Movement Disorders

运动障碍疾病MovementDisordersNeurologyDepartmentTheSecondAffiliatedHospitalHarbinMeicalUniversityExtrapyramidalDisordersGeneralConception:ThedysfunctionofbasalgangliaputamenglobuspalliduslentiformnucleuscaudatenucleussubthalamuscorpusstriatumsubstantianigrabasalgangliaGeneralConceptionTwotypesofsymptomsMuscularhypotonusassociatedwithhyperkinesia(F1)Muscularhypertonuscharacterizedwithhypokinesia(F2)Parkinson’sdisease---conceptionAlsocalledparalysisagitans.acommondegenerationdiseaseinnervoussystembecomesincreasinglycommonwithadvancingagecharacterizedbyresttremor,bradykinesia,rigidity,abnormalgaitandpostureParkinson’sdisease---EtiologyAging:overmiddleagedpersonsEnvironmentaltoxin:MPTP(l-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine)inastrocyteoxidatedMPP+mitochondrioninsubstantianigraneuronaldeathGeneticfactor:alpha-synucleingene-----susceptibilityParkinson’sdisease---PathologylossofpigmentationandcellsinthesubstantianigraLewybodyinthebasalgangalia,sympatheticgangliaandbrainstem.Parkinson’sdisease---BiochemistryL-tyrosine(blood)THL-DopaDDCDAMAOandCOMTHVANigrostriatalpathwayisthemostimportant.dopaminedepletiondopaminergicnigrostriatalsystemExtrapyramidalPathwaydopamineacetylcholineParkinsondisease’s---ClinicalfindingsOvermiddleaged&insidiousonsetandgradualprogression.Tremor:oftenappearasthefirstsign.Characters:4~6Hz,conspicuousatrest,increaseattimesofemotionalstress,improvesduringvoluntaryactivity,stopduringsleep,beginfromhand,“N”progression.Parkinsondisease’s---ClinicalfindingsRigidity(F1/F2):leadpipe/cogwheelphenomenon.Minimalrigidity:movethecontralaterallimb,headdroppingtest,roadmarksignParkinsondisease’s--ClinicalfindingsBradykinesia:slownessandreductionofvoluntarymovement,difficulttoinitiate,maskedface,hypophonia,micrographia.Abnormalgaitandposture:flexedposture;difficulttogetup,startwalking,turnorstop.Shufflinggaitandabsenceofthearmswing.Festinatinggait(latesign).Parkinsondisease’s--ClinicalfindingsOtherfeatures:Myerson’ssign,oilyface,intractableconstipation,posturalhypotension,cognitivedisturbance,hallucination,depressionParkinson’sdisease---DifferentialdiagnosisParkinsonism:postencephaliticparkinsonism,drugortoxin-inducedparkinsonism(CO,Mn),arterioscleroticparkinsonism.Depression:atrialofantidepressantdrugtreatmentmaybehelpful.Parkinson’sdisease---DifferentialdiagnosisEssential(benign,familial)tremor:familyhistory,earlyonset,nodorheadshake.Parkinsonismassociatedwithotherneurologicdiseases:DiffusLewybodydisease(hallucination),Wilsondisease,Huntingtonchorea,multiplesystematrophy(MSA),PSP,corticalbasalganglionicdegeneration(CBGD).Parkinson’sdisease---TreatmentBeginfromlowdoseandindividualized.Anticholinergicdrugs:helpfulinalleviatingtremorandrigidity.Artane(1~2mg,tid).•Amantadine:formildparkinsonism.50mg,bid~tid.•Levodopa:L-Dopa/DCI(4:1)---compound;sideeffects:nausea,hypotension(peripheral)dyskinesiaandmotorfluction(central)wearingofforon-offphenomenonParkinson’sdisease---Treatment•DA-Ragonist:bromocriptine•MAOB/COMTinhibitor•Surgery:pallidotomyanddeepbrainstimulation(highfrequencythalamicstimulation)•PhysicaltherapyandaidsfordailylivingPrognosisdeadfromcomplicationsSydenhamChorea---ConceptionAlsocalledrheumaticchorea.Ausualmanifestationofrheumaticfeverinnervoussystem.Presentsinchildhoodoryoungadultlife.ClinicalfindingsinvoluntarychoreadystoniaweaknessautomaticactiondisturbanceemotionalchangesSydenhamChorea---EtiologyRelatedwiththeinfectionofHemolyticStreptococcusA.Anti-neuronantibodiescanreactwiththeneuronsincaudateorsubthalamicnucleus.Mayberelatewithendocrinechanges.SydenhamChorea---Clinicalfindings5~15ys.G/B=3:1SubacuteorinsidiousonsetEarlysymptom:irritability,inattention,unsteadygait,easilydroppingobjects,etal.SydenhamChorea---ClinicalfindingsChorea:rapid,irregular,involuntarily,unpredictablyobviousonface(facialgrimacingandtonguemovements)prominentwhennervousanddisappearedduringsleep.SydenhamChorea---Clinicalfindings•Muscletoneandpower:reduced.Triad:limbweakness,choreaandataxia.milkmaidgrasp,wax-waningsign,dancinggait.•Psychiatricsymptom:agitation,hallucination•Rheumaticfeverfindings.Self-limitationSydenhamChorea---Testing1.Serumtest:rapiderythrocytesedimentation,increasedwbc.2.EEG:unspecific.3.Imagetest:29~85%patientspresentlowdensityfocusincaudatenucleusonCT.SydenhamChorea---Diagnosis&DifferentialdiagnosisOnsetage,triad,rheumaticfeverfindings1.Habitspasm:stereotypedactiononthesamemuscle2.Congenitalchorea:earlieronset3.Tourettesyndrome:ticsonfaceaccompaniedbystrangevoiceanddirtywords.SydenhamChorea---Diagnosis&Differentialdiagnosis4.HuntingtonChorea:onsetaftermiddleage,dementia,familialhistory5.Torsionspasm:continuous,hypermyotoniawhentorsionhappensSydenhamChorea---Treatment&Prognosisrest,keepquiet,avoidstimulationpenicillinorotherantibiotics(10~14days)anddeltacortone(untilthesymptomsdisapper)apozepam(5mg,bid/tid),haloperidol(0.5~1mg,bid/tid).sideeffectsofextrapyramidalsystemSelf-limited3~6monthsaftertheonsetAlsocalledhepatolenticulardegeneration(HLD)Akindofinheriteddisease•e