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Symptomatic narcolepsy in patients with neuromyelitis optica and multiple sclerosis: new neurochemical and
Takashi Kanbayashi1, Takayoshi Shimohata, Ichiro Nakashima
1Department of Neuropsychiatry, Akita University School of Medicine, Akita City, Japan.
Objective:
To characterize factors that contribute to symptomatic narcolepsy and excessive daytime sleepiness in neuromyelitis optica and multiple sclerosis.
Setting:
Japanese university hospitals.
Design:
Case study.
Patients:
Seven Japanese patients whose initial diagnoses were multiple sclerosis and who were exhibiting excessive daytime sleepiness.
Main Outcome Measures:
Lesions on magnetic resonance imaging, cerebrospinal fluid hypocretin-1 levels, and serum anti-aquaporin 4 (AQP4) antibody titer.
Results:
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.
Conclusion:
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.
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