Authors: Arthur L. Beaudet

The disorder now identified as aspartoacylase deficiency is equivalent to the condition variously called spongy degeneration of the brain, spongy degeneration of the central nervous system in infancy, or spongy degeneration of infancy, and many publications have used the eponymic designation, Canavan disease. The first definition of this condition as a distinct clinical entity is properly credited to van Bogaert and Bertrand in 1949.In retrospect, the first clinical description is attributed to Globus and Strauss in 1928.

In 1931, Canavan described an infant with prominent enlargement of the head and cerebral and cerebellar spongy degeneration under the designation "Schilder's encephalitis periaxialis diffusa."Eiselsberg is credited with the recognition of the familial nature of the disorder in 1937, but, like Jervis,she described the condition as Krabbe disease.

The reports of von Bogaert and Bertrand were comprehensive and described the essential pathologic and clinical features as well as the predilection for the occurrence of the disorder in Ashkenazic infants. In a more detailed review of the historical literature,Banker et al. pointed out that the Canavan eponym is hardly justified, because her report was not the first clinical description, did not recognize the familial or ethnic aspect to the disorder, and did not recognize spongy degeneration as the unique pathologic feature; the designation aspartoacylase deficiency may be most appropriate, but the eponym is widely used.