Ganapule Avinash, Garg Divyani, Agarwal Ayush, Gupta Anu, Rajan Roopa, Desai Soaham, Chandarana Mitesh, Sidharth S, Tripathi Madhavi, Garg Ajay, Radhakrishnan Divya M, Srivastava Achal Kumar
Department of Neurology, All India Institute of Medical Sciences, New Delhi, India.
Department of Neurology, Shree Krishna Hospital and Pramukhswami Medical College and Shree Krishna Hospital, Karamsad, Anand, Gujarat, India.
Ann Indian Acad Neurol. 2025 May 1;28(3):440-444. doi: 10.4103/aian.aian_1073_24. Epub 2025 Apr 22.
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.
抗IgLON5病是一种不断演变的疾病,处于自身免疫和神经退行性变的交汇点。来自印度的报道仍然很少。在本系列中,我们描述了7例患有抗IgLON5相关疾病的印度患者。患者年龄分布在50至80岁之间,平均病程为16个月。所有患者均有运动障碍,包括步态共济失调、帕金森综合征和舞蹈症。6例患者有睡眠障碍。5例有额叶执行功能障碍痴呆表型。2例有癫痫。4例有延髓受累,1例有肌萎缩侧索硬化(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。大多数患者使用了联合免疫疗法,3例有持续反应,2例因败血症相关并发症死亡。认识到IgLON5相关疾病不断增加的谱型很重要,以便在明显退变发生之前及时启动免疫治疗。