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伴皮质下梗死和白质脑病的脑常染色体显性遗传性动脉病(CADASIL)患者无症状性弥散加权成像病变的特征及其时间演变。

Characteristics and temporal evolution of asymptomatic diffusion-weighted imaging lesions in patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL).

机构信息

Department of Neurology, National Taiwan University Hospital, Taipei, Taiwan.

Department of Medical Imaging, National Taiwan University Hospital, Taipei, Taiwan.

出版信息

Eur J Neurol. 2024 Dec;31(12):e16519. doi: 10.1111/ene.16519. Epub 2024 Oct 11.

Abstract

BACKGROUND AND PURPOSE

The role of asymptomatic diffusion-weighted imaging-positive (aDWI+) lesions in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) patients remains unclear, and their radiographic features may differ from those of symptomatic diffusion-weighted imaging-positive (sDWI+) lesions. We aimed to investigate the clinicoradiographic characteristics of aDWI+ lesions in CADASIL patients.

METHODS

We conducted a retrospective analysis using data from the Taiwan CADASIL Registry. aDWI+ lesions were defined as incidentally detected DWI+ lesions without corresponding acute neurological deficits. We compared the baseline clinical characteristics of patients with and without aDWI+ lesions and analyzed their radiological features and evolution in relation to sDWI+ lesions.

RESULTS

Among 154 enrolled patients (mean age 62 ± 10 years), 17 (11%) had aDWI+ lesions. Baseline clinical characteristics were similar in the two groups, but those with aDWI+ lesions had more lacunes (median 8 vs. 2), multiple cerebral microbleeds (CMBs; 85% vs. 40%), and anterior temporal white matter hyperintensity (WMH; 47% vs. 14%). Multivariable analysis showed that aDWI+ lesions were associated with anterior temporal WMH (odds ratio 5.7, 95% confidence interval 1.5-21.0) after adjusting for multiple lacunes, multiple CMBs, and total WMH score. Compared to sDWI+ lesions, aDWI+ lesions were more often small infarcts (<1 cm; 89% vs. 23%) and less likely to involve the corticospinal tract (11% vs. 96%). Among the 11 aDWI+ lesions with follow-up magnetic resonance imaging, seven became microinfarcts, three became lacunes, and one disappeared.

CONCLUSIONS

aDWI+ lesions in CADASIL are not uncommon and are associated with higher burdens of small vessel disease and anterior temporal WMH. Further research is needed to assess their long-term impact on CADASIL.

摘要

背景与目的

无症状性弥散加权成像阳性(aDWI+)病变在伴有皮质下梗死和白质脑病的常染色体显性脑动脉病(CADASIL)患者中的作用尚不清楚,其影像学特征可能与有症状性弥散加权成像阳性(sDWI+)病变不同。我们旨在研究 CADASIL 患者中 aDWI+病变的临床影像学特征。

方法

我们使用来自台湾 CADASIL 登记处的数据进行了回顾性分析。aDWI+病变定义为无相应急性神经功能缺损的偶然发现的弥散加权成像阳性病变。我们比较了有和无 aDWI+病变的患者的基线临床特征,并分析了它们与 sDWI+病变的放射学特征和演变关系。

结果

在纳入的 154 例患者(平均年龄 62±10 岁)中,17 例(11%)有 aDWI+病变。两组的基线临床特征相似,但有 aDWI+病变的患者腔隙性梗死更多(中位数 8 个 vs. 2 个)、多发脑微出血(CMBs;85% vs. 40%)和前颞叶白质高信号(WMH;47% vs. 14%)。多变量分析显示,在校正多个腔隙性梗死、多发 CMBs 和总 WMH 评分后,aDWI+病变与前颞叶 WMH 相关(比值比 5.7,95%置信区间 1.5-21.0)。与 sDWI+病变相比,aDWI+病变更常为小梗死(<1cm;89% vs. 23%)且不太可能累及皮质脊髓束(11% vs. 96%)。在有随访磁共振成像的 11 个 aDWI+病变中,7 个变成微梗死,3 个变成腔隙性梗死,1 个消失。

结论

CADASIL 中的 aDWI+病变并不少见,与较高的小血管疾病和前颞叶 WMH 负担相关。需要进一步研究来评估其对 CADASIL 的长期影响。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e2d7/11554854/926b424a3e11/ENE-31-e16519-g003.jpg

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