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Globoid Cell Type of Leukodystrophy

ANTHONY N. D'AGOSTINO, M.D.; GEORGE P. SAYRE, M.D.; ALVIN B. HAYLES, M.D.

Arch Neurol. 1963;8(1):82-96.

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

The demyelinating diseases are characterized by a primary breakdown of myelin with more or less persistence of the axis cylinders and with secondary proliferation of astrocytes and microglial cells leading to sclerosis. The demyelination may be disseminated, as in multiple sclerosis, or diffuse. A disorder first described by Krabbe¹ in 1916 and known since then as Krabbe's disease is an example of the diffuse type of cerebral sclerosis.

In the disseminated type of sclerosis numerous discrete, demyelinating foci of variable size are scattered throughout the brain and spinal cord. In the diffuse type, the lesions begin in one focus in the cerebral hemispheres and spread to involve adjacent white matter in a continuous fashion. Ultimately the demyelination extends from the paraventricular areas to the cortex, and usually it becomes bilateral and symmetrical.

The classifications of the diffuse demyelinating diseases have undergone considerable revision since the development of histochemical . . . [Full Text PDF of this Article]

Author Affiliations
ROCHESTER, MINN.

Fellow in Pathology, Mayo Foundation (Dr. D'Agostino), Section of Pathologic Anatomy (Dr. Sayre), and Section of Pediatrics (Dr. Hayles), Mayo Clinic and Mayo Foundation.

Footnotes
Submitted for publication June 11, 1962.

This investigation was supported in part by Research Grant No. 2B5307 from the National Institutes of Health, Public Health Service.

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