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1.
J Neurol ; 270(5): 2370-2379, 2023 May.
Article in English | MEDLINE | ID: mdl-36869886

ABSTRACT

INTRODUCTION: COVID-19 restrictive containment was responsible for major psychological distress and alteration of quality of life (QoL) in the general population. Their impact in a group of patients having cerebral small vessel disease (SVD) and at high risk of stroke and disability was unknown. OBJECTIVE: We aimed to determine the potential psychological impact of strict containment during the COVID-19 pandemic in a sample of CADASIL patients, a rare SVD caused by NOTCH3 gene mutations. METHODS: Interviews of 135 CADASIL patients were obtained just after the end of the strict containment in France. Depression, QoL and negative subjective experience of the containment were analysed, as well as predictors of posttraumatic and stressor-related manifestations, defined as an Impact Event Scale-Revised score ≥ 24, using multivariable logistic analysis. RESULTS: Only 9% of patients showed a depressive episode. A similar proportion had significant posttraumatic and stressor-related disorder manifestations independently associated only with socio-environment factors, rather than clinical ones: living alone outside a couple (OR 7.86 (1.87-38.32), unemployment (OR 4.73 (1.17-18.70)) and the presence of 2 or more children at home (OR 6.34 (1.35-38.34). CONCLUSION: Psychological impact of the containment was limited in CADASIL patients and did not appear related to the disease status. About 9% of patients presented with significant posttraumatic and stressor-related disorder manifestations which were predicted by living alone, unemployment, or exhaustion related to parental burden.


Subject(s)
CADASIL , COVID-19 , Cerebral Small Vessel Diseases , Child , Humans , CADASIL/complications , CADASIL/epidemiology , CADASIL/genetics , Quality of Life , Pandemics , COVID-19/complications , Cerebral Small Vessel Diseases/complications , Receptor, Notch3/genetics , Mutation , Receptors, Notch/genetics
2.
AJNR Am J Neuroradiol ; 38(11): 2094-2099, 2017 Nov.
Article in English | MEDLINE | ID: mdl-28912283

ABSTRACT

BACKGROUND AND PURPOSE: Reaction time was recently recognized as a marker of subtle cognitive and behavioral alterations in the early clinical stages of CADASIL, a monogenic cerebral small-vessel disease. In unselected patients with CADASIL, brain atrophy and lacunes are the main imaging correlates of disease severity, but MR imaging correlates of reaction time in mildly affected patients are unknown. We hypothesized that reaction time is independently associated with the corpus callosum area in the early clinical stages of CADASIL. MATERIALS AND METHODS: Twenty-six patients with CADASIL without dementia (Mini-Mental State Examination score > 24 and no cognitive symptoms) and without disability (modified Rankin Scale score ≤ 1) were compared with 29 age- and sex-matched controls. Corpus callosum area was determined on 3D-T1 MR imaging sequences with validated methodology. Between-group comparisons were performed with t tests or χ2 tests when appropriate. Relationships between reaction time and corpus callosum area were tested using linear regression modeling. RESULTS: Reaction time was significantly related to corpus callosum area in patients (estimate = -7.4 × 103, standard error = 3.3 × 103, P = .03) even after adjustment for age, sex, level of education, and scores of depression and apathy (estimate = -12.2 × 103, standard error = 3.8 × 103, P = .005). No significant relationship was observed in controls. CONCLUSIONS: Corpus callosum area, a simple and robust imaging parameter, appears to be an independent correlate of reaction time at the early clinical stages of CADASIL. Further studies will determine whether corpus callosum area can be used as an outcome in future clinical trials in CADASIL or in more prevalent small-vessel diseases.


Subject(s)
CADASIL/diagnostic imaging , CADASIL/psychology , Corpus Callosum/diagnostic imaging , Reaction Time , Adult , Aged , Atrophy , Cerebral Small Vessel Diseases/diagnostic imaging , Disease Progression , Female , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Neuropsychological Tests
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