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Decreased water exchange rate across blood-brain barrier in hereditary cerebral small vessel disease.
Li, Yingying; Ying, Yunqing; Yao, Tingyan; Jia, Xuejia; Liang, Huilou; Tang, Weijun; Jia, Xiuqin; Song, Haiqing; Shao, Xingfeng; Wang, Danny J J; Wang, Chaodong; Cheng, Xin; Yang, Qi.
Afiliação
  • Li Y; Department of Radiology, Beijing Chaoyang Hospital, Capital Medical University, Beijing 100020, China.
  • Ying Y; Key Lab of Medical Engineering for Cardiovascular Disease, Ministry of Education, Beijing 100020, China.
  • Yao T; Department of Neurology, National Center for Neurological Disorders, National Clinical Research Centre for Aging and Medicine, Huashan Hospital, Fudan University, Shanghai 200040, China.
  • Jia X; Department of Neurology, Xuanwu Hospital, Capital Medical University, National Clinical Research Center for Geriatric Disorders, Beijing 100053, China.
  • Liang H; Department of Radiology, Beijing Chaoyang Hospital, Capital Medical University, Beijing 100020, China.
  • Tang W; Key Lab of Medical Engineering for Cardiovascular Disease, Ministry of Education, Beijing 100020, China.
  • Jia X; State Key Laboratory of Brain and Cognitive Science, Beijing MRI Center for Brain Research, Institute of Biophysics, Chinese Academy of Sciences, Beijing 100101, China.
  • Song H; Department of Radiology, Huashan Hospital, Fudan University, Shanghai 200040, China.
  • Shao X; Department of Radiology, Beijing Chaoyang Hospital, Capital Medical University, Beijing 100020, China.
  • Wang DJJ; Key Lab of Medical Engineering for Cardiovascular Disease, Ministry of Education, Beijing 100020, China.
  • Wang C; Department of Neurology, Xuanwu Hospital, Capital Medical University, National Clinical Research Center for Geriatric Disorders, Beijing 100053, China.
  • Cheng X; Laboratory of FMRI Technology (LOFT), USC Mark & Mary Stevens Neuroimaging and Informatics Institute, Keck School of Medicine, University of Southern California, Los Angeles, CA 90033, USA.
  • Yang Q; Laboratory of FMRI Technology (LOFT), USC Mark & Mary Stevens Neuroimaging and Informatics Institute, Keck School of Medicine, University of Southern California, Los Angeles, CA 90033, USA.
Brain ; 146(7): 3079-3087, 2023 07 03.
Article em En | MEDLINE | ID: mdl-36625892
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) and heterozygous HTRA1 mutation-related cerebral small vessel disease (CSVD) are the two types of dominant hereditary CSVD. Blood-brain barrier (BBB) failure has been hypothesized in the pathophysiology of CSVD. However, it is unclear whether there is BBB damage in the two types of hereditary CSVD, especially in heterozygous HTRA1 mutation-related CSVD. In this study, a case-control design was used with two disease groups including CADASIL (n = 24), heterozygous HTRA1 mutation-related CSVD (n = 9) and healthy controls (n = 24). All participants underwent clinical cognitive assessments and brain MRI. Diffusion-prepared pseudo-continuous arterial spin labelling was used to estimate the water exchange rate across the BBB (kw). Correlation and multiple linear regression analyses were used to examine the association between kw and disease burden and neuropsychological performance, respectively. Compared with the healthy controls, kw in the whole brain and multiple brain regions was decreased in both CADASIL and heterozygous HTRA1 mutation-related CSVD patients (Bonferroni-corrected P < 0.007). In the CADASIL group, decreased kw in the whole brain (ß = -0.634, P = 0.001), normal-appearing white matter (ß = -0.599, P = 0.002) and temporal lobe (ß = -0.654, P = 0.001) was significantly associated with higher CSVD score after adjusting for age and sex. Reduced kw in the whole brain was significantly associated with poorer neuropsychological performance after adjusting for age, sex and education in both CADASIL and heterozygous HTRA1 mutation-related CSVD groups (ß = 0.458, P = 0.001; ß = 0.884, P = 0.008). This study showed that there was decreased water exchange rate across the BBB in both CADASIL and heterozygous HTRA1 mutation-related CSVD patients, suggesting a common pathophysiological mechanism underlying the two types of hereditary CSVD. These results highlight the potential use of kw for monitoring the course of CADASIL and heterozygous HTRA1 mutation-related CSVD, a possibility which should be tested in future research.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: CADASIL / Doenças de Pequenos Vasos Cerebrais Limite: Humans Idioma: En Revista: Brain Ano de publicação: 2023 Tipo de documento: Article País de afiliação: China

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: CADASIL / Doenças de Pequenos Vasos Cerebrais Limite: Humans Idioma: En Revista: Brain Ano de publicação: 2023 Tipo de documento: Article País de afiliação: China