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. 2025 May 1;28(3):440-444.
doi: 10.4103/aian.aian_1073_24. Epub 2025 Apr 22.

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

Avinash Ganapule  1 , Divyani Garg  1 , Ayush Agarwal  1 , Anu Gupta  1 , Roopa Rajan  1 , Soaham Desai  2 , Mitesh Chandarana  3 , S Sidharth  1 , Madhavi Tripathi  4 , Ajay Garg  5 , Divya M Radhakrishnan  1 , Achal Kumar Srivastava  1
Affiliations

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

Avinash Ganapule et al. Ann Indian Acad Neurol. .

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.

Keywords: Autoimmune; chorea; dementia.

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Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1
Figure 1
F-18 FDG PET maximum intensity projection image shows that (a) no focal metabolically active lesion has been identified. Transaxial plain FDG PET image of the brain (b) at the level of BG reveals BG hypermetabolism and (c) occipital hypometabolism. Cortex ID maps showing minimal prefrontal hypometabolism (d). BG: basal ganglia, PET: positron emission tomography, ID: identification, FDG: fluorodeoxyglucose
Figure 2
Figure 2
F-18 FDG PET maximum intensity projection image shows that (a) no focal metabolically active lesion has been identified. Transaxial plain FDG PET image of the brain (b) at the level of BG reveals BG hypermetabolism and occipital hypometabolism (white arrow). Sagittal plain FDG PET image shows brainstem hypermetabolism (c, white arrow). Cortex id maps showing significant hypometabolism in the occipital lobe (d). BG: basal ganglia, PET: positron emission tomography, F-18 FDG: 18-fluorodeoxyglucose
See this image and copyright information in PMC

References

    1. Gaig C, Graus F, Compta Y, Högl B, Bataller L, Brüggemann N, et al. Clinical manifestations of the anti-IgLON5 disease. Neurology. 2017;88:1736–43. - PMC - PubMed
    1. Tao QQ, Wei Q, Song SJ, Yin XZ. Motor neuron disease-like phenotype associated with anti-IgLON5 disease. CNS Neurosci Ther. 2018;24:1305–8. - PMC - PubMed
    1. Bhatia M, Singh Y. Anti IgLON5 disease - first case report from India. Sleep Medicine. 2020;67:215–6. - PubMed
    1. Soman Pillai K, Maramattom BV, Gopalannair Santhamma S, Kishore A. Head drop and trunk flexion as an early manifestation of anti-IgLON5 disease. Mov Disord Clin Pract. 2022;10:135–7. - PMC - PubMed
    1. Gurram S, Holla VV, Kamath SD, Prakash SS, Dubbal R, Kamble N, et al. Facio-Lingual-Palatal Myorhythmic Presentation of Anti-IgLON5 Disease. Mov Disord Clin Pract. 2023;10:1427–9. - PMC - PubMed