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Neuronal Ceroid Lipofuscinosis 8 Northern Epilepsy Variant

Disease ID: disease_node_15961

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DbxrefGARD:2163, GARD:4010, ICD10CM:E75.4, MIM:610003, ORDO:1947
SubclassofDOID_0050737, DOID_14503
Data SourceDOID
SynonymsEPMR, northern epilepsy variant, neuronal ceroid lipofuscinosis, Northern epilepsy variant, progressive epilepsy with mental retardation, northern epilepsy, progressive epilepsy-intellectual disability syndrome, Finnish type
Doid Labelneuronal ceroid lipofuscinosis 8 northern epilepsy variant
Doid DescriptionA neuronal ceroid lipofuscinosis that is characterized by onset at 5 to 10 years of age of epilepsy followed by progressive mental retardation and a mixed combination of 'granular,' 'curvilinear,' and 'fingerprint' profile lipopigment patterns and has_material_basis_in a Finnish founder mutation in the CLN8 gene on chromosome 8p23.
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_15961
Doid IdDOID_0110724
LabelNeuronal Ceroid Lipofuscinosis 8 Northern Epilepsy Variant