Association of Glymphatic Function With Clinical Characteristics in Patients With Clinical and Asymptomatic Creutzfeldt-Jakob Disease

作者：Zhongyun Chen, Deming Jiang, Yu Kong, Jing Zhang, Chu Min, Sheng Bi, Shaozhen Yan, Hong Ye, Junjie Li, Lin Wang, Jie Lu, Liyong Wu · 发表于：Neurology · 年份：2024 · DOI：10.1212/wnl.0000000000210055 · 被引用次数：5 · 研究领域：Cerebrospinal fluid and hydrocephalus、Prion Diseases and Protein Misfolding、Genetic and Kidney Cyst Diseases

BACKGROUND AND OBJECTIVES: Abnormal glymphatic system-related proteins have been identified in a small-scale pathologic study of patients with Creutzfeldt-Jakob disease (CJD). However, it remains unclear whether glymphatic dysfunction occurs in vivo in patients with CJD and whether this decline begins during the preclinical stage. This study aimed to investigate the relationship between glymphatic dysfunction and clinical characteristics in patients with CJD, as well as potential glymphatic impairment in preclinical CJD. METHODS: F-fludeoxyglucose-PET to identify CJD-related metabolic patterns. Associations between the DTI-ALPS index and Medical Research Council Prion Disease Rating Scale (MRC-PDRS) score were evaluated using multiple linear regression. RESULTS: = 0.014) were associated with a lower DTI-ALPS index. The DTI-ALPS index of asymptomatic G114V carriers showed no significant difference compared with noncarriers. However, a preclinical CJD case exhibited an 8.2% decrease in the DTI-ALPS index 3.3 years before onset. No significant correlation was found between regional metabolic standardized uptake value ratios and DTI-ALPS index. DISCUSSION: Our study indicates that glymphatic dysfunction is associated with CJD severity and disease progression. Glymphatic dysfunction may occur in the preclinical stage, but these findings should be interpreted with caution because they are based on individual findings.