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VI. A Rapid Test for the Sulfatase A Deficiency in Metachromatic Leukodystrophy (MLD) Urine

JAMES AUSTIN, MD; DONALD ARMSTRONG, BS; LESLIE SHEARER; DONALD McAFEE, BS

Arch Neurol. 1966;14(3):259-269.

	Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings.

A RYLSULFATASE A activity is deficient in metachromatic leukodystrophy (MLD, sulfatide lipidosis). The activity of this hydrolytic enzyme is low in brain, liver, and kidney tissue obtained post mortem and in the urine of living MLD patients.^1-5 The deficiency of sulfatase A could explain some, if not all, of the excess of sulfatides in MLD. For example, it may be inferred from the findings of Mehl and Jatzkewitz that one result of a sulfatase A deficiency would be a deficient catabolism of sulfatides.^6,7

MLD itself is not common, but it does enter into the differential diagnosis of a wide variety of other neurological diseases not only in childhood, but also in adult life.⁸ On clinical grounds alone, the early diagnosis of MLD is not always straight-for-ward, nor is it sometimes clear even after biopsies and other tests. Hence, there is a need for a practical, rapid, laboratory . . . [Full Text PDF of this Article]

Author Affiliations
PORTLAND, ORE

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

Footnotes
Submitted for publication Aug 2, 1965; accepted Sept 13.

Reprint requests to Division of Neurology, University of Oregon Medical School, Portland, Ore 97201 (Dr. Austin).

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