Département de neurologie, Hôpital de la pitié salpêtrière, APHP, Sorbonne Université, Paris 75013, France.
Service des Pathologies du Sommeil, Centre de Référence narcolepsie et hypersomnies rares, Hôpital de la pitié salpêtrière, APHP, Sorbonne Université, Paris 75013, France.
Mult Scler Relat Disord. 2020 Feb;38:101869. doi: 10.1016/j.msard.2019.101869. Epub 2019 Nov 25.
The identification of AQP4-IgG, a specific and pathogenic antibody of NMO/SD has led to a broadening of the clinical spectrum of manifestations of NMO/SD including the presence of encephalic symptoms. Lesions are often distributed on peri‑ependymal area and sometimes affected the diencephalon leading to sleep disorders. We report a case of hypersomnia with polysomnographic documentation during the first attack of NMO/SD. Brain MRI revealed bilateral hypothalamic lesions around the third ventricle, whereas optic nerves and spinal cord were intact. The record of the nocturnal video-polysomnography followed by multiple sleep latency tests (MSLT) revealed an abnormal shortened sleep period with a single sleep onset in REM allowing secondary central hypersomnia diagnosis. The recovery of hypersomnia was complete within few months without psychostimulant treatment and the diencephalic lesion disappeared. Thus, diencephalic form of NMO/SD seems to cause narcolepsy or non-narcoleptic central hypersomnia and have a good recovery.
AQP4-IgG 的鉴定，一种 NMO/SD 的特定致病性抗体，导致了 NMO/SD 的临床表现谱的扩大，包括脑症状的出现。病变常分布在室管膜周围区域，有时累及间脑，导致睡眠障碍。我们报告了一例 NMO/SD 首次发作时伴有多导睡眠图记录的嗜睡症。脑 MRI 显示第三脑室周围双侧下丘脑病变，而视神经和脊髓正常。夜间视频多导睡眠图记录和多次小睡潜伏期试验（MSLT）显示，睡眠期异常缩短，快速眼动（REM）期单一起始，提示继发性中枢性嗜睡症的诊断。在没有使用精神兴奋剂治疗的情况下，嗜睡症在几个月内完全恢复，间脑病变消失。因此，NMO/SD 的间脑形式似乎引起发作性睡病或非发作性中枢性嗜睡症，并具有良好的恢复。
