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1、NewApproachestoNeuroimagingofProgressiveMultifocalLeukoencephalopathyAlexandraBinnie,HMSIIIGillianLieberman,M.D.AlexandraBinnie,HMSIIIGillianLieberman,MDWhatisProgressiveMultifocalLeukoencephalopathy(PML)?•AdemyelinatingdiseaseoftheCNScausedbyreactivation
2、oftheJCvirusinimmunocompromisedpatients•PMListypicallyseeninAIDSpatients,transplantrecipients,andleukemiapatients•80%ofnormaladultshaveanti-JCantibodiesindicatingthatexposuretoJCvirusisalmostuniversal•DiagnosisofPMLisbyPCRofcerebrospinalfluid(CSF)–70-100%
3、sensitive–92-100%specific•IfthePCRtestisnegativebutclinicalsuspicionishighabrainbiopsycanbeusedtoconfirmthediagnosis(Kelley,B.J.etal.,2000)AlexandraBinnie,HMSIIIGillianLieberman,MDWhatistheclinicalpresentationofPML?•Themostcommonpresentingsymptomsare:–foc
4、alorgeneralizedweakness(50-63%)–gaitdisturbance(32-43%)–cognitivedysfunction(29-55%)–speechdisturbance(21-50%)–seizures(5-23%)–visualdisturbance(30-50%)•InAIDSpatientstheincidenceis2-4%,typicallypresentingaftertheCD4countfallsbelow100cells/μl.•Theonlyeffe
5、ctivetreatmentishighlyactiveanti-retroviraltherapy.•Averagesurvivalwithouttreatmentis2.5months.•AveragesurvivalwithHAARTisover2yearsbut50%willdiewithin3monthsofdiagnosis.(Kelley,B.J.etal.,2000)AlexandraBinnie,HMSIIIGillianLieberman,MDPatient“C.A.”•A38yoHI
6、V+malepresentswith8weeksofprogressivedysarthria,dysphagia,andgaitdifficulty.•HewashospitalizedinChicagofor4weeksandthenrequestedatransfertoBostontobeclosetohisfamily.•PeroutsidehospitalrecordshewasdiagnosedwithPMLandstartedonhighly-activeantiretroviralthe
7、rapy(HAART)4weekspriortohistransfertoBoston.•Onexamhewasprofoundlydysarthricwithoutaphasia,andhadalateralrectuspalsyontherightaswellaspoorfinger-to-nosecoordinationbilaterally.AlexandraBinnie,HMSIIIGillianLieberman,MDPatient“C.A.”(cont.)AxialFLAIRimageFLA
8、IRimaging:thereisahyperintenselesionoftheponsbilaterallyextendingintotherightcerebellarhemispherebilateralpontinehyperintensityrightcerebellarhyperintensityCourtesyofDrSantoshKesari,BWHAlexandraBinnie,HMSIIIGillianL
