Abstract
I N A PREVIOUS report 2 we described for the first time an abnormal cerebrospinal fluid lipid profile in a patient with Tay-Sachs disease; it was found that the nonphosphorus sphingolipids were markedly elevated. Furthermore, if the data were calculated on the basis of lipid per milligram of cerebrospinal fluid protein, other lipid constituents, namely, total phospholipids, cephalins, and total lipids were also significantly increased. One of the purposes of this report is to present results on ten other children afflicted with Tay-Sachs disease. In the course of this investigation it was discovered that an abnormal cell was present in the cerebrospinal fluid of some cases of Tay-Sachs disease; the morphology of these cells will be presented. In order to evaluate further the significance of the abnormal cerebrospinal fluid lipid profile and the abnormal cells discovered, a child with terminal Tay-Sachs disease was studied while on a measured diet by
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