[Skip to Content]
[Skip to Content Landing]
September 1964

Electron Microscopic Observations on Metachromatic Leukodystrophy

Author Affiliations

From the divisions of neurology and neurosurgery, and the Department of Pathology, University of Minnesota School of Medicine.

Arch Neurol. 1964;11(3):273-288. doi:10.1001/archneur.1964.00460210051005

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