RETTS SYNDROME - CHARACTERIZATION OF RESPIRATORY PATTERNS AND SLEEP
RETTS SYNDROME - CHARACTERIZATION OF RESPIRATORY PATTERNS AND SLEEP
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DOI：
10.1002/ana.410210410
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发表时间：
1987-04-01
影响因子：
11.2
通讯作者：
PERCY, AK
中科院分区：
文献类型：
--
作者：
GLAZE, DG;FROST, JD;PERCY, AK
雷特综合征是一种发生在女性中的进行性疾病，其特征为自闭症行为、痴呆、共济失调、失去有目的地使用手和癫痫发作。观察到雷特综合征患者有刻板的手部运动（洗手），并表现出间歇性过度通气。为了更精确地描述与这种疾病相关的睡眠和呼吸模式，对11例这种综合征患者在睡眠和觉醒期间进行了多导睡眠描记研究。这些研究显示了清醒时异常的呼吸模式，以及异常的睡眠和脑电图特征。患者的快速眼动睡眠百分比下降，在清醒期间，在所有11名患者中观察到呼吸紊乱的模式，包括缺氧，随后是一段时间的呼吸频率和呼吸努力增加。在清醒期间发生紊乱的呼吸和代偿性呼吸过度，在睡眠期间有规律、持续的呼吸是雷特综合征的特征，表明自主/行为呼吸控制系统改变或受损。
Rett''s syndrome is a progressive disorder that occurs in females and is characterized by autistic behavior, dementia, ataxia, loss of purposeful use of the hands, and seizures. Patients with Rett''s syndrome have been observed to have stereotyped hand movements (hand-washing) and to exhibit intermittent hyperventilation. To characterize more precisely the sleep and respiratory patterns associated with this disorder, polygraphic studies were made during sleep and wakefulness in 11 patients with this syndrome. These studies showed abnormal respiratory patterns during wakefulness, and abnormal sleep and electroencephalographic characteristics. The patients had decreased percentages of rapid-eye-movement sleep, and during wakefulness, a pattern of disorganized breathing was observed in all 11 patients and consisted of hypoxia followed by a period of increased respiratory rate and effort. The occurrence of disorganized breathing and compensatory hyperpnea during wakefulness with regular, continuous breathing during sleep is characteristic of Rett''s syndrome and suggests an altered or impaired voluntary/behavioral respiratory control system.