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睡眠疾病的国际分类北京协和医院呼吸睡眠中心 肖毅睡眠疾病的分类对于疾病之间的区分是有必要的,并且有利于理解症状、病因、病理生理和治疗。因为许多疾病的病理生理还不清楚,早期的疾病主要是根据主要的症状(失眠、嗜睡、以及在睡眠中出现的事件)分类。这样的分类很容易被临床医师所理解,而且对于鉴别诊断亦很有用。由于现代睡眠研究的发展,某些睡眠疾病可以根据病理生理分类。睡眠疾病国际分类第二版(The International Classification of Sleep Disorders,ICSD-2)于2004年出版,它结合症状学(如失眠),病理生理(如昼夜节律)以及部位(如呼吸疾病)进行分类。由于睡眠疾病的不同特性,以及许多睡眠疾病的病理生理仍不明,因此是非常有必要的。ICSD-2不只是睡眠疾病的列表,而是一个手册,有很多的参考文献涉及疾病诊断的特点和流行病学,以帮助读者更容易进行鉴别诊断。自从睡眠疾病开始认识以来,临床医师就对睡眠疾病的分类产生了兴趣。第一次疾病分类(the Diagnostic Classification of Sleep and Arousal Disorders),颁布于1977年。这样一个分类是目前分类的基础。1990年,美国睡眠疾病协会(the American Sleep Disorders Association , ASDA)在经过五年的工作之后,制定了the International Classification of Sleep Disorders (ICSD)。有三个当时主要的国际睡眠组织共同参加,欧洲睡眠研究会,日本睡眠研究会和拉丁美洲睡眠研究会,出版了the International Classification of Sleep Disorders: Diagnostic and Coding Manual。该分类主要是根据诊断、流行病学和研究目的,对临床医师进行睡眠研究和国际交流起了重要的作用。2003年,美国睡眠医学会(the American Academy of Sleep Medicine),前身是ASDA,对ICSD进行了完全的修订和更新,在2005年发表了第二版的the International Classification of Sleep Disorders, ICSD-2。ICSD-2 列举了85种睡眠疾病,对每一种疾病都进行了详细的描述,包括诊断标准。它共有八个分类。1. 失眠 2. 睡眠呼吸疾病3. 不是由于呼吸疾病引起的嗜睡4. 昼夜节律睡眠障碍5. 异态睡眠 6. 睡眠相关运动失调. 7. 独立症状 8. 其他睡眠疾病ICSD-2 Sleep Disorder Categories and Individual Sleep DisordersInsomniasICD-9ICD-10Adjustment Sleep Disorder (Acute Insomnia)307.41F 51.01Psychophysiological Insomnia307.42F 51.03Paradoxical Insomnia (formerly Sleep State Misperception)307.42F 51.02Idiopathic Insomnia780.52F 51.04Insomnia due to mental disorder (Code also the associated mental disorder)307.42F 51.05Inadequate Sleep Hygiene (Code also any associated insomnia F51.0-, G47.0-)307.41Z72.821Behavioral Insomnia of Childhood307.42Z73.81Sleep-onset Association Type-Z73.810Limit-setting Sleep Type-Z73.811Combined Type-Z73.812Unspecified Type-Z73.819Insomnia due to a medical condition (Code also the associated medical condition)780.52G 47.03Insomnia due to a drug or substance (Code first T36-T65 with 6th character 1-4 to identify drug or substance, and use additional code T36-T50 with 6th character 5 to identify adverse effect of drug). Use F10-F19 codes when there is associated drug abuse or dependence.291.89G 47.02Insomnia not due to a substance or known physiological condition, unspecified (Nonorganic Insomnia, NOS)307.42F 51.00Physiological (organic) insomnia, unspecified; (Organic Insomnia, NOS)780.52G 47.00Sleep-Related Breathing DisordersCentral Sleep Apnea SyndromesPrimary Central Sleep Apnea780.51G 47.31Other Central Sleep Apnea due to a medical condition780.57G 47.39Cheyne Stokes Breathing Pattern-High Altitude Periodic Breathing-Central Sleep Apnea due to a medical condition, not Cheyne Stokes or High Altitude-Central Sleep Apnea due to a drug or substance995.2G 47.36Other Sleep-Related Breathing Disorder due to a drug or substance-Primary Sleep Apnea of Infancy (formerly Primary Sleep Apnea of Newborn)770.81P 28.3Obstructive Sleep Apnea SyndromesObstructive Sleep Apnea, Adult780.53G 47.32Obstructive Sleep Apnea, Pediatric780.53G 47.32Sleep-Related Hypoventilation/Hypoxemic SyndromesSleep-Related Non-obstructive Alveolar Hypoventilation, Idiopathic780.57G 47.34Congenital Central Alveolar Hypoventilation Syndrome780.57G 47.35Sleep-Related Hypoventilation/Hypoxemia due to a medical condition780.57G 47.37Sleep-Related Hypoventilation/Hypoxemia due to pulmonary parenchymal or vascular pathology-Sleep-Related Hypoventilation/Hypoxemia due to lower airways obstruction-Sleep-Related Hypoventilation/Hypoxemia due to neuromuscular or chest wall disorders-Other Sleep-Related Breathing DisorderSleep Apnea/Sleep-Related Breathing Disorder, unspecified (Sleep-Related Breathing Disorder, NOS)780.57G 47.30Hypersomnias Not Due to a Sleep-Related Breathing DisorderNarcolepsyNarcolepsy with Cataplexy347.01G 47.41Narcolepsy without Cataplexy347.00G 47.42Narcolepsy due to a medical condition347.1G 47.43Narcolepsy, unspecified347G 47.0Other HypersomniasRecurrent Hypersomnia780.54G 47.11Kleine-Levin Syndrome-Menstrual-Related Hypersomnia-Idiopathic Hypersomnia with long sleep time780.54G 47.12Idiopathic Hypersomnia without long sleep time780.54G 47.13Behaviorally Induced Insufficient Sleep Syndrome307.44F 51.11Hypersomnia due to a medical condition780.54G 47.15Hypersomnia due to a drug or substance995.2G 47.14Hypersomnia not due to a substance or known physiological condition (Nonorganic Hypersomnia, NOS)307.44F 51.10Physiological (Organic) Hypersomnia, unspecified (Organic Hypersomnia, NOS)780.54G 47.10Circadian Rhythm Sleep DisordersPrimary Circadian Rhythm Sleep DisordersCircadian Rhythm Sleep Disorder, Delayed sleep phase type780.55G 47.21Circadian Rhythm Sleep Disorder, Advanced sleep phase type780.55G 47.22Circadian Rhythm Sleep Disorder, Irregular sleep-wake type780.55G 47.23Circadian Rhythm Sleep Disorder, Free running (non-entrained) type780.55G 47.24Circadian Rhythm Sleep Disorders due to a medical condition780.55G 47.26Primary (Organic) Circadian Rhythm Sleep Disorders, unspecified other physiological (organic) circadian rhythm, unspecified (organic circadian rhythm disorder, NOS)780.55G 47.20Behaviorally Induced Circadian Rhythm Sleep DisordersCircadian Rhythm Sleep Disorder not due to a substance or known physiological condition, jet lag type307.45F 51.21Circadian Rhythm Sleep Disorder not due to a substance or known physiological condition, shift work type307.45F 51.22Circadian Rhythm Sleep Disorder not due to a substance or known physiological condition, delayed sleep phase type307.45F 51.23Circadian Rhythm Sleep Disorder not due to a substance or known physiological condition, unspecified (Nonorganic circadian rhythm sleep disorder, NOS)307.45F 51.20Other Circadian Rhythm Sleep Disorder not due to a substance or known physiological condition307.45F 51.29Other Circadian Rhythm Sleep Disorder due to a drug or substance291.89G 47.25ParasomniasDisorders of Arousal (From Non-REM Sleep)Confusional Arousals780.56G 47.51Sleepwalking307.46F 51.3Sleep Terrors307.46F 51.4Parasomnias Usually Associated with REM SleepREM Sleep Behavior Disorder780.56G 47.52Parasomnia Overlap Disorder-Status Dissociatus-Recurrent Isolated Sleep Paralysis780.56G 47.53Nightmare Disorder307.47F 51.5Other ParasomniasSleep-Related Dissociative Disorder300.15F 44.9Sleep-Related Enuresis788.36N 39.44Sleep-Related Groaning (Catathrenia)780.56G 47.50Exploding Head Syndrome780.56G 47.50Sleep-Related Hallucinations307.49R 29.81Sleep-Related Eating Disorder780.56G 47.59Parasomnia, unspecified780.56G 47.50Parasomnia due to a drug or substance780.56G 47.54Parasomnia due to a medical condition780.56G 47.55Sleep-Related Movement DisordersRestless Legs Syndrome (including Sleep-Related Growing Pains)333.99G 47.61Periodic Limb Movement Sleep Disorder780.58G 47.62Sleep-Related Leg Cramps780.58G 47.63Sleep-Related Bruxism780.58G 47.64Sleep-Related Rhythmic Movement Disorder780.58G 47.65Sleep-Related Movement Disorder, unspecified (no text)780.58G 47.60Sleep-Related Movement Disorder due to a drug or substance (no text)291.89G 47.66Sleep-Related Movement disorder due to a medical condition780.58G 47.67Isolated Symptoms, Apparently Normal Variants, and Unresolved IssuesLong Sleeper307.49R 29.81Short Sleeper307.49R 29.81Snoring786.09R 06.5Sleeptalking307.49R 29.81Sleep Starts, Hypnic Jerks781.01R 25.8Benign Sleep Myoclonus of Infancy781.01R 25.8Hypnagogic Foot Tremor and Alternating Leg Muscle Activation during Sleep781.01R 25.8Propriospinal Myoclonus at Sleep Onset781.01R 25.8Excessive Fragmentary Myoclonus781.01R 25.8Other Sleep DisordersOther Physiological (Organic) Sleep Disorder780.50G 47.8Physiological (Organic) Sleep Disorder, unspecified780.50G 47.8Other Sleep Disorder not due to a substance or physiological condition307.40F 51.8Environmental Sleep Disorder-Sleep Disorder not due to a substance or physiological condition, unspecified307.40F 51.9睡眠相关的呼吸疾病这组疾病的共同特点就是在睡眠中的病态呼吸。中枢性呼吸暂停疾病(Central apnea disorders)包括由于中枢神经系统功能失调,呼吸努力以间歇或周期的方式减少或消失的中枢性呼吸暂停;其他的中枢性呼吸暂停形式和潜在的病理和环境因素有关,如陈-施氏呼吸,高海拔周期呼吸。原发性中枢性呼吸暂停是一种原因不明的疾病,其特点是睡眠期间反复的呼吸停止的发作,而没有呼吸努力存在。主诉有白天嗜睡、失眠、睡眠期间呼吸困难,患者没有高碳酸血症 (Pco2 大于45 mm Hg),诊断是根据多导睡眠监测每小时有5次或更多的呼吸暂停发作。其他的中枢性呼吸暂停包括陈-施氏呼吸、其特点反复的呼吸暂停或低通气和呼吸过度交替出现,潮气量以逐渐增加和减少的方式变大或变小。这种形式主要出现在NREM,而在REM不会出现。这种形式的呼吸暂停典型的出现于心衰、脑血管病和肾衰。其他的中枢性睡眠呼吸暂停包括高海拔周期性呼吸,其特点是由于急性高原病引起的睡眠疾病。在呼吸暂停期间没有呼吸努力存在。典型的周期长度在12到34秒之间。诊断是根据睡眠期间每小时5次或更多的中枢性呼吸暂停,多数人在海拔7600米会出现这种通气形式,有些人

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