The Pathophysiologic Basis of Secondary Narcolepsy and Hypersomnia
The symptoms of narcolepsy can occur during the course of other neurologic conditions (ie, symptomatic narcolepsy). Inherited disorders, tumors, and head trauma were the three most frequent causes for symptomatic narcolepsy. Other causes include multiple sclerosis (MS), vascular disorders, and encep...
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Published in | Current neurology and neuroscience reports Vol. 11; no. 2; pp. 235 - 241 |
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Main Authors | , , , , , , |
Format | Journal Article |
Language | English |
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Current Science Inc
01.04.2011
Springer Nature B.V |
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Abstract | The symptoms of narcolepsy can occur during the course of other neurologic conditions (ie, symptomatic narcolepsy). Inherited disorders, tumors, and head trauma were the three most frequent causes for symptomatic narcolepsy. Other causes include multiple sclerosis (MS), vascular disorders, and encephalitis. Cerebrospinal fluid hypocretin-1 measures were carried out in some recent cases with symptomatic narcolepsy, and moderate decreases in hypocretin levels were seen in a large majority of these cases. Excessive daytime sleepiness (EDS) in these symptomatic cases was sometimes reversible with an improvement of the causative neurologic disorder and with an improvement of the hypocretin (orexin) status. Recently, we found that several symptomatic narcoleptic cases with MS show unique bilateral symmetric hypothalamic lesions associated with significant hypocretin ligand deficiency. In addition, these patients often share the clinical characteristics of neuromyelitis optica (NMO) and the detection of NMO-IgG (or anti-aquaporin-4 [AQP4] antibodies), suggesting a new clinical entity. Further studies of the involvement of the hypocretin system in symptomatic narcolepsy and EDS are helpful to understand the pathophysiologic mechanisms for occurrence of EDS and cataplexy. |
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AbstractList | The symptoms of narcolepsy can occur during the course of other neurologic conditions (ie, symptomatic narcolepsy). Inherited disorders, tumors, and head trauma were the three most frequent causes for symptomatic narcolepsy. Other causes include multiple sclerosis (MS), vascular disorders, and encephalitis. Cerebrospinal fluid hypocretin-1 measures were carried out in some recent cases with symptomatic narcolepsy, and moderate decreases in hypocretin levels were seen in a large majority of these cases. Excessive daytime sleepiness (EDS) in these symptomatic cases was sometimes reversible with an improvement of the causative neurologic disorder and with an improvement of the hypocretin (orexin) status. Recently, we found that several symptomatic narcoleptic cases with MS show unique bilateral symmetric hypothalamic lesions associated with significant hypocretin ligand deficiency. In addition, these patients often share the clinical characteristics of neuromyelitis optica (NMO) and the detection of NMO-IgG (or anti-aquaporin-4 [AQP4] antibodies), suggesting a new clinical entity. Further studies of the involvement of the hypocretin system in symptomatic narcolepsy and EDS are helpful to understand the pathophysiologic mechanisms for occurrence of EDS and cataplexy. The symptoms of narcolepsy can occur during the course of other neurologic conditions (ie, symptomatic narcolepsy). Inherited disorders, tumors, and head trauma were the three most frequent causes for symptomatic narcolepsy. Other causes include multiple sclerosis (MS), vascular disorders, and encephalitis. Cerebrospinal fluid hypocretin-1 measures were carried out in some recent cases with symptomatic narcolepsy, and moderate decreases in hypocretin levels were seen in a large majority of these cases. Excessive daytime sleepiness (EDS) in these symptomatic cases was sometimes reversible with an improvement of the causative neurologic disorder and with an improvement of the hypocretin (orexin) status. Recently, we found that several symptomatic narcoleptic cases with MS show unique bilateral symmetric hypothalamic lesions associated with significant hypocretin ligand deficiency. In addition, these patients often share the clinical characteristics of neuromyelitis optica (NMO) and the detection of NMO-IgG (or anti-aquaporin-4 [AQP4] antibodies), suggesting a new clinical entity. Further studies of the involvement of the hypocretin system in symptomatic narcolepsy and EDS are helpful to understand the pathophysiologic mechanisms for occurrence of EDS and cataplexy.[PUBLICATION ABSTRACT] |
Author | Ito, Sachiko-Uemura Takemura, Fumi Hishikawa, Yasuo Tsutsui, Ko Nishino, Seiji Kanbayashi, Takashi Sagawa, Yohei |
Author_xml | – sequence: 1 givenname: Takashi surname: Kanbayashi fullname: Kanbayashi, Takashi organization: Department of Neuropsychiatry, Akita University School of Medicine – sequence: 2 givenname: Yohei surname: Sagawa fullname: Sagawa, Yohei organization: Department of Neuropsychiatry, Akita University School of Medicine, Center for Narcolepsy, Stanford School of Medicine, Stanford University Sleep and Circadian Neurobiology Laboratory – sequence: 3 givenname: Fumi surname: Takemura fullname: Takemura, Fumi organization: Department of Neuropsychiatry, Akita University School of Medicine – sequence: 4 givenname: Sachiko-Uemura surname: Ito fullname: Ito, Sachiko-Uemura organization: Course of Physical Therapy, School of Health Sciences, Akita University – sequence: 5 givenname: Ko surname: Tsutsui fullname: Tsutsui, Ko organization: Department of Neuropsychiatry, Akita University School of Medicine – sequence: 6 givenname: Yasuo surname: Hishikawa fullname: Hishikawa, Yasuo organization: Department of Neuropsychiatry, Akita University School of Medicine – sequence: 7 givenname: Seiji surname: Nishino fullname: Nishino, Seiji email: nishino@stanford.edu organization: Center for Narcolepsy, Stanford School of Medicine, Stanford University Sleep and Circadian Neurobiology Laboratory |
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SubjectTerms | Adolescent Brain cancer Cerebrospinal fluid Child Disorders of Excessive Somnolence - pathology Disorders of Excessive Somnolence - physiopathology Encephalitis Head injuries Humans Hypothalamus Hypothalamus - metabolism Hypothalamus - pathology Hypotheses Intracellular Signaling Peptides and Proteins - metabolism Ligands Magnetic Resonance Imaging Male Medicine Medicine & Public Health Middle Aged Multiple sclerosis Narcolepsy - etiology Narcolepsy - pathology Narcolepsy - physiopathology Neurology Neuromyelitis Optica - immunology Neuromyelitis Optica - pathology Neuromyelitis Optica - physiopathology Neuropeptides - metabolism Neurosciences Orexins Sleep disorders Trauma Vein & artery diseases |
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Title | The Pathophysiologic Basis of Secondary Narcolepsy and Hypersomnia |
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