Department of Neurology, Amiens University Hospital, France (E.O.).
Department of Neurology (A.D., L.G., E.J.), Lariboisière Hospital, AP-HP, Paris, France.
Stroke. 2023 Apr;54(4):e138-e141. doi: 10.1161/STROKEAHA.122.040938. Epub 2023 Feb 23.
In CADASIL (Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy), clinical severity is not related to the total burden of white matter hyperintensities (WMHs), presumably because of heterogeneous underlying tissue alterations. We aimed to investigate whether WMHs in the corpus callosum (WMH) are due to secondary degeneration and related to clinical severity.
We evaluated data from 228 CADASIL patients included in an ongoing prospective cohort with available 3-dimensional fluid-attenuated inversion recovery magnetic resonance imaging sequences. We analyzed in a blind manner WMH and lacunes in presumably connected areas to determine whether WMH are related to secondary degeneration. We evaluated the links between WMH and the Mattis dementia rating scale and the modified Rankin Scale-widely used measures of global cognitive performances and disability, respectively. Linear regression models were adjusted for age, sex, level of education, brain volume, number of lacunes, and volume of WMH.
Among 228 patients, only 105 (46%) had WMH while all had WMH in the rest of the white matter. In 74% of cases, WMH crossed a presumably connected nearby lacune, which was significantly higher than the expected value if the spatial distributions of WMH and nearby lacunes were unrelated (11%; <0.001). Patients with WMH had worse Mattis dementia rating scale (median [P25-P75], 138 [122-142] versus 143 [140-143]; <0.001) and worse modified Rankin Scale (2 [1-3] versus 1 [0-1]; <0.001). In adjusted models, Mattis dementia rating scale was significantly associated with WMH (estimate, -6.2 [95% CI, -11.8 to -0.1]).
In CADASIL, WMH are likely related to secondary degeneration and are independently related to clinical severity, in contrast to the total burden of WMH.
在 CADASIL（伴有皮质下梗死和白质脑病的常染色体显性脑动脉病）中，临床严重程度与脑白质高信号（WMH）的总负担无关，这可能是由于潜在的组织改变具有异质性。我们旨在研究胼胝体（WMH）中的 WMH 是否由于继发性退行性变而与临床严重程度相关。
我们评估了 228 例纳入正在进行的前瞻性队列研究的 CADASIL 患者的数据，这些患者均具有可用的 3 维液体衰减反转恢复磁共振成像序列。我们以盲法分析了假定相关区域的 WMH 和腔隙，以确定 WMH 是否与继发性退行性变有关。我们评估了 WMH 与 Mattis 痴呆评定量表和改良 Rankin 量表之间的联系，这两个量表分别是用于评估整体认知功能和残疾的常用指标。线性回归模型调整了年龄、性别、教育程度、脑容量、腔隙数量和 WMH 体积。
在 228 例患者中，只有 105 例（46%）有 WMH，而其余所有患者的脑白质均有 WMH。在 74%的情况下，WMH 穿过了假定相关的附近腔隙，这明显高于 WMH 和附近腔隙的空间分布无关时的预期值（11%；<0.001）。有 WMH 的患者的 Mattis 痴呆评定量表评分更差（中位数[25%至 75%分位数]，138 [122-142]与 143 [140-143]；<0.001），改良 Rankin 量表评分也更差（2 [1-3]与 1 [0-1]；<0.001）。在调整模型中，Mattis 痴呆评定量表评分与 WMH 显著相关（估计值，-6.2 [95%置信区间，-11.8 至 -0.1]）。
在 CADASIL 中，WMH 可能与继发性退行性变有关，并且与临床严重程度独立相关，这与 WMH 的总负担不同。
