TY - JOUR
T1 - Symptomatic narcolepsy in patients with neuromyelitis optica and multiple sclerosis
T2 - New neurochemical and immunological implications
AU - Kanbayashi, Takashi
AU - Shimohata, Takayoshi
AU - Nakashima, Ichiro
AU - Yaguchi, Hiroaki
AU - Yabe, Ichiro
AU - Nishizawa, Masatoyo
AU - Shimizu, Tetsuo
AU - Nishino, Seiji
N1 - Copyright:
Copyright 2010 Elsevier B.V., All rights reserved.
PY - 2009/12
Y1 - 2009/12
N2 - 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.
AB - 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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U2 - 10.1001/archneurol.2009.264
DO - 10.1001/archneurol.2009.264
M3 - Article
C2 - 20008665
AN - SCOPUS:73549112697
VL - 66
SP - 1563
EP - 1566
JO - Archives of Neurology
JF - Archives of Neurology
SN - 0003-9942
IS - 12
ER -