使用[18F]PI-2620 PET 对抗 IgLON5 病中的 Tau 沉积进行体内测量。
In Vivo Measurement of Tau Depositions in Anti-IgLON5 Disease Using [18F]PI-2620 PET.
机构信息
From the Multimodal Neuroimaging Group (H.T., G.N.B., A.D., T.v.E.), Department of Nuclear Medicine, and Department of Neurology (H.T., T.v.E.), Faculty of Medicine and University Hospital Cologne, University of Cologne; Molecular Organization of the Brain (G.N.B., A.D.), Institute for Neuroscience and Medicine (INM-2), Forschungszentrum J̈lich; Department of Neurology (N.B.), Faculty of Medicine and University Hospital Schleswig Holstein (Lübeck), University of Lübeck; Institute of Clinical Chemistry (J.D., F.L., K.-P.W.), University Hospital Schleswig Holstein, Kiel/Lübeck German Center for Neurodegenerative Diseases (DZNE) (A.D.), Bonn-Cologne; Department of Neurology (T.G., I.A.), Faculty of Medicine and St. Josef-Hospital, Ruhr University Bochum; Department of Neurology (J.L.), Faculty of Medicine and University Hospital Ulm, Ulm University; Department of Neurology (F.L.), Faculty of Medicine and University Hospital Schleswig Holstein, Kiel University; Nuclear Chemistry (B.N.), Institute for Neuroscience and Medicine (INM-5), Forschungszentrum Jülich; and Institute of Radiochemistry and Experimental Molecular Imaging (B.N.), Faculty of Medicine and University Hospital Cologne, University of Cologne, Germany.
出版信息
Neurology. 2023 Nov 27;101(22):e2325-e2330. doi: 10.1212/WNL.0000000000207870.
OBJECTIVES
Anti-IgLON5 disease is a recently discovered neurologic disorder combining autoimmunity and neurodegeneration. Core manifestations include sleep disorders, bulbar symptoms, gait abnormalities, and cognitive dysfunction, but other presentations have been reported. Hallmarks are autoantibodies targeting the neuronal surface protein IgLON5, a strong human leukocyte antigen system Class II association, and brainstem and hypothalamus-dominant tau deposits. The purpose of this cohort study was to visualize tau deposition in vivo with the second-generation tau-PET tracer.
METHODS
A cohort of 4 patients with anti-IgLON5 disease underwent a dynamic PET scan with [18F]PI-2620. One patient received a follow-up scan. Z-deviation maps and a 2-sample test in comparison with healthy controls (n = 10) were performed. Antibody titers, neurofilament light chain, and disease duration were correlated with brainstem binding potentials.
RESULTS
Patients demonstrated increased [18F]PI2620 tau binding potentials in the pons, dorsal medulla, and cerebellum. The longitudinal scan after 28 months showed an increase of tracer uptake in the medulla despite immunotherapy. Higher antibody titers and neurofilament light chain correlated with higher tracer retention.
DISCUSSION
The results indicate that tau depositions in anti-IgLON5 disease can be visualized with [18F]PI-2620 and might correlate with the extent of disease. For validation, a larger longitudinal study is necessary.
目的
抗 IgLON5 病是一种新发现的神经疾病，其结合了自身免疫和神经退行性变。主要表现包括睡眠障碍、球部症状、步态异常和认知功能障碍，但也有其他表现的报道。其特征为针对神经元表面蛋白 IgLON5 的自身抗体，与人类白细胞抗原系统 II 类强关联，以及脑干和下丘脑为主的 tau 沉积。本队列研究的目的是使用第二代 tau-PET 示踪剂可视化体内 tau 沉积。
方法
4 名抗 IgLON5 病患者接受了 [18F]PI-2620 的动态 PET 扫描。一名患者接受了随访扫描。进行了 Z 偏移图和与健康对照组（n=10）的两样本 t 检验。抗体滴度、神经丝轻链和疾病持续时间与脑干结合势相关。
结果
患者在脑桥、背髓和小脑中显示出增加的 [18F]PI2620 tau 结合势。28 个月后的纵向扫描显示，尽管进行了免疫治疗，髓质中的示踪剂摄取仍增加。更高的抗体滴度和神经丝轻链与更高的示踪剂保留相关。
讨论
结果表明，[18F]PI-2620 可用于可视化抗 IgLON5 病中的 tau 沉积，并且可能与疾病的严重程度相关。为了验证，需要进行更大规模的纵向研究。
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