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Treatment With Arylsulfatase-A

Harry L. Greene, MD; George Hug, MD; William K. Schubert, MD

Arch Neurol. 1969;20(2):147-153.

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

INFANTILE metachromatic leukodystrophy (MLD) is a genetically determined fatal lipidosis, in which sulfatides (cerebroside sulfates) accumulate in various organs but chiefly affect the white matter of the nervous system.^1-3 The activity of the enzyme arylsulfatase-A (ASA) is deficient in patients with MLD. Mehl has shown that purified ASA can degrade cerebroside sulfate in vitro,⁴ and it is postulated that ASA deficiency results in the accumulation of cerebroside sulfate.⁵ Various forms of therapy have been attempted in an effort to alter the natural course of the disease, but none has been effective.^5-8

Replacement of ASA might degrade the accumulated cerebroside sulfate and there by be an effective treatment. This paper describes the results of both intravenous and intrathecal infusion of ASA in a patient with the late infantile form of metachromatic leukodystrophy.

Report of a Case

The boy was born uneventfully on Aug 20, 1963 with . . . [Full Text PDF of this Article]

Author Affiliations
Cincinnati

From the Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati.

Footnotes
Submitted for publication Aug 23, 1968; accepted Oct 24.

Reprint requests to The Children's Hospital, 240 Bethesda Ave, Cincinnati 45229 (Dr. Schubert).

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