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Leukoencephalopathy With Vanishing White Matter

Disease ID: disease_node_19949

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DbxrefGARD:231, ICD10CM:E75.2, MIM:PS603896, ORDO:135, ORDO:157713, ORDO:157716, ORDO:157719
SubclassofDOID_0050737, DOID_10579
Data SourceDOID
SynonymsCACH, CACH/VWM, childhood ataxia with central nervous system hypomyelination, vanishing white matter leukodystrophy, ovarioleukodystrophy
Doid Labelleukoencephalopathy with vanishing white matter
Doid DescriptionA leukodystrophy characterized by variable neurologic features resulting from deficiency in astrocyte maturation, including progressive cerebellar ataxia, spasticity, and cognitive impairment associated with white matter lesions on brain imaging with onset from early infancy to adulthood.
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_19949
Doid IdDOID_0060868
LabelLeukoencephalopathy With Vanishing White Matter