mitochondrial DNA depletion syndrome 4a true Alpers disease Alpers' disease or gray-matter degeneration Alpers syndrome DOID:0080122 SNOMEDCT_US_2018_03_01:20415001 MTHICD9_2006:330.8 Alpers progressive infantile poliodystrophy Alper's syndrome ORDO:726 NCI:C35257 A mitochondrial DNA depletion syndrome that is characterized by a clinical triad of psychomotor retardation, intractable epilepsy, and liver failure in infants and young children, and has_material_basis_in autosomal recessive inheritance of homozygous or compound heterozygous mutation in the nuclear gene encoding mitochondrial DNA polymerase gamma on chromosome 15q26. UMLS_CUI:C0205710 ICD10CM:G31.81 disease_ontology MESH:D002549 DOID:1442 progressive sclerosing poliodystrophy Intractable seizures with status epilepticus and epilepsia partialis continua occur, with developmental regression and liver dysfunction. Caused by mutations in POLG. OMIM:203700 Alpers-Huttenlocher syndrome GARD:5783 mitochondrial disease