Krabbe's disease

[krah´bez]

a familial form of leukoencephalopathy beginning in infancy, in which the sphingolipid ceramide galactoside accumulates in the tissues due to a deficiency of β-galactosidase, marked pathologically by cerebral demyelination and by the presence of large globoid bodies in the white substance.

Krabbe's disease

See GLOBOID CELL LEUKODYSTROPHY.

Collins Dictionary of Medicine © Robert M. Youngson 2004, 2005

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References in periodicals archive

Krabbe's disease is a rare and terminal genetic mutation that damages and attacks the nervous system.

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Globoid cell leukodystrophy, also known as Krabbe's disease, is an autosomal recessive white matter disorder caused by the deficiency of [beta]-galactocerebrosidase.

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