Neurology and Stroke Unit, Azienda Ospedaliera San G. Moscati, Contrada Amoretta, 83100, Avellino, Italy.
Neuroradiology Unit, Azienda Ospedaliera San G. Moscati, Contrada Amoretta, 83100, Avellino, Italy.
Neurol Sci. 2021 Aug;42(8):3419-3422. doi: 10.1007/s10072-021-05195-1. Epub 2021 Mar 22.
Sporadic cerebral amyloid angiopathy (CAA) is a common age-related cerebral small vessel disease characterized by progressive ß-amyloid deposition in the walls of small cortical arteries, arterioles, and capillaries in the cerebral cortex and overlying leptomeninges. CAA-related transient focal neurological episodes (CAA-TFNEs) represent a challenging clinical feature interesting from a pathophysiological point of view.
Here we present two cases of CAA-TFNEs in which we performed functional imaging with perfusion-weighted imaging MR and brain 18 F-FDG PET. In both cases, we found a topographic relationship between the involved cortical areas and the clinical expression of CAA-TFNEs. Cortical superficial siderosis in the first case and a convexity subarachnoid hemorrhage in the second case were found in the contralateral rolandic area corresponding to the clinical symptoms. The same areas showed a reduction of rCBV and rCBF on perfusion-weighted MR and were also associated in one case with hypometabolism on 18 F-FDG PET.
These new findings strengthen the hypothesis that CAA involves the superficial leptomeningeal arteries but also the short penetrating arterioles reaching different depths in the cortex generating hypoperfusion and altered vascular reactivity and consequently reduced neuronal activity.
Understanding CAA-TFNEs is pivotal because they carry a very high risk of subsequent lobar intracerebral hemorrhage but are frequently misdiagnosed as TIAs and treated with antithrombotics enhancing the bleeding risk associated with CAA.
散发性脑淀粉样血管病(CAA)是一种常见的与年龄相关的脑小血管疾病,其特征是β-淀粉样蛋白在大脑皮质的小动脉、小动脉和毛细血管壁以及脑皮质表面的软脑膜中进行性沉积。CAA 相关性短暂性局灶性神经功能障碍(CAA-TFNEs)是一种具有挑战性的临床特征,从病理生理学角度来看很有趣。
在此,我们报告了两例 CAA-TFNEs 病例,我们对其进行了灌注加权成像 MR 和脑 18 F-FDG PET 功能成像。在这两种情况下,我们发现受累皮质区域与 CAA-TFNEs 的临床表现之间存在一种局部关系。在首例病例中,发现皮质浅部铁沉积,在第二例病例中,发现对侧 Rolandic 区的凸面蛛网膜下腔出血,与 CAA-TFNEs 的临床表现相对应。灌注加权 MR 显示相同区域的 rCBV 和 rCBF 减少,并且在一例病例中还与 18 F-FDG PET 上的代谢减少相关。
这些新发现加强了 CAA 涉及浅脑膜动脉的假说,但也涉及到达皮质不同深度的短穿通小动脉,导致灌注不足和血管反应性改变,从而导致神经元活动减少。
理解 CAA-TFNEs 至关重要,因为它们有很高的继发脑叶脑出血风险,但常被误诊为 TIA 并接受抗血栓治疗,从而增加与 CAA 相关的出血风险。