PMID- 21532956
OWN - NLM
STAT- MEDLINE
DCOM- 20110831
LR  - 20211020
IS  - 1550-9109 (Electronic)
IS  - 0161-8105 (Print)
IS  - 0161-8105 (Linking)
VI  - 34
IP  - 5
DP  - 2011 May 1
TI  - Presentations of primary hypersomnia in Chinese children.
PG  - 627-32
AB  - 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 in 47 cases 
      to document hypocretin deficiency. A subgroup ("narcolepsy/hypocretin 
      deficiency") with likely hypocretin deficiency (low hypocretin-1 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 similar in presentations 
      across puberty groups except for sleep paralysis, which increased in frequency 
      with age/puberty. Mean sleep latency (MSL) decreased and the number of sleep 
      onset REM periods (SOREMPs) increased 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 criteria 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.
FAU - Han, Fang
AU  - Han F
AD  - Department of Pulmonary Medicine, Beijing University People's Hospital, Beijing, 
      China. hanfang1@hotmail.com
FAU - Lin, Ling
AU  - Lin L
FAU - Li, Jing
AU  - Li J
FAU - Aran, Adi
AU  - Aran A
FAU - Dong, Song X
AU  - Dong SX
FAU - An, Pei
AU  - An P
FAU - Zhao, Long
AU  - Zhao L
FAU - Li, Ming
AU  - Li M
FAU - Li, Qian Y
AU  - Li QY
FAU - Yan, Han
AU  - Yan H
FAU - Wang, Jie S
AU  - Wang JS
FAU - Gao, Hui Y
AU  - Gao HY
FAU - Li, Mei
AU  - Li M
FAU - Gao, Zhan C
AU  - Gao ZC
FAU - Strohl, Kingman P
AU  - Strohl KP
FAU - Mignot, Emmanuel
AU  - Mignot E
LA  - eng
GR  - P50 NS023724/NS/NINDS NIH HHS/United States
GR  - HL 047380/HL/NHLBI NIH HHS/United States
GR  - NS23724/NS/NINDS NIH HHS/United States
PT  - Journal Article
PT  - Research Support, N.I.H., Extramural
PT  - Research Support, Non-U.S. Gov't
DEP - 20110501
PL  - United States
TA  - Sleep
JT  - Sleep
JID - 7809084
RN  - 0 (HCRT protein, human)
RN  - 0 (Intracellular Signaling Peptides and Proteins)
RN  - 0 (Neuropeptides)
RN  - 0 (Orexins)
SB  - IM
MH  - Adolescent
MH  - Age of Onset
MH  - Analysis of Variance
MH  - Chi-Square Distribution
MH  - Child
MH  - China
MH  - Disorders of Excessive Somnolence/*diagnosis/physiopathology
MH  - Female
MH  - Humans
MH  - Intracellular Signaling Peptides and Proteins/deficiency
MH  - Male
MH  - Narcolepsy/diagnosis/physiopathology
MH  - Neuropeptides/deficiency
MH  - Orexins
MH  - Puberty/physiology
MH  - Retrospective Studies
MH  - Sleep/physiology
PMC - PMC3079942
OTO - NOTNLM
OT  - HLADQB1*0602
OT  - MSLT
OT  - Narcolepsy
OT  - childhood
OT  - hypocretin
EDAT- 2011/05/03 06:00
MHDA- 2011/09/01 06:00
PMCR- 2011/11/01
CRDT- 2011/05/03 06:00
PHST- 2011/05/03 06:00 [entrez]
PHST- 2011/05/03 06:00 [pubmed]
PHST- 2011/09/01 06:00 [medline]
PHST- 2011/11/01 00:00 [pmc-release]
AID - 10.1093/sleep/34.5.627 [doi]
PST - epublish
SO  - Sleep. 2011 May 1;34(5):627-32. doi: 10.1093/sleep/34.5.627.