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Electron Microscopic Observations on Metachromatic Leukodystrophy
GUNNBJORG AUREBECK, MD;
KENNETH OSTERBERG, MD;
MICHAEL BLAW, MD;
SHELLY CHOU, MD;
ERLAND NELSON, MD
Arch Neurol. 1964;11(3):273-288.
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Of all the diseases of unknown etiology and obscure pathogenesis that involve the nervous system, few have aroused more interest among pathologists, chemists, and clinicians than metachromatic leukodystrophy. The histopathology of this condition has been well documented by the classic observations of Scholz,1 Bielschowsky and Henneberg,2 Greenfield,3 and Brain and Greenfield.4 More recent papers, summarized by Helmstaedt,5 have pointed out some differences in the clinical and histological features according to the age of onset of the disease. An absolute increase in sulfatide content has been independently reported by Austin,6 Jatzkewitz,7 and Hagberg et al,8 not only in the brains, but in various organs of individuals with this disease. Austin's concept of this condition as a "sulfatide lipidosis," has found favor with a number of investigators.9-13
Although observations have been made on the fine structure of a sudanophilic variety of diffuse sclerosis
. . . [Full Text PDF of this Article]
Author Affiliations
MINNEAPOLIS
From the divisions of neurology and neurosurgery, and the Department of Pathology, University of Minnesota School of Medicine.
Footnotes
Submitted for publication March 28, 1964; accepted April 4.
Supported in part by research grants CA-05040 from the National Cancer Institute, and NB-02566 from the National Institute of Neurological Diseases and Blindness, Bethesda, Md.
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