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,1Bielschowsky and Henneberg,2Greenfield,3and Brain and Greenfield.4More recent papers, summarized by Helmstaedt,5have pointed out some differences in the clinical and histological features according to the age of onset of the disease. An absolute increase in content has been independently reported by Austin,6Jatzkewitz,7and Hagberg et al,8not 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