One day almost 25 years ago, when I was still a Privatdozent at Tübingen, I had unexpected visitors from Basel. The pathologist and a physician for children's diseases from that town had come to ask me to investigate some organs of an infant that died with a lipid storage disease. It was a case of Niemann-Pick's disease. My visitors only wanted me to find out which lipid was accumulated in this case, a task which was not difficult and which could be solved rather quickly.
The accumulated lipid was sphingomyelin, which together with the cerebrosides belongs to the sphingolipids. In normal spleen and liver the sphingomyelin is to be found only in traces, but in Niemann-Pick's disease it occurs in amounts of about 20% of the dry weight of the organs.
Almost simultaneously with the case of Niemann-Pick's disease a case of amaurotic idiocy of the Tay-Sachs type was admitted