腦損傷后“強哭強笑”的表現(xiàn)、機制、治療和診斷歸屬-單春雷

PK單朱1PK單朱1PK內(nèi)容勵：患者朱xx的“強哭強笑”屬于什么障礙？朱：屬于器質(zhì)性精神障礙，是腦損害導(dǎo)致的精神異常，不像是情緒障礙。單：屬于情緒控制障礙，可能為皮質(zhì)延髓束損傷導(dǎo)致，不像是精神障礙。2PK內(nèi)容勵：患者朱xx的“強哭強笑”屬于什么障礙？2幾個術(shù)語-中西方不統(tǒng)一情緒：與人的自然性需要相聯(lián)系，具有情景性、暫時性和明顯的外部表現(xiàn)；情緒發(fā)生時會出現(xiàn)一系列的機體內(nèi)部生理變化，并有各種外部表現(xiàn)（面部、動作、語言）。包括心境、激情和應(yīng)激。情感：與人的社會性需要相聯(lián)系，具有穩(wěn)定性、持久性，不一定有明顯的外部表現(xiàn)。包括道德感和價值感兩個方面，具體表現(xiàn)為愛情、友情、幸福、仇恨、厭惡、美感等等。情感的產(chǎn)生伴隨著情緒反應(yīng)，而情緒的變化也受情感的控制。情緒是情感的基礎(chǔ)和外部表現(xiàn)，情感是情緒的深化和本質(zhì)內(nèi)容。3幾個術(shù)語-中西方不統(tǒng)一情緒：與人的自然性需要相聯(lián)系，具有情景ICD10：Emotion：情緒；Mood：心境；Affect：情感Emotionisamentalandphysiologicalstateassociatedwithawidevarietyoffeelings,thoughts,andbehavior.Emotionsaresubjectiveexperiences,oftenassociatedwithmood,temperament,personality,anddisposition.

Moodisarelativelylonglastingemotionalstate.Moodsdifferfromsimpleemotionsinthattheyarelessspecific,lessintense,andlesslikelytobetriggeredbyaparticularstimulusorevent.Affectreferstotheexperienceoffeelingoremotion.Affectisakeypartoftheprocessofanorganism’sinteractionwithstimuli.Thewordalsoreferssometimestoaffectdisplay,whichis"afacial,vocal,orgesturalbehaviorthatservesasanindicatorofaffect."(APA2006)"Mood

istheemotionalfeelingstatedbyapatient,andaffect

istheemotionalappearanceofthepatient."幾個術(shù)語-中西方不統(tǒng)一4ICD10：幾個術(shù)語-中西方不統(tǒng)一4腦損傷后“強哭強笑”的表現(xiàn)、

機制、治療和診斷分類南京醫(yī)科大學(xué)一附院康復(fù)醫(yī)學(xué)科單春雷5腦損傷后“強哭強笑”的表現(xiàn)、

機制、治療和診斷分類南京醫(yī)科大Pseudobulbaraffect（PBA，假性延髓情緒）isadramaticdisorderofemotionalexpressionandregulationcharacterizedbyuncontrollableepisodesoflaughingandcryingthatoftencauseembarrassment,curtailmentofsocialactivities,andreductioninqualityoflife.Thedisorderoccursinpatientswithbraininjurycausedbymanytypesofneurologicaldisease,includingstroke,tumors,andneurodegenerativegrayandwhitematterdisorders.Althoughthepathophysiologyisunknown,PBAmayrelatetoreleaseofbrainstememotionalcontrolcentersfromregulationbythefrontallobes.單：患者朱XX的“強哭強笑”屬情緒控制障礙，可能為皮質(zhì)延髓束損傷（假性延髓麻痹）造成。6Pseudobulbaraffect（PBA，假性延髓情緒77PoeckcrystallizedthefeaturesofPBAintofourcriteria.First,theepisodesareinappropriatetothesituationandcanbeprecipitatedbynonspecificstimuli,suchascontractionoffacialmuscles,removalofbedcovers,ortheapproachofsomeonetowardthepatient.Second,thereisnotacloserelationbetweentheemotionalexpressionandhowthepatientisfeeling.Third,theepisodesarerelativelystereotyped,anditisdifficultforpatientstocontroltheextentanddurationoftheepisodes.Last,therearenoepisodicmoodchangescorrespondingtotheepisodes,andthereisnosenseofreliefastheemotionsareexpressed.Thislastcriteriontriestocapturethefactthattheepisodesappeartocomeunprovokedandoutofcontext.AllthesefeaturesservetodifferentiatePBAfromdepression,wherecryingusuallyiscontextappropriate.8PoeckcrystallizedthefeaturePBAhasbeenrecognizedforwellmorethanacentury.Infact,Darwinnotedthedisorderinhisstudiesofhumanemotion.Wilsonobservedthatitisfrequentlyassociatedwithdamagetodescendingmotorsystems.Wilsonlinkedthephenomenontonormal-appearing,involuntarilyexpressedemotionsthatoccurinthecontextofuppermotorneuronlesions,evenwithfacialparesis.HetheorizedthatPBArepresentsthereleaseofafasciorespiratorycontrolcenterforemotionalexpressioninthebrainstemfromvoluntarycontrolbyhighercorticalbraincenters.9PBAhasbeenrecognizedforweFocallesionscausingPBAhavebeendescribedinnearlyeverypartofthebrain,includingfrontalcorticalandsubcorticalstructures,brainstemregions,andanteriortemporalregions.Ithasbeenobservedinbothunilateralandbilateralinjury.PBAfromisolatedparietaloroccipitallesionsisrarelyreported.Thisstudyrevealedthatpoststroke“emotionalincontinence”occurredmorefrequentlyafterstrokeinthelenticulocapsularregion,basispontis,medullaoblongata,orthecerebellum.10FocallesionscausingPBAhaveNomenclature-命名法SeveraltermsareusedinterchangeablywithPBA.PBAisfrequentlyusedbecausethephenomenonoftenoccursinthesettingofpseudobulbarpalsycausedbydocumentedorputativefrontallobeinjury.SomehavearguedthatthelinkbetweenPBAandpseudobulbarpalsyisimperfect,andthatothertermsshouldbepreferred;however,thetermiscommonandfamiliartomostphysicians.Incontrast,moredescriptivetermssuchaspathologicallaughingandcrying（病理性哭笑）,affectivelability（情緒不穩(wěn)），emotionalincontinence（情緒失禁）,andemotionalism（易動情緒）mayhavesomeadvantagesoverPBAinthattheydonotimplyaspecificpathophysiologyorclinicalcontext,buttheymaybeoverlygeneral.11Nomenclature-命名法SeveralteRecently,theterminvoluntaryemotionalexpressiondisorder（不隨意性情緒表達(dá)障礙，IEED）wascoined.Involuntaryemotionalexpressiondisorder(IEED),alsocalledpseudobulbaraffect(PBA),pathologicallaughterandcrying(PLC)andaffectivelability,ischaracterizedbybrief,spontaneousanduncontrollableepisodesofcryingorlaughingthataretypicallyunrelatedtounderlyingmood.12Recently,theterminvoluntary情緒不穩(wěn)易動情緒情緒失控情緒失禁情緒不穩(wěn)過度情緒化強哭強笑不恰當(dāng)歡喜病理性情緒病理性情緒化病理性易動情緒病理性哭笑病理性流淚假性延髓情緒假性延髓哭泣不隨意性情緒表達(dá)障礙13情緒不穩(wěn)易動情緒情緒失控情緒失禁情緒不穩(wěn)過度情緒化強哭強Episodesoflaughingandcryingareconsideredpathologicalwhentheyoccurwithoutvoluntarycontrolandmodulation,arenotmeaningfullyrelatedtothestimulithatprovokethem(ie,contextuallyinappropriate),neitherreflectnorchangetheprevailingmood,andinvolveadissociationbetweenaffectiveexpressionandexperience(情緒表達(dá)和情緒體驗的分離).Theclassicexampleofsuchisapatientwithastrokewhoappearsemotionallynormalmostofthetime,butunpredictablyburstsintotearsandgrimaces,andvocalizesattheslightestprovocation.Aftertheseexcessivelyintenseanduncontrollableepisodesruntheircourseoverafewminutes,thepatientreturnstoanemotionally"neutral"baseline.Whenaskedhowhefeltduringtheepisode,thepatientrepliesthathefeltnothingatall--nosadness,anxiety,joy,oranyothersubjectiveemotionalexperienceoccursduringtheseepisodes.Thisformofaffectdysregulation--cryingwithoutfeelingsadandlaughingwithoutfeelingmirthoramusement--istheprototypeofPLC.ManypersonswithPLCexperiencebothepisodesofcryingandoflaughing.Whenonlyonetypeofepisodeoccursinanindividualpatient,pathologicalcryingaloneisthemorecommonpresentation.PathologicalLaughingandCrying（病理性哭笑）14EpisodesoflaughingandcryinAnyneurologicaldisorderthatinterfereswiththecorticobulbarorcortico-subcortical-thalamo-cerebellarcircuitsthatpermitregulationofaffectcanproducePLC.Commonunderlyingneurologicalconditionsincludestroke,amyotrophiclateralsclerosis,Parkinson'sdisease,multiplesclerosis,frontotemporaldementia,traumaticbraininjury,Alzheimer'sdisease,epilepsy,normalpressurehydrocephalus,progressivesupranuclearpalsy,Wilson'sdisease(hepatolenticulardegeneration),andneurosyphilis.15Anyneurologicaldisordertha

AffectiveLability(情緒不穩(wěn))EpisodesofaffectivelabilityaresimilartothoseofPLCinthattheyarebrief,excessivelyintensewithrespecttotheincitingstimulus,notfullyamenabletonormalvoluntarycontrol,andneitherreflectnorchangetheprevailingmood.However,theseepisodesareoftenlesssevereandmoreunderstandablyrelatedtosentimentalstimulithanareepisodesofPLC.Additionally,thesubjectiveandobjectivedimensionsofaffectarenotdissociatedduringepisodesofaffectivelability.Personswithaffectivelabilityfeelsadwhentheycryandfeelamusementwhentheylaugh,buttheyareunabletocontroltheintensity,duration,orfrequencyoftheseepisodes.Whilethestimulusforsuchepisodesmaycarrysomesentimentalvalence,thequalityoftheaffectiveresponseisinexcessofthatmeritedbythestimulusthatincitesit.Asaresult,episodesofaffectivelabilityaremorestereotypedthannormalaffectivevariability(ie,theyarepathological),althoughtheytendtobelessstereotypedthanepisodesofPLC.Anyneurologicaldisorderthatinterfereswiththecorticobulbarorcortico-subcortical-thalamo-cerebellarcircuitsinvolvedinaffectregulationcanproduceaffectivelability;notsurprisingly,thecausesofaffectivelabilityoverlapsubstantiallywiththosethatproducePLC.16AffectiveLability(情緒不穩(wěn))Episo1717治療AlthoughtherearenoUSFoodandDrugAdministration–approvedtreatmentsforPBA,severalagentshavebeenshowntobeeffective,includingtricyclicantidepressants（三環(huán)抗抑郁藥,阿米替林,25-300mg/d

）,selectiveserotoninreuptakeinhibitors（選擇性5-HT再攝取抑制劑,西酞普蘭,5-40mg/d）,andanewagentcontainingdextromethorphan（DM，右美沙芬，普西蘭,30mg,bid）andquinidine（奎尼丁,30mg,bid），（加：

金剛烷胺50-200mg,bid）.18治療AlthoughtherearenoUSFoo治療：DM/Q（右美沙芬）DM/Q（右美沙芬）hasbeenshowntobeeffectiveinamelioratingPBAinbothALS（肌萎縮性側(cè)索硬化癥）andMS（多發(fā)性硬化）,andithasbeenassessedinalargernumberofpatientsthananypreviousdrugusedtotreatPBA;however,ithasnotbeencomparedwiththeotheragentsthathavepreviouslyshownefficacy.ThemechanismbywhichDM/QhelpsPBAisunknown.DM,theactiveingredient(QisusedtoslowmetabolismofDM),isanN-methyl-D-aspartate(NMDA，N-甲基-D-天(門)冬氨酸)receptorantagonist.19治療：DM/Q（右美沙芬）DM/Q（右美沙芬）hasbe治療：DM/Q（右美沙芬）【其他名稱】右甲嗎喃；美沙芬；普西蘭；【藥物作用】為中樞性鎮(zhèn)咳藥，抑制延髓咳嗽中樞而產(chǎn)生鎮(zhèn)咳作用。鎮(zhèn)咳作用顯著，與相同劑量的可待因大體相同或稍強，但無止痛作用。長期服用無成癮性和耐受性，治療劑量不會抑制呼吸，作用快且安全?！具m應(yīng)癥狀】用于感冒、急性或慢性支氣管炎，上呼吸道感染時的咳嗽。20治療：DM/Q（右美沙芬）【其他名稱】右甲嗎喃；美沙芬；治療：拉莫三嗪LamotrigineInitiallyatthedoseof50mgaday,whichwasgraduallyincreasedto100mgadayovera4-weekperiod。規(guī)

格：25mg、50mg、100mg。

適應(yīng)癥：癲癇（簡單部分性發(fā)作、復(fù)雜部分性發(fā)作、續(xù)發(fā)性和原發(fā)性全身強直－陣攣性發(fā)作）。也可用于治療合并有Lennox-Gastaut綜合征的癲癇發(fā)作。

2003年6月，拉莫三嗪（lamotrigine）片劑獲美國FDA批準(zhǔn)，用于用標(biāo)準(zhǔn)藥物治療急性情緒發(fā)作的成人雙極失調(diào)患者的長期維持治療，以推遲情緒發(fā)作(抑郁、躁狂、輕躁狂、混合型發(fā)作)的時間。21治療：拉莫三嗪Lamotrigine21ICD-10F00－F09器質(zhì)性(包括癥狀性)精神障礙00阿爾采末氏病性癡呆01血管性癡呆02見于在它處歸類的其它疾病的癡呆03未特定的癡呆04器質(zhì)性遺忘綜合征、非酒和其它精神活性物質(zhì)所致05譫妄，非酒和其它精神活性物質(zhì)所致06腦損害和功能紊亂以及軀體疾病所致的其它精神障礙07腦疾病、損害和功能紊亂所致的人格和行為障礙08未特定的器質(zhì)性或癥狀性精神障礙22ICD-10F00－F09器質(zhì)性(包括癥狀性)精神障礙22F00－F09器質(zhì)性(包括癥狀性)精神障礙F06腦損害和功能紊亂以及軀體疾病所致的其它精神障礙

F06．0器質(zhì)性幻覺癥

F06．1器質(zhì)性緊張性障礙

F06．2器質(zhì)性妄想性（精神分裂癥樣）障礙

F06．3器質(zhì)性心境〔情感〕障礙

F06．4器質(zhì)性焦慮障礙

F06．5器質(zhì)性分離性障礙

F06．6器質(zhì)性情緒不穩(wěn)定（衰弱）障礙

F06．7輕度認(rèn)知障礙

F06．8腦損害和功能紊亂及軀體疾病所致的其它特定性精神障礙

F06．9腦損害和功能紊亂及軀體疾病所致的未特定的精神障礙

23F00－F09器質(zhì)性(包括癥狀性)精神障礙23F00－F09器質(zhì)性(包括癥狀性)精神障礙F06腦損害和功能紊亂以及軀體疾病所致的其它精神障礙

F06．3

器質(zhì)性心境〔情感〕障礙特征為心境或情感改變，常伴有總體活動水平的改變。這類障礙歸入本節(jié)的唯一標(biāo)準(zhǔn)是假定其病因為某種大腦或軀體疾病，通過檢查或者根據(jù)恰當(dāng)?shù)牟∈焚Y料能推測出這些疾病的存在。情感性障礙必須出現(xiàn)于設(shè)想的器質(zhì)性病因之后，此外尚需確定精神障礙不是病人對知道所患疾病或疾病的癥狀的情緒反應(yīng)。

診斷要點

器質(zhì)性病因的一般性標(biāo)準(zhǔn)見于F06之引言。除一般性標(biāo)準(zhǔn)外，還應(yīng)符合F30－F33所列出的各種障礙之一所需的條件。

不含：心境〔情感〕障礙，非器質(zhì)性或未特定（F30—F39）

下列第五位編碼可用于指明臨床障礙：

F06．30器質(zhì)性躁狂障礙

F06．31器質(zhì)性雙相障礙

F06．32器質(zhì)性抑郁障礙

F06．33器質(zhì)性混合性情感性障礙24F00－F09器質(zhì)性(包括癥狀性)精神障礙24F00－F09器質(zhì)性(包括癥狀性)精神障礙F06腦損害和功能紊亂以及軀體疾病所致的其它精神障礙

F06．6

器質(zhì)性情緒不穩(wěn)定（衰弱）障礙

特征為明顯和持續(xù)的情緒失禁或不穩(wěn)定、易疲乏或一系列不愉快的軀體感受（如頭暈）和疼痛，這些癥狀是由某種器質(zhì)性障礙所致，據(jù)認(rèn)為由腦血管病或高血壓癥所致的本癥遠(yuǎn)較其它病因為多。

（但，這是否包含了不伴情緒體驗的異常情緒表現(xiàn)？PBA）

不含：軀體形式障礙，非器質(zhì)性或未特定（F45．－）25F00－F09器質(zhì)性(包括癥狀性)精神障礙25F30－F39心境[情感]障礙躁狂發(fā)作雙相情感障礙抑郁發(fā)作復(fù)發(fā)性抑郁障礙持續(xù)性心境[情感]障礙:惡劣心境（原抑郁性神經(jīng)癥，2001分出）其它心境［情感］障礙未特定的心境[情感］障礙26F30－F39心境[情感]障礙26F40－F48神經(jīng)癥性、應(yīng)激相關(guān)的及軀體形式障礙恐怖性焦慮障礙其它焦慮障礙強迫性障礙嚴(yán)重應(yīng)激反應(yīng)，及適應(yīng)障礙分離(轉(zhuǎn)換)性障礙軀體形式障礙其它神經(jīng)癥性障礙27F40－F48神經(jīng)癥性、應(yīng)激相關(guān)的及軀體形式障礙27PK“強哭強笑”本質(zhì)：腦損傷所致的，容易被誘發(fā)、不能隨意控制、不伴有對應(yīng)情緒體驗（emotionalexperience）的異常情緒性表達(dá)（emotionalexpression）。勵：埃菲爾鐵塔在哪里？朱：在法國（不像是在巴黎）。單：在巴黎（不像是在法國）。勵：朱xx的“強哭強笑”屬于什么障礙？朱：屬于器質(zhì)性精神障礙（不像是情緒障礙）。單：屬于情緒控制障礙（不像是精神障礙）。28PK“強哭強笑”本質(zhì)：腦損傷所致的，容易被誘發(fā)、不能隨意控制Thankyou！29Thankyou！29PK單朱30PK單朱1PK內(nèi)容勵：患者朱xx的“強哭強笑”屬于什么障礙？朱：屬于器質(zhì)性精神障礙，是腦損害導(dǎo)致的精神異常，不像是情緒障礙。單：屬于情緒控制障礙，可能為皮質(zhì)延髓束損傷導(dǎo)致，不像是精神障礙。31PK內(nèi)容勵：患者朱xx的“強哭強笑”屬于什么障礙？2幾個術(shù)語-中西方不統(tǒng)一情緒：與人的自然性需要相聯(lián)系，具有情景性、暫時性和明顯的外部表現(xiàn)；情緒發(fā)生時會出現(xiàn)一系列的機體內(nèi)部生理變化，并有各種外部表現(xiàn)（面部、動作、語言）。包括心境、激情和應(yīng)激。情感：與人的社會性需要相聯(lián)系，具有穩(wěn)定性、持久性，不一定有明顯的外部表現(xiàn)。包括道德感和價值感兩個方面，具體表現(xiàn)為愛情、友情、幸福、仇恨、厭惡、美感等等。情感的產(chǎn)生伴隨著情緒反應(yīng)，而情緒的變化也受情感的控制。情緒是情感的基礎(chǔ)和外部表現(xiàn)，情感是情緒的深化和本質(zhì)內(nèi)容。32幾個術(shù)語-中西方不統(tǒng)一情緒：與人的自然性需要相聯(lián)系，具有情景ICD10：Emotion：情緒；Mood：心境；Affect：情感Emotionisamentalandphysiologicalstateassociatedwithawidevarietyoffeelings,thoughts,andbehavior.Emotionsaresubjectiveexperiences,oftenassociatedwithmood,temperament,personality,anddisposition.

Moodisarelativelylonglastingemotionalstate.Moodsdifferfromsimpleemotionsinthattheyarelessspecific,lessintense,andlesslikelytobetriggeredbyaparticularstimulusorevent.Affectreferstotheexperienceoffeelingoremotion.Affectisakeypartoftheprocessofanorganism’sinteractionwithstimuli.Thewordalsoreferssometimestoaffectdisplay,whichis"afacial,vocal,orgesturalbehaviorthatservesasanindicatorofaffect."(APA2006)"Mood

istheemotionalfeelingstatedbyapatient,andaffect

istheemotionalappearanceofthepatient."幾個術(shù)語-中西方不統(tǒng)一33ICD10：幾個術(shù)語-中西方不統(tǒng)一4腦損傷后“強哭強笑”的表現(xiàn)、

機制、治療和診斷分類南京醫(yī)科大學(xué)一附院康復(fù)醫(yī)學(xué)科單春雷34腦損傷后“強哭強笑”的表現(xiàn)、

機制、治療和診斷分類南京醫(yī)科大Pseudobulbaraffect（PBA，假性延髓情緒）isadramaticdisorderofemotionalexpressionandregulationcharacterizedbyuncontrollableepisodesoflaughingandcryingthatoftencauseembarrassment,curtailmentofsocialactivities,andreductioninqualityoflife.Thedisorderoccursinpatientswithbraininjurycausedbymanytypesofneurologicaldisease,includingstroke,tumors,andneurodegenerativegrayandwhitematterdisorders.Althoughthepathophysiologyisunknown,PBAmayrelatetoreleaseofbrainstememotionalcontrolcentersfromregulationbythefrontallobes.單：患者朱XX的“強哭強笑”屬情緒控制障礙，可能為皮質(zhì)延髓束損傷（假性延髓麻痹）造成。35Pseudobulbaraffect（PBA，假性延髓情緒367PoeckcrystallizedthefeaturesofPBAintofourcriteria.First,theepisodesareinappropriatetothesituationandcanbeprecipitatedbynonspecificstimuli,suchascontractionoffacialmuscles,removalofbedcovers,ortheapproachofsomeonetowardthepatient.Second,thereisnotacloserelationbetweentheemotionalexpressionandhowthepatientisfeeling.Third,theepisodesarerelativelystereotyped,anditisdifficultforpatientstocontroltheextentanddurationoftheepisodes.Last,therearenoepisodicmoodchangescorrespondingtotheepisodes,andthereisnosenseofreliefastheemotionsareexpressed.Thislastcriteriontriestocapturethefactthattheepisodesappeartocomeunprovokedandoutofcontext.AllthesefeaturesservetodifferentiatePBAfromdepression,wherecryingusuallyiscontextappropriate.37PoeckcrystallizedthefeaturePBAhasbeenrecognizedforwellmorethanacentury.Infact,Darwinnotedthedisorderinhisstudiesofhumanemotion.Wilsonobservedthatitisfrequentlyassociatedwithdamagetodescendingmotorsystems.Wilsonlinkedthephenomenontonormal-appearing,involuntarilyexpressedemotionsthatoccurinthecontextofuppermotorneuronlesions,evenwithfacialparesis.HetheorizedthatPBArepresentsthereleaseofafasciorespiratorycontrolcenterforemotionalexpressioninthebrainstemfromvoluntarycontrolbyhighercorticalbraincenters.38PBAhasbeenrecognizedforweFocallesionscausingPBAhavebeendescribedinnearlyeverypartofthebrain,includingfrontalcorticalandsubcorticalstructures,brainstemregions,andanteriortemporalregions.Ithasbeenobservedinbothunilateralandbilateralinjury.PBAfromisolatedparietaloroccipitallesionsisrarelyreported.Thisstudyrevealedthatpoststroke“emotionalincontinence”occurredmorefrequentlyafterstrokeinthelenticulocapsularregion,basispontis,medullaoblongata,orthecerebellum.39FocallesionscausingPBAhaveNomenclature-命名法SeveraltermsareusedinterchangeablywithPBA.PBAisfrequentlyusedbecausethephenomenonoftenoccursinthesettingofpseudobulbarpalsycausedbydocumentedorputativefrontallobeinjury.SomehavearguedthatthelinkbetweenPBAandpseudobulbarpalsyisimperfect,andthatothertermsshouldbepreferred;however,thetermiscommonandfamiliartomostphysicians.Incontrast,moredescriptivetermssuchaspathologicallaughingandcrying（病理性哭笑）,affectivelability（情緒不穩(wěn)），emotionalincontinence（情緒失禁）,andemotionalism（易動情緒）mayhavesomeadvantagesoverPBAinthattheydonotimplyaspecificpathophysiologyorclinicalcontext,buttheymaybeoverlygeneral.40Nomenclature-命名法SeveralteRecently,theterminvoluntaryemotionalexpressiondisorder（不隨意性情緒表達(dá)障礙，IEED）wascoined.Involuntaryemotionalexpressiondisorder(IEED),alsocalledpseudobulbaraffect(PBA),pathologicallaughterandcrying(PLC)andaffectivelability,ischaracterizedbybrief,spontaneousanduncontrollableepisodesofcryingorlaughingthataretypicallyunrelatedtounderlyingmood.41Recently,theterminvoluntary情緒不穩(wěn)易動情緒情緒失控情緒失禁情緒不穩(wěn)過度情緒化強哭強笑不恰當(dāng)歡喜病理性情緒病理性情緒化病理性易動情緒病理性哭笑病理性流淚假性延髓情緒假性延髓哭泣不隨意性情緒表達(dá)障礙42情緒不穩(wěn)易動情緒情緒失控情緒失禁情緒不穩(wěn)過度情緒化強哭強Episodesoflaughingandcryingareconsideredpathologicalwhentheyoccurwithoutvoluntarycontrolandmodulation,arenotmeaningfullyrelatedtothestimulithatprovokethem(ie,contextuallyinappropriate),neitherreflectnorchangetheprevailingmood,andinvolveadissociationbetweenaffectiveexpressionandexperience(情緒表達(dá)和情緒體驗的分離).Theclassicexampleofsuchisapatientwithastrokewhoappearsemotionallynormalmostofthetime,butunpredictablyburstsintotearsandgrimaces,andvocalizesattheslightestprovocation.Aftertheseexcessivelyintenseanduncontrollableepisodesruntheircourseoverafewminutes,thepatientreturnstoanemotionally"neutral"baseline.Whenaskedhowhefeltduringtheepisode,thepatientrepliesthathefeltnothingatall--nosadness,anxiety,joy,oranyothersubjectiveemotionalexperienceoccursduringtheseepisodes.Thisformofaffectdysregulation--cryingwithoutfeelingsadandlaughingwithoutfeelingmirthoramusement--istheprototypeofPLC.ManypersonswithPLCexperiencebothepisodesofcryingandoflaughing.Whenonlyonetypeofepisodeoccursinanindividualpatient,pathologicalcryingaloneisthemorecommonpresentation.PathologicalLaughingandCrying（病理性哭笑）43EpisodesoflaughingandcryinAnyneurologicaldisorderthatinterfereswiththecorticobulbarorcortico-subcortical-thalamo-cerebellarcircuitsthatpermitregulationofaffectcanproducePLC.Commonunderlyingneurologicalconditionsincludestroke,amyotrophiclateralsclerosis,Parkinson'sdisease,multiplesclerosis,frontotemporaldementia,traumaticbraininjury,Alzheimer'sdisease,epilepsy,normalpressurehydrocephalus,progressivesupranuclearpalsy,Wilson'sdisease(hepatolenticulardegeneration),andneurosyphilis.44Anyneurologicaldisordertha

AffectiveLability(情緒不穩(wěn))EpisodesofaffectivelabilityaresimilartothoseofPLCinthattheyarebrief,excessivelyintensewithrespecttotheincitingstimulus,notfullyamenabletonormalvoluntarycontrol,andneitherreflectnorchangetheprevailingmood.However,theseepisodesareoftenlesssevereandmoreunderstandablyrelatedtosentimentalstimulithanareepisodesofPLC.Additionally,thesubjectiveandobjectivedimensionsofaffectarenotdissociatedduringepisodesofaffectivelability.Personswithaffectivelabilityfeelsadwhentheycryandfeelamusementwhentheylaugh,buttheyareunabletocontroltheintensity,duration,orfrequencyoftheseepisodes.Whilethestimulusforsuchepisodesmaycarrysomesentimentalvalence,thequalityoftheaffectiveresponseisinexcessofthatmeritedbythestimulusthatincitesit.Asaresult,episodesofaffectivelabilityaremorestereotypedthannormalaffectivevariability(ie,theyarepathological),althoughtheytendtobelessstereotypedthanepisodesofPLC.Anyneurologicaldisorderthatinterfereswiththecorticobulbarorcortico-subcortical-thalamo-cerebellarcircuitsinvolvedinaffectregulationcanproduceaffectivelability;notsurprisingly,thecausesofaffectivelabilityoverlapsubstantiallywiththosethatproducePLC.45AffectiveLability(情緒不穩(wěn))Episo4617治療AlthoughtherearenoUSFoodandDrugAdministration–approvedtreatmentsforPBA,severalagentshavebeenshowntobeeffective,includingtricyclicantidepressants（三環(huán)抗抑郁藥,阿米替林,25-300mg/d

）,selectiveserotoninreuptakeinhibitors（選擇性5-HT再攝取抑制劑,西酞普蘭,5-40mg/d）,andanewagentcontainingdextromethorphan（DM，右美沙芬，普西蘭,30mg,bid）andquinidine（奎尼丁,30mg,bid），（加：

金剛烷胺50-200mg,bid）.47治療AlthoughtherearenoUSFoo治療：DM/Q（右美沙芬）DM/Q（右美沙芬）hasbeenshowntobeeffectiveinamelioratingPBAinbothALS（肌萎縮性側(cè)索硬化癥）andMS（多發(fā)性硬化）,andithasbeenassessedinalargernumberofpatientsthananypreviousdrugusedtotreatPBA;however,ithasnotbeencomparedwiththeotheragentsthathavepreviouslyshownefficacy.ThemechanismbywhichDM/QhelpsPBAisunknown.DM,theactiveingredient(QisusedtoslowmetabolismofDM),isanN-methyl-D-a