脑损伤后“强哭强笑”的表现、机制、治疗和诊断归属课件.ppt

1、1P K单单朱朱2PK内容 励：患者朱励：患者朱xx的的“强哭强笑强哭强笑”属于什么障碍？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。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。导致，不像是精神障碍。3几个术语几个术语-中西方不统一中西方不统一 情绪情绪：与人的自然性需要相联系，具有：与人的自然性需要相联系，具有情景性、暂时性和情景性、暂时性和明显的外部表现明显的外部表现；情绪发生时会出现一系列的机体内部；情绪发生时会出现一系列的机

2、体内部生生理变化理变化，并有各种外部表现（面部、动作、语言）。，并有各种外部表现（面部、动作、语言）。 包括包括心境、激情和应激。心境、激情和应激。 情感情感：与人的社会性需要相联系，具有：与人的社会性需要相联系，具有稳定性、持久性，稳定性、持久性，不一定有明显的外部表现不一定有明显的外部表现。包括道德感和价值感两个方面，。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。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的控情感的产生伴随着情绪反应，而情绪的变化也受情感的控制。情绪是情感的基础和外部表现，情感是情绪的

3、深化和制。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。本质内容。4ICD10： Emotion：情绪；：情绪； Mood：心境；：心境； Affect：情感：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 dispos

4、ition. Mood is a relatively 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 a

5、n organisms interaction with stimuli. 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.几个术语几个术语-中

6、西方不统一中西方不统一5脑损伤后脑损伤后“强哭强笑强哭强笑”的表现、的表现、机制、治疗和诊断分类机制、治疗和诊断分类南京医科大学一附院康复医学科 单春雷6 is a dramatic disorder of 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 di

7、sorder occurs in patients with 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

8、frontal lobes.单：患者朱单：患者朱XXXX的的“强哭强笑强哭强笑”属属情绪情绪控制障碍，可能控制障碍，可能为皮质延髓束损伤（假性延髓麻痹）造成。为皮质延髓束损伤（假性延髓麻痹）造成。78 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

9、, removal of bedcovers, or the approach of someone toward the patient. Second, 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 epi

10、sodes. Last, there are no episodic mood changes corresponding to the episodes, and 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 fr

11、om depression, where crying usually is context appropriate.9 PBA has been recognized 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 phenomeno

12、n to normal-appearing, involuntarily expressed emotions that occur in the context of upper 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 cor

13、tical brain centers. 10 Focal lesions causing PBA have been described in nearly every part of 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 o

14、r occipital lesions is rarely reported. This study revealed that poststroke “emotional incontinence” occurred more frequently after stroke in the lenticulocapsular region, basis pontis, medulla oblongata, or the cerebellum.11Nomenclature-命名法命名法 Several terms are used interchangeably with PBA. PBA is

15、 frequently used because the phenomenon often occurs in the setting of pseudobulbar palsy caused by documented 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 fa

16、miliar to most physicians. In contrast, more descriptive terms such as pathological laughing and crying（病理性哭笑病理性哭笑）, affective lability（情绪不稳情绪不稳）， emotional incontinence（情绪失禁情绪失禁）, and emotionalism（易动情绪易动情绪） may have some advantages over PBA in that they do not imply a specific pathophysiology or cl

17、inical context, but they may be overly general. 12 Recently, the term involuntary emotional expression disorder（不随意性情绪表达不随意性情绪表达障碍，障碍，IEED） was coined. Involuntary emotional expression disorder (IEED), also called pseudobulbar affect (PBA), pathological laughter and crying (PLC) and affective labili

18、ty, is characterized by brief, spontaneous and uncontrollable episodes of crying or laughing that are typically unrelated to underlying mood.13情绪不稳情绪不稳 易动情绪易动情绪情绪失控情绪失控 情绪失禁情绪失禁情绪不稳情绪不稳过度情绪化过度情绪化强哭强笑强哭强笑不恰当欢喜不恰当欢喜病理性情绪病理性情绪病理性情绪化病理性情绪化病理性易动情绪病理性易动情绪病理性哭笑病理性哭笑病理性流泪病理性流泪假性延髓情绪假性延髓情绪假性延髓哭泣假性延髓哭泣不随意性情绪表

19、达障碍不随意性情绪表达障碍14Episodes 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 inappropriate), neither reflect nor change the prevailing mood, and involve a dissociat

20、ion between affective expression and experience(情绪表达和情绪体验的分离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 at the slightest provocation. After these excessively int

21、ense 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 other subjective emotional experience occurs durin

22、g 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 episode occurs in an individual patient, pathologic

23、al crying alone is the more common presentation. Pathological Laughing and Crying（病理性哭笑）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 PLC. Common underlying neurological condi

24、tions 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 degeneration), and neurosyphili

25、s. 16 Affective Lability(情绪不稳情绪不稳)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. However, these episodes

26、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 feel amusement

27、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, episodes

28、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 in af

29、fect regulation can produce affective lability; not surprisingly, the causes of affective lability overlap substantially with those that produce PLC. 1718治疗治疗 Although there are no US Food and Drug Administration approved treatments for PBA, several agents have been shown to be effective, including

30、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）右美沙芬） DM/Q（右美沙芬右美沙芬）has been shown to be effe

31、ctive in ameliorating PBA in both ALS （肌萎缩性侧索硬化症肌萎缩性侧索硬化症）and MS（多发性硬化多发性硬化）,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 help

32、s PBA is unknown. DM, the active ingredient (Q is used to slow metabolism of DM), is an N-methyl-D-aspartate (NMDA，N-甲基甲基-D-天天(门门)冬氨酸冬氨酸) receptor antagonist.20治疗： DM/Q（右美沙芬）右美沙芬） 【其他名称】右甲吗喃；美沙芬；普西兰；【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，【药物作用】为中枢性镇咳药，抑制延髓抑制延髓咳嗽咳嗽中枢而产生镇咳作用。镇咳作用显著，与相同剂中枢而产生镇咳作用。镇咳作用显著，与相同

33、剂量的可待因大体相同或稍强，但无止痛作用。长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。吸，作用快且安全。 【适应症状】用于感冒、急性或慢性支气管炎，【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。上呼吸道感染时的咳嗽。21治疗：治疗： 拉莫三嗪拉莫三嗪 Lamotrigine Initially at the dose of 50 mg a day, which was gradually increased to 100 mg a day over a 4-week peri

34、od 。 规规 格：格：25 mg、50mg、100 mg。适适 应应 症：症： 癫痫（简单部分性发作、复杂部分性发作、续癫痫（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）。也可用于治疗合发性和原发性全身强直阵挛性发作）。也可用于治疗合并有并有Lennox-Gastaut综合征的癫痫发作。综合征的癫痫发作。 2003年年6月，拉莫三嗪（月，拉莫三嗪（lamotrigine）片剂获美国）片剂获美国FDA批准，用于用标准药物治疗批准，用于用标准药物治疗急性情绪发作急性情绪发作的成人双极失调的成人双极失调患者的长期维持治疗，以推迟情绪发作患者的长期维持治疗，以推迟情绪发作(抑郁

35、、躁狂、轻抑郁、躁狂、轻躁狂、混合型发作躁狂、混合型发作)的时间。的时间。 22ICD-10 F00F09器质性器质性(包括症状性包括症状性)精神障碍精神障碍 00阿尔采末氏病性痴呆阿尔采末氏病性痴呆 01血管性痴呆血管性痴呆 02见于在它处归类的其它疾病的痴呆见于在它处归类的其它疾病的痴呆 03未特定的痴呆未特定的痴呆 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

36、害和功能紊乱所致的人格和行为障碍脑疾病、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未特定的器质性或症状性精神障碍23 F00F09器质性器质性(包括症状性包括症状性)精神障碍精神障碍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 器质性幻觉症器质性幻觉症 F061 器质性紧张性障碍器质性紧张性障碍 F062 器质性妄想性（精神分裂症样）障碍器质性妄想性（精神分裂症样）障碍 F063 器质性心境情感障碍器质性心境情感障碍 F064 器质性焦虑障碍器质性焦虑障碍 F065 器质性分离性障碍器质性分离性障碍

37、F066 器质性情绪不稳定（衰弱）障碍器质性情绪不稳定（衰弱）障碍 F067 轻度认知障碍轻度认知障碍 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器质性器质性(包括症状性包括症状性)精神障碍精神障碍 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 器质性心境器质性心境障碍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

38、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。状的情绪反应。 诊断要点诊断要点 器质性病因的一般性标准见于器质性病因的一般性标准见于F06之引言。除一般性标准外，还应符合之引言。除一般

39、性标准外，还应符合F30F33所列出的各种障碍之一所需的条件。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（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：下列第五位编码可用于指明临床障碍： F0630器质性躁狂障碍器质性躁狂障碍 F0631器质性双相障碍器质性双相障碍 F0632器质性抑郁障碍器质性抑郁障碍 F0633器质性混合性情感性障碍器质性混合性情感性障碍 25 F00F09器质性器质性(包括症状性包括症状性)精神障碍精神障碍 F06 脑损害和功能紊乱以及躯体疾病所致的其它精脑损害和功能紊乱以及躯体疾病所致的其它精神障碍

40、神障碍 F066 器质性器质性不稳定（衰弱）障碍不稳定（衰弱）障碍 特征为明显和持续的特征为明显和持续的情绪失禁或不稳定情绪失禁或不稳定、易疲乏或一系列不、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症远较性障碍所致，据认为由脑血管病或高血压症所致的本症远较其它病因为多。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？（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（不含：躯体形式障碍，非器质性或未特定（F45） 2

41、6 F30F39心境心境情感情感障碍障碍 躁狂发作躁狂发作 双相情感障碍双相情感障碍 抑郁发作抑郁发作 复发性抑郁障碍复发性抑郁障碍 持续性心境持续性心境情感情感障碍障碍:恶劣心境（原抑郁性神恶劣心境（原抑郁性神经症，经症，2001分出）分出） 其它心境情感障碍其它心境情感障碍 未特定的心境未特定的心境情感障碍情感障碍27 F40F48神经症性、应激相关的及躯体形式障碍神经症性、应激相关的及躯体形式障碍 恐怖性焦虑障碍恐怖性焦虑障碍 其它焦虑障碍其它焦虑障碍 强迫性障碍强迫性障碍 严重应激反应，及适应障碍严重应激反应，及适应障碍 分离分离(转换转换)性障碍性障碍 躯体形式障碍躯体形式障碍 其它

42、神经症性障碍其它神经症性障碍28PK“强哭强笑强哭强笑”本质：脑损伤所致的，容易被诱发、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不能随意控制、不伴有对应情绪体验（emotional experience）的）的异常情绪性表达异常情绪性表达（emotional expression）。励：埃菲尔铁塔在哪里？励：埃菲尔铁塔在哪里？朱：在法国（不像是在巴黎）。朱：在法国（不像是在巴黎）。单：在巴黎（不像是在法国）。单：在巴黎（不像是在法国）。励：朱励：朱xx的的“强哭强笑强哭强笑”属于什么障碍？属于什么障碍？朱：属于器质性精神障碍（不像是情绪障碍）。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）。单：属于情绪控制障碍（不像是精神障碍）。29Thank you！