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September 1963

Studies in Globoid (Krabbe) LeukodystrophyI. The Significance of Lipid Abnormalities in White Matter in 8 Globoid and 13 Control Patients

Author Affiliations

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

Arch Neurol. 1963;9(3):207-231. doi:10.1001/archneur.1963.00460090013001

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

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