全三联征并不常见，这可能会混淆及时诊断和治疗决策的制定。头痛是 SuS 中最常见的症状，通常是早期特征，并且可以帮助将 SuS 与鉴别诊断中的其他疾病区分开来。然而，与 SuS 相关的头痛的特征和管理在文献中尚未系统定义。
