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1、NeurologyClinicalPracticeDiagnosisandmanagementofidiopathicnormal-pressurehydrocephalusMichaelA.WilliamsMDFAANNormanR.RelkinMDPhDSummaryThediagnosisandmanagementofidiopathicnormal-pressurehydrocephalus(iNPH)adisorderofgaitimpairmentincontinenceanddementiathataffectselderlypatientsincorporatesanorgan
2、izedapproachusingfamiliarprinciplesforneurologists.Thestartingpointisacomprehensivehistoryandneurologicexam-inationreviewofneuroimaginganduationofthedifferentialdiagnosis.CoexistingdisordersshouldbetreatedbeforespecificiNPHtestingispered.SpecificiNPHtestingincludesassessingpatientresponsetotemporary
3、CSFremovalandtestingCSFhydrodynamics.InproperlyselectedpatientsalliNPHsymptomsincludingdementiacanimproveaftershuntsurgery.ThelongitudinalcareofiNPHpatientswithshuntsincludesuationofthedifferentialdiag-nosisofworseningiNPHsymptomsandtreatmentofcoexistingdisorders.uationofshuntobstructionisoftenindic
4、atedandifitisfoundsurgicalcorrectionislikelytoresultinsymptomaticimprovement.Idiopathicnormal-pressurehydrocephalus(iNPH)isadisorderoftheelderlywithsymptomsofimpairedgaitandmobilityurinaryurgencyandincontinenceandmildcognitiveimpairmentordementiainthepresenceofventri-culomegaly.Theclinicalpresentati
5、onaloneisusuallynotsufficienttodiagnoseiNPHandrec-ommendshuntsurgeryaseachoftheprimaryiNPHsymptomscanhavemultiplepotentialetiologiesandenlargedventriclescanbeseenwitheitherhydrocephalusorbrainatrophy.TheSandraandMalcolmBermanBrain&SpineInstituteAdultHydrocephalusCenter(MAW)SinaiHospitalofBaltimoreMD
6、andClinicalNeurologyandNeuroscienceDepartmentofNeurologyandNeuroscience(NRR)WeillCornellMedicalCollegeNewYorkNY.Fundinginationanddisclosuresareprovidedattheendofthearticle.FulldisclosureinationprovidedbytheauthorsisavailablewiththefulltextofthisarticleatNeurology.orgcp.Correspondenceto:michwilllifeb
7、ridgehealth.orgNeurology:ClinicalPractice|October2013www.neurology.orgcp375OrganizedapproachtodiagnosisandtreatmentThegoaloftheuationofpossibleiNPHistopredictwhethershuntsurgeryislikelytobenefitthepatientsufficientlytojustifytherisksofsurgeryandpostoperativemorbidities.Followingisanorganizedapproach
8、touation.1.Clinicaluation2.TreatmentofotherdisordersbeforeundertakingspecifictestingforiNPH3.TestingthatisspecificforprognosticatingtreatmentresponseiniNPH4.Shuntsurgery5.Longitudinalfollow-upClinicaluationKeyneurologicfeaturesAcomparisonofkeyclinicalfindingsforiNPHfromtheInterna-tionalandJapanesegu
9、idelinesispresentedintable1.14BydefinitioniNPHisidiopathichowevertheneurologichistoryshouldincludeknownriskfactorsforcommunicatinghy-drocephalusincludingmeningitisencephalitistraumaticbraininjury(includingconcus-sion)subarachnoidhemorrhageandbrainradiation.Enlargedheadcircumferenceisalsoariskfactort
10、hatmayindicateacongenitalcomponenttothedisorder.5Patientswithsecondarycommunicatinghydrocephalusshouldbeuatedfortheneedforshuntsurgeryinthemanneroutlinedbelowexceptinclinicallyobviouscasessuchasthedevelopmentofhy-drocephalusduringhospitalizationforsubarachnoidhemorrhage.SymptomonsetTheonsetofiNPHsym
11、ptomsisinsidiousandshouldhavebeenevidentforatleast6months.Somepatientsandfamiliesarenotawareofsymptomsuntilaprecipitatingeventoccurs(e.g.afallorachangeinsymptomsafterasurgicalprocedure).Carefulquestion-ingcanclarifythenatureofsymptomonset.GaitIniNPHahigher-levelgaitdisorderisseenwithdisturbedpostura
12、landlocomotorreflsintheabsenceofprimarysensorimotordeficits.6Findingsincludedifficultywithtransitionalmovements(sittingtostandingorstandingtositting)gaitinitiationfailurepoorfootclearancewithshufflingtrippingfallingorfestinationmultistepturnswithinstabilityandretropulsionoranteropulsionofstance.7The
13、useofastandardizedgaituation(e.g.theTinettiscoreBoonScaleorthetimedup-and-gotest)canbehelpful.Spasticityhyperreflexiaandotheruppermotorneuronfindingsarenottypical.Symp-tomsofiNPHaresymmetricthereforelateralizingfindingsshouldincreasesuspicionofotherdisorders.BladderdysfunctionThebladderdysfunctionof
14、iNPHisusuallyurinaryurgencywithdif-ficultyinhibitingbladderemptying.8Intheearlystagespatientsmayexperienceurgencywithoutincontinenceorwithlossofafewdropsofurinebeforereachingthetoilet.Night-timeurinaryfrequencyiscommon.Patientsareusuallyawareoftheirneedtourinateandareconcernedabouttheiraccidents.Inc
15、ontinencewithoutawarenessofurinaryurgeorthatonesclothesarewetisnotcharacteristicofiNPH.DementiaThedementiaofiNPHincludesapathyoramotivationdaytimesleepinesspsy-chomotorslowingandotherfeaturesoffrontal-subcorticaldysfunction.911FunctionallossesThetermshydrocephalusandventriculomegalyarenotsynonymous.
16、AlthoughallpatientswithiNPHshouldhaveenlargedventriclesnotallelderlypatientswithenlargedventricleshaveiNPH.3762013AmericanAcademyofNeurologyMichaelA.WilliamsandNormanR.RelkinTable1Keyclinicalfeaturesofidiopathicnormal-pressurehydrocephalus:ComparisonbetweentheInternationalandtheJapaneseguidelinesFeatureInternationalguidelinesJapaneseguidelinesEssentialsymptomsFindingsofgaitbalancedisturbancemustbepresentplusatleastoneotherareaofimpairmentincognitionurinarysymptomsorbothMorethano
