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首例短暂性头痛伴神经功能缺损伴淋巴细胞增多症(HaNDL)的独特CT灌注成像:对HaNDL病理生理学及皮质扩散性抑制血管运动原理的新见解

Unique CT Perfusion Imaging in a Case of HaNDL: New Insight into HaNDL Pathophysiology and Vasomotor Principles of Cortical Spreading Depression.

作者信息

Burke Matthew J, Lamb Michael J, Hohol Marika, Lay Christine

机构信息

Division of Neurology, Department of Medicine, University of Toronto, Toronto, Canada.

Department of Internal Medicine, University of Toronto, Toronto, Canada.

出版信息

Headache. 2017 Jan;57(1):129-134. doi: 10.1111/head.12968. Epub 2016 Oct 24.

DOI:10.1111/head.12968
PMID:27774587
Abstract

BACKGROUND

The etiology of HaNDL is not known. Recent neuroimaging studies have suggested that there may be altered cerebrovascular blood flow during acute episodes. However, what exactly these vascular changes represent and how they may relate to the overall pathogenesis of HaNDL is uncertain.

CASE

A 42-year-old, right-handed male, presented with acute aphasia and right arm weakness. Urgent CT/CT-angiogram were normal except for an incidental hypoplastic right anterior cerebral artery (ACA) A1 segment. However, CT perfusion revealed global left hemisphere hypoperfusion in the range of oligemia. Also, the right ACA territory, supplied by the dominant left A1, shared the same pattern of hypoperfusion. Further investigations and clinical course were consistent with HaNDL.

DISCUSSION/CONCLUSIONS: The pattern of global left hemispheric hypoperfusion seen in this case of HaNDL supports a hypothesis of secondary oligemia induced by a hemispheric wave of cortical spreading depression (CSD). However, the extension of hypoperfusion to the right ACA territory represents a phenomenon not previously reported in this field. We speculate that the direct spread of CSD-induced vasomotor changes across the anomalous vasculature could account for this finding. This case provides a valuable contribution toward understanding HaNDL pathophysiology and in doing so may also yield broader implications regarding neurophysiological principles of CSD.

摘要

背景

短暂性单侧神经症状伴可逆性影像学病灶(HaNDL)的病因尚不清楚。最近的神经影像学研究表明,急性发作期间脑血管血流可能发生改变。然而,这些血管变化究竟代表什么以及它们与HaNDL的整体发病机制可能有何关联尚不确定。

病例

一名42岁右利手男性,出现急性失语和右臂无力。紧急CT/CT血管造影除偶然发现右侧大脑前动脉(ACA)A1段发育不全外均正常。然而,CT灌注显示左半球整体灌注不足,范围为缺血。此外,由优势侧左A1供血的右侧ACA区域也呈现相同的灌注不足模式。进一步检查和临床病程符合HaNDL。

讨论/结论:该例HaNDL中所见的左半球整体灌注不足模式支持皮层扩散性抑制(CSD)半球波诱发继发性缺血的假说。然而,灌注不足扩展至右侧ACA区域是该领域此前未报道过的现象。我们推测CSD诱发的血管舒缩变化直接通过异常血管系统扩散可解释这一发现。该病例为理解HaNDL病理生理学做出了宝贵贡献,同时也可能对CSD的神经生理学原理产生更广泛的影响。

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