Electron Microscopic Observations on Metachromatic Leukodystrophy

Abstract

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