title: Neuromyelitis optica spectrum disorder presenting with repeated hypersomnia due to involvement of the hypothalamus and hypothalamus-amygdala linkage.
abstract: We report the case of a 46-year-old Japanese woman with neuromyelitis optica spectrum disorder presenting with repeated hypersomnia accompanied by decreased CSF orexin level. First episode associated with hypothalamic-pituitary dysfunction showed bilateral hypothalamic lesions that can cause secondary damage to the orexin neurons. The second episode associated with impaired memory showed a left temporal lesion involving the amygdala. The mechanism remains unknown, but the reduced blood flow in the hypothalamus ipsilateral to the amygdala lesion suggested trans-synaptic hypothalamic dysfunction secondary to the impaired amygdala. A temporal lesion involving the amygdala and hypothalamus could be responsible for hypersomnia due to neuromyelitis optica spectrum disorder.
authors: Department of Gastroenterology and Neurology, Kagawa University Faculty of Medicine, Japan.
authors: Kodai
authors: K
authors: Kume
authors: Department of Gastroenterology and Neurology, Kagawa University Faculty of Medicine, Japan kdeguchi@med.kagawa-u.ac.jp.
authors: Kazushi
authors: K
authors: Deguchi
authors: Department of Neurological Intractable Disease Research, Kagawa University Faculty of Medicine, Japan.
authors: Kazuyo
authors: K
authors: Ikeda
authors: Department of Gastroenterology and Neurology, Kagawa University Faculty of Medicine, Japan.
authors: Tadayuki
authors: T
authors: Takata
authors: Department of Gastroenterology and Neurology, Kagawa University Faculty of Medicine, Japan.
authors: Yohei
authors: Y
authors: Kokudo
authors: Department of Neurological Intractable Disease Research, Kagawa University Faculty of Medicine, Japan.
authors: Masaki
authors: M
authors: Kamada
authors: Department of Health Sciences, Kagawa University Faculty of Medicine, Japan.
authors: Tetsuo
authors: T
authors: Touge
authors: Department of Neurology, Tohoku University Graduate School of Medicine, Japan; Department of Neurology, Yonezawa National Hospital, Japan.
authors: Toshiyuki
authors: T
authors: Takahashi
authors: Department of Neuropsychiatry, Akita University School of Medicine, Japan.
authors: Takashi
authors: T
authors: Kanbayashi
authors: Department of Gastroenterology and Neurology, Kagawa University Faculty of Medicine, Japan.
authors: Tsutomu
authors: T
authors: Masaki
mesh_terms: D000679:Amygdala / Q000473:pathology*; D006970:Disorders of Excessive Somnolence / Q000209:etiology*; D005260:Female; D006801:Humans; D007031:Hypothalamus / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D009471:Neuromyelitis Optica / Q000134:cerebrospinal fluid / Q000150:complications / Q000473:pathology*; D000068797:Orexins / Q000134:cerebrospinal fluid
chemical_list: D000068797:Orexins
keywords: Neuromyelitis optica spectrum disorder; amygdala; aquaporin 4; hypersomnia; hypothalamus; orexin