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Metachromatic Leucodystrophy

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Lipids and Lipidoses

Abstract

The term “diffuse cerebral sclerosis” (leucodystrophy) is used for changes which occur in a variety of disorders and consist of demyelination in cerebrum and cerebellum, whereby reactive gliosis leads to progressive sclerosis. The axis cylinders are rather well preserved, in contrast to their condition during the demyelination which occurs following disease of ganglion cells and axons as in Tay-Sachs disease. It appears therefore that, in the leucodystrophies, there is a primary disturbance in the metabolism of myelin components. Lipid abnormalities are found in several types of congenital diffuse sclerosis or leucodystrophy, such as “globoid-cell” leucodystrophy, type Krabbe (Diezel 1955; Lees and Moser 1962; Austin 1963 a, b; Cumings and Rozdilsky 1965; Austin and Lehfeldt 1965) and Pelizaeus-Merzbacher disease (Debuch 1957, 1958; Seitelberger 1957; Norman and Tingey 1963; Diezel et al. 1965; Nisenbaum et al. 1965).

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  1. W. Kahlke
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Kahlke, W. (1967). Metachromatic Leucodystrophy. In: Schettler, G. (eds) Lipids and Lipidoses. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-87367-6_11

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