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To the Editor: Shulman et al. [1] draw attention to the late presentation of Niemann-Pick disease, type C (NPC). Some aspects of their paper deserve amplification. The authors allude to abnormal intracellular cholesterol metabolism in their introduction but then distinguish NPC from Niemann-Pick disease types A and B on the basis of sphingomyelinase activity. Distinction between the subtypes of Niemann-Pick disease on the basis of sphingomyelinase activity is hazardous at best, although the statement that there is variable sphingomyelinase activity in cells from NPC patients is essentially correct. NPC is a lipidosis in which abnormal intracellular trafficking of exogenously derived cholesterol is associated with excess lysosomal storage of unesterified cholesterol. The variable depression of acid sphingomyelinase activity …
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