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A Neurochemical Study of a Case of Sudanophilic Leukodystrophy
BRUNO GERSTL, MD;
LUCIEN J. RUBINSTEIN, MD;
LAWRENCE F. ENG, PhD;
MILITIZA TAVASTSTJERNA, DSc
Arch Neurol. 1966;15(6):603-614.
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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
. . . [Full Text PDF of this Article]
Author Affiliations
PALO ALTO, CALIF
From the Department of Pathology, Stanford University School of Medicine, and the Laboratory Service, Veterans Administration Hospital, Palo Alto, Calif.
Footnotes
Submitted for publication June 15, 1966; accepted July 25.
Read in part before the Western Federation of Neurological Sciences Meeting, San Francisco, March 4, 1966.
Reprint requests to Department of Pathology, Stanford University School of Medicine, Palo Alto, Calif 94304 (Dr. Gerstl).
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