Neurology-Main神经内科-11-20

NeurologyMain11-20Q11A36-year-oldCaucasianmaleisbroughttotheemergencydepartmentduetoweaknessofhisupperandlowerextremities.Neurologicalexaminationrevealsweakness,atrophy,fasciculations,spasticityandhyperreflexiaoftheinvolvedmuscles.Hissensory,bowel，bladderandcognitivefunctionsareintact.Serumcreatinekinaseisnormal.Cerebrospinalfluidexaminationiswithinnormallimits.Electromyographyshowschronicpartialdenervation.Thepatientissubsequentlydiagnosedwithamyotrophiclateralsclerosis.Whichofthefollowinghasbeenapprovedforuseinpatientswithamyotrophiclateralsclerosis?A.RiluzoleB.CorticosteroidsC.IntravenousimmunoglobulinsD.PlasmapheresisE.DonepezilA11Correctanswer:ARiluzoleisaglutamateinhibitorthatiscurrentlyapprovedforuseinpatientswithamyotrophiclateralsclerosis.Althoughitcannotarresttheunderlyingpathologicalprocess,itmayprolongsurvivalandthetimetotracheostomy.Itssideeffectsaredizziness,nausea,weightloss,elevatedliverenzymesandskeletalweakness.(ChoicesB,C&D)Amyotrophiclateralsclerosisisaneurodegenerativedisease.Corticosteroids:intravenousimmunogiobuiinsandcyclophosphamidehavenorolehitsmanagement.Theseagentsareusedinthemanagementofimmunologically-mediaiedneurologicaldiseases,suchasmultiplesclerosisandGuillain-Barresyndrome.Corticosteroidsareusedtotreatacuteexacerbationsofmultiplesclerosis,whileplasmapheresisandintravenous丨mmunoglobulinsarethemsintreatmentmodalitiesofGuillain-Barresyndrome.(ChoiceE)DonepezilisacholinesteraseinhibitorthatisusedtoimprovecognitivefunctioninpatientswithAlzheimer'sdisease.Ithasnoroleinthemanagemeniofamyotrophiclateralsclerosis.EducationalObjective:Riluzoleisaglutamateinhibitorthatiscurrentlyapprovedforuseinpatientswithamyotrophiclateralsclerosis.Q12A35-year-oldmanisbroughttotheemergencydepartmentafterhejumpedfromthefourthfloorofaburningbuilding.Histemperatureis36.9°C(98.5°F);bloodpressureis90/40,pulseis90/min,andrespirationsare20/min.Examinationshowsafractureoftherighttibia.Heisconsciousandhispupilsarebilaterallyequalandreactivetolightandaccommodation.Hisneurologicalexaminationshowsparaplegia,withlossofpainandtemperatureinbothlegsbutnormalproprioception.Upperextremitiesdonotshowanyneurologicaldeficits.Passivestraightlegraisingtestisnegative.ACTscanofthespineshowsaburstfractureatthelevelofthefourththoracicvertebra.Whichofthefollowingisthemostlikelydiagnosis?A.CentralcordsyndromeB.AnteriorcordsyndromeC.BrownSequardsyndromeD.AcutediskprolapseE.CaudaequinesyndromeA12Correctanswer:BThisclinicalpresentationisacharacteristicofanteriorcordsyndrome.Anteriorcordsyndromeiscommonlyassociatedwithburstfractureofthevertebraandischaracterizedbytotallossofmotorfunctionbelowtheleveloflesionwithlossofpainandtemperatureonbothsidesbelowthelesion.MRIisthebestinvestigationtostudytheextentofneurologicaldamage(ChoiceB).(ChoiceA)Centralcordsyndromeischaracterizedbyburningpainandparalysisinupperextremitieswithrelativesparingoflowerextremities,itiscommonlyseeninelderlysecondarytoforcedhyperextensiontypeofinjurytotheneck.(ChoiceC)BrownSequardsyndromeisacutehemisectionofcordandischaracterizedbyipsilateralmotorandproprioceptionlossandcontralateralpainlossbelowtheleveloflesion.(ChoiceD)Acutediskprolapsewillbecharacterizedbysevereradicularpainwithpositivestraightlegraisingtest.(ChoiceE)Caudaequinesyndromeischaracterizedbyparaplegia：variablesensoryloss,urinaryandfecalincontinenceanditwouldnotoccursecondarytofractureoffourththoracicvertebra.EducationalObjective:Anteriorcordsyndromeiscommonlyassociatedwithburstfractureofthevertebraandischaracterizedbytotallossofmotorfunctionbelowtheleveloflesionwithlossofpainandtemperatureonbothsidesbelowthelesionandwithintactproprioception.Q13A29-year-oldwomancomestotheclinicforroutinefollow-up.Fiveyearsago,sheexperiencedanunprovokedgeneralizedtonic-clonicseizure.Atthatiime;phenytoinwasprescribed,andthepatienthasbeenonastabledosesince.Shehasnotexperiencedseizurerecurrence.MRIofthebrainperformedfollowingtheinitialepisodewasnormal.Shehasnoothermedicalproblemsandisalifetimenonsmoker.Thepatient'sfamilyhistoryis门egativeforneurologicdisease.Sheisinastablerelationshipandiscontemplatingpregnancy.Bloodpressureis123/72mmHgandpulseis75/min.Neurologicexaminationisunremarkable.Serumphenytoinlevelis11mcg/mL(therapeuticrange,10-20mcg/mL).Electroencephalogramisnormal.Whichofthefollowingisthebestnextstepinmanagementofthispatient?A.DiscontinuephenytoinnowB.IncreasephenytoindoseC.iRepeatMRIofthebrainD.SlowlytaperanddiscontinuephenytoinE.SwitchtovalproicacidA13Correctanswer:DDiscontinuingphenytoininthispatientwhohasahistoryofunprovokedseizurebuthasbeenseizurefree2yearsisareasonableconsideration.Givenherlackofidentifiablebraindisease,normalEEG,absenceoffamilialepilepsy,andpositiveinitialresponsetotherapy,thispatientisatalowriskforseizurerecurrence.Inaddition,phenytoinisaknownteratogencapableofcausingfetalhydantoinsyndrome(eg,orofacialclefts：microcephaly,nail/digithypoplasia,cardiacdefects,dysmorphicfacialfeatures).Givenitsknownteratogenicity,phenytoinshouldbediscontinuedinwomenofreproductiveagewhoareconsideringbecom