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May 1965

Metachromatic Form of Diffuse Cerebral Sclerosis: IV. Low Sulfatase Activity In the Urine Of Nine Living Patients With Metachromatic Lcukodystrophy (MLD)

Author Affiliations

From the Division of Neurology, University of Oregon Medical School. Associate Professor of Neurology (Dr. Austin); Research Assistants in Neurology (Mr. McAfee and Mr. Shearer).

Arch Neurol. 1965;12(5):447-455. doi:10.1001/archneur.1965.00460290003001

A STATISTICALLY significant decrease in sulfatase activity has been noted in autopsy tissues from four patients with metachromatic leukodystrophy (MLD) (sulfatide lipidosis).1,19 Arylsulfatase activity was low both in histochemical tests2,14 and in quantitative colorimetric analyses of brain, liver, and kidney. It would seem reasonable to relate a sulfatase deficiency in some way to the excess of cerebroside sulfates (sulfatides) found in the central nervous system and kidney in MLD.5

Enzyme studies on dead patients are limited by several important qualifications. These restrict the conclusions which can legitimately be drawn from postmortem studies alone even when the analyses are well controlled. Hence, the present study was designed to test a critical question—is sulfatase activity low in living MLD patients who are still in earlier stages of their disorder? Urines were selected for assay because sulfatase activity which is produced in the kidney normally enters the urine in detectable

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