Locked-in Syndrome (LiS): What It Is, Causes & Symptoms

The total immobility form: In this type of LiS, you have complete body paralysis and loss of eye movement, but you have your normal cognitive ... ComingtoaClevelandCliniclocation?Visitation,maskrequirementsandCOVID-19information Home / HealthLibrary / Disease&Conditions Locked-inSyndrome(LiS) Locked-insyndrome(LiS)isarareandseriousneurologicaldisorderthathappenswhenapartofyourbrainstemisdamaged,usuallyfromastroke.PeoplewithLiShavetotalparalysisbutstillhaveconsciousnessandtheirnormalcognitiveabilities.MostpeoplewithLiScancommunicatewitheyemovementsandleadmeaningfullives. Appointments866.588.2264 Appointments&Locations RequestanAppointment Overview SymptomsandCauses DiagnosisandTests ManagementandTreatment Prevention Outlook/Prognosis LivingWith Locked-inSyndrome(LiS) Overview SymptomsandCauses DiagnosisandTests ManagementandTreatment Prevention Outlook/Prognosis LivingWith BackToTop Overview Locked-insyndromeischaracterizedbycompleteparalysisofvoluntarymuscles,exceptforthosethatcontrolyoureyes. Whatislocked-insyndrome(LiS)? Locked-insyndrome(LiS)isarareneurologicaldisordercharacterizedbyparalysisofvoluntarymuscles,exceptforthosethatcontrolyourverticaleye(upanddown)movements.Peoplewithlocked-insyndromeareconscious,alertandhavetheirusualcognitiveabilities(thinkingandreasoning),butthey’reunabletoshowfacialexpressions,speakormove. PeoplewithLiScanhearandtypicallycommunicatethroughpurposefuleyemovements,blinkingorboth.Theycanalsouseassistivetechnologiestocommunicate. Locked-insyndromeiscausedbydamagetoaspecificpartofyourbrainstemknownasthepons. Whatarethetypesoflocked-insyndrome? Locked-insyndrome(LiS)hasthreemaintypes,orforms,including: Theclassicalform:InthistypeofLiS,youhavetotalimmobility(lackofvoluntarymovement)butcanmoveyoureyesvertically(upanddown),blinkandmaintainyourusualcognitiveabilities.Youcanalsohear. Theincompleteform:ThistypeofLiSisjustliketheclassicalformexceptyoucanhavesomesensationandmovementfunctionsincertainareasofyourbody. Thetotalimmobilityform:InthistypeofLiS,youhavecompletebodyparalysisandlossofeyemovement,butyouhaveyournormalcognitiveabilities.Healthcareproviderscantellapersonwiththisformstillhascognitive(thinkingandreasoning)functionbyexaminingcorticalfunctionwithanelectroencephalogram(EEG),atestthatmeasuresbrainwaves. Whodoeslocked-insyndromeaffect? Anyonecandeveloplocked-insyndrome(LiS),thoughitmorecommonlyaffectspeoplewhoare30to50yearsold. Howcommonislocked-insyndrome? Becausecasesoflocked-insyndrome(LiS)maygounrecognizedormisdiagnosed,it’sdifficultforresearcherstodeterminetheactualnumberofpeoplewhoareaffectedbyLiSeachyear.However,researchersagreethatLiSisrare. SymptomsandCauses Whatarethesignsandsymptomsoflocked-insyndrome? Theeffectsoflocked-insyndrome(LiS)onyourbodyvaryslightlydependingonwhichtypeyouhave. Peoplewithlocked-insyndromeareofteninitiallythoughttobeinacomabeforerecognitionofconsciousness. Mostpeoplewithlocked-insyndromecannotconsciouslyorvoluntarily: Chew. Swallow. Speak. Makefacialexpressions. Produceanybodymovementsbelowtheireyes. Mostpeoplewithlocked-insyndromecan: Movetheireyesupanddown(vertically)butnotsidetoside(horizontally,orlaterally). Blink. Allpeoplewithlocked-insyndromecan: Hear. Comprehendpeopletalkingorreadingtothem. Thinkandreasonastheydidbeforehavinglocked-insyndrome. Havesleep-wakecycles. Canyoufeelpainwithlocked-insyndrome? Dependingonwhichformoflocked-insyndrome(LiS)youhave,youmayormaynotbeabletofeelphysicalpain. IfyouhavethetotalimmobilityformofLiS,youwon’tbeabletofeelphysicalpainduetototalparalysisofyourbody. IfyouhavetheincompleteformofLiS,youmaybeabletofeelphysicalpainandothersensationsincertainareasofyourbody. Whatcauseslocked-insyndrome? Locked-insyndrome(LiS)iscausedbydamagetoaspecificpartofyourbrainstemcalledthepons. Theponsisabroadhorseshoe-shapedmassofnervefibersthatconnectsthemedullaoblongata(thelowestportionofyourbrainstem)withthecerebellum(apartofyourbrainthatplaysavitalroleinvirtuallyallphysicalmovement,particularlycoordination). Theponscontainsimportantneuronalpathwaysbetweenyourcerebrum,spinalcordandcerebellum.Inlocked-insyndrome,damagetoyourponsresultsininterruptionofallthenervesrunningfromyourbrain,throughyourspinalcordandtoyourbody’smuscles.Thisresultsinbodyparalysis.Thedamagetoyourponsalsoaffectsthecentersinyourbrainstemthatareimportantforfacialcontrolandspeaking,whichpreventsyoufrommakingfacialexpressions,chewing,swallowingandtalking. Severalspecificconditionsandsituationscandamageyourpons,causinglocked-insyndrome.Themostcommoncauseisischemicorhemorrhagicstrokeaffectingthecorticospinal,corticopontineandcorticobulbartractsinyourbrainstem. Anischemicstrokehappenswhenabloodvesselinyourbraindevelopsabloodclotandcutsoffthebloodsupplytoyourbrain.Ahemorrhagicstrokehappenswhenbloodfromanarterysuddenlybeginsbleedingintoyourbrain. Otherlesscommoncausesofdamagetoyourponsthatcanleadtolocked-insyndromeinclude: Infectionincertainportionsofyourbrain. Tumorsormassesonyourponsorbrainstem. Demyelination,whichisthelossoftheprotectiveinsulation(myelin)thatsurroundsnervecells. Certainconditions,suchasamyotrophiclateralsclerosis(ALS)andGuillain-Barresyndrome. Trauma(injury)toyourpons. Substancemisuse. DiagnosisandTests Howislocked-insyndromediagnosed? Aspeoplewithlocked-insyndrome(LiS)lackmotorresponses(suchaswithdrawalfrompainfulstimulation),whichhealthcareprovidersusetomeasureresponsiveness,healthcareprovidersmaymistakenlythinkpeoplewithLiSareunconscious.Becauseofthis,LiScanbedifficulttodiagnoseortakeawhiletocorrectlydiagnose. AsignificantaspectofanLiSdiagnosisisrulingoutotherconditionsthatcouldbecausingyoursymptoms.Healthcareprovidersalsoneedtodeterminethecauseoflocked-insyndromeasapartofthediagnosisandtreatment. Whattestswillbedonetodiagnoselocked-insyndrome? Healthcareprovidersuseseveralteststohelpdiagnoselocked-insyndrome,determinethecauseandruleoutotherpossibleconditions. Thesetestsinclude: Magneticresonanceimaging(MRI)scanorcomputedtomography(CT)scan:AnMRIorCTscancanshowifyouhavedamagetoyourponsand/orotherportionsofyourbrain. Cerebralangiography:Thistestcanrevealifyouhaveabloodclotinthearteriesofyourbrainstemorsomewhereelseinyourbrain. Electroencephalogram(EEG):Thistestmeasurestheelectricalactivityofyourbrain.Itcanhelphealthcareprovidersdetermineifyou’reexperiencingnormalbrainactivityandsleep-wakecycles,whichdistinguishesLiSfromotherconditions. Evokedpotentials:Theseareteststhatmeasuretheelectricalactivityincertainareasofyourbrainandspinalcordinresponsetostimulation,suchaspain,auditoryorvisualstimulation.Healthcareprovidersusetheseteststoassessthedamagedresponsesinyourbrainstemandthepreservedresponsesinyourbrain. Electromyography:Thistestmeasureshowwellyourmusclesandnerveswork.Healthcareprovidersmayusethistesttoruleoutdamagetoyourmusclesandnerves. Bloodtests:Ametabolicpanel,particularlycheckingbloodsodiumlevels,canhelpdetermineifpontinemyelinolysisisthecause. Cerebrospinalfluid(CSF)examination:Thistestcanhelpdetermineifaninfectionorautoimmuneconditioniscausingyoursymptoms. ManagementandTreatment Howislocked-insyndrometreated? Thereisnocureorspecifictreatmentsforlocked-insyndromeotherthantreatingthecauseandpreventingfurthercomplications,ifpossible. Managementoflocked-insyndromeincludessupportivetherapyandcommunicationtraining. Supportivetherapy Supportivetherapyforbreathingandfeedingisveryimportant,especiallyearlyon.Peoplewithlocked-insyndrome(LiS)oftenneedanartificialaidforbreathingandwillhaveatracheotomy(atubegoingintheirairwaythroughasmallholeintheirthroat). Becauseeatinganddrinkingusingyourmouthisn’tsafewithLiS,peoplewithLiSusuallyhaveasmalltubeinsertedintheirstomachcalledagastrostomytube(G-tube)toreceivefoodandwater. Othersupportivetherapiesinclude: Preventingcomplicationsduetoimmobilization,suchaspneumonia,urinarytractinfections(UTIs)andthrombosis. Preventingpressureinjuries(bedsores). Providingphysicaltherapytopreventlimbcontractures(lackofrangeofmotionduetojoint,muscleorsofttissuelimitations). Providingphysicaltherapyforrehabilitatingthesmallvoluntarymovementsthatremainorrecover,ifany. Communicationtraining Speechtherapistscanhelppeoplewithlocked-insyndrome(LiS)communicatemoreclearlywitheyemovementsandblinking.Themethodofcommunicationisuniquetoeachperson.Forexample,lookingupcouldmean“yes”andlookingdowncouldmean“no”orviceversa.PeoplewithLiScanalsoformwordsandsentencesbysignalingdifferentlettersinthealphabetwhilesomeoneelseverballylistseachletter. Inadditiontocodedeyemovementsforcommunication,electroniccommunicationdevices,suchasinfraredeyemovementsensorsandcomputervoiceprosthetics,allowpeoplewithlocked-insyndrometocommunicatemorefreelyandhaveaccesstotheinternet. Canapersonrecoverfromlocked-insyndrome? Dependingonthecauseoflocked-insyndrome(LiS),youmayrecovermotorabilities(movement),butacompleterecoveryishighlyunlikely.Somepeoplewithlocked-insyndromehaveminormotorfunctionandsensationincertainareasoftheirbody. ThemajorityofpeoplewithLiSdon’trecovertheirlostnervefunctions,buttheycanlearntocommunicateusingeyemovements. Prevention CanIpreventlocked-insyndrome? Unfortunately,mostcasesoflocked-insyndromearen’tpreventable.Butyourriskoflocked-insyndromemayincreaseifyouhaveahigherriskofstroke. Talktoyourhealthcareprovideraboutyourriskofstrokeandwaysyoucanloweryourrisk. Outlook/Prognosis Whatistheprognosis(outlook)forlocked-insyndrome? Theprognosis(outlook)forpeoplewithlocked-insyndrome(LiS)dependsonthecauseandformofthecondition,aswellasthelevelofsupportandcarethey’reabletoreceive. ArecentsurveyrevealedthatpeoplewithLiSreportedhavingahappyandmeaningfullife,especiallywhentheyhavepropersocialservicesandadaptivetechnologytohelpthemhaveanormalroleathome,aswellasintheircommunity. ManypeoplewithLiScanuseamotorizedwheelchairandacomputerwithadaptivetechnology. Howlongcanyoulivewithlocked-insyndrome? Somepeoplewithlocked-insyndromedon’tlivebeyondtheearlystageoftheconditionduetomedicalcomplications.Butothersliveforanother10to20yearsandreportagoodqualityoflife. LivingWith HowdoItakecareofmyselfortakecareofsomeonewithlocked-insyndrome? Ifyouhavelocked-insyndrome,it’sessentialtoensureyou’regettingqualitymedicalcaretopreventcomplicationsfromimmobilization,suchaspneumoniaandbedsores. It’salsoimportanttofindwaystocommunicate—whetherit’sthrougheyemovementsorassistivetechnology,suchaseye-trackingtechnology.Considerjoiningasupportgrouptomeetotherswhocanrelatetoyourexperiences,too. Ifyou’retakingcareofsomeonewithlocked-insyndrome,it’simportanttoadvocateforthemtoensuretheygetthebestmedicalcareandaccesstotechnologiesthatcanhelpthemcommunicateandbemoreindependent. AnotefromClevelandClinic Locked-insyndrome(LiS)isararebutseriouscondition.Itcanbeveryoverwhelmingtoreceivethiskindofdiagnosis.Knowthatyourhealthcareteamistheretosupportyouandyourlovedones.It’sessentialtoreceivecontinuousqualitymedicalcarewhenlivingwithLiSandtolearnnewwaysofcommunicating.Duetosupportivecareandadvancementsintechnology,manypeoplewithLiSlivefullandmeaningfullives. 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