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December 1966

A Neurochemical Study of a Case of Sudanophilic Leukodystrophy

Arch Neurol. 1966;15(6):603-614. doi:10.1001/archneur.1966.00470180043005

THE NOSOLOGICAL definition of diffuse sudanophilic cerebral sclerosis has had a long and confused history. In 1956 Poser and van Bogaert1 reached, on morphological grounds, the conclusion that the eponymic entity commonly termed "Schilder's disease" consisted of three kinds of conditions: (1) Schilder's cerebral sclerosis, a disease closely resembling, if not identical with, multiple sclerosis; (2) The leukodystrophies, a group of heredodegenerative diseases, related to metabolic disorders such as the lipidoses and glycogenoses2,3; and (3) Examples of subacute encephalitis, including Dawson's inclusion body encephalitis, van Bogaert's subacute sclerosing leukoencephalitis, and Pette-Doring's nodular pancephalitis.

The exclusion of Schilder's disease and its transitional forms to multiple sclerosis from the complex group of the leukodystrophies was endorsed by Norman.4 In the latter group, however, there remained a heterogeneous collection of sporadic and familial cases in which the demyelinative process, though having the morphological features of a leukodystrophy, was characterized