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A 72-year-old woman with a seven-year history of Parkinson's disease, characterized by gait instability, rigidity, and postural instability, was admitted to our department for evaluation of recurrent episodes of hypoxemia and altered consciousness. During hospitalization, she experienced recurrent episodes of respiratory failure, prompting the measurement of anti-IgLON5 antibodies, which were found to be positive, leading to a diagnosis of anti-IgLON5 disease. The respiratory failure could not be attributed to vocal cord paralysis or respiratory muscle weakness, leading to the hypothesis that subglottic laryngeal spasm was the cause. Treatment with steroid pulse therapy resulted in improved ventilation. While several cases of anti-IgLON5 disease presenting with respiratory failure due to respiratory muscle weakness have been reported, this case suggests that subglottic laryngeal spasm may also be an underlying cause of respiratory failure in anti-IgLON5 disease.
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http://dx.doi.org/10.5692/clinicalneurol.cn-002014 | DOI Listing |
Clin Neurol Neurosurg
October 2025
Department of Neurology, Albany Medical Center, 43 New Scotland Ave. MC-70, Albany, NY 12208, USA. Electronic address:
We present a 67-year-old male with anti-IgLON5 disease with cervical and upper trunk dystonic spasms. Intermittent hallucinations, occurring both with infections and without a precipitant, and hand tremors were also present. The MRI of the brain was nonspecific.
View Article and Find Full Text PDFJ Sleep Res
August 2025
Department of Neurology, National Neuroscience Institute, Singapore General Hospital Campus, Singapore, Singapore.
This scoping review aims to synthesise prevailing scientific evidence on the sleep-related manifestations of anti-IgLON5 disease to identify distinctive sleep and/or polysomnographic patterns which may facilitate diagnosis. A systematic search was conducted to identify studies from 2014 to 2023 which included patients with sleep disturbances and immunohistochemically-proven anti-IgLON5 disease. Eligible studies included clinical trials, observational studies, and case series that contained descriptions of their sleep disturbances and/or polysomnographic features.
View Article and Find Full Text PDFJAMA Neurol
August 2025
Department of Neurology, St Josef Hospital, Ruhr-University Bochum, Bochum, Germany.
Importance: Anti-IgLON5 disease is an autoimmune encephalopathy that often leads to severe disability or death. The efficacy of immunotherapy remains unknown.
Objective: To investigate whether early immunotherapy is associated with disability and death in anti-IgLON5 disease.
Brain
July 2025
Department of Neurology and Experimental Neurology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität Berlin, Berlin, 10117, Germany.
Anti-IgLON5 disease is an autoimmune encephalitis that presents with a heterogenous clinical phenotype, including sleep disorders, movement abnormalities and bulbar involvement. It is characterised by autoantibodies against IgLON5, 85% association with HLA-DQB1*05:∼ and a brainstem-dominant tauopathy. Cellular and murine models report pathogenic effects of the autoantibodies, and neurodegenerative factors suggest progressive atrophy as a common sequela.
View Article and Find Full Text PDFAnn Indian Acad Neurol
May 2025
Department of Neurology, All India Institute of Medical Sciences, New Delhi, India.
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.
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