Abstract
A case of familial amaurotic idiocy in a child which died at the age of two and one half years is described. The pathological findings in the brain were typical for the disease. Histochemical examination revealed that the substance stored in the neurones was a mixture containing mainly a neutral glycolipid and cholesterol. The findings in this case, taken together with other reports in the literature indicate that the composition of the substance stored in various cases of amaurotic idiocy may vary to a great extent. The possibility is envisaged that amaurotic idiocy might be a syndrome which is due to different metabolic derangements in the various cases.
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