[Clinical and sleep EEG monitoring characteristics and long-term follow-up study on narcolepsy].
OBJECTIVE
Narcolepsy is a sleep disorder characterized by excessive daytime sleepiness, cataplexy, hypnagogic hallucination and sleep paralysis, with abnormal characteristics of shorter rapid eye movement (REM) sleep latency. The management of the patients is very important. The present study focused on the clinical characteristics, diagnostic methods and long-term prognosis of this particular syndrome.


METHODS
The clinical data of 39 narcoleptic children were analyzed. Sleep EEG monitoring was performed in all patients. Among the 39 cases, 23 were followed up.


RESULTS
All the patients manifested with excessive daytime sleepiness, with disrupted nocturnal sleep occurring in 35 cases. Cataplexy appeared in 36 cases, and sleep paralysis in 9, hypnagogic hallucination in 19, and automatic behavior in 6 cases, respectively. Sleep EEG monitoring demonstrated a short mean sleep latency (< 5 minutes) and two or more sleep onset REM periods (SOREMPs) in 38 cases. Twenty-three of the 39 cases were followed-up. Seventeen cases were followed-up for over one year. The longest follow-up duration was 14 years. Methylphenidate was administered in 10 cases. The excessive daytime sleepiness had been improved in 7 cases (70%). No obvious adverse effects were found. Psychosocial and academic problems appeared in most cases.


CONCLUSIONS
Narcolepsy is a chronic neurological disorder. A definite diagnosis is established when the symptoms of cataplexy and excessive daytime sleepiness occur in association with the characteristic findings on sleep EEG monitoring. Appropriate drug therapy and psychosocial management are of help for such patients. Stimulant medication is an important component of the overall treatment program. A comprehensive approach is necessary to meet the needs of children with narcolepsy. Family education and emotional support are key elements in the management plan. The overall goal for managing childhood narcolepsy is to assist the child and family in achieving optimal quality of life.
Wang
Rong
R
Department of Pediatrics, First Hospital, Beijing University, Beijing 100034, China.
Qin
Jiong
J
Liu
Xiao-yan
XY
Feng
Bao-rong
BR
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D018670:Monitoring, Ambulatory; D008991:Monitoring, Physiologic; D009290:Narcolepsy / Q000188:drug therapy* / Q000473:pathology*; D017286:Polysomnography