Kallmann综合征的MRI表现

《Kallmann综合征的MRI表现》由会员上传分享，免费在线阅读，更多相关内容在教育资源-天天文库。

1、MRIfindingsinKallmannsyndromeH.ZAGHOUANIBENALAYA,Z.ACHOUR,M.BHOURI, M.LIMEME,S.MAJDOUB,H.AMARA,D.BAKIR,CH.KRAIEMHN27INTRODUCTION:Kallmannsyndromeisaninheriteddisordercharacterizedbyhypogonadotrophichypogonadismandanosmiaorhyposmia.KSisduetoabnormalmigr

2、ationofgonado-tropin-releasinghormone(GnRH)aswellasolfactoryneuronsfromolfactoryplacodetotheforebrainandhypothalamusduringfetallife.StructuralolfactorytractabnormalitiesarewellseenonMRIPURPOSE:theaimofthisworkistodescribetheMRappearanceoftheolfactorybu

3、lbsandtractsinpatientswithKallmannsyndrome,MIETHODS:High-resolutionMRscanswereperformedintwopatientswithKallmannsyndrome.CoronalT2weightedcontiguoussectionswereobtainedthroughtheolfactorybulbsandtracti.RESULTS:Cases:A15-year-oldfemaleand20-year-oldmale

4、adressedtoMRIforhypogonadotrophichypogonadism((SerumLH<1.5IU/L,SerumFSH<1.5IU/L,Testosterone<20ng/dl)andhyposmia.CoronalT2-weightedMRimagesthroughtheanteriorfossashows:aplasiaofbotholfactorybulbandabsenceoftheleftolfactorysulcus(case1).Bilateralaplasia

5、ofolfactorybulbwithnormalolfactorytract(case2)Noothercranialabnormalityfound.CASE1:CoronalT2-weightedMRimagethroughtheanteriorfossa.olfactorybulbsareabsentandtheleftolfactorysulcusishypoplastic.CASE2:coronalT2imagesthroughthefrontallobesdemonstrateabno

6、rmalanatomywithabsenceoftheolfactorybulbs.Olfactorysulcus,thegyrusrectusandmedialorbitalgyrusarenormal.DISCUSSION:Kallmannsyndromeisaninheriteddisordercharacterizedbyhypogonadotrophichypogonadismandanosmiaorhyposmia.TheinheritancemaybeX-linked,Autosoma

7、lrecessiveorAutosomaldominantwithvariablepenetrance.Thereportedincidenceis1in10,000menand1in50,000women.Kallmannsyndromeisananomalyofneuronalmigration.CellsthatdifferentiateintoGonadotrophinreleasinghormone(GnRH)secretingneuronsoriginatefromwithinembry

8、onicolfactoryepitheliumandmigratealongfasciclesofvomeronasalandterminalisnervesintoforebrain.ThismigrationofGnRHneuronsisarrestedinKSresultinginGnRHdeficiencyfollowedbydifferentdegreesofluteinizinghormone(LH)andfolliclestimulatinghormon