Neurology-Main神经内科-161-170

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NeurologyMain161-170Q161A10-hour-oldnewbornisnotedtohaveaswellinginthescalpwhichwasnotpresentatbirth.Theswellingislimitedtothesurfaceofonecranialbone.Therearenovisiblepulsations,indentationsoftheskull,ordiscolorationoftheoverlyingscalp.Whatisthemostlikelydiagnosis?A.CephalohematomaB.CaputsuccedaneumC.CranialmeningoceleD.IntracranialhemorrhageE.DepressedskullfractureA161Correctanswer:AThisisaclassicpresentationofacephalohematoma.Cephalohematomaisasubperiostealhemorrhage;henceritisalwayslimitedtothesurfaceofonecranialbone.Thereisnodiscolorationoftheoverlyingscalp,andswellingisusuallynotvisibleuntilseveralhoursafterbirthbecausesubperiostealbleedingisaslowprocess.Mostcasesdonotrequireanytreatmentandresorbspontaneouslywithin2weeksto3months,dependingonthesize.Rarely,phototherapymaybenecessarytoimprovethehyperbilirubinemia.(ChoiceB)Caputsuccedaneumisadiffuse,sometimesecchymotic:swellingofthescalp.Itusuallyinvolvestheportionoftheheadpresentingduringvertexdelivery.Itmayextendacrossthemidlineandacrosssuturelines.(ChoiceC)Crania!meningoceleischaracterizedbythepresenceofpulsations,increasedpressureuponcrying,androentgenographicevidenceofbonydefects.(ChoiceD)Intracranialhemorrhageinneonatesclassicallypresentsasperiodsofapnea,pallororcyanosis,poorsuckling,abnormaleyesigns,high-pitchedcry,musculartwitching,convulsions,decreasedmuscletoneorparalysis,decreasedhematocrit,metabolicacidosis,andshock.Fontanelsmaybetenseandbulging.Swellingofthescalpdoesnotoccur,(ChoiceE)Depressedfracturesareindentationsofthecalvariumsimilarioadentinaping-pongball.Theseareusuallycomplicationsofforcepsdeliveryorfetalheadcompression.Althoughunderlyingskullfracturesareoccasionallyassociatedwithcephalohematoma,theseareusuallylinearandnotdepressed.EducationalObjective:Cephalohematomaisasubperiostealhemorrhage,andpresentsafewhoursafterbirthasscalpswellinglimitedtoonecranialbone.Mostcasesdonotrequireanytreatmentandresorbspontaneouslywithin2weeksto3months,dependingonthesize.Q162A10-month-oldboyisbroughttothephysicianbecausehehasnotyetbeguntostand.Heisabletocommandocrawlusinghisarmsbutdragshislegsaround.Theboywasbornat31weeksgestationbycesareandeliveryforseverepreeclampsia,whichwasunresponsivetomaternaltreatmentwithmagnesiumsulfate.HisApgarscoreswere3and7at1and5minutes,respectively.Maternalprenatallaboratorytestingwasnormal.Hismotherdidnotusealcohol,tobacco,orillicitdrugsduringherpregnancy.Theboy'svaccinationsareuptodate.Vitalsignsarenormal.Onphysicalexamination,heisalertandwell-appearing.Heisnon-dysmorphicandsays”mama”andudada.ITBilaterallowerextremitiesarehypertonicwithsignificantresistancewithpassiveattemptsatextension.Patellarreflexesarebriskandbothfeethaveequinovarusdeformities.Mildhypertoniaoftheupperextremitiesisalsonoted.Babinskireflexispresentbilaterally.Whichofthefollowingisthegreatestriskfactorforthischild’sexaminationfindings?A.AcuteintrapartumhypoxiaB.AdverseeffectsfromvaccinationC.ChromosomalabnormalityD.ExposuretomagnesiumsulfateinuteroE.HistoryofprematurityF.IntrauterineinfectionG.MaternalalcoholuseA162Correctanswer:ECerebralpalsy(CP)isagroupofclinicalsyndromescharacterizedprimarilybynonprogressivemotordysfunction.The3primarysubtypes-spastic,dyskinetic,andataxic-areoftenmultifactorialinetiology.CPisusuallycausedbyprenatalinsultstobraindevelopment,withprematurebirthbefore32weeksgestationasthegreatestriskfactorSpasticdiplegiaistheformmostcommonlyseeninpreterminfants.Itpresentsashypertoniaandhyperreflexiathatinvolvepredominantlythelowerextremitieswithbothfeetpointingdownandinward(equinovarusdeformity).Resistancetopassivemusclemovementincreaseswithmorerapidmovementoftheaffectedextremity(clasp-knife).ManypatientswithCPsufferfromvision,hearing,speech,orotherimpairments(Table).Approximately50%ofpatientsalsohaveintellectualdisability.Managementinvolvesmultidisciplinarytherapiesandanti-spasticmedicationstopreventandimprovecontractures.(ChoiceA)AcuteintrapartumhypoxiaisresponsibleforaminorityofcasesofCP.ApgarscoresareapoorpredictorofneurologicoutcomeasmostneonateswithlowscoresdonotdevelopCP.(ChoiceB)CPisusuallyduetoprenatalfactorsandhasnorelationtovaccinations.(ChoiceC)Althoughchromosomalabnormalitiescanpresentwithhyper-orhypotoniaanddevelopmentaldelay,thischildisnotdysmorphic;hisphysicalexaminationfindingsareclassicforspasticdiplegia.(ChoiceD)Magnesiumsulfateistheprimarymedicationpreeclampsiatreatment.IntrapartummagnesiumsulfateactuallydecreasestheriskofCPandseveremotordysfunction,(ChoiceF)Intrauterineinfectionswithtoxoplasmosis,syphilis,rubella,cytomegalovirus,andvaricellaareassociatedwithincreasedCPrisk.Thenormalprenatallaboratoryresultsandlackofotherabnormalphysicalfindingsmakesthisetiologylesslikely.(ChoiceG)HeavymaternalalcoholuseincreasestheriskofCPapproximately3-fold.However,thischildhasnodysmorphicfeaturesoffetalalcoholsyndromeandhismotherdidnotusealcoholduringherpregnancy.Educationalobjective:Cerebralpalsyisagroupofsyndromescharacterizedbynonprogressivemotordysfunction.Theetiologyisoftenmultifactorial,withprematurityastheleadingriskfactor.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