The relevance of the socio-emotional deficits in cerebral small vessels disease (CSVD): An exploratory study with sporadic CSVD and CADASIL patients.

Background: 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.

Objective: 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).

Methods: 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.

Results: 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.

Conclusions: 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.