P014 IgLON5 Disease – A Review of 2 Cases and Sleep Manifestations

Abstract Background IgLON5 disease is a novel autoimmune tauopathy with diverse sleep and neurologic manifestations. Sleep disorder common initial presentation may precede progression. Neurological symptoms frequently overlap extrapyramidal syndromes, motor neuron dementia. Diagnosis confirmed by anti-IgLON5 IgG in CSF and/or serum. Strong HLA-DRB1*1001 HLA-DQB1*0501 association supports mechanism. Video-PSG recommended to identify treatable sleep-disordered breathing parasomnia, present up 90% of cases. Treatment empirical immunomodulation plus supportive cares. Mortality high due sudden cardiorespiratory arrest Methods Case reports. Progress date 1 - 70-year-old male recurrent respiratory arrests apparent mixed aetiology progressive dysphagia over 6 months. Preliminary investigations were unremarkable. Tracheostomy was inserted immunoglobulin empirically commenced. antibodies identified on CSF. positive. Pulse methylprednisolone rituximab administered. Intermittent failure subsided but bulbar progress. PSG showed NREM REM parasomnia ataxic breathing. 2 58-year-old man presenting altered level consciousness. Examination findings included unsteady gait, strabismus, myoclonus, truncal instability. study Patient treated IVIG CPAP. History suspicious for evolving parasomnia. Intended outcome impact newly recognized The has not been well described. teases between autoimmunity neurodegeneration.