Choroid plexus enlargement in amyotrophic lateral sclerosis patients and its correlation with clinical disability and blood-CSF barrier permeability

作者：Tingjun Dai, Jianwei Lou, Deyuan Kong, Jinyu Li, Qingguo Ren, Yujing Chen, Sujuan Sun, Yan Yun, Xiaohan Sun, Yiru Yang, Kai Shao, Wěi Li, Yuying Zhao, Xiangshui Meng, Chuanzhu Yan, Pengfei Lin, Shuangwu Liu · 发表于：Fluids and Barriers of the CNS · 年份：2024 · DOI：10.1186/s12987-024-00536-6 · 被引用次数：25 · 研究领域：Amyotrophic Lateral Sclerosis Research、Cerebrospinal fluid and hydrocephalus、Alzheimer's disease research and treatments

BACKGROUND: Using in vivo neuroimaging techniques, growing evidence has demonstrated that the choroid plexus (CP) volume is enlarged in patients with several neurodegenerative diseases, including Alzheimer's disease and Parkinson's disease. However, although animal and postmortem findings suggest that CP abnormalities are likely important pathological mechanisms underlying amyotrophic lateral sclerosis (ALS), the third most common neurodegenerative disease, no available study has been conducted to thoroughly assess CP abnormalities and their clinical relevance in vivo in ALS patients to date. Thus, we aimed to determine whether in vivo CP enlargement may occur in ALS patients. We also aimed to identify the relationships of CP volume with clinical disabilities and blood-CSF barrier (BCSFB) permeability in ALS patients. METHODS: In this retrospective study, based on structural MRI data, CP volume was assessed using a Gaussian mixture model and underwent further manual correction in 155 ALS patients and 105 age- and sex-matched HCs from October 2021 to April 2023. The ALS Functional Rating Scale-Revised (ALSFRS-R) was used to assess clinical disability. The CSF/serum albumin quotient (Qalb) was used to assess BCSFB permeability. Moreover, all the ALS patients completed genetic testing, and according to genetic testing, the ALS patients were further divided into genetic ALS subgroup and sporadic ALS subgroup. RESULTS: We found that compared with HCs, ALS patients had a significan...