Objective sleep profile in LGI1/CASPR2 autoimmunity

Sleep

article

Devine, Michelle F¹  Feemster, John C¹  Lieske, Elizabeth A⁵  McCarter, Stuart J¹  Sandness, David J¹  Steele, Tyler⁵  Timm, Paul C¹  Mandrekar, Jay³  Boeve, Bradley F¹  Silber, Michael H¹  Dubey, Divyanshu³  McKeon, Andrew³  St. Louis, Erik K¹ 

[1] Mayo Clinic Center for Sleep Medicine, Mayo Clinic and Foundation;Department of Medicine, Division of Pulmonary, Critical Care, and Sleep Medicine, Mayo Clinic and Foundation;Department of Neurology, Mayo Clinic and Foundation;Olmsted Medical Center;Mayo Sleep Behavior and Neurophysiology Research Laboratory, Mayo Clinic and Foundation;Department of Biostatistics, Mayo Clinic and Foundation;Department of Pathology/Laboratory Medicine, Mayo Clinic and Foundation;Mayo Clinic Health System Southwest Wisconsin-La Crosse, Mayo Clinic and Foundation

关键词: LGI1;    CASPR2;    Morvan syndrome;    REM behavioral disorder;    autoimmune sleep;   

DOI  :  10.1093/sleep/zsab297

学科分类：生理学

来源: American Academy of Sleep Medicine

【 摘 要 】

Study Objectives Rapid eye movement (REM) sleep behavior disorder (RBD) and other sleep disturbances are frequent in leucine-rich, glioma inactivated protein 1-IgG (LGI1) and contactin-associated protein 2-IgG (CASPR2) autoimmunity, yet polysomnographic analyses of these disorders remain limited. We aimed to characterize clinical presentations and analyze polysomnographic manifestations, especially quantitative REM sleep without atonia (RSWA) in LGI1/CASPR2-IgG seropositive (LGI/CASPR2+) patients.Methods We retrospectively analyzed clinical and polysomnographic features and quantitative RSWA between LGI1+/CASPR2+ patients and age-sex matched controls. Groups were compared with Wilcoxon rank-sum and chi-square tests. Combined submentalis and anterior tibialis (SM + AT) RSWA was the primary outcome.Results Among 11 (LGI1+, n = 9; CASPR2+, n = 2) patients, Morvan syndrome sleep features were present in seven (63.6%) LGI1+/CASPR2+ patients, with simultaneous insomnia and dream enactment behavior (DEB) in three (27.3%), and the most common presenting sleep disturbances were DEB (n = 5), insomnia (n = 5), and sleep apnea (n = 8; median apnea-hypopnea index = 15/hour). Median Epworth Sleepiness Scale was nine (range 3–24; n = 10), with hypersomnia in four (36.4%). LGI1+/CASPR2+ patients had increased N1 sleep (p = .02), decreased REM sleep (p = .001), and higher levels of SM + AT any RSWA (p < .001). Eight of nine (89%) LGI1+ exceeded RBD RSWA thresholds (DEB, n = 5; isolated RSWA, n = 3). RSWA was greater in AT than SM. All 10 LGI1+/CASPR2+ patients treated with immunotherapy benefitted, and 5/10 had improved sleep disturbances.Conclusions LGI1/CASPR2-IgG autoimmunity is associated with prominent dream enactment, insomnia, RSWA, sleep apnea, and shallower sleep. Polysomnography provides objective disease markers in LGI1+/CASPR2+ autoimmunity and immunotherapy may benefit associated sleep disturbances.

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