title: A patient with anti-aquaporin 4 antibody presenting hypersomnolence as the initial symptom and symmetrical hypothalamic lesions.
abstract: Here we report a case with positive serum anti-aquaporin 4 (AQP4) antibody who presented with hypersomnolence, symmetrical hypothalamic lesions and a reduced CSF orexin (hypocretin) level without optic nerve and spinal cord lesions on MRI. All of the symptoms, MRI finding and CSF orexin level improved simultaneously after steroid therapy. AQP4 is a member of the AQP superfamily which is strongly expressed in the hypothalamus where orexin (hypocretin)-containing neurons are primarily concentrated. Although there have been only a few reports similar to our case, the present case suggests a close relationship between the positive serum anti-AQP4 antibody and symmetrical hypothalamic lesions with hypersomnolence and without optic /spinal lesion, which is improved by steroid treatment.
authors.affiliation: Department of Neurology, Graduate School of Medicine and Dentistry, Okayama University, Okayama, 700-8558 Japan.
authors.forename: Kentaro
authors.initials: K
authors.lastname: Deguchi
authors.forename: Syoichiro
authors.initials: S
authors.lastname: Kono
authors.forename: Shoko
authors.initials: S
authors.lastname: Deguchi
authors.forename: Nobutoshi
authors.initials: N
authors.lastname: Morimoto
authors.forename: Masami
authors.initials: M
authors.lastname: Ikeda
authors.forename: Tomoko
authors.initials: T
authors.lastname: Kurata
authors.forename: Yoshio
authors.initials: Y
authors.lastname: Ikeda
authors.forename: Tohru
authors.initials: T
authors.lastname: Matsuura
authors.forename: Takashi
authors.initials: T
authors.lastname: Kanbayashi
authors.forename: Toshiyuki
authors.initials: T
authors.lastname: Takahashi
authors.forename: Koji
authors.initials: K
authors.lastname: Abe
mesh_terms: D000328:Adult; D051401:Aquaporin 4 / Q000276:immunology*; D001323:Autoantibodies / Q000097:blood*; D003937:Diagnosis, Differential; D006970:Disorders of Excessive Somnolence / Q000175:diagnosis / Q000276:immunology* / Q000473:pathology*; D005260:Female; D006801:Humans; D007031:Hypothalamus / Q000276:immunology / Q000473:pathology*
chemical_list: C496030:AQP4 protein, human; D051401:Aquaporin 4; D001323:Autoantibodies