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The Pathophysiologic Basis of Secondary Narcolepsy and Hypersomnia

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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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Authors and Affiliations

  1. Department of Neuropsychiatry, Akita University School of Medicine, Akita, Japan

    Takashi Kanbayashi, Yohei Sagawa, Fumi Takemura, Ko Tsutsui & Yasuo Hishikawa

  2. Center for Narcolepsy, Stanford School of Medicine, Stanford University Sleep and Circadian Neurobiology Laboratory, 1201 Welch Road, MSLS, P213, Palo Alto, CA, 94304, USA

    Yohei Sagawa & Seiji Nishino

  3. Course of Physical Therapy, School of Health Sciences, Akita University, Akita, Japan

    Sachiko-Uemura Ito

Authors
  1. Takashi Kanbayashi
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  2. Yohei Sagawa
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  3. Fumi Takemura
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  4. Sachiko-Uemura Ito
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  5. Ko Tsutsui
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  6. Yasuo Hishikawa
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  7. Seiji Nishino
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Corresponding author

Correspondence to Seiji Nishino.

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Kanbayashi, T., Sagawa, Y., Takemura, F. et al. The Pathophysiologic Basis of Secondary Narcolepsy and Hypersomnia. Curr Neurol Neurosci Rep 11, 235–241 (2011). https://doi.org/10.1007/s11910-011-0178-y

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