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重视脑损伤后发作性交感过度兴奋中重度脑损伤患者的临床表现中,常可见到一组发作性的高热、多汗、呼吸急促、心动过速、血压升高、瞳孔改变、烦躁并全身强直、阵挛等肌张力障碍等症候群。PerkesI,BaguleyIJ,NottMT,MenonDK.Areview1.ofparoxysmal

sympathetichyperactivityafteracquiredbraininjury.AnnNeurol

2010;68:126–135.tachycardia(>120beats/min),tachypnea(>30/min),systolichypertension(>160mmHg),hyper/hypothermia,excessivesweating,decerebration/decortication,increasedmuscletone,horripilation鸡皮疙瘩

and/orflushing皮肤发红

iscollectivelyreferredtoas“dysautonomia”or“paroxysmalsympathetichyperactivity”syndrome发病率高低不一9.3–33%.KishnerS,AugustinJ,StrumS.Postheadinjuryautonomiccomplications.Lastupdated4October2006October4.Accessed18June2007.FearnsideMR,CookRJ,McDougallP,McNeilRJ.Thewestmeadheadinjuryprojectoutcomeinsevereheadinjury.Acomparativeanalysisofpre-hospital,clinicalandCTvariables.BritishJournalofNeurosurgery1993;7:267–279.Inthefirstpost-injuryweekinICU.Ofthewholesample,33%developedheartrates>120/minandrespiratoryrates>30/minand25%hadbloodpressure>160mmHgandtemperature>39Catsometimeinthefirstweek.LemkeDM.Sympatheticstormingafterseveretraumaticbrain

injury.CritCareNurse2007;27(1):30–7.onsetofdysautonomicparoxysmsandvariousafferentstimuli,bothnoxiousandnon-noxious.Suchstimulihaveincludedpain,endotrachealsuctioning,passivemovementssuchasturning,bathingandmusclestretching,constipation便秘

orakinkedcatheter导尿,emotionalstimuli,aswellasenvironmentalstimulisuchasloudnoises

LaxeS,TerréR,LeónD,BernabeuM.

Howdoesdysautonomiainfluencetheoutcomeoftraumaticbraininjuredpatients

admittedinaneurorehabilitationunit?BrainInj.2013;27(12):1383-7.AllpatientshadbeenreferredtotheS.AnnaInstitute–RANintheyears1998–2005forbeinginaVS/UWScondition.PSHoccurredin26.1%ofthem,withgreaterincidenceaftertraumaticthannon-traumaticbraininjury(31.9%vs15.8%).Outcomewasworsefollowingnon-traumaticbraindamageirrespectiveofPSHandworstamongnon-traumaticsubjectswithPSH.untreatedDysautonomiaincreasesmortalitythroughprolongedhyperthermia,excessivecatabolism分解代谢,highcatecholamine儿茶酚胺

levelsandspasticity/dystonia临床值得关注和重视!BaguleyIJ,HeriseanuRE,GurkaJA,NordenboA,CameronID.

GabapentininthemanagementofDysautonomiafollowing

severetraumaticbraininjury:acaseseries.JNeurolNeurosurg

Psychiatr2007;78(5):539–41itisnotpossibletocompletelyexcludeanepileptogenicaetiologyforallcasesofDysautonomiamultipleattemptstoeitheridentifyortreatepilepsyinDysautonomicpatientshaveproduced

negativeresults

Dysautonomia临床涵盖多个综合征ThesesyndromesincludeNMS,SS,Parkinsonian-HyperpyrexiaSyndrome(PHS)intrathecalbaclofenwithdrawalAutonomicDysreflexiaMalignantCatatonia紧张症MalignantHyperthermiaStiffManSyndromeandIrukandjiSyndrome.

病因区别脑损伤后发作性自主神经功能障碍家族性遗传性自主神经功能障碍、病毒感染后自主神经功能障碍、Guillain-Barre综合征伴发的自主神经功能障碍、脊髓损伤后的自主神经功能障碍目前较为接受的名称Paroxysmalsympathetichyperactivityaftertraumaticbraininjury

PSHFernandez-OrtegaJF,Prieto-PalominoMA,Garcia-CaballeroM,Galeas-LopezJL,Quesada-GarciaG,BaguleyIJ.Paroxysmalsympathetichyperactivityaftertraumaticbraininjury:clinicalandprognosticimplications.JNeurotrauma.2012;29(7):1364-70.

诊断标准—争议Baguley等以具有上述7项中的5项作为诊断依据。Blackman等拟定了更为严格的诊断标准,要求有严重脑损伤(RanchoLosAmigos量表认知功能≤Ⅳ)、体温>38.5&、脉搏>130次/min、呼吸>20次/min、躁动、多汗、肌张力障碍,上述症状每天最少发作1次、持续最少3d,并排除其他疾病。Rabinstein认为该标准过于严格,漏诊的患者会因得不到相应处理而对预后不利。鉴别诊断需要与感染(尤其是颅内感染)、间脑癫痫、颅内压升高(减压窗膨出、脑脊液压力升高)、抗精神病药物引起的恶性综合征(使用多巴胺受体阻滞剂或激动剂)、抗抑郁药引起的5-羟色胺综合征、脊髓损伤(T6~8以上)后自主神经反射异常(尤其合并脑外伤时)、脑外伤后精神障碍、恶性高热、麻醉药物戒断、药物撤离综合征(如巴氯芬的减量过快或突然撤药)等鉴别。而当与上述疾病交织存在时诊断更加复杂,但上述疾病应首先给予排除以免延误病情处理。BaguleyIJ,

HeriseanuRE,

CameronID,

NottMT,

Slewa-YounanS.ACriticalReviewofthePathophysiologyofDysautonomiaFollowingTraumaticBrainInjury.NeurocritCare.

2008;8(2):293-300.

下丘脑自主神经功能损伤或与皮质、皮质下、脑干

神经核团联系中断;交感、副交感平衡失调;

DisconnectiontheoriessuggestthatDysautonomiafollowsthereleaseofoneormoreexcitatorycentresfromhighercentrecontrol脑干和间脑在失去皮质、皮质下结构控制后的释放现象disconnectiontheory,theExcitatory:InhibitoryRatio(EIR)Model,suggeststhecausativebrainstem/diencephaliccentresareinhibitoryinnature,withdamagereleasingexcitatoryspinalcordprocesses.可能的机制AnatomicalmechanismanatomicalandphysiologicalevidencesuggeststhatDysautonomicparoxysmsaremoreconsistentlyassociatedwithmesencephalic

ratherthandiencephaliclesionsparoxysmalepisodescanbetriggeredbyenvironmentaleventsandminimisedbyvariousbutpredictableneurotransmittereffects.NeurotransmitterEffectsOpiateanddopaminergicpathways:

Morphinesettledbothhyperdynamiccardiacfunctionandposturing;bromocriptinedecreasedtemperatureandsweatingclonidinecontrolledbloodpressurebutdidnotobviouslyaffecteitherthenumberofDysautonomicepisodesorthesubject’stemperaturepropanololdecreasescirculatingcatecholamines,andreducesbothcardiacworkandcatabolicdriveGABAßagonistbaclofen;ITBactsoninhibitoryinterneuronsinthespinalcord,gabapentin

(GABAɑ2δ)appearedtoreducethenumberandseverityofparoxysmsandallowedanoverallreductioninothermedications,includingITB,withoutarecurrenceofsymptoms典型病例病例简介:男,27岁,外伤致左额硬膜下血肿清除术后16天,睁眼昏迷(VS),GCS4分。

PSH表现:呼吸↑心率↑大汗↑体温40肌紧张血压↑

1次/4~6h。

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