Intraneuronal lipid deposits in metachromatic leukodystrophy

Abstract

The histochemical characteristics and topographic distribution of intraneuronal metachromatic lipid deposits were studied in frozen and paraffin sections of formalin-fixed material in 4 cases of infantile metachromatic leukodystrophy. Concurrently, a case of amaurotic idiocy (Batten''s type) and a brain with abundant lipofuscin were studied with identical staining technics. The previous observations on the sulfatide nature of the intraneuronal lipid in metachromatic leukodystrophy was again confirmed; however, a striking difference between the lipid contained within neurons, processes of astrocytes, and macrophages was found. Lipofuscin and the ganglioside of amaurotic idiocy could easily be distinguished from the intraneuronal lipid of metachromatic leukodystrophy. The topographic distribution of affected neurons appeared quite selective with almost complete sparing of the cerbral and cerebellar cortex, claustrum, putamen, caudate nucleus, and certain brain stem nuclei. This distinctive distribution is a sharp contrast to that described in amaurotic idiocy.