In this article, Dr. Carles Gaig of Hospital Clínic of Barcelona discusses anti-IgLON5 disease, a newly identified neurologic disorder associated with antibodies in serum and CSF against IgLON5, a neuronal surface protein of unknown function. The disease is characterized by a distinctive sleep disorder associated with symptoms of brainstem dysfunction, gait instability, and a variety of other neurologic symptoms (eg, chorea, cognitive impairment). Anti-IgLON5 disease is associated with specific HLA haplotypes, but neuropathological examinations show a novel neuronal tauopathy predominantly involving the tegmentum of the brainstem and hypothalamus. At present, the exact pathogenesis is unclear, but these findings put anti-IgLON5 disease at the confluence of neurodegenerative and autoimmune mechanisms.
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