|Presentations of Primary Hypersomnia in Chinese Children|
|Han, Fang1; Lin, Ling2; Li, Jing1; Aran, Adi1,2; Dong, Song X.1; Zhao, Long1; Li, Ming3; Li, Qian Y.; Yan, Han1; Wang, Jie S.1; Gao, Hui Y.1; Li, Mei1; Gao, Zhan C.1; Strohl, Kingman P.4,5; Mignot, Emmanuel2|
|关键词||Narcolepsy Childhood Hypocretin Mslt hladbqb1*0602|
|WOS标题词||Science & Technology|
|类目[WOS]||Clinical Neurology ; Neurosciences|
|研究领域[WOS]||Neurosciences & Neurology|
|关键词[WOS]||NARCOLEPSY-CATAPLEXY ; CHILDHOOD NARCOLEPSY ; KOREAN PATIENTS ; HYPOCRETIN ; ONSET ; HLA ; DIAGNOSIS|
Objective: To retrospectively describe childhood presentations of primary hypersomnia with an emphasis on narcolepsy-cataplexy in a Chinese population.
Methods: A total of 417 children (< 18 years old) successively presenting with complaints of hypersomnia without anatomic cause or sleep apnea risk were evaluated using the Stanford Sleep Inventory, human leukocyte antigen (HLA) DQB1*0602 typing, and MSLT recordings. CSF hypocretin-1 was measured or HLA positive with clear-cut cataplexy) was further examined for presentations prior to , around, or after puberty.
Results: Narcolepsy with (n = 361) or without (n = 17) cataplexy presented at an earlier age and with increased male predominance when compared to idiopathic hypersomnia (n = 39, P < 0.01). Nearly 70% of those with narcolepsy/hypocretin deficiency (n = 271) had disease onset before age 10 y, and 15% had onset before age 6, an unusually young age distribution. Onset was prior to puberty in 78% of cases. Clinical features were decreased and the number of sleep onset REM periods (SOREMPs) increased in frequency with age/puberty, but MSLT diagnosis criteria (MSL <= 8 min, >= 2 SOREMPs) were similarly positive across groups. Familial clustering was present in only 1.7% of probands.
Conclusion: In children presenting with a complaint of primary hypersomnia to a sleep clinic in China, 86% (361/417) meet criterial for narcolepsy with cataplexy. Puberty did not affect positivity on the MSLT as a diagnostic feature. Sleep paralysis was the only symptom that increased with increasing age. In addition, narcolepsy with cataplexy in our clinic population appeared to begin at a younger age than usually reported in other studies.
|项目编号||81070069 ; GZ538 ; HL 047380 ; NIH-NS23724|
|资助机构||NSFC ; Sino-German Center for Research Promotion ; VA Research Service ; NIH|
|作者单位||1.Cleveland Louis Stokes VA Med Ctr, Cleveland, OH USA|
2.Beijing Univ, Peoples Hosp, Dept Pulm Med, Beijing 100044, Peoples R China
3.Stanford Univ, Ctr Narcolepsy, Howard Hughes Med Inst, Palo Alto, CA 94304 USA
4.Beijing Union Med Coll Hosp, Beijing Union Med Coll, Dept Endocrinol, Beijing 100730, Peoples R China
5.Case Western Reserve Univ, Dept Med, Div Pulm Crit Care & Sleep Med, Cleveland, OH 44106 USA
|Han, Fang,Lin, Ling,Li, Jing,et al. Presentations of Primary Hypersomnia in Chinese Children[J]. SLEEP,2011,34(5):627-632A.|
|APA||Han, Fang.,Lin, Ling.,Li, Jing.,Aran, Adi.,Dong, Song X..,...&Mignot, Emmanuel.(2011).Presentations of Primary Hypersomnia in Chinese Children.SLEEP,34(5),627-632A.|
|MLA||Han, Fang,et al."Presentations of Primary Hypersomnia in Chinese Children".SLEEP 34.5(2011):627-632A.|