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In 2014, a novel tauopathy characterized by REM sleep behavior disorder (RBD), sleep apnea, and stridor was first reported.1 Association with serum and CSF antibodies against IgLON5, a neuronal cell adhesion molecule of unknown function, was found in all patients.1 Neuropathologic examinations revealed neuronal accumulation of hyperphosphorylated 3-repeat (3R) and 4-repeat (4R) tau deposits in the hypothalamus and tegmental brainstem nuclei as the most relevant finding in the absence of inflammatory changes.2
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Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
- Received December 4, 2017.
- Accepted in final form April 5, 2018.
- © 2018 American Academy of Neurology
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