Objective sleep profile in LGI1 / CASPR2 autoimmunity

This article was originally published here

Sleep. December 25, 2021: zsab297. doi: 10.1093 / sleep / zsab297. before printing.

ABSTRACT

STUDY AIMS: Rapid Eye Movement (REM) sleep behavior disorder (RBD) and other sleep disorders occur frequently in leucine-rich, gliomine-activated protein-1-IgG () and contactin-associated protein-2-IgG (CASPR2) autoimmunity, but in polysomnographic analyzes these disturbances remain limited. Our aim was to characterize clinical presentations and analyze polysomnographic manifestations, especially quantitative REM sleep without atony (RSWA) in / CASPR2-IgG-seropositive (LGI / CASPR2 +) patients.

METHODS: We retrospectively analyzed clinical and polysomnographic features and quantitative RSWA between + / CASPR2 + patients and age- and sex-matched controls. The groups were compared using Wilcoxon’s sum of ranks and chi-square tests. The combined submental and tibial anterior RSWA (SM + AT) was the primary endpoint.

RESULTS: Out of 11 (LGI1 +, n = 9; CASPR2 +, n = 2) patients, Morvan syndrome sleep characteristics were present in 7 (63.6%) LGI1 + / CASPR2 + patients, with simultaneous insomnia and DEB in 3 (27.3%) ) and the most frequently presented sleep disorders in dream behavior (DEB, n = 5), insomnia (n = 5) and sleep apnea (n = 8; mean apnea-hypopnea index = 15 / hour). The median Epworth Sleepiness Scale (ESS) was 9 (range 3-24; n = 10), with hypersomnia in 4 (36.4%). LGI1 + / CASPR2 + patients had increased N1 sleep (p = 0.02), decreased REM sleep (p = 0.001), and higher SM + AT values ​​for each RSWA (p <0.001). Eight out of 9 (89%) LGI1 + exceeded RBD-RSWA thresholds (DEB, n = 5; isolated RSWA, n = 3). The RSWA was larger in the tibialis anterior than in the submentalis. All 10 LGI1 + / CASPR2 + patients treated with immunotherapy benefited and 5/10 had an improvement in sleep disorders.

CONCLUSION: LGI1 / CASPR2-IgG autoimmunity is associated with prominent dream staging, insomnia, RSWA, sleep apnea, and shallower sleep. provides objective disease markers for LGI1 + / CASPR2 + autoimmunity, and immunotherapy can be beneficial for associated sleep disorders.

PMID: 34953167 | DOI: 10.1093 / sleep / zsab297

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