62 research outputs found

    Presentations of Primary Hypersomnia in Chinese Children

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    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.Clinical NeurologyNeurosciencesSCI(E)16ARTICLE5627-632A3
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