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July 1966

Metachromatic Leukodystrophy (MLD): VII. Elevated Sulfated Acid Polysaccharide Levels in Urine and Postmortem Tissues

Author Affiliations

From the Division of Neurology, University of Oregon Medical School, Portland.

Arch Neurol. 1966;15(1):13-28. doi:10.1001/archneur.1966.00470130017002

ACID polysaccharides (APS)* have become a focus of general biomedical interest since large excesses were reported in Hurler's syndrome (gargoylism).1,2,7 Large excesses of APS have also been noted in urine of two children who had a form of metachromatic leukodystrophy (MLD).3 Apart from their relationships with diseases, the APS in the normal nervous system have negative charge characteristics which may be of neurophysiological interest. For example, the net charge of APS molecules might influence the distribution of cations and positively charged molecules. In this manner APS might have some general bearing on the bioelectric potentials involved in neuronal excitation and transmission.

The purpose of the present study is three-fold: (1) to document and extend the APS observations in MLD; (2) to correlate them with the excess APS isolated from the brain and kidney of the same MLD children; and (3) to review a sizable expansion of the

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