Objective: To review demographic, clinical features, treatment trends, and responses in Anti-IgLON5 disease with a focus on movement disorders

Background: Anti-IgLON5 antibody disease has emerged as a rare autoimmune cause of movement disorders. Knowledge regarding its clinical spectrum is expanding, with scarcity of data on treatment options and responses.

Method: A PubMed search was conducted to identify all English language articles reporting confirmed cases (serum/CSF) of Anti-IgLON5 antibody disease from inception to March 2024. Articles which were animal studies or full texts were not available were excluded. Descriptive statistical analysis was performed using Microsoft Excel to analyze data with available relevant information.

Results: We identified 220 articles, with 73 articles meeting inclusion criteria, reporting on 256 patients. Mean age was 62.1 ± 13.1 years with 56.7% males. The first case was reported in 2014. Movement disorders occurred at any point during the disease course in 55.7% (73/131) of cases. Median disease duration was 24 months (Range 0.1-204). The most frequently observed movement disorder was chorea (20.7%), followed by tremor (13.7%), parkinsonism (11.7%), ataxia (10.9%), dystonia (10.5%), and myoclonus (8.2%). Other reported neurological conditions included sleep disorders (75.7%), bulbar symptoms (58.7%), gait disturbance (47.7%), cognitive impairment (40.9%), dysautonomia (35.9%), abnormal eye movements (38.3%), and psychiatric symptoms (22.3%). MRI abnormalities were seen in 38.2% (55/144) cases. The treatments used included initial methylprednisolone in 48.2%, rituximab in 21.7%, IVIG in 30.2%, plasma exchange in 15.9%, azathioprine in 6.9%, cyclophosphamide in 5.3%, and mycophenolate mofetil in 4.1% of cases. Clinical outcomes were as follows (n 198): partial or good improvement in 29.3% of cases, no change in 15.2%, and poor outcomes in 22.7%, including 18.9% deaths.

Conclusion: Anti IGLON5 antibody disease can present with movement disorders affecting over half of the cases with sleep, gait, bulbar, cognitive and autonomic involvement as other major manifestations. Chorea emerges as the most prevalent movement disorder. Common treatments that have been utilized include methylprednisolone, rituximab, and IVIG, yet a significant portion of patients still experience poor outcomes, including mortality. These findings underscore the need for improved identification and therapeutic strategies in the management of IGLON5 disease.

References: 1. Sabater L, Gaig C, Gelpi E, et al.. A novel non-rapid-eye movement and rapid-eye-movement parasomnia with sleep breathing disorder associated with antibodies to IgLON5: a case series, characterisation of the antigen, and post-mortem study. Lancet Neurol. 2014;13(6):575-586.

2. Gaig C, Compta Y, Heidbreder A, et al. Frequency and Characterization of Movement Disorders in Anti-IgLON5 Disease. Neurology. 2021;97(14):e1367-e1381. Published 2021 Oct 4. doi:10.1212/WNL.0000000000012639

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MDS Abstracts - https://www.mdsabstracts.org/abstract/movement-disorders-in-anti-iglon5-antibody-disease/