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Subcellular Localization in Metachromatic Leukodystrophy

Michael J. Malone, MD; Jane Conole, BS

Arch Neurol. 1969;21(1):32-43.

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

METACHROMATIC leukodystrophy (MLD) is a progressive, demyelinating disorder which is characterized chemically by the accumulation of a class of acidic glycolipids: the sulfatides. Pathologically, demyelination involves both the central nervous system (CNS) and peripheral nervous system, and granular, metachromatic deposits are spread throughout the neuraxis. Similar metachromatic granular material is found both intracellularly and as deposits in the tubules of the kidneys, adrenal glands, gallbladder, and liver.¹

Austin et al² first suggested the presence of an enzyme defect in MLD, and Mehl and Jatzkewitz,³ after demonstrating desulfation of sulfatides to cerebrosides in vitro, have shown a deficiency in a degradative enzyme system, cerebroside sulfatase, in kidney tissue from a patient with MLD.^4,5 Moser et al⁶ studied the turnover of injected sodium sulfate S-35 in patients with MLD. In comparison with control groups, a greatly prolonged isotope turnover was found in MLD. They concluded that . . . [Full Text PDF of this Article]

Author Affiliations
Boston

From the Department of Neurology, Boston University School of Medicine (Dr. Malone) and the Neurology Research Department, Boston Veterans Administration Hospital (Miss Conole) and the Research Laboratory, Joseph P. Kennedy Jr. Memorial Hospital, Boston.

Footnotes
Submitted for publication Sept 21, 1968; accepted Jan 7, 1969.

Reprint requests to Kennedy Memorial Hospital, 30 Warren St, Boston 02135 (Dr. Malone).

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