prion专题

AsofDecember31,1998,35deathshadbeenattributedtonewvariantCreutzfeldt-Jakobdisease（nvCJD）intheUnitedKingdom,ofwhich33caseshadbeenneuropathologicallyconfirmedand2classifiedasprobablenvCJD.Fifteencasesweremaleand20female.Themedianillnessdurationwas14months（range,8-38months）andthemedianageatdeathwas29years（range,18-53years）.Thedinicalfeatureswereconsistentwithpreviousdescriptions.Innearlyallcases,therewereearlypsychiatricsymptomsafteramedianperiodof6monthsataxiadeveloped,followedbyinvoluntarymovementsandcognitiveimpairment.Electroencephalogramsdidnotshowthe"typical"appearancesfoundinsporadicCJD,abouthalfthecasestestedhadapositive14-3-3immunoassay,andover70%ofcaseshadbilateralpulvinarhighsignalonmagneticresonancebrainscanning.Prionproteingeneanalysisshowedthatallcaseswerehomozygousformethionineatcodon129.DiagnosticcriteriafornvCJDhavebeenformulated,whichhaveahighsensitivityandspecificity.

[引自AnnNeurol2000May;47（5）:575-82]