globoid cell leukodystrophy

leukodystrophy /leu·ko·dys·tro·phy/ (-dis´trah-fe) disturbance of the white substance of the brain; see also leukoencephalopathy.

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globoid cell leukodystrophy  Krabbe's disease.

hereditary adult-onset leukodystrophy  an inherited leukoencephalopathy characterized by progressive degeneration of the white matter, with motor disturbances, bowel and bladder incontinence, and orthostatic hypotension.

metachromatic leukodystrophy  an inherited disorder due to accumulation of sulfatide in tissues with a diffuse loss of myelin in the central nervous system; it occurs in several forms, with increasing age of onset correlated to decreasing severity, all initially presenting as mental regression and motor disturbances.

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glo·boid cell leukodystrophy (glboid)

n.

An inherited metabolic encephalopathy of infancy with rapidly progressive cerebral degeneration, massive loss of myelin, severe astrocytic gliosis, and infiltration of the white matter with characteristic multinucleate globoid cells. Also called Krabbe's disease.

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globoid cell leukodystrophy

see globoid cell leukodystrophy.

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globoid cell leukodystrophy

Krabbe's disease Neurology An AR defect in sphingolipid metabolism due to galatocerebroside β-galactosidase deficiency, resulting in in utero demyelinization, and death in early infancy Clinical Spastic paralysis, seizures, pyrexia, vomiting, cortical blindness, deafness, dysphagia, pseudobulbar palsy, quadriplegia, mental deterioration Management CNS disease may be reversed by allogeneic hematopoietic stem cell transplantation. Cf Leukodystrophy.