Fan Siyuan, Jia Chenhao, Liang Menglin, Ren Haitao, Zhang Tianhao, Li Qijun, Huang Zhaoxia, Yen Tzu-Chen, OuYang Chujun, Cui Ruixue, Guan Hongzhi
Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100730, China.
Department of Nuclear Medicine, Peking Union Medical College Hospital Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100730, China.
J Neurol. 2025 Jan 15;272(2):115. doi: 10.1007/s00415-024-12874-4.
Anti-IgLON5 disease is a rare autoimmune neurological disorder with prominent Tau protein deposits in the brainstem and hypothalamus. The aim of this study was to visualize the in vivo distribution patterns of Tau protein in patients with anti-IgLON5 disease using the second-generation Tau PET tracer, Florzolotau (18F) PET imaging.
Patients diagnosed with anti-IgLON5 disease were enrolled consecutively. Age- and sex-matched healthy controls (HCs) were also enrolled. The uptake of Florzolotau (18F) and F-FDG was assessed using both visual and semi-quantitative analysis techniques.
A total of 10 patients with anti-IgLON5 disease and 40 HCs were included in the study. All ten patients with anti-IgLON5 disease underwent Florzolotau (18F) PET scans, and five of them underwent F-FDG PET scans. Twenty HCs underwent Florzolotau (18F) PET scans, and the remaining 20 HCs underwent F-FDG PET scans. In patients with anti-IgLON5 disease, significant uptake of Florzolotau (18F) was observed predominantly in the midbrain, pons, cerebellum, caudate, and putamen. This uptake pattern was notably absent in the control group. Moreover, semi-quantitative analysis techniques demonstrated widespread hypometabolism in the cerebral cortex in patients with anti-IgLON5 disease.
This study indicates distinct Tau protein deposition patterns in patients with anti-IgLON5 disease, potentially serving as imaging biomarkers.
抗IgLON5病是一种罕见的自身免疫性神经疾病,脑干和下丘脑有明显的 Tau 蛋白沉积。本研究的目的是使用第二代 Tau 正电子发射断层显像(PET)示踪剂 Florzolotau(18F)PET 成像来可视化抗 IgLON5 病患者体内 Tau 蛋白的分布模式。
连续纳入诊断为抗 IgLON5 病的患者。还纳入了年龄和性别匹配的健康对照(HCs)。使用视觉和半定量分析技术评估 Florzolotau(18F)和 F-FDG 的摄取情况。
本研究共纳入 10 例抗 IgLON5 病患者和 40 例健康对照。所有 10 例抗 IgLON5 病患者均接受了 Florzolotau(18F)PET 扫描,其中 5 例还接受了 F-FDG PET 扫描。20 例健康对照接受了 Florzolotau(18F)PET 扫描,其余 20 例健康对照接受了 F-FDG PET 扫描。在抗 IgLON5 病患者中,主要在中脑、脑桥、小脑、尾状核和壳核观察到 Florzolotau(18F)的显著摄取。对照组中明显没有这种摄取模式。此外,半定量分析技术显示抗 IgLON5 病患者大脑皮质广泛代谢减低。
本研究表明抗 IgLON5 病患者存在独特的 Tau 蛋白沉积模式,可能作为影像学生物标志物。