Baumann C R, Khatami R, Werth E, Bassetti C L
Department of Neurology, University Hospital Zürich, Frauenklinikstrasse 26, 8091 Zürich, Switzerland.
J Neurol Neurosurg Psychiatry. 2006 Mar;77(3):402-4. doi: 10.1136/jnnp.2005.067207.
Cerebrospinal fluid (CSF) hypocretin-1 deficiency is associated with definite ("clear cut") cataplexy in patients with narcolepsy. The relationship between CSF hypocretin-1 levels and other narcoleptic symptoms (including excessive daytime sleepiness, EDS) is not properly understood. In a consecutive series of 18 subjects with narcolepsy and definite cataplexy, patients with undetectable CSF hypocretin-1 (n = 12) were found to have significantly lower mean sleep latencies (p = 0.045) and a higher frequency of sleep onset REM periods (SOREMPs, p = 0.025) on multiple sleep latency test than patients (n = 6) with detectable levels. Conversely, Epworth sleepiness scale scores, the frequency of hallucinations/sleep paralysis, and the frequency and severity of cataplexy were similar in both groups. These results suggest that hypocretin deficiency identifies a homogenous group of patients with narcolepsy characterised by the presence of definite cataplexy, severe EDS, and frequent SOREMPs.
脑脊液（CSF）中食欲素-1缺乏与发作性睡病患者明确的（“明显的”）猝倒相关。脑脊液中食欲素-1水平与其他发作性睡病症状（包括日间过度嗜睡，EDS）之间的关系尚未得到充分理解。在连续的18例发作性睡病且有明确猝倒的受试者中，发现脑脊液中食欲素-1检测不到的患者（n = 12）在多次睡眠潜伏期试验中的平均睡眠潜伏期显著更低（p = 0.045），且睡眠开始快速眼动期（SOREMPs，p = 0.025）的频率更高，相比之下，食欲素-1水平可检测到的患者（n = 6）则不然。相反，两组的爱泼沃斯嗜睡量表评分、幻觉/睡眠瘫痪的频率以及猝倒发作频率和严重程度相似。这些结果表明，食欲素缺乏确定了一组发作性睡病患者，其特征为存在明确的猝倒、严重EDS以及频繁的SOREMPs。
