The Expanding Spectrum of Anti-IgLON5 Disease: A Case Series from an Indian Cohort.

IF 1.9 4区医学 Q3 CLINICAL NEUROLOGY

Annals of Indian Academy of Neurology Pub Date : 2025-04-22 DOI:10.4103/aian.aian_1073_24

Avinash Ganapule, Divyani Garg, Ayush Agarwal, Anu Gupta, Roopa Rajan, Soaham Desai, Mitesh Chandarana, S Sidharth, Madhavi Tripathi, Ajay Garg, Divya M Radhakrishnan, Achal Kumar Srivastava

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引用次数: 0

Abstract

Abstract: Anti-IgLON5 disease is an evolving entity that lies at the confluence of autoimmunity and neurodegeneration. Reports from India remain sparse. In this series, we describe seven Indian patients with anti-IgLON5-related disease. Patients presented across the fifth to eighth decades with a mean duration of illness of 16 months. All had movement disorders, which included gait ataxia, parkinsonism, and chorea. Six patients had sleep disturbances. Five had a frontal dysexecutive dementia phenotype. Two had epilepsy. Bulbar involvement was present in four, and one had amyotrophic lateral sclerosis (ALS)-like features. Magnetic resonance imaging was abnormal in two cases. Positron emission tomography of the brain also contributed to diagnosis. Combination immunotherapies were used in most of the patients, with three showing a sustained response and two deaths reported due to sepsis-related complications. It is important to recognize the increasing spectrum of IgLON5-related disease to enable timely initiation of immunotherapy before marked degeneration occurs.

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抗iglon5疾病的扩展谱：来自印度队列的病例系列

摘要：抗iglon5疾病是自身免疫和神经退行性变共同作用下的一种进化实体。来自印度的报道仍然很少。在本系列文章中，我们描述了7名患有抗iglon5相关疾病的印度患者。患者的年龄跨度为五到八十岁，平均病程为16个月。所有人都有运动障碍，包括步态共济失调、帕金森病和舞蹈病。6名患者有睡眠障碍。其中5人有额叶执行障碍痴呆表型。其中两人患有癫痫。其中4例受累于球部，1例有肌萎缩侧索硬化症（ALS）样特征。2例磁共振成像异常。脑部正电子发射断层扫描也有助于诊断。大多数患者使用联合免疫疗法，其中3例显示持续反应，2例因败血症相关并发症死亡。重要的是要认识到iglon5相关疾病的频谱增加，以便在显著变性发生之前及时启动免疫治疗。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Annals of Indian Academy of Neurology Nervous System Diseases-

CiteScore

2.20

自引率

11.80%

发文量

293

审稿时长

29 weeks

期刊介绍： The journal has a clinical foundation and has been utilized most by clinical neurologists for improving the practice of neurology. While the focus is on neurology in India, the journal publishes manuscripts of high value from all parts of the world. Journal publishes reviews of various types, original articles, short communications, interesting images and case reports. The journal respects the scientific submission of its authors and believes in following an expeditious double-blind peer review process and endeavors to complete the review process within scheduled time frame. A significant effort from the author and the journal perhaps enables to strike an equilibrium to meet the professional expectations of the peers in the world of scientific publication. AIAN believes in safeguarding the privacy rights of human subjects. In order to comply with it, the journal instructs all authors when uploading the manuscript to also add the ethical clearance (human/animals)/ informed consent of subject in the manuscript. This applies to the study/case report that involves animal/human subjects/human specimens e.g. extracted tooth part/soft tissue for biopsy/in vitro analysis.