Neuroscience Center, CNMC, George Washington University School of Medicine, Washington, DC, USA.
Sleep. 2009 Dec;32(12):1645-8. doi: 10.1093/sleep/32.12.1645.
Patients with SSADH deficiency, a disorder of chronically elevated endogenous GABA and GHB, were studied for sleep symptoms and polysomnography. We hypothesized that patients would have excessive daytime somnolence and decreased REM sleep.
Polysomnography and MSLT were performed on patients enrolled for comprehensive clinical studies of SSADH deficiency.
Sleep studies were obtained in the sleep laboratories at CNMC and NIH.
Sleep recordings were obtained in 10 patients with confirmed SSADH deficiency.
Thirteen overnight polysomnograms were obtained in 10 patients (7 male, 3 female, ages 11-27 y). Eleven MSLT studies were completed in 8 patients.
Polysomnograms showed prolongation of REM stage latency (mean 272 +/- 89 min) and decreased percent stage REM (mean 8.9%, range 0.3% to 13.8%). Decreased mean sleep latency was present in 6 of 11 MSLTs.
SSADH deficiency is associated with prolonged latency to stage REM and decreased percent stage REM. This disorder represents a model of chronic GABA and GHB accumulation associated with suppression of REM sleep.
患有 SSADH 缺乏症（一种慢性内源性 GABA 和 GHB 升高的疾病）的患者进行了睡眠症状和多导睡眠图研究。我们假设患者会出现日间过度嗜睡和 REM 睡眠减少。
对参加 SSADH 缺乏症综合临床研究的患者进行多导睡眠图和 MSLT 检查。
睡眠研究在 CNMC 和 NIH 的睡眠实验室进行。
在 10 名确诊为 SSADH 缺乏症的患者中获得了睡眠记录。
在 10 名患者（7 名男性，3 名女性，年龄 11-27 岁）中获得了 13 个过夜多导睡眠图。在 8 名患者中完成了 11 次 MSLT 研究。
多导睡眠图显示 REM 期潜伏期延长（平均 272+/-89 分钟）和 REM 期百分比减少（平均 8.9%，范围 0.3%至 13.8%）。11 次 MSLT 中有 6 次出现平均睡眠潜伏期缩短。
SSADH 缺乏症与 REM 期潜伏期延长和 REM 期百分比减少有关。这种疾病代表了一种与 REM 睡眠抑制相关的慢性 GABA 和 GHB 积累的模型。
