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1、1,P K,单,朱,2,PK内容,励：患者朱xx的“强哭强笑”属于什么障碍？朱：属于器质性精神障碍，是脑损害导致的精神异常，不像是情绪障碍。单：属于情绪控制障碍，可能为皮质延髓束损伤导致，不像是精神障碍。,3,几个术语-中西方不统一,情绪：与人的自然性需要相联系，具有情景性、暂时性和明显的外部表现；情绪发生时会出现一系列的机体内部生理变化，并有各种外部表现（面部、动作、语言）。包括心境、激情和应激。情感：与人的社会性需要相联系，具有稳定性、持久性，不一定有明显的外部表现。包括道德感和价值感两个方面，具体表现为爱情、友情、幸福、仇恨、厌恶、美感等等。情感的产生伴随着情绪反应，而情绪的变化也受情感
2、的控制。情绪是情感的基础和外部表现，情感是情绪的深化和本质内容。,4,ICD10：Emotion：情绪；Mood：心境；Affect：情感Emotion is a mental and physiological state associated with a wide variety of feelings,thoughts,and behavior.Emotions are subjective experiences,often associated with mood,temperament,personality,and disposition.Mood is a relativel
3、y long lasting emotional state.Moods differ from simple emotions in that they are less specific,less intense,and less likely to be triggered by a particular stimulus or event.Affect refers to the experience of feeling or emotion.Affect is a key part of the process of an organisms interaction with st
4、imuli.The word also refers sometimes to affect display,which is a facial,vocal,or gestural behavior that serves as an indicator of affect.(APA 2006)Mood is the emotional feeling stated by a patient,and affect is the emotional appearance of the patient.,几个术语-中西方不统一,5,脑损伤后“强哭强笑”的表现、机制、治疗和诊断分类,南京医科大学一附
5、院康复医学科 单春雷,6,Pseudobulbar affect（PBA，假性延髓情绪）is a dramatic disorder of emotional expression and regulation characterized by uncontrollable episodes of laughing and crying that often cause embarrassment,curtailment of social activities,and reduction in quality of life.The disorder occurs in patients w
6、ith brain injury caused by many types of neurological disease,including stroke,tumors,and neurodegenerative gray and white matter disorders.Although the pathophysiology is unknown,PBA may relate to release of brainstem emotional control centers from regulation by the frontal lobes.,单：患者朱XX的“强哭强笑”属情绪
7、控制障碍，可能为皮质延髓束损伤（假性延髓麻痹）造成。,7,8,Poeck crystallized the features of PBA into four criteria.First,the episodes are inappropriate to the situation and can be precipitated by nonspecific stimuli,such as contraction of facial muscles,removal of bedcovers,or the approach of someone toward the patient.Secon
8、d,there is not a close relation between the emotional expression and how the patient is feeling.Third,the episodes are relatively stereotyped,and it is difficult for patients to control the extent and duration of the episodes.Last,there are no episodic mood changes corresponding to the episodes,and
9、there is no sense of relief as the emotions are expressed.This last criterion tries to capture the fact that the episodes appear to come unprovoked and out of context.All these features serve to differentiate PBA from depression,where crying usually is context appropriate.,9,PBA has been recognized
10、for well more than a century.In fact,Darwin noted the disorder in his studies of human emotion.Wilson observed that it is frequently associated with damage to descending motor systems.Wilson linked the phenomenon to normal-appearing,involuntarily expressed emotions that occur in the context of upper
11、 motor neuron lesions,even with facial paresis.He theorized that PBA represents the release of a fasciorespiratory control center for emotional expression in the brainstem from voluntary control by higher cortical brain centers.,10,Focal lesions causing PBA have been described in nearly every part o
12、f the brain,including frontal cortical and subcortical structures,brainstem regions,and anterior temporal regions.It has been observed in both unilateral and bilateral injury.PBA from isolated parietal or occipital lesions is rarely reported.This study revealed that poststroke“emotional incontinence
13、”occurred more frequently after stroke in the lenticulocapsular region,basis pontis,medulla oblongata,or the cerebellum.,11,Nomenclature-命名法,Several terms are used interchangeably with PBA.PBA is frequently used because the phenomenon often occurs in the setting of pseudobulbar palsy caused by docum
14、ented or putative frontal lobe injury.Some have argued that the link between PBA and pseudobulbar palsy is imperfect,and that other terms should be preferred;however,the term is common and familiar to most physicians.In contrast,more descriptive terms such as pathological laughing and crying（病理性哭笑）,
15、affective lability（情绪不稳），emotional incontinence（情绪失禁）,and emotionalism（易动情绪）may have some advantages over PBA in that they do not imply a specific pathophysiology or clinical context,but they may be overly general.,12,Recently,the term involuntary emotional expression disorder（不随意性情绪表达障碍，IEED）was co
16、ined.Involuntary emotional expression disorder(IEED),also called pseudobulbar affect(PBA),pathological laughter and crying(PLC)and affective lability,is characterized by brief,spontaneous and uncontrollable episodes of crying or laughing that are typically unrelated to underlying mood.,13,情绪不稳,易动情绪,
17、情绪失控,情绪失禁,情绪不稳,过度情绪化,强哭强笑,不恰当欢喜,病理性情绪,病理性情绪化,病理性易动情绪,病理性哭笑,病理性流泪,假性延髓情绪,假性延髓哭泣,不随意性情绪表达障碍,14,Episodes of laughing and crying are considered pathological when they occur without voluntary control and modulation,are not meaningfully related to the stimuli that provoke them(ie,contextually inappropriat
18、e),neither reflect nor change the prevailing mood,and involve a dissociation between affective expression and experience(情绪表达和情绪体验的分离).The classic example of such is a patient with a stroke who appears emotionally normal most of the time,but unpredictably bursts into tears and grimaces,and vocalizes
19、 at the slightest provocation.After these excessively intense and uncontrollable episodes run their course over a few minutes,the patient returns to an emotionally neutral baseline.When asked how he felt during the episode,the patient replies that he felt nothing at all-no sadness,anxiety,joy,or any
20、 other subjective emotional experience occurs during these episodes.This form of affect dysregulation-crying without feeling sad and laughing without feeling mirth or amusement-is the prototype of PLC.Many persons with PLC experience both episodes of crying and of laughing.When only one type of epis
21、ode occurs in an individual patient,pathological crying alone is the more common presentation.,Pathological Laughing and Crying（病理性哭笑）,15,Any neurological disorder that interferes with the corticobulbar or cortico-subcortical-thalamo-cerebellar circuits that permit regulation of affect can produce P
22、LC.Common underlying neurological conditions include stroke,amyotrophic lateral sclerosis,Parkinsons disease,multiple sclerosis,frontotemporal dementia,traumatic brain injury,Alzheimers disease,epilepsy,normal pressure hydrocephalus,progressive supranuclear palsy,Wilsons disease(hepatolenticular deg
23、eneration),and neurosyphilis.,16,Affective Lability(情绪不稳),Episodes of affective lability are similar to those of PLC in that they are brief,excessively intense with respect to the inciting stimulus,not fully amenable to normal voluntary control,and neither reflect nor change the prevailing mood.Howe
24、ver,these episodes are often less severe and more understandably related to sentimental stimuli than are episodes of PLC.Additionally,the subjective and objective dimensions of affect are not dissociated during episodes of affective lability.Persons with affective lability feel sad when they cry and
25、 feel amusement when they laugh,but they are unable to control the intensity,duration,or frequency of these episodes.While the stimulus for such episodes may carry some sentimental valence,the quality of the affective response is in excess of that merited by the stimulus that incites it.As a result,
26、episodes of affective lability are more stereotyped than normal affective variability(ie,they are pathological),although they tend to be less stereotyped than episodes of PLC.Any neurological disorder that interferes with the corticobulbar or cortico-subcortical-thalamo-cerebellar circuits involved
27、in affect regulation can produce affective lability;not surprisingly,the causes of affective lability overlap substantially with those that produce PLC.,17,18,治疗,Although there are no US Food and Drug Administration approved treatments for PBA,several agents have been shown to be effective,including
28、 tricyclic antidepressants（三环抗抑郁药,阿米替林,25-300mg/d）,selective serotonin reuptake inhibitors（选择性5-HT再摄取抑制剂,西酞普兰,5-40mg/d）,and a new agent containing dextromethorphan（DM，右美沙芬，普西兰,30mg,bid）and quinidine（奎尼丁,30mg,bid），（加：金刚烷胺50-200mg,bid）.,19,治疗：DM/Q（右美沙芬）,DM/Q（右美沙芬）has been shown to be effective in amel
29、iorating PBA in both ALS（肌萎缩性侧索硬化症）and MS（多发性硬化）,and it has been assessed in a larger number of patients than any previous drug used to treat PBA;however,it has not been compared with the other agents that have previously shown efficacy.The mechanism by which DM/Q helps PBA is unknown.DM,the active
30、ingredient(Q is used to slow metabolism of DM),is an N-methyl-D-aspartate(NMDA，N-甲基-D-天(门)冬氨酸)receptor antagonist.,20,治疗：DM/Q（右美沙芬）,【其他名称】右甲吗喃；美沙芬；普西兰；【药物作用】为中枢性镇咳药，抑制延髓咳嗽中枢而产生镇咳作用。镇咳作用显著，与相同剂量的可待因大体相同或稍强，但无止痛作用。长期服用无成瘾性和耐受性，治疗剂量不会抑制呼吸，作用快且安全。【适应症状】用于感冒、急性或慢性支气管炎，上呼吸道感染时的咳嗽。,21,治疗：拉莫三嗪,LamotrigineIn
31、itially at the dose of 50 mg a day,which was gradually increased to 100 mg a day over a 4-week period。规 格：25 mg、50mg、100 mg。适 应 症：癫痫（简单部分性发作、复杂部分性发作、续发性和原发性全身强直阵挛性发作）。也可用于治疗合并有Lennox-Gastaut综合征的癫痫发作。2003年6月，拉莫三嗪（lamotrigine）片剂获美国FDA批准，用于用标准药物治疗急性情绪发作的成人双极失调患者的长期维持治疗，以推迟情绪发作(抑郁、躁狂、轻躁狂、混合型发作)的时间。,22,I
32、CD-10,F00F09器质性(包括症状性)精神障碍00阿尔采末氏病性痴呆01血管性痴呆02见于在它处归类的其它疾病的痴呆03未特定的痴呆04器质性遗忘综合征、非酒和其它精神活性物质所致05谵妄，非酒和其它精神活性物质所致06脑损害和功能紊乱以及躯体疾病所致的其它精神障碍07脑疾病、损害和功能紊乱所致的人格和行为障碍08未特定的器质性或症状性精神障碍,23,F00F09器质性(包括症状性)精神障碍F06 脑损害和功能紊乱以及躯体疾病所致的其它精神障碍F060 器质性幻觉症F061 器质性紧张性障碍F062 器质性妄想性（精神分裂症样）障碍F063 器质性心境情感障碍F064 器质性焦虑障碍F0
33、65 器质性分离性障碍F066 器质性情绪不稳定（衰弱）障碍F067 轻度认知障碍F068 脑损害和功能紊乱及躯体疾病所致的其它特定性精神障碍F069 脑损害和功能紊乱及躯体疾病所致的未特定的精神障碍,24,F00F09器质性(包括症状性)精神障碍F06 脑损害和功能紊乱以及躯体疾病所致的其它精神障碍F063器质性心境情感障碍特征为心境或情感改变，常伴有总体活动水平的改变。这类障碍归入本节的唯一标准是假定其病因为某种大脑或躯体疾病，通过检查或者根据恰当的病史资料能推测出这些疾病的存在。情感性障碍必须出现于设想的器质性病因之后，此外尚需确定精神障碍不是病人对知道所患疾病或疾病的症状的情绪反应。诊
34、断要点 器质性病因的一般性标准见于F06之引言。除一般性标准外，还应符合F30F33所列出的各种障碍之一所需的条件。不含：心境情感障碍，非器质性或未特定（F30F39）下列第五位编码可用于指明临床障碍：F0630器质性躁狂障碍 F0631器质性双相障碍 F0632器质性抑郁障碍 F0633器质性混合性情感性障碍,25,F00F09器质性(包括症状性)精神障碍F06 脑损害和功能紊乱以及躯体疾病所致的其它精神障碍F066器质性情绪不稳定（衰弱）障碍特征为明显和持续的情绪失禁或不稳定、易疲乏或一系列不愉快的躯体感受（如头晕）和疼痛，这些症状是由某种器质性障碍所致，据认为由脑血管病或高血压症所致的本
35、症远较其它病因为多。（但，这是否包含了不伴情绪体验的异常情绪表现？PBA）不含：躯体形式障碍，非器质性或未特定（F45）,26,F30F39心境情感障碍躁狂发作双相情感障碍抑郁发作复发性抑郁障碍持续性心境情感障碍:恶劣心境（原抑郁性神经症，2001分出）其它心境情感障碍未特定的心境情感障碍,27,F40F48神经症性、应激相关的及躯体形式障碍恐怖性焦虑障碍其它焦虑障碍强迫性障碍严重应激反应，及适应障碍分离(转换)性障碍躯体形式障碍其它神经症性障碍,28,PK,“强哭强笑”本质：脑损伤所致的，容易被诱发、不能随意控制、不伴有对应情绪体验（emotional experience）的异常情绪性表达（emotional expression）。,励：埃菲尔铁塔在哪里？朱：在法国（不像是在巴黎）。单：在巴黎（不像是在法国）。,励：朱xx的“强哭强笑”属于什么障碍？朱：属于器质性精神障碍（不像是情绪障碍）。单：属于情绪控制障碍（不像是精神障碍）。,29,Thank you！,
