Kanbayashi Takashi, Shimohata Takayoshi, Nakashima Ichiro, Yaguchi Hiroaki, Yabe Ichiro, Nishizawa Masatoyo, Shimizu Tetsuo, Nishino Seiji
Department of Neuropsychiatry, Akita University School of Medicine, Akita City, Japan.
Arch Neurol. 2009 Dec;66(12):1563-6. doi: 10.1001/archneurol.2009.264.
To characterize factors that contribute to symptomatic narcolepsy and excessive daytime sleepiness in neuromyelitis optica and multiple sclerosis.
Japanese university hospitals.
Case study.
Seven Japanese patients whose initial diagnoses were multiple sclerosis and who were exhibiting excessive daytime sleepiness.
Lesions on magnetic resonance imaging, cerebrospinal fluid hypocretin-1 levels, and serum anti-aquaporin 4 (AQP4) antibody titer.
Bilateral and symmetrical hypothalamic lesions associated with marked or moderate hypocretin deficiency were found in all 7 cases. Four of these patients met the International Classification of Sleep Disorders 2 narcolepsy criteria. Three patients, including 2 patients with narcolepsy, were seropositive for anti-AQP4 antibody and diagnosed as having neuromyelitis optica-related disorder.
Since AQP4 is highly expressed in the hypothalamic periventricular regions, an immune attack on AQP4 may be partially responsible for the bilateral and hypothalamic lesions and hypocretin deficiency in narcolepsy/excessive daytime sleepiness associated with autoimmune demyelinating diseases.
明确视神经脊髓炎和多发性硬化症中导致症状性发作性睡病和日间过度嗜睡的因素。
日本大学医院。
病例研究。
7例最初诊断为多发性硬化症且表现出日间过度嗜睡的日本患者。
磁共振成像上的病变、脑脊液中食欲素-1水平以及血清抗水通道蛋白4（AQP4）抗体滴度。
所有7例患者均发现双侧对称性下丘脑病变，伴有明显或中度食欲素缺乏。其中4例患者符合《国际睡眠障碍分类第2版》发作性睡病标准。3例患者，包括2例发作性睡病患者，抗AQP4抗体血清学呈阳性，被诊断为患有视神经脊髓炎相关疾病。
由于AQP4在下丘脑室周区域高表达，针对AQP4的免疫攻击可能部分导致了与自身免疫性脱髓鞘疾病相关的发作性睡病/日间过度嗜睡中的双侧下丘脑病变和食欲素缺乏。
