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I. The Significance of Lipid Abnormalities in White Matter in 8 Globoid and 13 Control Patients

JAMES AUSTIN, MD

Arch Neurol. 1963;9(3):207-231.

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

Introduction

Globoid leukodystrophy is a fatal, genetically determined disorder of myelin which chiefly affects infants and children. Its eponym, "Krabbe's disease," is derived from the report of the late Knud Krabbe who, in 1916, clearly described its infantile form in two siblings.¹ Bullard and Southard (1906)² and Beneke (1908)³ may well have encountered similar cases.

Distinctive cellular structures are the hall-mark of this disease. These multinuclear and mononuclear structures may be termed "globoid" elements (Fig 1). They are present in large numbers in the devastated white matter. Their descriptive name, "globoid," is derived from the report of Collier and Greenfield⁴ who, by this term, drew attention to their globular, distended appearance. After their report, the terms "Krabbe" or "globoid" have come to be used synonymously to refer to this separate category of leukodystrophy.

The globular shape and distended appearance of globoid elements is of obvious . . . [Full Text PDF of this Article]

Author Affiliations
NEW DELHI, INDIA

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

Footnotes
Submitted for publication, April 3, 1963; accepted May 29, 1963.

With the technical assistance of Winston Maxwell, MA, and Richard Anderson, MA.

These studies were supported by grant B-1430 from the National Institute of Neurological Diseases and Blindness, United States Public Health Service.

The results in series I were presented at the American Association of Neuropathologists' meeting, Atlantic City, June, 1960; series II results were presented at the American Neurological Association meeting, Atlantic City, June, 1963.

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