<prompt> A severely retarded infant is noted to have hepatosplenomegaly and a cherry-red spot in the macula. Of the following, which is most likely the cause of these findings?Tay-Sachs diseaseNiemann-Pick diseaseGauchers diseaseMetachromatic leukodystrophy  <response>: Niemann-Pick Disease is an autosomal recessive disorder that results from defects in acid sphingomyelinase. Types A and B are distinguished by an early age of onset and progressive CNS disease in type A. Type A typically has onset in the first 6 months, with rapidly progressive CNS deterioration, spasticity, failure to thrive, and massive hepatosplenomegaly.A cherry-red spot in the macula is noted in about 30 of patients. Tay-Sachs Disease defective hexosaminidase A is associated with psychomotor retardation and a cherry-red spot, but no hepatosplenomegaly. Macular Degeneration and a cherry-red spot are NOT pa of the clinical picture of Gauchers disease, metachromatic leukodystrophy, or globoid-cell leukodystrophy. Ref: Hopkin R., Grabowski G.A. 2012. Chapter 361. Lysosomal Storage Diseases. In D.L. Longo, A.S. Fauci, D.L. Kasper, S.L. Hauser, J.L. Jameson, J. Loscalzo Eds, Harrisons Principles of Internal Medicine, 18e. 