Silva Ana Rita, Santos Irina, Fernandes Carolina, Silva Cristiana, Pereira Daniela, Galego Orlando, Queiroz Henrique, Almeida Maria do Rosário, Baldeiras Inês, Santo Gustavo
CINEICC - Center for Research in Neuropsychology and Cognitive Behavioral Interventions of the University of Coimbra, Colegio Novo Street, N/A, Coimbra 3000-115, Portugal.
Neurology Department, Academic and Clinical Centre - Coimbra University Hospitals, Coimbra, Portugal.
Cereb Circ Cogn Behav. 2023 Sep 27;5:100186. doi: 10.1016/j.cccb.2023.100186. eCollection 2023.
Cerebral Small Vessels Disease (CSVD) is categorized in different forms, the most common being the sporadic form and a genetic variant - Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL). Amongst the most frequent clinical manifestations are the neuropsychological changes of cognitive, behavioral, and emotional nature, whose features are still under debate.
This exploratory study aimed to compare the neuropsychological profile of a sporadic CSVD sample and a CADASIL sample with an age, education, and gender matched control group, between the ages of 30-65 YO (total sample mean age=51.16; SD=4.31).
20 patients with sporadic CSVD, 20 patients with CADASIL and 20 matched controls completed a neuropsychological assessment battery. Global cognitive state, processing speed, working memory, attention, executive dysfunction, episodic memory, social cognition, impulsivity, apathy, alexithymia, depression, and anxiety were measured. White matter hyperintensities (WMH) volume were quantified and measured as lesion burden.
The cognitive differences found between the clinical groups combined (after confirming no differences between the two clinical groups) and matched controls were restricted to speed processing scores ( = 0.32 95 % CI [.12-.47]). The socio-emotional and behavioral profile revealed significantly higher levels of depression ( = 0.21, 95 % CI [.16-.33]). and anxiety ( = 0.25 95 % CI [.19-.32]) in CADASIL and sporadic CSVD groups, and the same for the alexithymia score ( = 0.533 95 % CI [.32-.65]) were the clinical groups revealed impoverished emotional processing compared to controls. WMH only significantly correlated with the cognitive changes and age.
In our study, CADASIL and sporadic cSVD patients combined, present multiple emotional-behavioral symptoms - alexithymia, anxiety, depression, and in a lower extent apathy and impulsivity - suggesting for the presence of emotion dysregulation behaviors, present independently of age and of the presence of cognitive deficits. Despite of the small sample size that could underpower some findings, this exploratory research supported that these symptoms may have a significant impact in disease monitoring, progression, and prognosis, requiring further investigation regarding their neurophysiological substrates.
脑小血管病（CSVD）有不同的形式，最常见的是散发性形式和一种遗传变异型——伴有皮质下梗死和白质脑病的常染色体显性遗传性脑动脉病（CADASIL）。最常见的临床表现包括认知、行为和情感方面的神经心理变化，但其特征仍存在争议。
本探索性研究旨在比较散发性CSVD样本和CADASIL样本与年龄、教育程度和性别匹配的对照组（年龄在30 - 65岁之间，总样本平均年龄 = 51.16；标准差 = 4.31）的神经心理特征。
20例散发性CSVD患者、20例CADASIL患者和20例匹配的对照组完成了一套神经心理评估。测量了整体认知状态、处理速度、工作记忆、注意力、执行功能障碍、情景记忆、社会认知、冲动性、冷漠、述情障碍、抑郁和焦虑。对白质高信号（WMH）体积进行量化并作为病变负荷进行测量。
在确认两个临床组之间无差异后，合并的临床组与匹配对照组之间发现的认知差异仅限于处理速度得分（t = 0.32，95%置信区间[.12 -.47]）。社会情感和行为特征显示，CADASIL组和散发性CSVD组的抑郁水平（t = 0.21，95%置信区间[.16 -.33]）和焦虑水平（t = 0.25，95%置信区间[.19 -.32]）显著更高，述情障碍得分也是如此（t = 0.533，95%置信区间[.32 -.65]），表明与对照组相比，临床组的情感处理能力较差。WMH仅与认知变化和年龄显著相关。
在我们的研究中，CADASIL患者和散发性CSVD患者合并出现多种情绪行为症状——述情障碍、焦虑、抑郁，以及程度较低的冷漠和冲动性——提示存在情绪调节障碍行为，这些行为独立于年龄和认知缺陷而存在。尽管样本量较小可能使一些研究结果的说服力不足，但这项探索性研究支持这些症状可能对疾病监测、进展和预后有重大影响，需要进一步研究其神经生理基础。
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