<prompt> Splenomegaly seen in AE: PGI Dec 2006Neimann picks diseaseKrabbes diseaseGM2, gangliosidosisGauchers disease  <response>: Ans: B Krabbes disease Lysomal Storage Diseases Without AR inheritance MPS -II Hunter Fabrys diseaseBoth are X-linkedHepa tospleenomegaly Toy Sachs diseaseQ Fabryrs diseasesQMucolipidosis III; psued-o-Hurler polydystrophyKrabbes diseaseQ Metachromatic leukodystrophyCholesteryl ester storage disease only hepatomegaly. Table Harrison 17th24S2-53: Selected Lysosomal Storage DiseasesDisorderEnzyme Deficiency Specific TherapyStored MaterialClinical Types OnsetInheritanceNeurologicLiver Spleen EnlargementSkeletal DysplasiaOphthalmologicHematologicUnique FeaturesGaucherdiseaseAcid -gluco- sidase ET, SRTGlucosyl - ceramideType 1Type 2Type 3ARNone NoneEye movements Eye movementsGaucher cells in bone marrow, cytopeniasAdult form highly variableNiemann-Pickdisease A and BSphingomy - elinaseSphingo - myelinNeuronopathic type A Nonneu- ronopath- ic type BARMental retardation and seizuresNone OsteoporosisMacular degenerationFoam cells in bone marrowPulmonary-infiltratesLung failureSialidosisNeuraminidaseSialyloli - gosaccha- ridesType I. congenitalType II, infantile and Juvenile formsARMyoclonus,Mental retardation, less in type 1, less in type 1Cherry red spotVacuolatedlymphocytesMPS phenotype in type IIKrabbe diseaseGalactosyl- ceramidase B MTGalac -tosylce - ramide,GalactosylsphingosineInfantileARMentalretardationNoneNoneNoneNoneWhite matter globoid cellsTay-Sachs diseaseHexosamini- dae AGM2 gan- gliosidesInfantile juvenileARMental retardation, seizures, later juvenile formNoneNoneCherry- red spot in infantile formNoneMacrocephaly, hyper - acusis in infantile formSandhoff diseaseHexosaminidases A and BGM2 gan- gliosidesGM2 gan- gliosides InfantileARMental retardation, seizures-Cherry red spotNoneMacrocephaly, hyperatusis Krabbes DiseaseIt is a lysosomal storage disorder classified under leukodystrophies.Enzyme deficient is Galactosylceramidease with resultant accumulation of galactosyl cermamide and galactosyl sphingosine.It has AR inheritance aw mental retardation.There is no hepatospleenomegaiyQ skeletal, opthmotogical or hematologic manifestations. 