702 VI. OCCURRENCE OF LIPIDS IN THE ANIMAL 



sides were present in normal brain as well, their concentration in this tissue 

 was increased in Tay-Sachs' disease, in which they partially replaced the 

 cerebrosides. Thus, the gangliosides made up 4 to 8% of the solids in the 

 brain of the Tay-Sachs infant, while they are normally present to the ex- 

 tent of only 0.3%. In a sub-type of this disease, known as the juvenile 

 type of amaurotic idiocy, the ganglioside content was increased to 1.5%; 

 slightly higher than normal amounts of ganglioside were also noted in 

 Niemann-Pick's disease. 



d. Gaucher 's Disease (Reticular and Histiocytic Cerebrosidosis). 

 The first recognition of Gaucher's disease is to be credited to E. Gaucher 

 who, in 1882, described a disease characterized by enlargement of the 

 spleen. 947 However, it was not until twenty-three years later that Brill, 

 Mandelbaum, and Libmann 948 and Schlaugenhaufer 949 called attention to 

 the fact that the liver and osseous system were affected, in addition to the 

 spleen. It is now known that the lymph-nodes are also involved. Before 

 a clear picture of the nature of this disorder was available, it was considered 

 to be neoplastic in nature. After Schlaugenhaufer 949 discovered that 

 Gaucher's disease was centered in the lymphohemopoietic system, it was 

 proved by histologic examination that it was not neoplastic in origin, and 

 did not arise as a result of simple hyperplasia of the tissues. The enlarge- 

 ment of the organs was a result of deposition of a foreign substance in the 

 area involved. 



Lieb, 950 Lieb and Mladenovic, 951 and Epstein and Lorenz 952 identified the 

 foreign substance as a cerebroside, which was believed to be identical with 

 cerasine. This finding was confirmed by Cushing and Stout, 953 by Bloom 

 and Kern, 954 and by Pick. 919 These workers were the first to associate 

 Gaucher's disease with xanthomatosis and Niemann-Pick's disease; each of 

 these three abnormalities was characterized by the deposition of a particular 

 and specific type of lipid. Although the storage of lipids in the usual form 

 of Gaucher's disease is confined to the reticulum, Oberling and Woringer 955 



947 E. Gaucher, De V epithelioma primitif de la rate, These de Paris, 1882; cited by S. J. 

 Thannhauser and G. Schmidt, Physiol. Revs., 26, 275-317 (1946), p. 305. 



948 N. E. Brill, F. S. Mandelbaum, and E. Libmann, Am. J. Med. Sci., 129, 491-504 

 (1905). 



949 F. Schlaugenhaufer, Arch, pathol. Anat. u. Physiol. (Virchow's), 187, 125-163 

 (1907). 



95 ° H. Lieb, Z. physiol. Chem., U0, 305-313 (1924); 170, 60-67 (1927). 

 951 H. Lieb and M. Mladenovic, Z. physiol. Chem., 181, 208-220 (1929). 

 962 E. Epstein and K. Lorenz, Z. physiol. Chem., 192, 145-170 (1930). 

 953 E. H. Cushing and A. P. Stout, Arch. Surg., 12, 539-560 (1926). 

 964 W. Bloom and R. Kern, Arch. Internal Med., 39, 456-461 (1927). 

 955 C. Oberling and P. Woringer, Rev. franc, pediat., 3, 475-532 (1927). 



